,Doctor's Note,Summary
0,"This 60-year-old male was hospitalized due to moderate ARDS from COVID-19 with symptoms of fever, dry cough, and dyspnea. We encountered several difficulties during physical therapy on the acute ward. First, any change of position or deep breathing triggered coughing attacks that induced oxygen desaturation and dyspnea. To avoid rapid deterioration and respiratory failure, we instructed and performed position changes very slowly and step-by-step. In this way, a position change to the 135° prone position () took around 30 minutes. This approach was well tolerated and increased oxygen saturation, for example, on day 5 with 6 L/min of oxygen from 93% to 97%. Second, we had to adapt the breathing exercises to avoid prolonged coughing and oxygen desaturation. Accordingly, we instructed the patient to stop every deep breath before the need to cough and to hold inspiration for better air distribution. In this manner, the patient performed the breathing exercises well and managed to increase his oxygen saturation. Third, the patient had difficulty maintaining sufficient oxygen saturation during physical activity. However, with close monitoring and frequent breaks, he managed to perform strength and walking exercises at a low level without any significant deoxygenation. Exercise progression was low on days 1 to 5, but then increased daily until hospital discharge to a rehabilitation clinic on day 10.","60-year-old man was admitted to the hospital with COVID-19 causing breathing problems and a fever. The physical therapy team worked carefully to help him breathe easier and improve his oxygen levels, taking small steps to avoid triggering coughing fits. They used a slow, step-by-step approach to position changes and breathing exercises, which helped him increase his oxygen saturation. With close monitoring and gentle exercise, he was able to regain some strength and mobility before being transferred to a rehabilitation clinic."
1,"A 39-year-old man was hospitalized due to an increasingly reduced general health condition, after persistent fever and dry cough for 2 weeks. The patient initially needed 4 L/min of oxygen, had a rapid and shallow breathing pattern at rest and became severely breathless during minor physical activities. In the beginning, physical therapy focused on patient education about dyspnea-relieving positions, the importance of regular mobilization, and deep-breathing exercises. However, it quickly became evident that his anxiety from fear of dying and worries about his future aggravated his dyspnea and vice versa. The patient was so dyspneic, anxious, and weak that he was barely able to walk to the toilet. To counter this vicious circle, the physical therapist actively listened to the patient, explained why he was experiencing breathlessness, and tested suitable positions to relieve his dyspnea. He seemed to benefit from the education and the relaxing breathing exercises, as seen on day 2, when his respiratory rate could be reduced from 30 breaths/min to 22 breaths/min and his oxygen saturation increased from 92% to 96% on 4 L/min oxygen after guiding him through some deep-breathing exercises. Over the next days, his dyspnea and anxiety started to alleviate and he regained his self-confidence. Therapy was progressively shifted to walking and strength training and the patient rapidly advanced to walk 350 m without a walking aid or supplemental oxygen before his discharge home.","This 39-year-old man was hospitalized because he had a persistent fever and cough, and was struggling to breathe. Initially, he needed a lot of oxygen and his breathing was very fast and shallow. Physical therapy helped him learn how to breathe better and relax, which reduced his anxiety and improved his breathing. As he felt more confident, his breathing became easier, and he was able to walk without needing extra oxygen. He quickly regained his strength and was able to walk a significant distance before going home."
2,"This 69-year-old male was admitted to the ICU after a dry cough for 2 weeks, oxygenation was poor and computer tomographic imaging showed typical COVID-19 pneumonia. Initially the patient received lung-protective ventilation and targeted sedation, but was otherwise stable. Treatment interventions included passive range of motion and positioning including passive mobilization into a side-edge position (). Over the next days, the patient deteriorated with hemodynamic instability and severe ARDS leading to intermittent prone positioning and continuous renal replacement therapy. The role of physical therapists during proning was to ensure correct joint positioning and pressure prophylaxis to prevent secondary complications such as nerve lesions, contractures, or pressure ulcers. Nevertheless, the long duration and repeated positioning resulted in a small pressure ulcer on the patient’s forehead. After tracheostomy, passive range-of-motion exercises, and passive side-edge mobilization were slowly resumed, whereby asynchronous ventilation and hemodynamic instability remained 2 major problems leading to further sedation and relaxation, thus inhibiting any active participation. After 24 days in the ICU, the patient scored 1/50 points on the Chelsea Critical Care Physical Assessment Tool (CPAx) and showed severe signs of muscle loss. The patient died soon after withdrawal of life support.","This 69-year-old man was admitted to the ICU after developing a cough and low oxygen levels, which were confirmed as COVID-19 pneumonia. He received supportive care, including special positioning to help his breathing and prevent pressure sores. Unfortunately, his condition worsened, and he needed more intensive treatments like kidney dialysis. Despite the efforts of physical therapists, he experienced complications and ultimately passed away after 24 days in the ICU."
3,"This 57-year-old male was admitted to the ICU with dyspnea, heavy dry cough, and fever 6 days after testing positive for COVID-19. Initially, he was able to exercise and sit in a chair with a physical therapist, but progressive respiratory failure necessitated intubation and proning. The patient had large amounts of bronchial mucus and required regular suctioning along with respiratory therapy. Secretions were assessed with pulmonary auscultation (presence of crackles) and by analyzing expiratory flow on the ventilator (sawtooth pattern). When suctioning failed to improve these clinical signs, 1 to 2 physical therapists used manual airway clearance techniques. The goal of these techniques was to sufficiently increase expiratory flow for effective airway clearance while avoiding alveolar collapse. To achieve this, manual compressions on the chest and abdomen were performed with just enough intensity to modify expiratory flow. After extubation, the patient was still unable to effectively clear his mucus due to weak cough. He continued to need intensive manual airway clearance techniques, nasal rinsing to induce cough and to help expectoration as well as upper and lower airway suctioning. To this end, the patient was treated up to 6 times per day/night. Additional physical therapist interventions included passive range of motion, assisted exercising, and mobilization. At the time of writing, the patient was still in the ICU without ventilatory support.","A 57-year-old man was admitted to the ICU after testing positive for COVID-19, experiencing difficulty breathing and a persistent cough. His breathing worsened, requiring him to be placed on a ventilator and positioned on his stomach to help him breathe more easily. Physical therapists worked with him to clear mucus from his lungs using special hand movements and techniques. Despite these efforts, he continued to struggle with clearing his mucus, and he needed ongoing support with suctioning and other therapies to help him breathe better. Currently, he remains in the ICU and is still receiving intensive care to improve his breathing."
4,"This 52-year-old male tested COVID-19 positive 4 days after the beginning of a dry cough, fever, and head and limb pain. One day later, he was hospitalized with exertional dyspnea. He was diagnosed with pneumonia that developed into moderate ARDS needing mechanical ventilation and intermittent dialysis. After extubation, oxygenation was stable with 2 to 3 L/min of oxygen. However, the patient was disoriented and could not communicate verbally. His global weakness (CPAx 11/50) was accompanied by oral and pharyngeal weakness and paresthesia. Spontaneous swallowing frequency and tongue control were severely reduced, and the patient showed insufficient protection from aspiration. This was confirmed by a specialized physical therapist with the Gugging Swallowing Screen, which confirmed severe dysphagia with 2/20 points. He was treated nil by mouth and received dysphagia therapy such as intensive oral stimulation, facilitation of swallowing, and training of protection mechanisms. After initial agitation and disorientation, the patient started to communicate in single-word phrases, but dysphagia continued to be severe with massive oral and pharyngeal dry saliva residuals that compromised his paresthesia and required regular mouth care. Over the next days, the patient managed to swallow pureéd food and mildly thick fluids under supervision, although cough strength was still weak (Gugging Swallowing Screen 13/20, CPAx 30/50). Nevertheless, he continued to progress and became capable of independent food ingestion (Gugging Swallowing Screen 20/20, CPAx 39/50) before his discharge to a rehabilitation clinic 25 days after admission.","This 52-year-old man was diagnosed with COVID-19, which led to pneumonia and eventually ARDS, requiring him to be placed on a ventilator. After he was taken off the ventilator, he had difficulty swallowing and speaking, and struggled to protect himself from choking. With intensive therapy, including exercises and special techniques, he gradually improved his ability to swallow and communicate. Finally, he was able to eat solid foods on his own and was discharged to a rehabilitation clinic to continue his recovery."
5,"This 33-year-old female patient had typical COVID-19 symptoms such as high fever, dry cough, headache, and dyspnea about 1 week before ICU admission. She was intubated and proned due to rapid respiratory deterioration. For the following 6 days, her situation was unstable, and physical therapy consisted of prone positioning and prevention of secondary damage. From day 7 onwards, she started to improve rapidly and could be mobilized passively into a side-edge position. After extubation, she presented postextubation dysphagia and severe ICU-acquired weakness (MRC-SS 36/60). She also suffered from pronounced delirium and anxiety and said repeatedly that she had been abducted and that she believed she had to die. She seemed to feel threatened by us and it was difficult to calm her down. Due to the pandemic measures of the Swiss government, hospital visits were not generally allowed, but because her anxiety was limiting her rehabilitation, her husband was granted an exceptional permission to visit her. This seemed to give the patient a short sense of security, and she started to participate in some basic functional activities (CPAx 21/50). Nevertheless, the delirium did not resolve upon her transfer to a peripheral acute hospital.","This woman came to the hospital about a week ago with symptoms of COVID-19, including fever and difficulty breathing, and needed to be put on a ventilator and placed on her stomach to help her breathe. For the next six days, her condition remained unstable, and physical therapy focused on keeping her comfortable and preventing further lung damage. She gradually improved and was able to move around with help, but she also experienced difficulty swallowing and weakness after being in the ICU. She was also struggling with confusion, anxiety, and frightening thoughts, and her husband was allowed to visit to help her feel more secure. Despite some progress, she still had difficulty with her thinking and needed to be transferred to another hospital for continued care."
6,"This 66-year-old male patient was admitted to the hospital due to an ischemic left-hemispheric stroke in addition to a dry cough and fever. He tested positive for SARS-CoV-2 the following day but continued to deteriorate resulting in severe ARDS, intubation, and ICU admission. Despite repeated proning, gas exchange did not improve sufficiently and the patient was placed on veno-venous extracorporeal membrane oxygenation for 7 days. After sedation was stopped, the patient continued to be somnolent and unable to communicate or to follow commands. Physical therapy therefore focused on perception training, movement exercises, airway-clearing techniques, dysphagia therapy, and mobilization. A first SOEB trial had to be discontinued due to hemodynamic instability. Instead, the patient was positioned in a side-edge position (), which he tolerated better and where an intensive exercise training including trunk and head control was conducted. Nevertheless, muscle tone and strength remained severely reduced, particularly on his hemiplegic side, and a second SOEB trial failed again. Physical therapy was also limited because of reduced self-activity and suspected impaired perception and visual acuity. Consequently, occupational therapy was involved to create a basis of communication, to support functional initiation of upper limb movements, and to integrate perception-training into activities of daily living. Currently, the patient tolerates spontaneous breathing trials, shows signs of being alert during therapy, but cannot communicate. He is hemodynamically stable, even in an SOEB position, but remains functionally dependent (CPAx 6/50).","This 66-year-old man was admitted to the hospital after having a stroke and testing positive for COVID-He developed severe lung problems, requiring him to be put on a breathing machine for a week. Physical and occupational therapists worked with him to improve his breathing, movement, and ability to communicate, starting with simple exercises and positioning. While he’s still having difficulty with his arm and speech, he’s showing some signs of alertness and is responding to therapy."
7,"A 66-year-old male started to present symptoms of fever, dyspnea, coughing, asthenia, lack of appetite, nausea, and vomiting. He was admitted to the acute care unit for observation and oxygen therapy, but his oxygen requirements constantly increased due to moderate ARDS. After 12 days of deep sedation, neuromuscular blocking agents, and proning with daily passive range of motion, the patient finally started to initiate active movements and was passively transferred to a chair. However, due to a persisting difficult weaning status, probably related to respiratory muscle weakness, tracheostomy was performed [ventilator settings: pressure support 10 cmH2O, positive end-expiratory pressure (PEEP) 8 cmH2O]. Subsequently, the patient showed significant improvement in his physical functions with active SOEB, chair-transfer with the help of 2 physical therapists, and active in-bed cycling against resistance for 20 minutes (). The strategy was to increase pressure support (by 5 cmH2O) during efforts to reinforce exercise training effects, unloading respiratory muscles. This strategy along with a highly collaborative patient culminated in his rapid improvement in physical function (MRC-SS 58/60, physical function ICU test score 10/12, walking distance 10 m), although he was still experiencing fatigue, inspiratory muscle weakness (maximal inspiratory pressure of −45 cmH2O) and dysphagia upon his transfer to a step-down unit.","This 66-year-old man was admitted to the hospital because he was struggling to breathe and had a high fever. He developed a serious lung condition called ARDS, requiring him to be placed on a ventilator and positioned on his stomach to help him breathe. After several weeks of treatment, he started to regain strength and was able to move around with assistance. To help him breathe easier, a tracheostomy was performed, and he gradually improved his ability to walk and perform daily activities, though he still experiences some fatigue and weakness."
8,"This 77-year-old male patient was transferred to our ICU 1 week after his COVID-19 diagnosis due to continuing respiratory decompensation requiring intubation. Following the acute phase, with intermittent proning, the patient continued to be hemodynamically unstable and was difficult to wean. Rehabilitation proved challenging under these conditions, and physical therapists had to reevaluate and adapt their interventions daily according to his condition. After 2 weeks, he was tracheotomized and started to improve very slowly. One week after tracheostomy, the patient was able to speak for the first time after a cuff-down trial and with the help of a speaking valve. But the patient spoke only a few words with us and it was often difficult to involve him in exercises. Two days later, he was able to communicate with his relatives via video telephony. This was a very emotional moment for everyone involved, but it improved his communication and he was able to express to his wife that he had no strength left to continue. However, through the family’s active participation in his early rehabilitation process, they were able to reinforce his confidence and motivation. He was discharged to a rehabilitation clinic severely weak (MRC-SS 40/60) and functionally impaired (CPAx 22/50), but continued to progress in slow steps.","This 77-year-old man was admitted to the ICU after a difficult COVID-19 illness, needing a breathing tube to help him breathe. Despite efforts to improve his condition, he remained unstable and required further treatment, including a tracheostomy. After two weeks, he began to improve and was able to speak again, bringing a joyful moment for his family. He was discharged to a rehabilitation clinic to continue regaining strength and function, with his family actively supporting his recovery."
9,"A 45-year-old female was brought in by ambulance after collapsing at home secondary to a hypoglycemic event (capillary blood glucose of 1 mmol/L with paramedics). She had a history of restrictive AN, binge-purge behaviour, and an old traumatic brain injury, leaving her with memory problems. She was well known to mental health services, having been admitted multiple times to eating disorder centres for nasogastric feeding. She had never smoked in her life and denied any alcohol intake. The patient was on ferrous fumarate, fexofenadine, fluoxetine, ibuprofen, lansoprazole, quetiapine, supplemental vitamins, regular morphine (modified release), and gabapentin.\nOn admission, her blood pressure was 106/85 mmHg, respiratory rate was 20 breaths/minute, heart rate was 64 beats/minute, temperature was 35.1 °C, and capillary blood glucose was 6 mmol/L. Her weight on admission was 37.3 kg (body mass index [BMI] = 12.6). On examination, she was clearly malnourished, cachexic, and dehydrated. The rest of the clinical examination was normal, as shown in Table . Her chest radiograph showed patchy consolidations in the right middle and lower lobes (Figure ). She was prescribed appropriate antibiotics. She was refusing treatment and was deemed to lack the capacity to make that decision. Therefore, Section 5(2) under the Mental Health Act was put in place. She was commenced on oral supplements as per guidance from the dietitian, and then switched to nasogastric feeding.\nOn the night of the second day, she had an episode of decreased consciousness, bradypnoea (RR-6), and hypotension (83/64). Her blood sugar level was 6.6 mmol/L. After receiving Naloxone, her symptoms improved, and her opiates were discontinued. The following day she mentioned right upper quadrant pain. Blood tests showed worsening liver enzymes, as shown in Table . An abdominal ultrasound was performed on the fourth day of admission, which showed multiple small calculi and biliary debris, with gallbladder oedema, and a small amount of peri-cholecystic fluid (Figure ).\nShe was treated for cholecystitis with amoxicillin, clavulanic acid, and clarithromycin, which led to improvement in her inflammatory markers in the following days. She underwent an inpatient magnetic resonance cholangiopancreatography (MRCP) to rule out intra-biliary pathology, which was negative. Subsequently, she was referred to surgeons for consideration of elective laparoscopic cholecystectomy once deemed fit.\nShe had a protracted and difficult admission. Due to the coronavirus disease 2019 pandemic, her transfer to an Eating Disorders Unit proved difficult. By the fourth week of admission, her liver enzymes had improved, as can be seen in Table . Nasogastric feeding was successfully weaned as her weight improved. She was discharged on day 51 to an Eating Disorders Unit weighing 56.4 kg (BMI = 18.1) from her admission weight of 37.3 kg (BMI = 12.6).","This 45-year-old woman collapsed at home due to dangerously low blood sugar. She has a history of eating problems and a brain injury, which have made her very underweight and vulnerable. During her hospital stay, she experienced several concerning episodes, including decreased consciousness and difficulty breathing, and her liver enzymes were elevated. After treatment for an infection and a change in her feeding method, she gradually improved and was discharged to an eating disorder unit, significantly healthier and stronger."
10,"A 29-year-old male was brought to the hospital by ambulance after collapsing at home. He was found to be bradycardic and hypoglycaemic with a capillary blood glucose level of 2.3 mmol/L. He had a history of eating and anxiety disorders and was not on any regular medications. On admission, his weight was 37.3 kg (BMI = 11.6). His blood pressure was initially un-recordable but subsequently was recorded to be 104/72 mmHg. His capillary blood glucose level was 4.7 mmol/L, and his Glasgow Coma Scale score was 15/15. On examination, he was noted to be severely malnourished and cachexic. The rest of the clinical examination was normal. LFTs were very abnormal, as shown in Table .\nSince admission, he seemed to lack insight. Due to problems keeping him compliant with medication and intravenous glucose, he had a number of hypoglycaemic events in the first two days of admission. The following day, he was deemed not to have the capacity. He underwent Mental Capacity Assessment and Deprivation of Liberty Safeguards. He was ultimately placed under Mental Health Act 5(2) and was started on nasogastric feeding. His liver enzymes worsened further after the introduction of nasogastric feeding, but we were reassured by a normal non-invasive liver screen and ultrasound.\nHis condition, liver tests, and liver synthetic function improved over the course of his 24-day admission (Table ), but his stay was associated with difficult behaviour. He was eventually discharged to an Eating Disorders Unit.","This 29-year-old man collapsed at home and was rushed to the hospital because he was very weak and his blood sugar was dangerously low. He had a history of eating problems and anxiety, and he was severely malnourished. During his stay, he struggled to take his medication and eat, leading to repeated low blood sugar episodes. After a 24-day hospital stay, his condition improved, and he was transferred to an eating disorder unit for continued care."
11,"A 36-year-old G4P2 premenopausal woman with a family history of colorectal, hepatobiliary cancers felt an abnormal right breast lump. Diagnostic mammogram and ultrasound showed a hypoechoic lesion in the upper outer quadrant of right breast measuring 14 mm x 13 mm x 18 mm and 5 x 4 mm satellite lesion is noted 6 mm inferior to the dominant mass, BI-RADS 5 highly suggestive of malignancy. Due to concern for multifocal disease, MRI breast with without contrast was done and it showed 2.3 x 1.1 x 2.7 cm irregular-shaped, heterogeneous mass with irregular margins in the upper outer quadrant of right breast, 7 cm from the nipple, 1.2 cm from the skin and there was an additional mass measuring 8 mm x 4 mm x 1.6 cm at 12:00 along with 4 mm lesion, 7 mm from the nipple at 10:00 (Figures (a) and (b)). Right breast biopsy from the dominant lesion showed invasive mammary carcinoma with features of both lobular and ductal carcinoma, Nottingham histological grade 2, estrogen receptor 90%, progesterone receptor 100%, HER2 2+ by IHC but negative by FISH, Ki-67 50%.\nStatus post right breast simple mastectomy and axillary lymph node evaluation. Surgical pathology showed a multifocal invasive mammary carcinoma of the breast with ductal and lobular features, size of largest invasive carcinoma was 55 mm, size of additional invasive foci was 1.5 mm, Nottingham histological grade 2 of 3, low to intermediate nuclear grade DCIS without central necrosis measuring at least 6 mm, margins uninvolved, one benign sentinel lymph node. Pathological staging (m)pT3 (sn)N0. Oncotype DX breast recurrence score of 16 (for patients <50 years of age, benefit from chemotherapy 1.6%). Genetic testing did not reveal any clinically significant mutations. The patient has received adjuvant PMRT 5000 cGy dose, 25 fractions along with 1000 cGy scar boost. Based on TEXT/SOFT data ovarian suppression could be considered in patients with high-risk features. The patient's tumor was multifocal, and the largest lesion measured 5.5 cm. The patient elected against surgical menopause. The patient was started on ovarian suppression with goserelin 3.6 mg every 28 days along with letrozole 2.5 mg once daily.\nSixteen months after initiation of ovarian suppression patient started having a regular menstrual cycle while on goserelin. No medication interaction or missed doses were noted. The patient's BMI was 41.1 and BSA was 2.19 m2. Estradiol was 92 pg/mL and follicle-stimulating hormone (FSH) was 4.3 mIU/mL in the premenopausal range. Few prior case reports showing failure of goserelin ovarian ablation in a premenopausal woman were reported. Given high-risk disease, discussed benefit of continued ovarian suppression plus AIs versus tamoxifen alone, and discussed the role of oophorectomy which results are reliable and prompt reduction in circulating estrogens. The patient was hesitant and wished to wait before the oophorectomy. She was started on triptorelin 3.75 mg IM once every 28 days.","A 36-year-old woman found a lump in her breast and underwent tests that showed it was likely cancer. Further imaging revealed multiple areas of concern, and a biopsy confirmed invasive breast cancer with features of both ductal and lobular types. After surgery to remove her breast and some lymph nodes, doctors determined the cancer was more advanced than initially thought. To reduce the risk of recurrence, she started taking medication to suppress her ovaries and will continue to monitor her condition closely."
12,"The patient was a 62-year-old male with a past medical history of liver cirrhosis secondary to hepatitis C, tobacco use, and post-stent coronary artery disease, who initially came to the hospital for elective left and right heart catheterization as a pre-transplant evaluation. Physical examination showed abdominal distension and diffuse tenderness with the presence of prominent superficial abdominal veins. A computed tomography (CT) scan of the abdomen with contrast was obtained immediately. The CT showed an occlusive thrombus of the IVC extending from the renal veins to the level of the cavoatrial junction. Thrombus was also observed in the portal vein, and multiple subcutaneous varicosities were found. Initially, a plan was made to start the patient on anticoagulation, but because of the patient’s history of advanced cirrhosis, large esophageal varices on recent endoscopy, and thrombocytopenia, we concluded that the patient was not a candidate for anticoagulation. Given that the patient had significant abdominal distention with pain that did not improve even after therapeutic paracentesis; we inserted a stent in the IVC to relieve the patient’s pain as a palliative procedure to improve his quality of life.\nRight internal jugular (IJ) and right femoral vein accesses were obtained for the procedure. A 6F pigtail diagnostic catheter was advanced from the right IJ to the right atrium, and contrast was injected into the right atrium that showed an occluded IVC at the junction of the right atrium. Another pigtail catheter was advanced through the right femoral vein and an inferior venogram was performed that showed the IVC was 100% occluded 2 cm above the renal veins (Figures , ).\nA Glidewire advantage was advanced through the right femoral vein but was unable to cross the 100% occluded IVC. Then, a 7-French Swan-Ganz catheter was tried and was successfully advanced through the IVC all the way to the right atrium. A Swan wire was inserted through the Swan-Ganz catheter. Then, a multipurpose catheter was advanced over the Swan wire into the right atrium, and the Swan-Ganz wire was successfully replaced with a Glidewire Advantage (Figure ). Initially, a 9 × 80 Mustang balloon was used to perform the IVC ballooning; after IVC ballooning; however, the inferior venogram showed no flow to the IVC, which was still 100% occluded. Then, a 22 × 70 mm Wallstent was advanced via the right femoral vein to the right atrial side. The stent was deployed with a considerable waist in the mid area (Figure ). After stent placement, an 18 × 60 Boston Scientific esophageal balloon was successfully used to post-dilate the stent (Figure ).\nThe final venogram (Figures , ) and digital subtraction imaging mode showed excellent flow in the IVC to the right atrium. Small clots floating around at the lower edge of the stent above the renal artery were successfully removed with a 7-Fr snare. Usually, if IVC occlusion is of thrombosis in etiology anticoagulation is advised after venous stent placement, since our patient had an advanced liver failure with elevated INR, anticoagulation was not started [].","This 62-year-old man came to the hospital for a test to see if he could receive a liver transplant. During the evaluation, doctors found a major blockage in his inferior vena cava (IVC), a large vein that carries blood from the legs and abdomen to the heart. To relieve his pain and improve his quality of life, a stent was placed in the IVC to open up the blockage. The procedure was successful, and the stent restored blood flow, allowing for a better outcome."
13,"We report a case of a 45-year-old woman, a non-smoker, treated for type II diabetes under insulin and primary hyperparathyroidism. Her medical history dates back to 2006 with an infiltrated nodule associated with ulcers that grew gradually in her right thigh. The patient underwent surgery with an anatomopathological study. Skin biopsy objectified ulcerated epidermotropic dermo-hypodermal tumor proliferation whose morphological appearance and immunohistochemical data are in favor of LCH. Langerhans cells present positivity of the anti-PS100 antibody, anti-CD1a antibody, and anti-Ki67 antibody (60%), and negativity of the anti-CD68 antibody. The patient subsequently received 25 sessions of radiotherapy followed by six courses of chemotherapy with a low dose of oral methotrexate. After these treatments, skin lesions are stable. In 2020, the patient complained of an increase in skin lesions, with the appearance of several infiltrating nodules, scaling, crusted papules, and ulcerated plaques. The patient received chemotherapy as single-system LCH (SS-LCH) based on methotrexate, associated with prednisone and vinblastine, with the obtainment of stable response and limited regression of the lesions. After two courses, the patient was lost to follow-up. One year later, the oncologist referred the patient to the hematology department for a major increase in skin lesions, with the appearance of new ones on her thigh. Dermatological examination found ulcers and necrotic lesions, purplish, well-limited, of variable size, not painful, not warm to the skin, and localized to the right thigh. The mucous membranes and integuments were unharmed (Figure ).\nFurthermore, the examination found an afebrile, obese patient (body mass index (BMI) at 44.8). Abdominal and cardiovascular examinations were unremarkable. The physical examination did not find any tumoral syndromes, and the patient did not present have B signs (fever, weight loss, and night sweats). A complete assessment was carried out in favor of a cutaneous location without visceral involvement. A CT scan of the chest and abdomen was normal. Extensive investigation revealed no systemic involvement, central nervous system (CNS) evaluation was not done, serum thyroid hormone levels were normal, endocrine workup was performed, including cortisolemia, and parathormone assessment were done in favor of primary hyperparathyroidism. A skin biopsy was performed. The microscopic examination revealed a cutaneous tissue with an infiltrate of large cells involving the dermis and realizing an epidermotropism. The infiltrate was composed of atypical cells with abundant, pale eosinophilic cytoplasm, irregular and elongated or lobed nuclei with prominent nuclear grooves and folds, fine chromatin, and indistinct nucleoli. Multinucleated giant tumor cells, high cytonuclear atypia, and marked nuclear pleomorphism were seen. The mitotic figures were increased. The neoplastic cells also showed a mixed inflammatory background with variable numbers of reactive lymphocytes, plasma cells, benign histiocytes, and eosinophils associated (Figure ).\nThe immunohistochemical stains had shown a diffuse strong and heterogeneous positivity for CD1a and S100 and variable positivity for CD45. All other markers studied were negative, including CD68, cytokeratins (AE1/AE3), and melanocytic marker (MelanA). The Ki-67 proliferation index was high (50%) (Figure ).\nIn the light of microscopic and phenotypic results, the diagnosis of Langerhans cell sarcoma was confirmed. The patient did not receive any maintenance therapy and she received only two courses of chemotherapy; consequently, the patient was included in the LCH protocol as single-system LCH (SS-LCH), and she received the same chemotherapy combining prednisone (60 mg / m2), high-dose of methotrexate (5000 mg/m2), and vinblastine (10 mg/m2). The evolution was favorable after five cycles of chemotherapy with significant regression of lesions in the thigh without the appearance of any new lesions (Figure ).","This patient is a 45-year-old woman with a history of type 2 diabetes and primary hyperparathyroidism who has been battling a rare skin cancer called Langerhans cell sarcoma for many years. Initially diagnosed in 2006, the cancer had spread to her thigh and was treated with surgery, radiation, and chemotherapy. However, the cancer returned in 2023, with new lesions appearing on her thigh. Further tests revealed the cancer was still localized to the skin, with no signs of spreading to other parts of her body. After receiving chemotherapy, the patient experienced a significant reduction in the size of the skin lesions, indicating a positive response to treatment."
14,"We describe the case of a 55-year-old male who presented to the emergency department via emergency medical services for the chief complaint of sudden onset shortness of breath that woke him from his sleep just prior to arrival. He reported three days of non-radiating lumbar back pain and two episodes of non-bloody emesis leading up to this event. His medical history included hypertension and type 2 diabetes mellitus. His current medications were metformin, amlodipine, losartan, and atenolol. Initial vital signs revealed heart rate (HR) 75, respiratory rate (RR) 29, blood pressure (BP) 119/62, and oxygen saturation 99% on 2L nasal cannula. Temperature was 36.3°C.\nPhysical examination was significant for an ill-appearing male patient who was anxious and tachypneic. He also had significant work of breathing with retractions and abdominal breathing. Lungs were clear to auscultation, with no wheezing, rhonchi, or rales. Abdominal exam revealed mild epigastric tenderness with no rebound, guarding, or palpable pulsatile mass. No costovertebral angle (CVA) tenderness or midline tenderness was elicited. Neurological exam revealed no focal deficits.\nDue to his presentation and multiple comorbidities we had significant concern for the possible acute coronary syndrome, dissection, sepsis, or pulmonary embolism. Blood work including complete blood count (CBC), comprehensive metabolic panel (CMP), lactic acid, troponin, urine analysis (UA) was ordered. CT-angiogram of his chest, abdomen, and pelvis was also ordered.\nComplete blood count revealed a white blood cell count of 20.4 x 103/uL, hemoglobin of 11.2 g/dL, and platelet count of 376 x 103/uL. Comprehensive metabolic panel was significant for a sodium of 145 mmol/L, potassium 6.1 mmol/L, chloride 100 mmol/l, bicarbonate <7 mmol, blood urea nitrogen (BUN) 67 mg/dl, creatinine 14.7 mg/dL, calcium 9.1 mg/dL, and glucose of 165. Troponin I was <0.012 ng/mL. Initial lactic acid was recorded at 13.9 mmol/L. An arterial blood gas revealed severe anion gap metabolic acidosis with a pH of 6.9, pCO2 of 22.3 mmHg, pO2 of 105.5 mmHg, HCO3 of 5.3 meq/L, and a Base Excess of -24.8 mmol/l. The patient was anuric at this time. Initial EKG showed normal sinus rhythm (NSR), nonspecific ST abnormalities, vent rate 73, QTC 471. The CT scan revealed an obstructing stone at the left proximal ureteropelvic junction (Figures , ) and at the right ureterovesical junction (Figure ), with resultant bilateral hydronephrosis (Figure ).\nWhile in the ED the patient progressively became hypotensive and was given broad-spectrum antibiotics, Vancomycin and Cefepime, due to concern for sepsis and aggressive fluid resuscitation was initiated. The patient did not respond to fluid resuscitation and was started initially on Levophed. In addition, the patient received 100 mL of 8.4% sodium bicarbonate and 2 grams of calcium gluconate for severe metabolic acidosis and hyperkalemia. Due to the expected clinical decline, the patient was intubated in the emergency department. Critical Care, Nephrology, and Urology were consulted emergently in the ED. After initial stabilization, the patient was taken for immediate bilateral percutaneous nephrostomy (PCN) placement by Interventional Radiology (IR) and placement of a trialysis catheter for initiation of hemodialysis afterward. Urinalysis from the PCN revealed hematuria, but no evidence of infection.\nAfter successful placement of bilateral PCN, the patient was transferred to the intensive care unit (ICU) in critical condition. He was maintained on multiple vasopressors consisting of combinations of norepinephrine, vasopressin, epinephrine, and phenylephrine along with stress dose hydrocortisone. Repeat blood gas and labs revealed a worsening metabolic and lactic acidosis with a repeat pH of 6.7 and a lactic acid of 15.9. The patient was started on hemodialysis with a high bicarbonate dialysate for emergent clearance of hyperkalemia and refractory acidosis. After hemodialysis, the patient’s pH was 7.1, despite a worsening lactic acidosis of 17.0 mmol/L. Overnight the patient was converted to continuous venous-venous hemodialysis, a form of continuous renal replacement therapy (CRRT). Within 24 hours the patient’s acidemia corrected and blood pressure stabilized allowing for a de-escalation of vasopressors. A repeat chemistry revealed recovering renal function along with urine production in the nephrostomy tubes. On hospital day 3, the patient was successfully extubated, weaned off all vasopressors, and CRRT was stopped. Blood and urine cultures taken prior to dialysis were negative after five days. On hospital day 6, Urology performed a cystoscopy with bilateral ureteral stent placement and removal of bilateral percutaneous nephrostomy tubes. On hospital day 8, the patient had made a remarkable recovery from being critically ill. His creatinine was 1.5, BUN 27, and he was discharged home with urology follow-up in 1-2 weeks.","This man was rushed to the hospital after suddenly having trouble breathing. He also had back pain and felt sick to his stomach. Doctors were worried he might be having a heart problem or a blood clot, so they did several tests, including scans of his chest, abdomen, and pelvis. The scans showed a large stone blocking his kidneys, which was causing a buildup of acid in his blood and leading to his breathing problems. Because of this, he needed immediate treatment with fluids, antibiotics, and dialysis to help his kidneys function. He was also given medications to help lower his blood pressure and correct the acid imbalance. After several days of intensive care, he started to improve and was eventually able to breathe on his own. Finally, a urologist placed stents in his ureters to help drain the stone, and he was discharged home with a follow-up appointment to monitor his recovery. He is now recovering well and is expected to make a full recovery."
15,"A 58-year-old Caucasian (American) male with an unremarkable past medical history presented for evaluation of nausea, vomiting, and a 30-pound weight loss over the past two months at our hospital. He also reported dark-colored urine and intermittent episodes of hemoptysis during the same period. Specifically, he stated that his symptoms started four days after receiving his second dose of the mRNA-1273 (Moderna) vaccine for COVID-19. His first dose taken three weeks earlier was well tolerated. He denied any flank or abdominal pain, melena, fever, cough, hematuria, urinary frequency or urgency, and trauma. He denied smoking. Vital signs were stable upon admission. Physical examination was insignificant for any lower extremity pitting edema, petechiae, or rash. The patient was not on any medication prior to his hospitalization.\nLaboratory analysis was remarkable for serum creatinine of 4.1 mg/dL (0.8-1.4 mg/dL) along with hematuria and sub-nephrotic proteinuria of 1796 g/24 hours (<150 mg/24 hours). Our differential diagnosis at this point was wide including all nephritic syndromes given AKI, hematuria and proteinuria. All serological workup was subsequently sent. C-ANCA (anti-neutrophil cytoplasmic antibodies) were elevated 160 AU/mL (20-25 AU/mL) and anti-proteinase 3 (anti-PR3) antibodies were also elevated >100 EU/ mL (normal <3.5 EU/mL) (Table ). Immunohistochemical staining for the SARS-CoV-2 spike protein was not performed. All previous routine laboratory parameters including urinalysis were within normal range.\nHe underwent computed tomography (CT) scan of the chest for evaluation of hemoptysis that showed a right upper lobe consolidation and moderate bilateral pleural effusion.\nThe renal ultrasound was unremarkable. Renal biopsy was subsequently performed and showed acute, pauci immune, focal necrotizing, and diffuse crescentic glomerulonephritis (Figures , ).\nThe patient was diagnosed with anti-PR3-associated ANCA glomerulonephritis. He received intense immunosuppression with plasma exchange x 5 cycles, intravenous prednisone 1 gram x 3 doses followed by prednisone 60 mg daily, cyclophosphamide x 2 doses, and rituximab x 4 doses. The patient achieved remission after 10 weeks of diagnosis with a resolution of symptoms and improvement in renal function with a creatinine of 1.5 mg/dl. He is following in our nephrology office regularly since discharge.","A 58-year-old man came to the hospital because he was experiencing nausea, vomiting, and unintentional weight loss, along with dark urine and coughing up blood. These symptoms started shortly after he received his second COVID-19 vaccine. Tests revealed that he had kidney damage and inflammation, and his blood tests showed signs of an autoimmune reaction. After receiving intensive treatment, including plasma exchange and medications, his condition improved, and his kidney function returned to normal."
16,"A 67-year-old Caucasian female presented to our hospital with a chief complaint of persistent bright red blood per rectum. Her medical history was significant for hypertension, hyperlipidemia, diabetes mellitus type 2, coronary artery disease with three prior myocardial infarctions, recurrent cerebrovascular accidents requiring anticoagulation with warfarin, gastroesophageal reflux disease, asthma, and endometrial cancer status post radiation therapy. Fifteen months prior to the current presentation, the patient was noted to have a grade 1 endometrial adenoma but was not considered a good surgical candidate due to multiple comorbidities. Vaginal hysterectomy was considered but due to her long and narrow vagina, this option was deferred initially. Her only treatment option was radiation therapy and brachytherapy. She eventually underwent total abdominal hysterectomy with bilateral salpingo-oopherectomy due to continued pelvic pain. The patient denied any prior history of gastrointestinal (GI) bleeding. Her bleeding was described as one large episode of bright red blood per rectum associated with blood clots. She denied any abdominal pain, nausea, vomiting, diarrhea, constipation, or melena. The most recent colonoscopy was performed four months ago and revealed three diminutive polyps in the transverse colon with pathology confirming tubular adenoma.\nHer physical examination was significant for mild left-sided abdominal tenderness but was otherwise unremarkable. Rectal examination was notable for nonbleeding hemorrhoids and no visible blood. Blood work revealed white blood cells of 14.3k/uL (normal range 4.3-10.0 k/uL) and hemoglobin of 9.6 g/dL (normal range 11.8-14.8 g/dL), which is similar to the patient’s baseline. Creatinine was slightly elevated to 1.2 and blood urea nitrogen was elevated to 39. International normalized ratio was 2.0. Due the large volume of hematochezia and presence of anemia, the patient was admitted to the hospital and underwent a colonoscopy, which revealed a large, fungating, friable, and ulcerated nonobstructing mass in the sigmoid colon. The mass was noncircumferential, measured 4 cm in length, and was located 15-19 cm from the anal verge (Figures , ). Biopsies were obtained with cold forceps for histology and the proximal and distal margins of the mass were tattooed. Histology showed invasive and moderately differentiated carcinoma without visible goblet cells. Given the patient’s history of endometrial cancer, immunohistochemistry was performed and was consistent with an endometrial (endometrioid subtype) primary.\nPan-computed tomography was negative for any obvious distant disease. The tumor marker carcinoembryonic antigen level was normal as well. The patient subsequently underwent surgical resection of the affected colon followed by radiation therapy. At a follow-up visit one year later, the patient was doing well without active GI complaints.","67-year-old woman came to the hospital because she was experiencing bright red blood in her stool. She has a history of several health problems, including heart disease, diabetes, and cancer. A colonoscopy revealed a large tumor in her colon that was causing the bleeding, and it was determined to be a type of cancer. After surgery and radiation therapy, she is doing well and has no active symptoms."
17,"A 71-year-old African American female with a medical history of hypertension, type 2 diabetes mellitus, stage 3 chronic kidney disease, and osteoarthritis initially presented to the emergency room with intermittent bilateral hand tingling and numbness, which was gradual in onset over months. Her symptoms were associated with seeing red spots and experiencing a burning sensation in the bottom of her feet. She was concerned because she was having difficulty picking up objects due to her hand symptoms. Her basic lab work was unremarkable, and she was provided gabapentin and magnesium oxide with close follow-up with her primary care physician. At follow-up, she complained of one to two months of unsteady gait as well as increased confusion. Her reported medications included losartan and metformin. Physical exam revealed normal pupils with reaction and accommodation (3mm diameter bilaterally), no cranial nerve deficits, normal strength throughout, and normal reflexes throughout except for diminished reflexes in the bilateral knees and ankles. She endorsed blurry vision; no ophthalmologic exam was performed, but she was able to read a name badge from one foot away. She additionally was found to have decreased vibratory and proprioception in a stocking pattern as well as a wide-based and unsteady gait.\nTo investigate further, tests for thyroid-stimulating hormone (TSH), folate, B12, and rapid plasma regain (RPR) titer were ordered. Her TSH, folate, and B12 levels were within normal limits, but her RPR titer was reactive at 1:1. A subsequent reflex Treponema pallidum particle agglutination (TP-PA) test was reactive. She was told to go to the hospital for further workup and treatment. Further questioning revealed that she had had two sexual partners in her life, both ex-husbands. However, she noted that her husbands committed adultery several times and that she was not currently sexually active; her last sexual encounter occurred several years ago. She endorsed that she did not notice any ulcers or skin lesions, and she had not been treated for syphilis. During hospitalization, she received a lumbar puncture that revealed a negative venereal disease research laboratory (VDRL) test result. Her glucose level (61 mg/dL) and white blood cell level (3 x 109/L) were normal, but her CSF protein level was markedly elevated at 156 mg/dL. Due to her exposure history, a constellation of symptoms, RPR titer with positive TP-PA, and protein elevation in her CSF, our infectious disease colleagues believed that she contracted syphilis earlier in life from one of her husbands and was never treated. The decision was made to start treatment with intravenous (IV) penicillin (24 million units, continuous infusion) for two weeks. At follow-up, her vision had improved and she no longer had sensory symptoms in her hands. Her gait was somewhat improved, but still unsteady, and she still had burning pain in her feet. Her physical exam showed improvement in sensation in hands and improved gait; all other neurological exam was unchanged. It was thought that neuropathy in feet could have been due to poorly controlled diabetes; although, no formal nerve conduction study was pursued.","A 71-year-old woman came to the hospital because she was experiencing tingling and numbness in her hands, along with blurry vision and difficulty with balance. Tests revealed she had a past infection with syphilis, likely from years ago, which was causing these symptoms. She started treatment with intravenous antibiotics, and her symptoms, including vision and hand sensations, began to improve. Although her balance is still a bit unsteady, she is now feeling much better overall, and her doctors are monitoring her condition closely."
18,"Our patient is a 78-year-old male with a past medical history of cutaneous T-cell lymphoma/mycosis fungoides (on regular outpatient extracorporeal photopheresis), type II diabetes mellitus, atrial flutter on Xarelto, and sick sinus syndrome on dual-chamber pacemaker, presented to the hospital with right upper quadrant abdominal pain. The patient was a former smoker and denied any alcohol use.\nIn the emergency department, he was hemodynamically stable. Laboratory workup was significant for abnormally elevated liver function tests including aspartate aminotransferase/alanine aminotransferase (AST/ALT) of 204/188 U/L, alkaline phosphatase (ALP) of 550 U/L, and total bilirubin of 2.5 mg/dL. Ultrasound of the abdomen was negative for any focal liver or gallbladder lesions. There was no evidence of intrahepatic or extrahepatic biliary duct dilation. Hepatobiliary iminodiacetic acid (HIDA) scan was normal, and hence cholecystitis was ruled out. CT abdomen and pelvis and CT angiography of the chest were negative for acute pathology. As the patient had a pacemaker, magnetic resonance cholangiopancreatography (MRCP) could not be performed. Further laboratory evaluation for elevated liver enzymes, including viral hepatitis panel, thyroid-stimulating hormone (TSH), iron panel, antinuclear antibody (ANA), anti-mitochondrial antibody, alpha-1-antitrypsin antibody, anti-smooth muscle antibody, and ceruloplasmin was negative.\nGiven that the patient has a history of cutaneous T-cell lymphoma, the important differential diagnosis included leukemic infiltration of the liver and adverse reaction to the prior chemotherapy. However, the patient received only a short course of the chemotherapeutic regimen mogamulizumab (due to insurance issues), and hence it was unlikely to cause this current clinical picture. Subsequently, a percutaneous liver biopsy was performed to confirm the diagnosis, which showed replacement of the normal liver parenchymal cells by high-grade tumor cells with a high nuclear-cytoplasmic ratio (Figures -). The tumor cells showed positive immunohistochemical staining for cytokeratin AE1/AE3, cytokeratin 20 (CK20), synaptophysin, chromogranin, and negative for CK7, caudal type homeobox transcription factor 2 (CDX-2), and thyroid transcription factor 1 (TTF-1) (Figures -). All these features were suggestive of metastatic Merkel cell carcinoma. There was no evidence of leukemic infiltrates. As the patient had no evidence of MCC involvement of the skin, he was diagnosed with metastatic MCC of the liver of unknown primary.\nHematology/Oncology and Dermatology was consulted. Considering the medical comorbidities, the patient and family opted for comfort care measures and were discharged home.","A 78-year-old man with a history of skin cancer, diabetes, and a heart condition came to the hospital because of pain in his upper right abdomen. Tests revealed high levels of liver enzymes and a concerning pattern of cells in a liver biopsy, leading to a diagnosis of metastatic Merkel cell carcinoma, a rare type of cancer. Because of his other health problems, the doctors discussed treatment options with him and his family and decided to focus on comfort care and discharge him home. The patient will continue to be monitored for his cancer."
19,"A 24-year-old healthy woman presented with difficulty breathing and dissatisfaction with her facial appearance. She had a history of childhood trauma resulting in nasal septum deviation and external nasal deformity. Four months after a successful and uneventful septorhinoplasty, she presented to the emergency department with blunt nasal trauma resulting in a septal hematoma, which was drained successfully; the patient was discharged with no adverse sequelae.\nFour months later, the patient sustained nasal trauma again, this time accompanied by clear nasal discharge, raising suspicion of cerebrospinal fluid (CSF) leak. The patient was discharged after managing the nasal injury, as the CT brain showed an intact cribriform plate with no evidence of a CSF leak. Ten days later, she presented at the emergency department with dizziness and an unstable gait. She also had complaints of paresthesia for the past two months, beginning in her right hand and progressing to the right shoulder, arm and leg, associated with some difficulty in the execution of movements in the first and second finger of the right hand. Her right leg was quite stiff with difficulty in walking. On close inquiry, she gave history of pain in the right eye and double vision many months back, which had resolved spontaneously. Examination showed a positive Romberg’s and Lhermitte’s sign, with right-sided sensory impairment.\nMagnetic resonance imaging (MRI) of the brain, cervical and thoracic spine demonstrated demyelinating lesions in the brain and cervical segment of the spinal cord (Figure ). Some of the lesions demonstrated enhancement on post gadolinium administration sequences, suggestive of active demyelinating diseases like MS. A lumbar puncture was performed which demonstrated the presence of oligoclonal bands in the CSF. The diagnosis of MS was confirmed by a neurologist and treatment was initiated.\nThe initial neurological symptoms have largely vanished with only persistent light paresthesia in the right hand. Two years later she has had no new symptoms and continues with the same medication with good tolerance.","This 24-year-old woman came to the hospital because she was having trouble breathing and wasn't happy with her face. She had previously had surgery to correct a problem with her nose, which was successful. However, she recently experienced another nose injury and developed a CSF leak, leading to dizziness, difficulty walking, and numbness in her right hand and leg. MRI scans revealed that she has multiple sclerosis, an autoimmune disease affecting the brain and spinal cord. With treatment, her symptoms have improved, and she is now doing well."
20,"A 64-year-old Caucasian male smoker with a horseshoe kidney with a history of open pyelolithotomy 18 years ago, presented to King Abdulaziz Medical City in mid-2020 with a report from another hospital stating that he developed gross hematuria six months prior, which was treated as a urinary tract infection. A CT of the abdomen and pelvis was performed in that hospital, showing a horseshoe kidney with severe left hydronephrosis and enlarged retroperitoneal lymph nodes, with the largest one located in the posterior part of the left renal artery measuring 4.7 × 3.5 × 2.6 cm. Additionally, there were multiple stones (Figures , , ). Urine culture was performed and revealed that various organisms were isolated (10-100,000 CFU/ml). Urinalysis showed a small amount of blood with a moderate presence of leukocytes and a trace protein.\nAt the end of 2020, the patient underwent magnetic resonance imaging (MRI). The MRI showed a horseshoe kidney with chronic hydronephrosis of the left kidney and a large mass within it centrally with further satellite lesions, which all likely represent UC and associated lymphadenopathy along the para-aortic chain (Figure ). Additionally, a finding of chronic pancreatitis was noted with dilated duct and stone, for which the patient was referred to the gastroenterology department. Furthermore, a bone scan and chest CT were performed, and no significant abnormality or metastasis was found.\nAfter a couple of days, the patient presented to the emergency department with non-radiating progressive lower abdominal and left colicky flank pain for three days with hematuria and constipation with fullness. The patient denied any history of fever or vomiting. There were no other genitourinary symptoms, scrotal pain, or change in the level of consciousness. Vital signs were measured and were as follows: blood pressure, 151/71 mmHg; heart rate, 109; respiratory rate, 20; and temperature, 37.1℃. The weight of the patient was 48.4 kg, and height was 166 cm. The chest was clear, while the abdomen was tender over the left side and the left flank area. Lab results were obtained (Table ). Urine culture was performed and showed more than 100,000 CFU/ml of Staphylococcus aureus being isolated. Abdomen and pelvis CT was performed to rule out any intra-abdominal collections, but it was negative. The urology team was consulted for his chief complaint, as his case is already known to them. On examination, they found that the abdomen was soft with lower left quadrant tenderness. They concluded that the pain was less likely to be from the left kidney as the CT findings were stable. Furthermore, based on the urine culture result, the patient was prescribed Bactrim and was given an appointment for admission a week later to perform a flexible ureteroscopy with biopsy.\nAfter one week, the patient underwent ureteroscopy with cytology and culture based on his MRI findings. Cystoscopy and retrograde pyelography showed normal bilateral ureters. However, the left kidney was severely dilated, and cloudy urine was noticed from the left ureteric orifice. Thus, culture and cytology were taken; then, a double-J (DJ) stent was inserted (Figure ). Urine culture was positive for S. aureus, and the patient was already on Bactrim. The urine cytology result was negative for high-grade UC, but benign urothelial cells, squamous cells, inflammatory cells, and red blood cells were present. Furthermore, since cancer could not be excluded, the patient was discharged and given an appointment for a left nephroureterectomy.\nAfter two weeks, the patient presented to the emergency room complaining of left flank pain for five days with hematuria and constipation. The urology team was again consulted and suspected urinary tract infection at the site of the double J stent. Thus, the patient was admitted, given antibiotics, and was planned for the open left radical nephroureterectomy the following day. The surgery was successfully performed, and samples were sent to the histopathology department.\nMacroscopic examination showed a fairly-circumscribed, bulging, gray-white mass located mainly in the renal pelvis and mostly replacing the entire kidney. It measured 14 cm in maximum dimension. The mass cut sections were homogenous with areas of necrosis, with a stent present (Figure ). The mass is away from margins and Gerota’s fascia by 2 cm. The remaining kidney parenchyma showed dilated cystic spaces containing multiple brownstones. Furthermore, the resected left renal hilum lymph node measured 7 cm in maximum dimension. Microscopically, the tumor showed urothelial carcinoma of the renal pelvis with dysplasia in the background. In addition, the tumor had a predominance of sarcomatoid differentiation. Furthermore, foci of squamous formation containing keratin formation, and other foci of glands with mucinous and goblet cells lining were seen. The tumor was focally extending to the perinephric fat. Margins were negative; however, the ureteric margin showed dysplasia. The left renal hilum lymph node was positive for metastatic carcinoma. The final diagnosis based on the examination was UC of the renal pelvis with sarcomatoid differentiation (70%) and unusual histological differentiation including squamous (25%), and glandular (<5%) (Figure ). In addition, there was no outside pathology confirmation done. Immunohistochemistry profile was positive for CK7, epithelial membrane antigen (EMA), vimentin, and focally for smooth muscle antigen (SMA). However, it was negative for desmin, cytokeratin (AE1/3), and GATA-3 (Figure ).\nAccording to the American Joint Committee on Cancer (AJCC), the pathological stage is pT3N2Mx. After a couple of days, the patient underwent a cystourethrogram, and there was no leak. The patient was eventually discharged in good health the next day and was given an appointment. The plan of the oncology team was to start adjuvant chemotherapy and perform positron emission tomography-CT (PET-CT) for the suspicious distant lymphadenopathy. In addition, CT, cystoscopy, and urine cytology were supposed to be done regularly. However, the patient missed the appointment, so the current status of the patient is unknown.","This man has a history of kidney problems, including a horseshoe kidney and previous surgery to remove stones. He recently started experiencing severe pain and blood in his urine, which led to further tests revealing a large tumor in his left kidney and signs of infection. After several tests and consultations, doctors determined that the tumor was urothelial carcinoma (a type of cancer) and performed surgery to remove the kidney and surrounding tissues. The surgery revealed more concerning findings, including cancer in nearby lymph nodes, and the patient is now undergoing chemotherapy and will have further scans to check for any spread of the cancer."
21,"A 47-year-old Caucasian male with a history of an aortic valve replacement, Factor V Leiden anomaly, migraines, and a competitive cycling hobby presented with new paracentral blind spots in the right eye following a fishing trip in Florida on August 28, 2014. The patient reported that the vision loss began during a fishing trip when he became dehydrated and had not resolved. He described three to four similar events that occurred previously following episodes of extreme physical activity, however, all resolving. On presentation in 2016, visual acuity was 20/20 in both eyes. No fundus abnormalities were noted. Amsler grid testing revealed two scotomas about 1 and 4 degrees superior nasal to fixation in the right eye. Spectral-domain OCT imaging also revealed several hyperreflective bands in the middle retina of the right eye (Figure ). In Figure , the hyporeflective lesions seen at the border of the fovea inferior temporal and slightly further out were consistent with his subjective superior nasal scotomas on Amsler grid testing. Spectral-domain OCT findings of PAMM were corroborated with the Chief of the Retinal Service at the New York Eye and Ear Infirmary. The patient was diagnosed with findings consistent with PAMM. At that time, no treatment was given. While diagnostic measures were not taken during this patient’s first few described episodes, it was thought that the previous episodes were also consistent with PAMM, given their similar presentation on the Amsler grid testing and symptomatology.\nIn mid-2018, the patient presented with a left-sided visual scotoma that had developed following a recent episode of febrile gastroenteritis. Treatment started with niacinamide OTC minerals, which helped initially over 30-40 minutes. After consulting with his cardiologist, nitroglycerin was prescribed but not taken. The patient was treated with 325 mg of aspirin and 200 mg of ibuprofen every 4 hours, with minimal relief. Aspirin and ibuprofen were administered because they are anti-inflammatory and blood thinners. The scotoma improved over the course of the next week but did not fully resolve. A permanent small scotoma remained.\nIn 2019, the patient began to experience similar symptoms after vigorous exercise. The patient was treated with 0.4 mg nitroglycerin sublingually previously prescribed by his cardiologist. Within 45 minutes, the visual scotoma improved, and within a few hours, it completely resolved. Since this occasion, the patient experienced two subsequent episodes of the development of visual scotomas, both resolving within minutes of the administration of sublingual nitroglycerin.\nThe patient described the lesions as permanent obstructions in the field of vision. They appear as small opaque structures, with a purple hue. The structures blend into the background without a distinct outline. The right eye lesions seen in Figure appeared at about 11 o’clock in the field of the patient's vision and did not move.","A 47-year-old man with a history of heart problems and migraines has been experiencing recurring blind spots in his vision. These blind spots started after a period of dehydration during a fishing trip and have happened several times before, always following intense physical activity. Tests have shown that he has a condition called PAMM, which causes small, permanent areas of vision loss. Fortunately, he has found that taking nitroglycerin helps to quickly clear up the visual disturbances, and he is being closely monitored."
22,"A 54-year-old Caucasian female, without significant past medical history, unvaccinated for COVID-19 presented with shortness of breath, cough, myalgias, nausea, vomiting, diarrhea, and fevers a week starting with headache. Upon initial evaluation in the emergency room, vital signs were as follows: blood pressure (BP) was 115/77 mmHg, heart rate (HR) was 103 beats per minute (bpm), temperature was 99.0°F, and oxygen saturation was 84% on room air. Lab work showed nasopharyngeal swab positive for SARS-CoV-2, elevated D-dimer (772 ng/mL), elevated international normalized ratio (INR) (1.3), hyperglycemia (117 mg/dL), hyponatremia (130 mmol/L), hypokalemia (3.3 mmol/L), hypochloremia (91 mmol/L), elevated liver enzymes (aspartate aminotransferase {AST}: 157 U/L, alanine aminotransferase {ALT}: 87 U/L), elevated N-terminal pro b-type natriuretic peptide (NT-proBNP) (508 pg/mL), and elevated troponin (13 ng/L). Chest x-ray showed bilateral infiltrates. CT chest with contrast showed bilateral pneumonia. The patient was admitted to the telemetry unit and started on ceftriaxone, azithromycin, dexamethasone, and remdesivir. Initial EKG on admission showed sinus tachycardia and left axis deviation with HR of 101 bpm (Figure ). After three days of remdesivir, EKG was repeated and showed sinus bradycardia with nonspecific intraventricular conduction delay, with HR of 57 bpm (Figure ). Third day after discontinuing remdesivir, the patient developed a transient arrhythmia noted on telemetry which resolved within a few seconds. This prompted nurse to get an EKG which showed normal sinus rhythm (Figure ). Potassium levels were low initially and after repletion potassium normalized on day two of hospital stay. Magnesium levels were normal throughout the hospital stay.","A 54-year-old woman with no prior health problems was admitted to the hospital after a week of feeling unwell with symptoms like fever, cough, and shortness of breath. Tests confirmed she had COVID-19 and developed pneumonia, along with other issues like low potassium and elevated heart enzymes. She received several medications to treat the infection and support her heart function, and her heart rhythm was monitored closely. After a few days, her heart rhythm returned to normal, and she was able to recover and be discharged home."
23,"A 54-year-old Hispanic female with a past medical history of type 2 diabetes mellitus, unvaccinated for COVID-19 presented with shortness of breath, cough, and pleuritic chest pain for four days. Upon initial evaluation in the emergency room, vital signs were as follows: BP was 118/63 mmHg, HR was 80 bpm, temperature was 103.1°F, and oxygen saturation was 91% on room air. Lab work showed nasopharyngeal swab positive for SARS-CoV-2, leukopenia (WBC: 3.8x103/uL), elevated D-dimer (514 ng/mL), hyperglycemia (126 mg/dL), elevated liver enzymes (AST: 224 U/L, ALT: 175 U/L), elevated c-reactive protein (CRP) (129.8 mg/L), and elevated respiratory procalcitonin (0.26 ng/mL). Chest x-ray showed patchy bilateral lung opacities. CT chest with contrast showed moderate bilateral pulmonary infiltrates. The patient was admitted to the telemetry unit and started on ceftriaxone, azithromycin, and dexamethasone. EKG on admission showed normal sinus rhythm with HR of 80 bpm (Figure ). The day following admission, the patient was started on remdesivir. After two doses of remdesivir, the patient developed severe sinus bradycardia with HR of 30-40 bpm, and remdesivir was discontinued (Figure ). She continued to have bradycardia with HR of 45-60 bpm persistently throughout the hospitalization. Potassium and magnesium levels stayed within normal limits for this patient throughout the hospital stay.","6 A 54-year-old woman with diabetes and no COVID-19 vaccination was admitted to the hospital after experiencing shortness of breath, cough, and chest pain. Tests confirmed she had COVID-19 and showed inflammation in her lungs, along with other signs of infection and high blood sugar. She received several medications to treat the infection and support her breathing, but developed a dangerously slow heart rate after receiving remdesivir. Despite this complication, she remained under close monitoring and received supportive care until she was discharged."
24,"A 59-year-old female, current smoker with 20 pack-years history, with a past medical history only significant for hypertension, gradually developed anorexia, nausea, fatigue, and weight loss. She initially presented to the emergency department with left flank pain and on CT scan of the abdomen was found to have diffuse osteosclerotic lesions in visualized bones. She was then followed up in primary care clinic where workup for an occult malignancy was initiated. There was no palpable mass or axillary adenopathy on breast examination. She had multiple mammograms in the past, some of which had shown suspicious architecture, which was followed up with multiple breast ultrasounds that had revealed benign findings. Mammogram was repeated and was reported benign with BI-RADS 2. Nuclear bone scan was unremarkable. CT chest revealed no pulmonary lesions but there were small mediastinal, submental, and axillary lymphadenopathy and several subcutaneous lesions on the back (one of which was excised and showed inclusion epidermal cyst). Multiple myeloma workup was negative.\nWhile the workup was ongoing, the patient started to experience lower back pain associated with weakness of lower extremities, numbness, tingling, and balance issues. She developed constipation as well as urinary incontinence. MRI of the brain and spine redemonstrated similar bony lesions in vertebrae, and also revealed abnormal leptomeningeal enhancement in the brainstem extending along the entire spinal cord (Figure ). Due to this finding, the patient was admitted to the hospital for further workup. Her mentation was normal. Deep tendon reflexes were absent in lower extremities, Babinski was positive bilaterally, and gait was ataxic. Strength was overall 5/5 in upper extremities and 4/5 in lower extremities. Sensations to touch, pain, temperature, and vibration were normal. Cranial nerve examination was normal, and cerebellar signs were absent. Her thyroid-stimulating hormone was normal. Lumbar puncture showed increased protein (1187 mg/dL) and white blood cells 43 cells/uL (lymphocytes 70%, monocytes 30%) in CSF. CSF cytology was negative for carcinoma and showed numerous lymphocytes but the negative finding could also have been due to delay in processing the specimen. Peripheral blood flow cytometry revealed monoclonal B cell lymphocytosis, non-CLL type. Infectious workups including human immunodeficiency virus, rapid plasmin reagent, Epstein-Barr virus, herpes simplex virus, cytomegalovirus, lyme, and cryptococcal antigen were negative.\nFinally, right iliac bone biopsy was done that revealed metastatic adenocarcinoma, GCDFP-15 and GATA-3 positive, indicative of breast carcinoma. Estrogen (ER) positive (90%), progesterone (PR) positive (40%), human epidermal growth factor receptor 2 (HER-2)/neu non-amplified IHC 1+, and Ki-67 10%. Tumor markers CA 15-3, CA 27.29, and carcinoembryonic antigen were raised at 85 U/mL, 84.2 U/mL, and 7.1 ng/mL, respectively. She was started with letrozole initially with dexamethasone taper, followed by palliative radiation therapy. The radiation was given to lumbosacral spine with 30 Gy in 10 fractions, which improved her lower back pain. After completion of the radiation therapy, abemaciclib was added to letrozole as the systemic therapy. She showed excellent response to the treatment and her previously seen leptomeningeal enhancements are no longer seen in the follow-up scans (Figure ). She does continue to have sclerotic changes in her bones; however, she deferred from bone-targeted therapy. The patient has been on abemaciclib and letrozole for about two years, her neurological symptoms have improved, and now she has a good quality of life. She is able to walk half a mile on flat ground and is able to perform light household chores.","This 59-year-old woman started experiencing unexplained symptoms like loss of appetite, fatigue, and pain in her side. After further testing, doctors discovered that she had cancer that had spread to her bones and brain. Despite extensive workup, including bone scans and biopsies, the cancer was identified as breast cancer that had spread. She responded well to treatment with medications and radiation, and her symptoms have improved significantly, allowing her to regain some mobility and enjoy a better quality of life."
25,"A 33-year-old female with no prior medical comorbidities, who recently gave birth to a healthy girl child four months ago, was brought to the emergency department with sudden onset weakness of both upper and lower limbs that started four days prior and rapidly progressed to a state of quadriplegia. She was conscious and obeyed simple commands with eyes and mouth; however, she had severe dysarthria. She had bilateral facial palsy and bulbar palsy. She had flaccid, hyporeflexic, pure motor quadriplegia with limbs showing only a subtle withdrawal flicker to pain. MRI of the brain revealed hyperintensity in the central pons in diffusion-weighted images (Figure ), T2-weighted images (Figure ), and fluid-attenuated inversion recovery (FLAIR) images (Figure ) without abnormal contrast enhancement (Figure ), consistent with central pontine myelinolysis (CPM) (Figure ).\nThe biochemical analysis showed hypernatremia while the remaining electrolytes were normal. The rest of the blood workup was unremarkable. Relatives denied an antecedent history of hyponatremia with rapid correction. The patient was started on sodium correction and was given five days intravenous (IV) pulse methylprednisolone 1 g/day to stabilize the blood-brain barrier. The patient recovered significantly to normal power. She was then considered to have idiopathic hypernatremic osmotic demyelination and was discharged with a modified Rankin Scale score (mRS) of 0.\nOne year later, she presented to the neurology department with a one-week history of generalized fatigue, diffuse myalgias, and three days history of rapidly progressive weakness of all four limbs making her wheelchair-bound one day before the presentation. Her initial vital signs were unremarkable. She was noted to have a pure motor flaccid symmetric quadriparesis with proximal more than distal weakness and generalized hyporeflexia. Clinical examination of other systems was normal. Nerve conduction studies (NCS) done on day three of onset of weakness demonstrated reduced compound muscle action potential (CMAP) amplitudes of bilateral tibial and peroneal nerves with absent F waves and H reflexes. CMAP of tested nerves in upper limbs showed preserved amplitudes with normal distal latency and absent F waves. There were no conduction blocks. The sensory conduction study of all the tested nerves in all four limbs was normal. Cerebrospinal fluid (CSF) analysis did not show albumin-cytological dissociation. Therefore, acute motor axonal neuropathy (AMAN) variant of Guillain-Barré syndrome (GBS) or hypokalemia-related electrophysiological abnormalities were considered. A basic metabolic panel revealed severe hypokalemia (potassium 2.2 mEq/L). Arterial blood gas (ABG) and 24-hour urine analysis showed metabolic acidosis, consistent with renal tubular acidosis type-1 (distal). Autoimmune workup was positive anti-SSA/Ro autoantibodies. The biopsy of the minor salivary gland was pathognomonic. The patient was diagnosed with pSS according to the new classification criteria proposed by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Overall clinical, electrical, and biochemical data suggest the presence of renal tubular acidosis with secondary hypokalemia-related quadriparesis due to pSS.\nThe patient was given intravenous (IV) potassium supplementation through a peripheral vein at a rate not exceeding 10 mEq/hour and subsequently was shifted to oral liquid formulation in the form of a syrup. Oral sodium bicarbonate supplementation was given at a dose of 1 mEq/kg/day for renal tubular acidosis. With potassium correction, there was a rapid recovery in the power of all four limbs within 24 hours of admission. The patient was initiated on 1 mg/kg/day of oral prednisolone and was referred to a rheumatologist for further management. She remained asymptomatic on her six-month follow-up.","This 33-year-old woman experienced a sudden and rapid loss of movement in her arms and legs, leading to quadriplegia, after recently giving birth. Tests revealed she had a condition called central pontine myelinolysis, which damaged the part of her brain controlling movement. She was treated with medication and sodium correction, and her condition improved significantly. One year later, she returned with new symptoms of fatigue and weakness, and tests showed she had a rare autoimmune disease called primary Sjögren’s syndrome, which caused kidney problems and muscle weakness."
26,"A 67-year-old female with a past medical history of chronic obstructive pulmonary disease and history of long-term tobacco abuse, who recently quit smoking, presented with shortness of breath, cough, myalgias, and malaise for one week. The patient had received two doses of Pfizer COVID vaccine, with the second dose in February 2021. In the ER, her vital signs were blood pressure (BP) 120/71, heart rate (HR) 78 bpm, respiratory rate (RR) 20 breaths/min, oxygen saturation 85% on room air, and afebrile. Laboratory assessment on admission is in Table . Nasopharyngeal swab for SARS-CoV-2 was positive. Chest X-ray on admission shows mildly patchy bibasilar pulmonary infiltrates and a calcified pulmonary nodule in the mid-right lung (2.0 cm) (Figure ). The patient was admitted to the general medical ward and started on 6 L per minute of supplemental oxygen via nasal cannula, remdesivir, dexamethasone, furosemide, azithromycin, and enoxaparin for venous thromboembolism prophylaxis. Despite multiple measures, the patient did not improve, requiring more aggressive management. Repeat chest X-ray showed slight interval improvement of bilateral pulmonary infiltrates and needed 4-5 L per minute via nasal cannula (Figure ).","67-year-old woman with COPD and a history of smoking came to the hospital with shortness of breath, cough, and muscle aches after testing positive for COVID-Her breathing was initially low, requiring supplemental oxygen, and a chest X-ray showed some inflammation in her lungs and a small nodule. She received several medications to treat the COVID-19 and improve her breathing, but her condition didn't improve significantly. Further imaging showed slight improvement, but she continued to need oxygen support."
27,"An 84-year-old female with a past medical history of hypertension presented with weakness, dry cough, and shortness of breath for four days. The patient had received two doses of the COVID vaccine, with the second dose in March 2021. In the ER, her vital signs were BP 133/93, HR 103 bpm, RR 22 breaths/min, oxygen saturation of 96% on 40 L per minute of supplemental oxygen via high-flow nasal cannula, and afebrile. Laboratory assessment is in Table . Nasopharyngeal swab for SARS-CoV-2 RNA was positive. Chest X-ray on admission shows worsening right pleural effusion with new opacity obscuring the lower two-third of the right lung and a new pleural-based opacity in the left upper lobe (Figure ). CT chest with contrast shows large right pleural effusion and associated right basilar consolidation and abdominal ascites. The patient was admitted to the telemetry unit and started on methylprednisolone, piperacillin-tazobactam, remdesivir, and baricitinib. The patient clinically deteriorated on Day 2 and was transferred to the intensive care unit for thoracentesis and possible intubation. Thoracentesis removed 1.95 L of bloody, serosanguineous fluid obtained, with partial resolution of the effusion (Figure ). On Day 3, the patient developed septic shock, florid renal failure, and lethargy and was started on intravenous fluids and norepinephrine drip. Chest X-ray showed near-complete opacification of bilateral lung fields and subsequently was intubated. On Day 4, tense ascites were noted and the patient underwent paracentesis, which removed 4.25 L of bloody, serosanguinous fluid. Renal replacement therapy started. The patient was deemed to have a guarded prognosis with multiorgan failure.","An 84-year-old woman with high blood pressure was admitted to the hospital after four days of feeling weak, having a cough, and struggling to breathe. She tested positive for COVID-19, and tests revealed fluid buildup in her lungs and abdomen, along with signs of infection. She was treated with medications and underwent procedures to drain the fluid, but her condition worsened, leading to septic shock and kidney failure. Sadly, her prognosis is guarded, and she was intubated and started on dialysis."
28,"A 23-year-old Caucasian male with a history of exercise-induced asthma presented to the emergency department complaining of left-sided chest pain which started two days after receiving the second dose of the mRNA-1273 Moderna vaccine. The patient described the pain as sharp, intermittent with radiation to the left upper back and left arm with 10/10 severity and worsening with deep inspiration. Fever and chills were also present. The patient did not report any recent history of tick bites, upper respiratory symptoms, paroxysmal nocturnal dyspnea (PND), orthopnea, arthralgias or rashes.\nOn physical examination the patient was in no distress, with normal vital signs, normal S1/S2 heart sounds without any murmurs, rubs, or gallops and no jugular vein distention (JVD). There was no palpable tenderness of the chest wall. The lungs were clear to auscultation. There was no pitting edema in the lower extremities.\nDiagnostic testing revealed elevated troponin T of 475ng/L (<22ng/L) which trended upward reaching a peak of 910ng/L (<22ng/L). Initial electrocardiogram (ECG) showed right axis deviation with left posterior fascicular block without any ST elevations as well as premature atrial contractions (PACs) in trigeminy (Figure ). A bedside ultrasound showed trace pericardial effusion. CT angiography (CTA) of the chest was negative for pulmonary embolism (PE). Lyme serology, antinuclear antibodies (ANA) and respiratory viral panel were negative and thyroid stimulating hormone (TSH) was normal. Pertinent leukocytosis of 11.09 K/ul (3.8-10.5 K/Ul) with absolute neutrophil count of 8.09 K/uL, elevated erythrocyte sedimentation rate (ESR) of 37mm/hr (0-15mm/hr), c-reactive protein (CRP) of 11.6mg/L (<4.0mg/L). Urine toxicology was positive for recreational marijuana drug use but negative for cocaine use.\nTransthoracic echo (TTE) revealed abnormal motion and increased thickening of the septal wall with preserved ejection fraction (EF) of 65% and normal diastolic function (Video ). Trace pericardial effusion was also noted. Based on the patient’s clinical presentation, ECG, cardiac markers and TTE findings a presumptive diagnosis of peri-myocarditis was made.\nWe did not pursue cardiac MRI since the patient had clinically improved within 48 hrs. He received aspirin 325 mg once followed by indomethacin 50mg twice a day and discharged on day three to complete a total of two weeks indomethacin and three months of colchicine 0.6mg daily. Complete resolution of his symptoms, normalization of troponins and ECG, was demonstrated within two weeks during follow up. At his 60-day follow-up visit, TTE confirmed resolution of the wall motion abnormality and pericardial effusion and he remains completely symptom free at 128 days.","A 23-year-old man with a history of asthma went to the hospital because of sudden, severe chest pain after getting his second COVID vaccine dose. Tests showed high levels of a protein called troponin, indicating possible inflammation around the heart, and an ultrasound revealed a small amount of fluid around the heart. Although other tests ruled out blood clots and infections, the patient’s symptoms improved with medication, and follow-up tests confirmed his heart function had returned to normal. He was discharged with a plan to continue medication for several months and is now completely symptom-free."
29,"A 35-year-old male presented with a foreign body in his left ear caused by a trauma to the left parietal area by a fishhook. The patient was in a boat on a fishing trip when the fishhook accidentally pierced his upper neck behind the left auricle and pierced the auricle of the left ear. On examination, the patient was conscious and oriented and no bleeding, swelling, hematoma or bruises were noticed. Vital measurements and systemic review revealed normal findings. The patient received an intramuscular injection of 0.5 mL tetanus toxoid adsorbed vaccine and was referred for surgical assessment and foreign body removal. Under local anesthesia, the triple needle fishhook was removed and cut by a bone nipper from left pinna and post-auricular area (Figure ). The lacerated wound was stitched by 05 Ethilon suture, left mastoid dressing was applied and the patient was discharged after prescribing per-oral cefuroxime and diclofenac for five days.\nTwo weeks later, the patient returned to the hospital for follow-up. On examination, left pinna and post-auricular area were normal. However, a small, non-tender, firm 2 x 2 mm subcutaneous swelling was noticed below the ear lobule. Amoxicillin/clavulanate and diclofenac sodium were prescribed, and the patient was discharged.\nIn the follow-up visit two months after the injury, the patient was assessed for a localized small, non-tender, 2 x 2 mm parotid swelling at the angle of mandible on the left side. The swelling appeared after the removal of the foreign body two months ago and did not get resolved. Ultrasound (US) of the neck showed a linear hypoechoic focus in the superficial parotid gland extending to the subcutaneous tissue (Figure ). The presence of scar or granulation tissue was suspected and no focal mass lesions were detected. In addition, a few oval-shaped, non-specific cervical lymph nodes were noted with preserved fatty hilum and a short axis diameter of 0.8 cm. Magnetic resonance imaging (MRI) confirmed the presence of a post-traumatic scar and granulation tissue involving the superficial part of the left parotid gland and extending to the subcutaneous soft tissue (Figure ). The presence of bilateral non-specific lymph nodes was noted in both cervical regions.","A 35-year-old man was injured when a fishhook pierced his ear. He sought medical attention after the injury, and the hook was successfully removed with stitches. However, two weeks later, he returned with a small swelling under his ear, which was treated with antibiotics. Finally, after two months, a larger swelling developed in his parotid gland, which was confirmed by ultrasound to be a scar and tissue growth from the original injury. The doctors prescribed more antibiotics and monitored the area closely."
30,"An 18-year-old male patient presented to the emergency department with right hip pain for two weeks. Examination revealed pain and mild to moderate tenderness in the right hip joint. The range of motion was decreased, and trying to initiate movement caused severe pain to the extent that the patient could not walk for gait assessment. Past history revealed similar but less severe episodes for the last four years. There was no history of fever, skin rash, or acne. His inflammatory laboratory investigations, including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), were within the normal limits. X-rays of the pelvis and right thigh were unremarkable except for a small lucency in the right greater trochanteric region (Figure ).\nSubsequently, an MRI of the right thigh showed trochanteric bursa effusion and right hip joint synovitis (Figures , ).\nNon-steroidal anti-inflammatory drugs (NSAIDs) and paracetamol were started for the management, and the patient showed excellent improvement for the first three months.\nHe remained well for four months, after which he developed swelling of the right sternoclavicular joint. X-ray of the joint and laboratory investigations were found to be normal (Figure ).\nDue to the previous history of the right hip joint, the lesion was investigated further with MRI (Figures , , ).\nA patchy area of hyperintensity on T2 and hypointensity on T1 images close to the sternoclavicular junction was observed. On post-contrast images, there was a mild accentuated heterogeneous enhancement. Traces of fluid was also noted in the joint space, and marrow edema was seen along the articular margin and body of the sternum. MRI also showed subcutaneous edema and changes related to cellulitis in the overlying soft tissues. Clinical history and imaging data were suggestive of SAPHO syndrome. The patient was again started on paracetamol and NSAIDs based on the excellent previous response. At the one-month follow-up, the swelling was found to have improved. The patient was continued on the therapy and his symptoms resolved within three months.","This 18-year-old man came to the hospital because of pain in his right hip that had been bothering him for two weeks. Tests, including X-rays and an MRI, showed inflammation in the hip joint and a fluid buildup around the joint. The doctors believe he has SAPHO syndrome, a condition that can cause recurring joint problems. With medication and a follow-up visit, the swelling in his hip joint improved, and he was able to return to normal activities."
31,"A 35-year-old male patient presented with a six-month history of pain in the anterior chest and neck. His pain initially had been mild but had become severe in the last three weeks. He was afebrile and did not have any constitutional symptoms. On examination, marked tenderness of the right sternoclavicular joint was noted. Initial laboratory investigations, tuberculosis workup, and chest X-rays were unremarkable. However, further study with MRI revealed subchondral bone marrow edema and enhancement involving the medial end of the right clavicle. In addition, mild effusion of the right sternoclavicular joint and surrounding soft-tissue edema was also seen (Figure , ).\nGiven the patient's radiological and clinical findings, suspicion of SAPHO syndrome was raised. He was given an initial trial of paracetamol and NSAIDs and he showed remarkable improvement on his monthly follow-up visits. Though a biopsy was offered, the patient refused to undergo one and has shown no relapse to date thanks to the dramatic response to NSAIDs.","A 35-year-old man has been experiencing increasing pain in his chest and neck for six months. After initial tests showed nothing unusual, an MRI revealed changes in the bone around his collarbone, suggesting a possible condition called SAPHO syndrome. He responded very well to medication, and his pain has significantly improved. He is now doing well and has not experienced a return of his symptoms."
32,"A previously healthy 49-year-old female with a past medical history of well-controlled hypertension and body mass index (BMI) of 30.37 kg/m2 presented to the emergency department with altered mental status, abdominal pain, hematemesis, and hypotension. According to family, the patient complained of abdominal pain earlier that morning and was later found at home minimally responsive and recurrently vomiting blood.\nIn the emergency department, the patient’s vitals included a blood pressure of 94/50 mmHg, a temperature of 87.1 Fahrenheit, and a respiratory rate of 34 breaths per minute. The patient was intubated for airway protection. She received 5L of fluid as well as one unit of packed red blood cells for suspected large fluid volume loss. Esophagogastroduodenoscopy was performed and was remarkable for a Mallory-Weiss tear with portohypertensive gastropathy. This was thought to be caused by the repeated vomiting reported by her family. CT scan showed peripancreatic edema and fat stranding, consistent with acute pancreatitis (Figure ). A repeat CT scan was done to evaluate the progression of her pancreatitis, which showed worsening pancreatitis with developing ascites. Labs were remarkable for a glucose up to 955 mg/dL, hemoglobin A1c (HgbA1c) of 13.7%, and a triglyceride level up to 1608 mg/dL (Table ). The patient was then placed on an insulin drip for her significantly elevated blood glucose. After her glucose normalized, she was continued on an insulin drip until her triglycerides dropped below 500 mg/dL. The patient was then downgraded to the general medical floor and discharged after being able to tolerate a regular diet without significant pain or discomfort.","This 49-year-old woman was rushed to the hospital after she became confused and started vomiting blood. She had low blood pressure and was found unresponsive at home. Tests revealed a tear in her esophagus, which caused bleeding, and signs of inflammation in her pancreas. Her blood sugar was very high, so she received insulin to bring it down, and she was eventually discharged home after her condition improved."
33,"A 53-month-old Sudanese female presented with progressive bilateral breast enlargement and accelerated growth since the age of 9 months. Her family had sought medical advice several times in different primary health care facilities and were reassured. She had no vaginal bleeding and no pubic or axillary hair.\nExamination showed a well-looking girl, vitally stable with normal blood pressure. Her weight was 17 kg (50th centile) and height 108 cm (90th centile) using the Centers for Disease Control and Prevention growth chart. Mid-parental height was 175 cm and predicted adult height was 167 cm using the JM Tanner formula. No previous documented follow-up growth data were available. Her Tanner staging was A1, P1, and B3. She had reddish mucoid vagina. She had no clitoromegaly, acne, hirsutism, or palpable abdominal mass (Table ).\nLeft wrist X-ray revealed a bone age of 8 years.\nThe hormonal evaluation using fluorometric enzyme immunoassay showed basal luteinizing hormone of 3.1 mIU/L, which increased to 8.8 mIU/L 45 minutes post-gonadotrophin-releasing hormone stimulation. Elevated levels of estradiol E2 29,000 pg/ml (5–15 pg/ml), and dehydroepiandrosterone sulfate 90 ng/mL (2.3 ng/mL), with normal early morning cortisol level 16 ng/mL (7–28 ng/mL). Due to financial difficulties, we did not measured the follicular-stimulating hormone level.\nAbdominal ultrasound revealed a right-sided hypoechoic suprarenal mass, an ovarian volume of 1.8 cm3, uterine volume of 3 cm3, and endometrial thickness of 1.2 cm. The abdominal CT scan showed a 25 × 22 mm well-defined rounded focal lesion with a smooth outline, at the level of the right adrenal gland with homogeneous attenuation, HU-7 on a noncontrast scan, and no evidence of local tissue invasion (Fig. ). The left adrenal gland and ovaries were normal.\nBrain magnetic resonance imaging was done to exclude a central cause of precocious puberty and was normal. Complete hemogram, liver, and renal functions were normal.\nA diagnosis of an estrogen-secreting right adrenocortical tumor was made, and we referred the patient to surgery.\nDuring laparotomy a 3 cm diameter tumor in the right adrenal gland was completely excised. The histopathological examination showed well-circumscribed tumor forming nests with trabeculae and sheets of polygonal cells with eosinophilic cytoplasm. The tumor was not infiltrating the capsule, with no mitosis, atypia, or necrosis (Wieneke index score = 0). Findings suggestive of benign adrenal cortical adenoma.\nThe postoperative evaluation revealed normal cortisol and dehydroepiandrosterone sulfate. Estradiol E2 40 pg/mL was consistent with secondary central precocious puberty, so the patient was started on monthly gonadotrophin-releasing hormone agonist, with a regular follow-up plan for the possibility of recurrence of adrenal adenoma.\nSix months after the operation, the patient was well and compliant to monthly gonadotrophin agonist injections. She showed partial regression of her secondary sexual characters and a growth velocity of 5 cm/year. The abdominal CT scan was normal with no evidence of recurrence.","This 53-month-old girl has been growing and developing faster than expected, with her breasts getting bigger. After seeing several doctors, we found that her body is producing too much estrogen, which is causing this early development. Tests showed a small tumor on her right adrenal gland is the cause of this excess estrogen. Surgery to remove the tumor was successful, and now she is receiving medication to slow down her growth and prevent the tumor from returning."
34,"A 66-year-old male with back pain and cough for two weeks was admitted to the First Hospital of Jiaxing on 8 August 2019. A thoracic computed tomography (CT) scan revealed that the malignant tumor on the left upper lobe was complicated by distal obstructive inflammation, the enlargement of the left hilar and mediastinal lymph nodes, and the multiple bone metastases on 25 August 2019 (Fig. A). Immunohistochemical (IHC) results of the posterior iliac bone marrow biopsy specimen showed the positive expression of CD3, CD20, CD34, CD235a, and NPO, and the negative CD61 expression. IHC results of an endoscopic biopsy specimen of the bronchial mucosa on the upper left lobe showed the positive expression of TTF1, CK7, NapsinA, Ki67, CK, and EMA, and the negative expression of CK5/6, P40, CgA, Syn, and CD45 on 6 September 2019. Histopathologic observations showed infiltration of atypia cells in mucosal and fibrous tissues. The detection tools of pathology and cytology included automatic IHC staining (BenchMark XT, Roche, The United States), digital slice scanner, image analysis software (Pannoramic 250, 3DHistech, Hungary), and microscope (Eclipse Ci-S, Nikon, Japan). Finally, the patient was diagnosed with stage IVb lung adenocarcinoma combing with bone metastases.\nTo seek potential therapeutic opportunities, the FFPE tissue and control sample (white blood cell) of the patient were detected using a 733-gene NGS panel in a College of American Pathologists (CAP) and Clinical Laboratory Improvement Amendments (CLIA) certificated lab. Sequencing reads were mapped against the hg19/GRCh37 genome, and duplicate reads were removed, followed by variants calling in targeted regions using an in-house developed bioinformatics algorithm. The algorithm utilized a filtering model containing background error correction, strand bias, base quality, mapping quality, short tandem repeat regions, and low-quality mapping ratio 25 []. The NGS analysis results indicated that the patient had an EGFR exon 19 p.L747_S752del somatic mutation with an allelic fraction of 73.19% and TP53 p.H179L germline mutation. Besides, the whole chromosome 7 and 5q deletion were detected using NGS-based copy number variation (CNV) analyses (Fig. ), which were classical abnormalities associated with MDS. According to the clinical inquiry, the patient was a farmer by occupation, which means that he has long been exposed to certain chemicals such as pesticides, fertilizers, and solvents containing benzene. In addition, he smoked for 40 years. Taking all these findings together, we suspected the patient with secondary MDS. The results of the physiological blood indexes and the bone marrow aspiration test showed a decrease of platelet counts and the elevation of myelocyte counts. The proportion of blast was 5%, and the morphology of the cells presented the characteristics of MDS, which further confirmed our speculation (Fig. and Additional file : Fig. S1).\nIn terms of treatment, the EGFR-positive mutation patient was administered with gefitinib starting from 20 August 2019. Due to decreasing platelet counts, azacitidine was administrated for four courses of treatment. However, the thrombocytopenia had not been significantly improved, and the size of the maximal tumor in the lung lesion did not decrease after seven months’ treatment (Fig. B). On 10 April 2020, a pathological report showed the infiltration of poorly differentiated cancer cells into the fibrous tissue. IHC results suggested the neuroendocrine tumor, in which small cell carcinoma and atypical carcinoid tumor accounted for about 40% and 60%, respectively. On 7 April and 28 April 2020, considering positive PD-L1 expression and high tumor mutation burden (TMB) in the tissue sample, the patient was administrated with toripalimab (a PD-1 inhibitor) and bevacizumab. Platelet counts returned to near-normal levels gradually, and the tumor lesion shrank obviously (Fig. ). It was concluded that the patient reached a partial response (PR) (Fig. C). The progression-free survival was four months.","A 66-year-old man was admitted to the hospital after two weeks of experiencing back pain and a cough. Tests revealed he had lung cancer that had spread to his bones, and the cancer cells were identified through genetic testing. The tests also showed a genetic mutation that makes the cancer more responsive to a specific drug called gefitinib. After trying different treatments, the patient responded well to a combination of drugs, including an immunotherapy medication, and showed signs of shrinking tumor growth."
35,"A 22-year-old male presented in the emergency department with acute onset of swelling and redness over the right side of the neck and chest wall for the last three days. He had features of septicemia such as drowsiness or Glasgow Coma Scale score of 11/15, respiratory rate of 26 breaths per minute, pulse rate of 130 beats per minute, blood pressure of 84/56 mmHg, and urine output of 15 mL/hour. He had no history of chronic disease, drug reaction, trauma, unknown bite, or significant familial disease. Blood investigations revealed low hemoglobin of 7.6 g/dL, raise leukocyte count of 28000/mm3, low albumin of 2.2 g/dL, raised serum creatinine of 2.23 mg/dL, serum urea of 174 mg/dL, and low sodium of 125 mEq/L. Serological markers including erythrocyte sedimentation rate (95 mm/hour) and procalcitonin (25.2 ng/mL) were higher. X-ray of the chest was grossly normal (Figure ), and contrast-enhanced computed tomography (CECT) of the neck revealed irregular, well-defined, hypodense, non-enhancing area in the right parotid gland with extension into the neck spaces, larynx, and subcutaneous planes (Figure ). CECT of the chest revealed mild effusion in bilateral pleural space secondary to acute infection and no evidence of lymphadenopathy or osteomyelitis (Figures , ).\nZiehl-Neelsen (ZN) staining from pleural fluid was negative for acid-fast bacillus (AFB bacilli). The patient was managed in the intensive care unit with ventilator support due to acute respiratory distress syndrome. He was diagnosed with acute progressive necrotizing fasciitis with multiple organ dysfunction syndromes due to an unknown cause of septicemia. He underwent multiple aggressive debridements of the neck and chest wall (Figure ).\nThe cartridge-based nucleic acid amplification test (CBNAAT) and ZN staining revealed positive mycobacteria and AFB bacilli for pathological tissue. Therefore, the pus collected in the parotid region and neck space was likely secondary to tubercular infection. A split-thickness graft was applied in multiple fractions after three weeks of anti-tubercular chemotherapy. The patient had a long hospital stay due to the difficult location and complexity of the ulcer over the neck. He was followed up for six months in good clinical condition after discharge.","This 22-year-old man came to the hospital with a serious infection that started as swelling and redness on his neck and chest. He was very sick, with a fast heart rate, low blood pressure, and difficulty breathing. Tests showed he had a severe infection in his neck and chest, and a CT scan revealed a large area of tissue damage. After aggressive treatment and surgery, the infection was identified as a rare type of tuberculosis, and he has been recovering well."
36,"A 41-year-old Japanese woman presented to our clinic with a 4 month history of bilateral groin pain and right buttock pain. Her right hip was more painful than her left hip. There was no history of trauma, alcohol abuse, or steroid use. Her medical history included iron-deficiency anemia diagnosed 2 years earlier, after which she had been on iron supplements. She had no fracture episodes, including fragility fractures.\nHer height, body weight, and body mass index were 155 cm, 42 kg, and 18.7 kg/m2, respectively. She was able to walk for approximately 10 minutes without a stick, albeit at a slow speed. Limitations in the passive motion of her bilateral hip joint were observed thus: flexion, 100°, internal rotation 5°, external rotation 15°, and abduction 20°, on both sides. She was able to perform a straight-leg raise of the right limb with substantial pain. The neurovascular status of both lower extremities was intact. The Japanese Orthopaedic Association scoring system for the evaluation of hip-joint function (JOA hip score) was 46 points for her right hip and 56 points for her left hip. The score was based on a total of 100 points, comprising 40 for pain, 20 for range of motion, 20 for the ability to walk, and 20 for activities of daily living [].\nStandard radiographs of both hips (Fig. a–c) demonstrated no characteristic findings such as the crescent sign, sclerotic band pattern, and collapse of the femoral head, and no joint space narrowing was seen in either femoral head. MRI of both hips (Fig. d, e) presented a low signal line in the subchondral region of the femoral head in the T1 weighted image and high signal region in almost all of the femoral head in the short tau inversion recovery (STIR). The oblique axial views of the proton density-weighted image showed a low-signal sinuous line in the anteromedial region of the femoral head (Fig. ). Dual-energy X-ray absorptiometry (DEXA) values were low in both femoral necks. Bone mineral density was 0.909 g/cm2 (T-score –0.9, Z-score −0.8) in the lumbar spine, 0.594 g/cm2 (T-score −1.8, Z-score −1.4) in the right femoral neck, and 0.529 g/cm2 (T-score −2.4, Z-score −2.0) in the left femoral neck. Laboratory findings were as follows: C-reactive protein (CRP) 0.03 mg/dl (normal range: 0–0.5 mg/dl); alkaline phosphatase 608 IU/l (40–150 IU/l); calcium 9.0 mg/dl (8.4–10.2 mg/dl); albumin 4.4 g/d (3.9–4.9 g/d); and hemoglobin 9.7 g/dl (12–16 g/dl). Bone turnover markers were as follows: tartrate-resistant acid phosphatase 5b (TRACP-5b) 463 mU/dl (premenopausal normal range: 120–420 mU/dl) and total procollagen type 1 N-terminal propeptide (Total P1NP) 72.7 ng/ml (26.4–98.2 ng/ml). We diagnosed bilateral osteonecrosis of the femoral head and classified it as stage 1 in both femoral heads, according to the Association Research Circulation Osseous (ARCO) classification [].\nThe patient was instructed not to take as much weight as possible using sticks on the right side. Her symptoms improved immediately. However, 6 months later, her symptoms increased slightly without any traumatic episodes. Radiographic findings indicated almost no change (Fig. a–c). However, a slight signal change in the medial subcapital region was observed in the MRI scan (Fig. d, e). Her pain gradually increased thereafter, and 10 months after her first visit, walking became difficult. The JOA hip score decreased to 34 points in both hips; still, no changes could be observed in the radiograph (Fig. a–c). MRI of both hips showed a nondisplaced subcapital fracture on the medial side of both femoral necks, with bone marrow edema around the fracture (Fig. d, e). Because the cause of the fractures was not identified, the DEXA and bone turnover markers were measured again, and 25-hydroxy (OH) vitamin D was measured for the first time. All DEXA values decreased. The bone mineral density was 0.849 g/cm2 (T-score −1.5, Z-score −1.2) in the lumbar spine, 0.527 g/ cm2 (T-score −2.4, Z-score −2.1) in the right femoral neck, and 0.490 g/cm2 (T-score −2.7, Z-score −2.4) in the left femoral neck. TRACP-5b level increased to 607 mU/dl, and total P1NP decreased to 52.7 ng/ml. Her 25(OH) vitamin D level was 11.1 ng/dL and she was diagnosed with vitamin D deficiency. Based on the above results, our diagnosis was bilateral stress fracture of the femoral neck secondary to osteonecrosis of the femoral head. The patient underwent internal fixation of both hips with sliding hip screws (Dual SC screw system; Kisco, Kobe, Japan) to stabilize the stress fractures. In addition, the reaming performed before inserting of the sliding hip screw served as core decompression for the femoral heads [, ]. The specimens obtained from the reaming were examined histologically. Definitive findings of osteonecrosis such as bone marrow necrosis and loss of osteocyte nuclei in the femoral heads were observed (Fig. ). Postoperative radiographs showed no evidence of displacement of the fractures (Fig. ). We administered eldecalcitol 0.75 μg per day orally for vitamin D deficiency, and daily subcutaneous injections of teriparatide acetate. In the immediate postoperative period, the patient began to bear weight as tolerated with the use of an assistive device bilaterally. One month postoperatively, she was able to walk without pain and used a cane part time. She eventually regained full walking ability without a cane 3 months after surgery. Furthermore, her JOA hip score improved to 90 points in both hip joints at 5 months after surgery. Radiographs showed no evidence of recurrent stress fracture in the femoral neck or progression of osteonecrosis (Fig. ). Sequential oblique axial MRI showed that the necrotic region of the femoral head had decreased 5 months after surgery (Fig. ).","This 41-year-old woman has been experiencing pain in her hips and buttock for several months. After a series of tests, including X-rays and MRIs, doctors discovered that she had a condition called osteonecrosis of the femoral head, which means the bone in her hips was breaking down. Further investigation revealed that she was deficient in vitamin D, which contributed to the problem. To stabilize the fractures and prevent further damage, she underwent surgery to insert screws into her hips. Following surgery, she gradually regained her ability to walk and her hip pain decreased significantly."
37,"A 14-year-old male adolescent presented to the emergency department with subfebrile temperatures for 1 week and localized pain in his right popliteal fossa for 3 days. Prior to the onset of these symptoms, he had been immobilized for several days following a minor sports injury.\nThe adolescent had no permanent medication and no prior medical history except for an asymptomatic ATD diagnosed at the age of six by functional antithrombin assay (antithrombin activity of 57%, age adapted reference: 77–125%). Since at that time, there were no clinical signs of thrombosis, the diagnosis of ATD did not lead to any therapeutic consequences. Screening for ATD at this early age had been carried out on parental request, as his mother was diagnosed with ATD in her early adulthood. Interestingly, his mother now reported that she had very recently been diagnosed with IVCA (preexisting chromogenic test results showed an antithrombin activity of 50% for the mother, the age adjusted reference range being 80 to 130%).\nThe coexistence of other hereditary thrombophilic disorders in our patient and his mother (protein S deficiency, protein C deficiency, factor V Leiden mutation, prothrombin-mutation, antiphospholipid syndrome) was ruled out by respective laboratory analyses.\nLaboratory blood analysis in the emergency department showed markedly elevated D-dimers of 25 mg/l FEU (reference: < 0.5 mg/l FEU) and of C-reactive protein (CrP) of 184 mg/l (reference < 5 mg/l). Antithrombin activity on admission was reduced to 61% (age adjusted reference 83–118%).\nA vascular ultrasound examination upon admission confirmed the clinically suspected thrombosis of the right lower extremity involving the external iliac, common and superficial femoral as well as the popliteal vein. The ultrasound examination of the left lower extremity veins did not give evidence of thromboses upon admission. The patient was treated with continuous infusion of unfractionated heparin at a therapeutical dose including several bolus administrations and antithrombin (4000 IE within the first 36 h) was substituted reaching levels in the target range between 80 and 100%. Subsequent monitoring of the activated partial thromboplastin time (aPTT) showed an insufficient response to heparin treatment (aPTT was 29 s before the administration of heparin and did not exceed a maximum value of 33.6 s during heparin treatment). The anticoagulative therapy was therefore switched to direct thrombin inhibition by argatroban at a dose of 1.5–2.0 μg/kg/min, which led to target aPTT values of 50–60s.\nDue to the elevated CrP levels and subfebrile temperatures, empirical antibiotic treatment with intravenous cefuroxim was initiated.\nA magnetic resonance imaging (MRI) scan on day three confirmed a complete DVT of the right lower extremity, and now additionally extended thromboses of the left lower extremity and thrombosis of both iliac vessels. Furthermore, it revealed an occlusion of the inferior vena cava (IVC) with prominent venous lumbar collaterals, suggestive of IVCA. Surrounding the lumbar vertebrae, several of these collaterals appeared to be fully thrombotic, which was confirmed by angiography (Fig. A).\nThe following day, a catheter-directed local thrombolysis using recombinant tissue plasminogen activator (rt-PA) at a daily dose of 0.25 mg/kg was initiated in both extremities and carried out for a total of 48 h. For the right leg, an additional ultrasound-accelerated thrombolysis using the EKOS® system was performed.\nDuring hospitalization, the anticoagulative therapy was switched from argatroban to the vitamin K antagonist phenprocoumon with a target International Normalized Ratio (INR) of 2.0–2.5. Oral anticoagulation will most likely be continued lifelong due to the combination of extended multilocal thromboses with underlying ATD, as well as IVCA, which in itself is a risk factor for thrombosis.\nThe patient was discharged on day eight after hospital admission. An ultrasound examination upon discharge showed full recanalization of the femoral and iliac veins of both legs.\nDuring a routine follow-up 1 week later, the patient presented free of any clinical complaints. However, despite continued oral anticoagulation since hospital discharge, vascular ultrasound now revealed extended re-thrombosis of both the right and left pelvic axis and the right lower extremity, thus leading to a second hospital admission and re-induction of therapy with both intravenous antithrombin and catheter-directed thrombolysis.\nRecanalization of the right lower extremity and pelvic axis was subsequently achieved, whereby IVC occlusion was confirmed (Fig. B). However, the left pelvic vessels remained thrombotic even after 5 days of catheter-directed thrombolysis.\nSurprisingly, one of the angiographies allowed for location of the occluded area of the IVC by rendezvous-technique between the superior and inferior vena cava, which prior to this had been thought to be atretic (Fig. C). The vessel was successfully recanalized using balloon dilation and subsequent stenting (Fig. D).\nThroughout the clinical stay, the patient’s oral anticoagulation with phenprocoumon was intensified to a new target INR of 2.5–3.0 in order to prevent re-thrombosis.\nThe patient was discharged from hospital on day seven after admission. Regular follow-up examinations at our outpatient clinic did not give evidence for any new thrombotic events to date (time since first occurrence of thrombosis was 9 months).\nConcerning the thrombotic left iliac vessels, a follow-up angiographic intervention relying on special equipment was performed, but recanalization could not be achieved.\nFortunately, the IVC of the patient’s mother could also be recanalized by angiographic intervention.","This 14-year-old boy was admitted to the hospital after experiencing pain and fever in his leg, which turned out to be a blood clot in his veins. He also has a family history of blood clotting problems, and tests showed his blood was not clotting properly. Because of the clots and his family history, doctors started him on strong medications to prevent more clots from forming, and they used a special technique to break up the existing clots. After several treatments and monitoring, the clots in his leg and IVC were successfully cleared, and he was able to go home. He will need to continue taking medication to prevent future clots."
38,"The patient is a 68-year-old retired male, born in Aloag and resident of Tambillo (a rural locality in the vicinity of the capital of Ecuador, Quito). His medical history was significant only for being a heavy smoker until 2016 (with a calculated 20 pack-year), copious alcohol consumption every 15 days until 2010 and a myocardial infarction in 2015, successfully treated with stenting, acetylsalicylic acid and atorvastatin, a medication that he continues until this day. There is no family history of cancer or other pathologies of interest.\nIn February 2020, he presented dysesthesias in the right hemithorax associated with pain and a mass-like sensation in the same region. This prompted a visit to his local healthcare center (part of the public health network) where a chest CT scan was ordered in March 2020 revealing a solitary pulmonary mass located in the right inferior lobule with an invasion of both the pleura and thoracic wall. However, due to the beginning of the COVID-19 pandemic in Ecuador, all further studies were suspended for two to three months, resulting in a significant delay of the biopsy, which was undertaken on May 17, 2020. The histopathological study reported a neuroendocrine carcinoma. The patient was subjected to a thoracotomy and inferior pulmonary lobectomy on June 7, 2020 and was afterward treated with four cycles of chemotherapy consisting of cisplatin and etoposide until November of the same year. In December, the patient presents with neurologic symptoms consisting of loss of balance, ataxic gait, headaches, and nausea, prompting the necessity of a brain MRI. The study revealed a mass on the right lobe of the cerebellum (2.66 x 2.61 x 2.48cm) with perilesional edema, compressing the fourth ventricle. A progression of his primary lung cancer was diagnosed, the original chemotherapy regimen was suspended and replaced with adjuvant Temozolomide maintenance therapy, and he is referred to the radiotherapy service for WBRT with 20 Gy divided into five fractions, which improved his symptoms.\nHowever, in March 2021, the patient comes to our hospital’s radiotherapy department to request a second opinion due to the reappearance of his neurological symptoms (loss of balance, headaches, nausea, dizziness, and photophobia). The physical examination is relevant for a Glasgow Coma Scale of 15, oriented in time, place, and person, incapacitating ataxic gait (the patient needs to be mobilized in a wheelchair), and severe photophobia requiring the constant use of sunglasses. The rest of the neurological examination is normal including cranial nerves, reflexes, and somatosensory function. Relevant prognostic indexes were calculated and included the Karnofsky Performance Scale (KPS) of 80%, and the Graded Prognostic Assessment (GPA), specific for patients with brain metastasis, of 3. A CT scan of the thorax, abdomen and pelvis was obtained, on February 22, 2021, in order to detect any new tumoral or metastatic activity; however, none was found (Figures -). Therefore, a follow-up MRI was ordered on February 23, 2021, which revealed two intra-axial lesions on the right cerebellar hemisphere and near the right lateral ventricle, respectively (Figures -).\nDue to the aforementioned clinical and imaging findings, radio-surgical management of the disease with 21Gy, in one fraction, delivered with a linear particle accelerator (LINAC), TrueBeam® (Varian Medical Systems, Inc., Palo Alto, CA) was offered to the patient; immobilization was performed with a thermoplastic mask and Precise Bite mouthpiece (Solstice™ SRS Immobilization System, Orange City, IA); treatment simulation was done on the CT images and volume definition was performed in the contrasted T1 MRI with SPACE sequence; Gross tumor volume (GTV) was defined as the signal enhancing hyperintense tumoral regions observed on MRI and planning target volume (PTV) was prolonged 1mm in all directions. Dosimetric planning was based on HyperArc (Varian Medical System) which is a relatively new isocentric VMAT technique developed specifically for non-coplanar, multileaf collimator (MLC)-based stereotactic radiotherapy with automated treatment optimization and dose delivery; the clinical and dosimetric planning can be found in Figures -. Dosage and organ constraints, evaluation of the iso-dosage curve goals as well as the evaluation of the Paddick index were performed in accordance with published guidelines [,]. Before treatment, we administer a 16mg intramuscular deposit dexamethasone injection in order to reduce the risk of cerebellar tonsil herniation due to the important edema from the cerebellar metastasis. Tomographic images obtained with the Cone Beam CT system, integrated into the LINAC, showed a complete (100%) correlation of the images obtained during simulation and planning. Finally, the effective time of treatment was of four minutes.\nTreatment was delayed as per the patient’s request for more time to consider and on April 5, 2021, the procedure was finally performed without complications and in an outpatient setting. A follow-up was programmed 15 days after treatment at which the patient presented significant clinical improvement (absence of headaches, nausea, dizziness, photophobia, and normal balance and gait). There was no evidence of radiation toxicity, and the cognitive function was normal. A control MRI on July 17, 2021, showed a clear reduction in the size of both lesions as well as complete resolution of the mass effect produced by the cerebellar mass; a comparison of the pre- and post-treatment scans can be found in Figures -.\nAt the moment, the patient remains in continuous follow-up, with the last reported telephonic follow-up, on September 2, 2021, in which the patient reported being asymptomatic and carrying out all of his regular day-to-day activities without any limitations.","This 68-year-old man has had a challenging medical journey. He started with smoking and heavy drinking, then suffered a heart attack, and later discovered a cancerous tumor in his lung. After initial treatment for the lung cancer, he began experiencing new neurological symptoms like balance problems and headaches, which led to the discovery of more tumors in his cerebellum. Despite these setbacks, he received focused radiation therapy that significantly improved his symptoms and helped to shrink the tumors. Currently, he is doing well and is being closely monitored to ensure the cancer remains under control."
39,"A 68-year-old female patient was admitted to the hospital on December 2, 2020, due to being “anxious and easily frightened for 3 months, psychomotor retardation, and affected by urinary incontinence for half a month.” The patient had no mental illness before and developed symptoms 3 months before admission. These included waking up early, being nervous and afraid for no apparent reason, and being fearful of leaving the house. The patient was upset, sensitive, and cried occasionally. In addition, the patient needed walking support (e.g., hands on the wall) at home to prevent falling. The patient had been hospitalized at a local mental health center 2 months prior to the present admission, where she was diagnosed with “GAD.” She received paroxetine (20 mg/d), tandospirone (30 mg/d), and oxazepam (15 mg/d). Her symptoms improved, and so she was discharged. She reported that she took the medications regularly according to the instructions. However, half a month prior to admission at our hospital, the patient experienced a relapse characterized by anxiety, fear, small steps while walking, reluctance to come out of her home, speaking less, and being slow to respond, as well as urinary incontinence. In addition, her social skills declined significantly, and the patient could not take care of herself independently. The patient had been diagnosed with type-II diabetes 4 years previously. No other comorbidities were reported. The patient had no abnormalities in her personal history, menstrual history, marriage and childbirth history, or family history.\nPhysical examination after admission revealed she had normal limb muscle strength and tone. The finger-to-nose, rapid alternating movement, heel-to-shin tests, and Romberg’s sign were normal. However, her gait was not stable and she took small steps. She was negative for pathological signs and meningeal irritation. In a psychological assessment, the patient exhibited clear consciousness, disorientation to place and time, passivity during interactions with few answers to questions, no hallucinations or delusions, a decline in memory and cognition; and a reduction in daily physical activities and energy levels. She felt distraught with a slight tremor in her hands.\nHer level of thyroid-stimulating hormone (TSH) was 6.280 µIU/mL (0.270-4.200); and she was normal for total T3, total T4, free T3, and free T4. Her level of anti-thyroglobulin antibody (anti-Tg) was 235.60 IU/mL (<115.00), and that of anti-thyroid peroxidase antibody (anti-TPO) was 238.70 IU/mL (<34.00). A color Doppler ultrasound showed diffused thyroid lesions with nodular changes (nodules in the right lobe of the thyroid). Both a CT examination (Figure ) and an MRI (Figure ) of the head showed brain atrophy and leukoaraiosis. A chest CT and pelvic ultrasonography were normal. EEG recordings revealed diffuse slow waves. No epileptic activity or extreme delta brush was observed. The patient’s Hamilton Anxiety Scale (HAMA) score was 22, indicating moderate anxiety. Her Hamilton Depression Scale (HAMD) score was 13, indicating a likelihood of depression. Her Mini-Mental State Examination (MMSE) score was 14, indicating dementia.\nThe differential diagnosis was as follows: 1) rapidly progressive dementia, 2) type-II diabetes, and 3) subclinical hypothyroidism. After admission, the patient was treated with venlafaxine (75 mg/d) and oxazepam (15 mg/d). She showed a rapidly progressive decline in cognitive function, emotional dysregulation, paroxysmal body shaking, slow performance, abnormal gait, constipation, and urinary incontinence. However, the reason for the rapidly progressive dementia was unknown. Thus, lumbar puncture and cerebrospinal fluid examination were performed with the following results: cerebrospinal fluid glucose: 6.30 mmol/L (2.50-4.50); protein: 0.87 g/L (0.15-0.45); and anti-glutamate receptor (NMDA) ratio: 1:3.2 (IgG).\nBased on these results, the diagnosis was changed to AE. Accordingly, the patient was transferred to the neurology department and treated with immunoglobulin (IVIG, 20 g/d via intravenous infusion for 5 days) and methylprednisolone (1000 mg/d via intravenous infusion for 3 days). The dosage of methylprednisolone was dropped by half every 3 days (till 48 mg/d) and was given orally. After treatment, the patient showed a gradual recovery in cognitive and emotional function, her body shaking disappeared, and her gait was stable. Her EEG was normal. Both medications were stopped gradually. The patient was discharged 24 days after administration and had resumed a normal life without relapse at a 6-month follow-up.","This 68-year-old woman had been feeling anxious and fearful for months, and recently started having trouble with her bladder and walking. She had been treated for anxiety before, but her symptoms returned, and she became increasingly confused and slow to respond. Tests revealed she had some changes in her brain and thyroid, and a possible autoimmune disorder. After receiving specific treatments, she gradually improved and was able to return home and resume her normal life."
40,"A 44-year-old female with a history of asthma, essential hypertension, class 3 obesity, depression, and poor social and economic background was intermittently followed during the previous four years for persistent cutaneous candidiasis with intertrigo in the inframammary, inguinal, and lower abdominal regions (Figure ).\nShe had been treated with topical antifungal, oral fluconazole and oral itraconazole with no improvement, which was believed to be because of poor hygiene and questionable therapeutic compliance. A worsening in the skin rash with exudate, pruritus, and a change to a violaceous colour, with scaly papules and vesicles (Figures , ) led to the performance of a skin biopsy which revealed (Figure ) orthokeratotic hyperkeratosis in the epidermis with areas of parakeratosis and, in the papillary dermis, there was an infiltrate of cells with eosinophilic cytoplasm and reniform nuclei that showed positive CD1a and S100 proteins on the immunohistochemistry and negative CD163 (Figure ).\nThe patient denied constitutional, musculoskeletal, neurological, or urinary complaints. She underwent a complete blood count, complete metabolic panel, brain magnetic resonance imaging (MRI), thoracic-abdominal-pelvic computed tomography (CT), and bone scintigraphy. Brain MRI depicted mild chronic microvascular changes in the white matter, unchanged from a prior study. CT demonstrated a thickening of the renal pelvis (4 mm) in the right kidney with a slight urothelial dilation (Figure ). The rest of the exams did not reveal further organ involvement.\nAfter considering the skin histology, the extensive cutaneous involvement, and the infiltrative urothelial involvement, it was evident this was a multi-system process. A consultation with Hematology/Oncology, led to induction treatment with prednisolone and vinblastine-based chemotherapy. At six weeks of chemotherapy, there was a partial regression of the skin lesions (Figure ) and a resolution of the urothelium lesion in imaging exam (CT).\nThe disease was in continuous regression and considering the extension of affected skin tissue a second round of chemotherapy with prednisolone and vinblastine was administered for six weeks. There was a resolution of all the lesions following this second round, and the patient underwent maintenance therapy consisting of administrating mercaptopurine daily and prednisolone/vinblastine every three weeks during 12 months, staying in remission (Figure ).\nSix months after the end of maintenance therapy the patient had a recurrence of the disease and started second-line chemotherapy with clofarabine and cytosine arabinoside (ARA-C). The patient did not comply with the treatment and the disease progressed. As a result of skin ulceration, she developed skin and soft tissue infection that evolved into septic shock and did not survive despite intensive care support.","This 44-year-old woman had a persistent skin rash for several years that didn't respond to typical treatments. A skin biopsy revealed a rare condition involving the skin and kidneys, and she was referred to a specialist. She started chemotherapy, which initially helped to reduce the rash and kidney problems. Sadly, the disease returned, leading to a serious infection and ultimately her passing away."
41,"A 72-year-old male, known hypertensive on medication, non-smoker, no family history of cancer presented with complaints of pain in the right hip with difficulty in walking in January 2019. On evaluation, a pathological fracture was found at the neck of the right femur. Magnetic resonance imaging of the spine was done, which revealed osteophyte complexes at C3-C4, C4-C5, C5-C6 vertebrae causing narrowing of neural foramina. Multiple T2-hyperintense lesions in lung parenchyma were an incidental finding. Upon further evaluation with positron emission tomography-computed tomography (PET-CT) scan of the whole body, mass in the apex of the right lung, right hilum, mediastinal lymph node, soft tissue wall thickening in the proximal stomach along with multiple liver and bone metastases were found. Upper gastrointestinal (GI) endoscopy revealed a proximal gastric growth from which a biopsy was taken. Histopathology showed poorly differentiated adenocarcinoma. A provisional diagnosis of carcinoma stomach with distant metastasis was reached. But immunohistochemistry came out to be positive for thyroid transcription factor-1 (TTF-1) and cytokeratin-7 (CK-7), while negative for cytokeratin 20 (CK-20) (Figures -).\nSo, a possibility of metastasis from a lung primary was considered. It was confirmed by a biopsy from the lung mass that revealed adenocarcinoma as the histopathology. On immunohistochemistry, it was positive for TTF-1 and CK-7 while negative for CK-20 and synaptophysin. Analysis for anaplastic lymphoma kinase, epidermal growth factor receptor, and receptor tyrosine kinase 1 were all negative but programmed death ligand 1 (PD-L1) tumor proportion score (TPS) was 90%. So, the final diagnosis was adenocarcinoma lung with multiple lung, liver, femur, as well as gastric metastases.\nFor the pathological fracture, he underwent fixation by intramedullary nailing followed by palliative external beam radiotherapy 8 Gy in a single fraction. He received 10 three-weekly cycles each of Inj. pembrolizumab 200 mg as intravenous infusion and Inj. denosumab 120 mg subcutaneously from March 2019 to September 2019. An interim PET-CT scan done after four cycles of immunotherapy in June 2019 showed a near-complete metabolic response (Figures -).\nAfter 10 cycles, Inj. pembrolizumab was discontinued as the patient was unwilling to continue due to personal reasons. He was then started with chemotherapy, Inj. pemetrexed 500 mg/m2 and Inj. bevacizumab 15 mg/kg in October 2019. Following this, the patient developed grade 1 maculopapular rash, pedal edema, and grade 2 diarrhea, which were managed conservatively. After that the patient did not opt for the continuation of chemotherapy. The next visit of the patient was in February 2021 with complaints of cough and breathlessness. On re-evaluation with PET-CT scan, progressive disease was found in the primary site (Figures , ).\nPalliative radiotherapy of 30 Gy in 10 fractions to the primary lesion was delivered through anteroposterior and posteroanterior portals of 10 megavolt energy each in April 2021 via linear accelerator (Elekta-Versa HD) which was tolerated well (Figure ).\nIn June, the patient developed an altered sensorium and contrast-enhanced magnetic resonance imaging of brain revealed multiple brain metastases for which the patient received palliative whole-brain radiotherapy of 30 Gy in 10 fractions over two weeks from the same linear accelerator. His condition further deteriorated, and in July, the patient succumbed to disease progression.","A 72-year-old man with high blood pressure experienced pain in his hip and difficulty walking. Tests revealed a broken hip bone and signs of cancer spreading to his lungs, liver, and stomach. Further investigation confirmed a lung cancer with metastases in other parts of his body, originating from his stomach. He received treatment with immunotherapy and chemotherapy, which initially showed promise, but eventually, he developed complications and passed away due to the progression of the cancer."
42,"A 90-year-old male was found in his home slumped to his right side and unable to be awakened. Paramedics calculated an 8/15 Glasgow Coma Scale (GCS) score, which remained the same on admission. On presentation to the hospital, the patient had left facial droop, bilateral pinpoint pupils, and right-sided weakness. The National Institutes of Health Stroke Scale (NIHSS) score on admission was 26, suggesting a severe stroke. All other observations on admission were non-revealing, including vital signs, biochemical tests, and systems review.\nThe patient’s neurological medical history included two previous transient ischemic attacks and suspected dementia. Other past medical history included chronic obstructive pulmonary disease, hypertension, abdominal aortic aneurysm repair, stage 3 chronic kidney disease, adult polycystic kidney disease, hypercholesterolemia, and aortic valve sclerosis. He was an ex-smoker of unknown pack-years and prior to admission was living independently.\nInitial head CT performed only showed chronic small vessel disease with periventricular leukoaraiosis, consistent with his age. Thrombolysis was initiated with an intravenous tissue-plasminogen activator, however, the patient’s GCS declined to 6/15 approximately 40 minutes after the start of thrombolysis. A repeat head CT was performed, again showing no acute findings. To prevent further deterioration, a decision to monitor the patient’s condition conservatively was made.\nOver the next few days, the patient remained very somnolent. When the patient was able to awaken, he was only capable of responding to simple commands. Speech and language therapists noted that fatigue was limiting conversations, with the patient speaking with imprecise articulation and at a low volume. Occupational therapists concurred that the patient was poorly engaged during sessions, while physiotherapists classified this patient as having limited rehabilitation potential.\nStroke was finally confirmed upon a third head CT several days after admission, which showed bilateral thalamic infarcts. Other findings included hyperdensity at the basilar tip and left posterior cerebral artery, likely due to wall calcification of vessels. An MRI revealed bilateral hyperintensities in the thalami, consistent with acute bilateral thalamic stroke, with small left parietal cortical infarction (Figure ).\nImaging also confirmed the arterial location of the infarction, a single branch of the pre-communicating (P1) segment of the left posterior cerebral artery known as the artery of Percheron (AOP). Over the next month, the patient participated in multidisciplinary rehabilitation sessions. He had a gradual return of physical function and with it, his sense of humor returned, much to the delight of his family and the attending staff. He was unable to care for himself independently and was eventually discharged to a care home. Six months later, he was mobile with a walking stick and had a reasonable quality of life. One year after the initial presentation to the hospital, he passed away from an intercurrent chest infection.","A 90-year-old man was found unresponsive at home and was rushed to the hospital with a severe stroke. Doctors discovered he had significant weakness on one side of his body and difficulty with his eyes, and confirmed the stroke with further imaging. Despite initial treatment, his condition worsened, and doctors determined he had suffered a stroke in the thalamus and a small area of the parietal cortex. He underwent rehabilitation to regain some movement and speech, but eventually needed to move to a care home and passed away six months later from an unrelated illness."
43,"A 47-year-old male with a past medical history notable for hypertension on metoprolol succinate, morbid obesity, and pre-diabetes presented to the emergency department (ED) with a chief complaint of generalized weakness. The patient tested positive for COVID-19 and exhibited mild unspecified respiratory symptoms. He was subsequently discharged home to recover in isolation per the CDCs COVID-19 response protocols. One week later, the patient returned to the ED for ongoing symptoms and was admitted requiring supplemental oxygen for hypoxia. Upon admission to the hospital, the patient was noted to have difficulty standing and ambulating. Two days into his inpatient stay, the patient developed urinary retention issues requiring intermittent catheterization. Three days later, he developed facial weakness and numbness. The clinical diagnosis of GBS was suspected and subsequent EDX studies reported AIDP. The patient was treated with a five-day course of intravenous immune globulin (IVIG). Three days after the completion of his IVIG treatment, the patient noted improvement with right upper extremity anti-gravity strength.\nThe patient was transferred to inpatient rehabilitation with significant proximal lower extremity weakness. The bilateral upper extremities demonstrated slight weakness. The bilateral lower extremities demonstrated a significant loss of strength, 1/5 dorsiflexion, and 3/5 plantar flexion bilaterally. The patient also reported diminished sensation to light touch in bilateral upper extremities in all dermatomes. His blood pressure upon rehabilitation admission was 110/73 mmHg.\nPrior to hospitalization, the patient was independent with mobility and all activities of daily living. Upon evaluation in the inpatient rehabilitation, the patient was at a significant functional decline from baseline, requiring dependent assistance with toileting hygiene, showering, upper body dressing, lower body dressing, footwear management, rolling left and right, and all transfers. The patient was unable to ambulate due to his level of impairment. In inpatient rehabilitation, the patient completed three hours total of physical, occupational, and speech therapy per day five days a week with exercises aimed to improve balance, mobility, activities of daily living, fine motor skills, cognition, and breath support.\nAfter completing four weeks of inpatient therapy, the patient was independent with supine to sit, upper and lower body dressings, and rolling right and left in bed. He required minimal assistance to lift the left lower extremity into the bed and moderate assistance with wheelchair-to-bed transfers and bed mobility. He was able to stand with the assistance of his spouse and by using significant reliance on bilateral upper extremity support on a walker for stability and offloading lower extremities. He required a power wheelchair for mobility and was unable to transfer in and out of the car thereby requiring a wheelchair van for transportation. Final muscle strength grading was not documented. His blood pressure remained stable throughout his stay on metoprolol succinate and was 121/65 mmHg on discharge.","This 47-year-old man was initially admitted after testing positive for COVID-19 and experiencing weakness. After recovering at home, he returned to the hospital with worsening symptoms, including difficulty walking and urinary problems, and was diagnosed with Guillain-Barré syndrome (GBS). He received treatment with IVIG, which helped improve his strength, but he experienced significant weakness and difficulty with daily tasks. After four weeks of rehabilitation, he regained some independence with basic movements and activities, but still needs a wheelchair and assistance for transfers."
44,"The patient was a 31-year-old male, a right-hand-dominant construction worker who was referred to our plastic surgery unit from a local minor injuries unit. He complained of pain in his left ring finger, following an inadvertent injury with a spray gun containing Newton 103-S (a type of liquid cement). The patient had been wearing protective gloves at the time of injury. He had no past medical history, took no regular medications, and had no allergies.\nThe patient had been given tetanus immunisation by the referring hospital. He had a plastic surgery review approximately three hours post-injury. On examination, his left ring finger was swollen, erythematous with necrotic skin on the volar aspect of the finger (Figure ). There was no evidence of distal vascular compromise and the dorsal skin was well-perfused. He had a complete loss of sensation in the distribution of the ulnar digital nerve; however, the radial digital nerve was clinically intact. There was decreased range of movement at both the proximal interphalangeal joint (PIPJ) and distal interphalangeal joint (DIPJ). The palmar skin was not involved.\nBlood results and observations were within normal ranges. No X-rays were taken at the time of presentation. Broad-spectrum IV antibiotics were commenced, and the patient was taken to theatre for urgent debridement and washout under general anaesthetic.\nThe initial surgical approach consisted of Bruner incisions. Significantly hardened concrete was identified in the subcutaneous tissues (Figure ). It was evident that skin and subcutaneous tissues were non-viable and were debrided, improving access. The pH of the wound was 8.5, in keeping with the alkaline substance injected; irrigation continued until pH returned to 7. The ulnar digital artery was thrombosed, but the radial digital artery was patent. The concrete had penetrated the flexor sheath, surrounding both flexor tendons. Subsequent flexor sheath washout from A1 to A5 confirmed that both tendons were in continuity but had undergone surface changes secondary to contact with the concrete.\nA second look was performed at 48 hours; the distal digit remained viable with the flexor tendons appearing healthy. Tendon glide was impeded by friction within the flexor sheath. Access incision was made into the palm, which revealed further cement material. The passive range of movement was reassessed, which confirmed PIPJ and DIPJ to be in good condition. Further debridement of devitalised soft tissue resulted in a large defect of almost the full length of the volar aspect of the ring finger exposing the intact flexor apparatus pulleys and tendons (Figure ).\nThe patient was counselled extensively regarding ongoing management, which principally consisted of either reconstruction of the volar soft tissue defect or potential amputation through the middle phalanx. He was counselled that the reconstructive option would result in a stiff and potentially insensate finger but could preserve length, while amputation might provide a faster recovery and better overall functional outcome. The patient opted for reconstruction and preservation of as much length of the digit as possible.\nAt one week from the initial injury, a cross-finger flap from the ipsilateral middle finger was performed, reconstructing the volar soft tissue defect (Figure ). The patient was discharged with oral antibiotics.\nAfter three weeks, the flap was divided and inset. However, the patient developed a wound infection five weeks after reconstruction, necessitating further washout and IV antibiotics.\nAt four months post-injury, the digit had an active range of movement of 20-84 degrees at the metacarpophalangeal joint (MCPJ); both PIPJ and DIPJ were in fixed 30 degrees of flexion. The protective sensation was maintained in the radial aspect of the digit, and the patient did not develop issues with pain subsequently. He was followed up via telephone consultation at 18 months postoperatively, and he reported that he had no functional limitations on daily activities, and was back to work. The skin overlying the volar defect of the ring finger and grafted area on the dorsum of the middle finger has healed well (Figures , ).","A 31-year-old construction worker injured his left ring finger with a spray gun containing cement. The finger became severely swollen and damaged, with some of the skin and tissue dying. Doctors performed surgery to remove the damaged tissue and clean the wound, and the patient received antibiotics. After several weeks, a skin flap was used to reconstruct the finger, and the patient is now healing well with good range of motion and sensation."
45,"A 46-year-old male patient was admitted to the hospital with intermittent back pain and chest tightness for 2 weeks. The patient works in ozone disinfection. Prior to symptom onset, he had a history of acute ozone inhalation. He recalled smelling something more pungent than usual for several days. Before coming to our hospital, he had not undergone any treatment. In addition, he denied any history of chest trauma. He had suffered from hypertension for over a decade and was treated with oral nifedipine and metoprolol. However, medication poorly controlled his blood pressure. The highest recorded systolic blood pressure with treatment was 180 mmHg. His blood pressure upon admission was 148/91 mmHg.\nAn echocardiogram revealed a left SVA that ruptured into the left-ventricular myocardium, forming an echo-lucent cavity (). The left-ventricular wall had thickened resulting in uncoordinated motion and reduced systolic function. Moreover, moderate eccentric aortic regurgitation was also noted. Furthermore, CT angiograms better captured a large, left-ventricular, IPA arising from a small perforation in the left SVA (). The adjacent left ventricle and interventricular septum were compressed. With CMR examination, late gadolinium enhancement (LGE) clearly demonstrated the left-ventricular IPA with distal thrombus and a linear enhancement of the IPA wall, compatible with myocardial fibrosis ().\nSubsequently, the patient underwent surgery where the perforation was sutured and repaired, and the left aortic valve was lengthened with pericardial patches. Three weeks after surgery, a follow-up echocardiogram demonstrated the cessation of the abnormal blood flow in the left sinus of Valsalva ().","6 This 46-year-old man was admitted to the hospital because of chest pain and back pain that had been bothering him for two weeks. He works with ozone and has a history of breathing in ozone, which may have caused this problem. Tests showed a hole in his heart’s valve, which was causing pressure and weakening his heart muscle. Surgery was performed to repair the hole, and follow-up tests showed that the problem was resolved."
46,Patient 1: A 64-year-old man with a left inferior visual field deficit and headache. The CT angiography showed a left superior carotid-ophthalmic aneurysm (4 × 5 mm) ().,"This 64-year-old man is experiencing blurry vision in his lower field of vision and headaches. A scan revealed a small aneurysm (a bulge in a blood vessel) in his eye, located on the left side. This aneurysm is causing the visual problems. The doctor will discuss treatment options to prevent it from growing or causing further issues."
47,"Patient 2: A 43-year-old woman with subarachnoid hemorrhage, classified as Fisher I, Hunt, and Hess II. In the digital 3D angiographic reconstruction multiple aneurysms are observed: a right M1 aneurysm and a right superior carotid-ophthalmic aneurysm ().","This 43-year-old woman recently suffered a subarachnoid hemorrhage, a type of bleeding in the brain. The scans show a small aneurysm (a bulge in a blood vessel) in the right side of her brain, as well as another smaller aneurysm in the eye area. These aneurysms were identified using advanced imaging techniques. The medical team will continue to monitor her condition and discuss treatment options."
48,"A 77-year-old woman was hospitalized in the cardiology department due to atrial fibrillation. Contraindications were excluded, and atrial fibrillation (AF) ablation was performed on an optional schedule. However, abdominal contrast-enhanced computed tomography (CT) scan found a circular and low-density lesion in the Segment 4 of the liver with unclear border, approximately 15 mm× 12 mm in size, which was mild progressive enhanced in the arterial phase and portal phase. HCC was suspected, but no hepatic cirrhosis and history of hepatitis (). Tumor markers, including CEA, CA199, CA125, AFP, were within normal range, hepatitis panel was negative, and hepatic function was normal.\nTo confirm the diagnosis, the patient underwent further examinations. Abdominal magnetic resonance imaging (MRI) disclosed one nodule in the segment 7, one nodule in the segment 5, and two nodules in the segment 4 of the liver (4, 6, 17, and 6 mm in diameter), respectively. The larger one was located in the segment 4 of liver, with hypointense on T1-weighted images (T1WI) and hyperintense on T2-weighted images (T2WI), which showed significant enhancement on the arterial phase and slight washout on the portal phase. In the delayed phase, the edge of the tumor was underscored as a circular enhancement which is unsimilar to the enhancement method of dynamic CT. It showed a significant restriction of diffusion on diffusion weighted images (DWI) and apparent diffusion coefficient (ADC). Other lesions in segments 7 and 5 showed the same hemodynamic characteristics as the tumor in segment 4 (). Based on MRI findings, it is considered as tumorous lesions of the liver.\nFor further differential diagnosis, the patient underwent positron emission tomography-computer tomography (PET-CT) examination, founding that the segment 4 of liver had a slightly low-density lesion with increased uptake of 18F-fluoro-deoxy-glucose (FDG), and SUVmax of early and delayed enhancement imaging was 4.81 and 5.24, respectively. The detention index was 8.9%. However, the lesions in segments 7 and 5 of the liver showed no significant increase in FDG uptake (). As all images suggested HCC, the medial segment of the liver was finally surgically removed.\nSurgical removal of a piece of left medial lobe of liver tissue, about 6 cm × 4 cm × 2.5 cm in size, the cut surface shows an expanded lumen structure, and a gray-white mass in the liver parenchyma. The size is about 2.5 × 2cm, part of the boundary is unclear, and the texture is soft. The HE-stained sections under low-power microscope showed that massive lymphoid tissues proliferated in the lesion and formed massive lymphoid follicles, and the peripheral lymphoid tissues of the lesion grew around the small bile ducts, but no clear lymphoepithelial lesions were seen. The interfollicular area is dominated by small lymphocytes, and many plasma cells can be seen. In the lymphatic tissue, there were homogeneous red-stained collagen-like deposits around some small blood vessels. Immunohistochemical results were as follows: Bcl-2(−), Bcl-6(germinal center +), CD10(germinal center +), CD20(+), CD23(+), CD3(+), Ki-67(germinal center +), CD5(+), CyclinD1(−), TDT(−), kappa/lambda ratio about 2:1. EBV negative by qRT-PCR method (). A genetic study of the immunoglobulin heavy chain (IgH) clonality on the DNA of tumor tissues was performed using capillary electrophoresis method, which was reported previously (). The results showed that no clonal IgH gene rearrangement was detected: IGH (−), IGK (−), IGL (−). Therefore, the possibility of extranodal marginal-zone lymphoma (MALT) was ruled out, and the final diagnosis was RLH. Combined with imaging, pathology, and clinical manifestations, the clinical diagnosis was multifocal RLH in the liver. Three months after the operation, a re-examination of the abdominal ultrasound showed no signs of recurrence.","This 77-year-old woman was admitted to the hospital because her heart was beating irregularly. During her stay, doctors found a small, concerning spot in her liver and performed additional tests, including MRI and PET scans, to determine if it was cancer. These tests revealed that the spot was actually a type of lymphoma, a type of cancer that affects the immune system, and not a typical liver cancer. The doctors removed a piece of the affected liver tissue, and follow-up tests have shown no signs of recurrence, so she is doing well."
49,"Patient 1 was a 45-year-old right-handed woman, who sought treatment for a depressive episode. The current episode onset was placed after a switch from sertraline to vortioxetine due to unbearable side effects (nausea and headache). She suffered from her first depressive episode at the age of 25, then alternating depressive and hypomanic episodes, which led clinicians to a diagnosis of BD-II.\nThe patient has also been suffering from EDs since her adolescence: she was diagnosed with anorexia nervosa at the age of 13; she then shifted to a bulimic eating pattern, with binge episodes followed by purging behaviors. This phase lasted for 10 years, followed by a complete remission until the age of 38. At this age she developed BED. Once a week or more she used to wake up at night and eat everything she could find in the fridge, including raw food. After these episodes she used to feel guilty and nauseated, but she did not show purging behavior anymore. She felt very uncomfortable due to either the loss of control or her weight gain. Her private psychiatrist then diagnosed her with BED, but apparently, she did not receive any psychological or pharmacological specific support. In the following years she had alternating periods of remission with periods of active disease. She denied alcohol or other psychoactive substances consumption. In the 2 months previous to our study, she had gained 12 kg, with 3–4 binge episodes per week on average. She denied medical comorbidities. When she first came to our attention, her therapy was the following: lamotrigine (150 mg daily), vortioxetine (20 mg daily). She was suffering from a depressive episode defined as mild according to the Hamilton Rating Scale for Depression (HAMD) and moderate according to the Montgomery-Åsberg Depression Rating Scale (MADRS) ().","This 45-year-old woman is experiencing a depressive episode after switching medications. She has a history of alternating periods of depression and mania, and has struggled with eating disorders since adolescence, including anorexia, bulimia, and now binge eating disorder. She has been experiencing frequent binge eating episodes, leading to weight gain and feelings of guilt. Currently, she is being treated with medication and will continue to work with her doctor to manage her mood and eating habits."
50,"The second patient we are reporting is a 28-year-old right-handed woman, who came to our attention for a severe depressive episode. Her psychopathological onset is placed 10 years ago; she developed her first depressive episode with comorbid panic attacks. From that moment the patient has alternated phases of depression with sporadic episodes of elation, thus a sign of hypomanic episodes, which led clinicians to a diagnosis of BD-II. Her depressive phases used to have a seasonal pattern, with autumn or winter worsening. When she came to our attention (November 2020), the current episode had been lasting for 3 months, according to her seasonal pattern. She reported low consumption of alcohol in social circumstances and sporadic use of cannabis in her adolescence.\nRegarding her ED, binging behaviors were reported to happen from the first diagnosis of depression, with various degrees of intensity and severity, and appeared to be more intense in depressive phases. Binge eating episodes during depressive phases used to be daily. No compensatory behaviors were ever observed. Notably, her first BED diagnosis was given in our center during her last depressive episode, as she had always been trying to hide her eating behaviors, even with physicians. Along with the current depression, she referred almost daily binge eating: after her dinner she used to go out and then buy and rapidly eat large amounts of high-fat food. This used to cause both physical and psychological distress.\nIn her past pharmacological history, many pharmacological therapies had been prescribed (i.e., valproate, fluoxetine, citalopram, venlafaxine, and bupropione) and when she came to our attention her therapy was clomipramine (150 mg daily) and pregabalin (225 mg daily). Her depressive symptoms at baseline were severe according to both MADRS and HAMD ().","This 28-year-old woman has struggled with depression and periods of feeling overly happy (hypomania) for the past 10 years, alternating between these states. She also has a history of binge eating, which is worse when she’s feeling down. She’s currently experiencing a severe depressive episode that has lasted for three months, and is being treated with medication, including clomipramine and pregabalin. Her doctors are working with her to manage her depression and binge eating behaviors."
51,"Patient 1: A 65-year-old male was diagnosed with AML in January 2020 with the manifestation of leukocytosis, thrombocytopenia and anaemia. BM aspiration revealed a hypercellular BM with 50% blasts. Flow cytometry showed the immunophenotype of myeloid blasts. Cytogenetics revealed a complex karyotype, and a molecular panel identified aberrations in ASXL1, CEBPA, JAK2, and RUNX1. None of the 41 gene fusions were detected by using multiple RT–PCR assay (). Therefore, AML with adverse risk was diagnosed according to genetic risk stratification (). After one course of induction treatment with the IA regimen [idarubicin 12 mg/m2 day1-3, cytarabine 100 mg/m2 continuous infusion day1-7.], the patient achieved complete remission with minimal residual disease as low as 5.8x10-4 by flow cytometry analysis. Genetic analyses showed that all gene mutations were negative. Subsequently, the patient refused bone marrow transplant for financial reasons. He received one course of the IA regimen and 3 courses of the high-dose Ara-c (HiDAC) regimen [cytarabine 2 g/m2 over 3 h every 12 h on day1–3.] as consolidation therapies. However, the remission duration only lasted for 11 months. In November 2020, relapsed BM morphology was detected, with 17.5% blasts concurrent with molecular aberration recurrence. Therefore, relapsed AML was diagnosed. Initially, venetoclax combined with azacitidine [VA, venetoclax once daily (100 mg day1, 200 mg day2, 400 mg day3-28) and azacitidine 75 mg/m2 day1-7.] was administered as a salvage therapy, an effective regimen recommended for the treatment of R/R AML patients who are ineligible for intensive salvage chemotherapy, but progressive disease was observed. Then, his treatment plan switched to a chidamide combined with venetoclax plus azacitidine regimen [chidamide 5 mg daily day1-7, venetoclax 100 mg day1, 200 mg day2, 400 mg day3-21; azacitidine 75 mg/m2 daily day1-7.], and CR was achieved after one course of therapy. The patient treatment process is shown in . After 3 months of follow-up, unfortunately, the patient gave up further treatment and passed away due to disease progression. The overall survival time was 1.5 years from first diagnosis.","A 65-year-old man was initially diagnosed with a type of leukemia (AML) in 2020 and received treatment that initially put him into remission. However, the leukemia returned after about a year, and despite further treatments, including a combination of medications, he eventually passed away after 5 years. Genetic testing revealed specific changes in his leukemia cells that influenced the treatment options. Despite the efforts of his medical team, the disease ultimately progressed, and he was unable to continue treatment."
52,"Patient 2: A 57-year-old male was diagnosed with AML in July 2016. Investigation revealed a pancytopenia. BM examination showed a hypercellular marrow with 65% myeloid blasts. Flow cytometry analysis showed the immunophenotype of myeloid blasts. Cytogenetics revealed del () (q22q36), and a molecular panel identified aberrations in DNMT3A and IDH2. The patient achieved CR after 1 cycle of the IA regimen [idarubicin 12 mg/m2 day1-3, cytarabine 100 mg/m2 continuous infusion day1-7.] and received 6 courses of the HiDAC regimen [cytarabine 2 g/m2 over 3 h every 12 h on day1–3.] as consolidation therapies while not adopting allogeneic haematopoietic stem cell transplantation. The patient experienced his first relapse 2 years after first remission with a 9% immature cell level in the BM and was treated with the CAG [cytarabine 10 mg/m2 every 12 h, day1-14; aclarubicin 5-7 mg/m2, daily on day1-8; and concurrent use of G-CSF 200 µg/m2/day.] regimen for 3 cycles, resulting in a second CR in March 2019. A second relapse occurred 20 months later. The patient began the VA regimen [venetoclax once daily (100 mg day1, 200 mg day2, 400 mg day3-28) and azacitidine 75 mg/m2 day1-7.], but no response was observed after 2 courses of therapies. Finally, the patient received a chidamide combined with venetoclax plus azacitidine regimen [chidamide 5 mg daily day1-7, venetoclax 100 mg day1, 200 mg day2, 400 mg day3-21; azacitidine 75 mg/m2 daily day1-7.] as salvage therapy as described above. The patient achieved his third CR. Treatment process is shown in . There were no infectious complications observed with the combination, and the duration of neutropenia was 10 days. After 1 month of follow-up, the patient remains in CR and is preparing for transplantation.","This 57-year-old man was originally diagnosed with acute myeloid leukemia (AML) in After several rounds of chemotherapy, he achieved remission and remained in remission for two years. However, he experienced two subsequent relapses, which were treated with different chemotherapy regimens. Finally, he responded to a new combination of medications, achieving a third remission and is now preparing for a stem cell transplant."
53,"Patient 3: A 60-year-old female was diagnosed with AML in December 2020. BM examination showed a hypercellular marrow with 32% myeloid blasts. A molecular panel identified aberrations in RUNX1. Karyotype was normal. All patient baseline characteristics at diagnosis and treatment characteristics are shown in . For induction therapy, the patient received the VA [venetoclax once daily (100 mg day1, 200 mg day2, 400 mg day3-28) and azacitidine 75 mg/m2 day1-7.] regimen and achieved CR after one course. Subsequently, she continued two courses of VA as consolidation therapy, but progressive disease was observed during the second course, with 67% blasts in BM. Then, a chidamide combined with venetoclax plus azacitidine regimen [chidamide 5 mg daily day1-7, venetoclax 100 mg day1, 200 mg day2, 400 mg day3-21; azacitidine 75 mg/m2 daily day1-7.] was given. After one course, the patient obtained CR. Treatment process is shown in . During the whole course, no severe adverse events occurred. After 1 month of follow-up, the patient remains in CR at the time of writing.","This 60-year-old woman was diagnosed with a type of leukemia (AML) in After initial treatment with a combination of medications, she achieved a complete remission, meaning the cancer disappeared. However, the cancer returned during a second treatment course, and a new combination of drugs, including chidamide, was used successfully. She has now achieved a complete remission again after one course of this new treatment, and is being closely monitored."
54,"A 54-year-old Caucasian male patient was referred to our institution, initially with the diagnosis of a T-cell lymphoma, not otherwise specified, which was refractory to two courses of chemotherapy (CHOEP: cyclophosphamide, doxorubicine, vincristine, etoposide and prednisolone). At presentation an erythroderma involving >90% of the integument was predominant (). Computer tomography (CT) scans showed enlarged axillary, inguinal and cervical lymph nodes. The complete blood counts showed a leukocytosis of 24,300/µl. Flow cytometry of the pb revealed 11,664 Sézary cells/µl with CD4+CD7- phenotype and with a CD4:CD8 ratio of 85.5. Flow cytometry of the bone marrow aspirate confirmed CD30 positivity with expression of 7% in Sézary cells. Polymerase chain reaction of pb confirmed the clonality in T-cell receptor beta and gamma showing monoclonal Vβ-β2 and two clonal Vγ1-8-Jγ1.1 and 2.1 rearrangements. While conventional cytogenetics showed a normal male karyotype, fluorescent in situ hybridization (FISH) detected the deletion of chromosome 17p in 22 of 200 interphases with deletion of TP53 gene. Immunohistochemistry of both trephine biopsy () and skin histology revealed infiltrations with Sézary cells (). The skin histology also confirmed CD30 positivity with 5-10%, and the diagnosis was revised to SS.\nThe diagnosis was thus revised to Sézary Syndrome in Stage IVA (pT4 Nx M0 B2) according to the updated classification of International Society for Cutaneous Lymphomas (ISCL) and the European Organization of Research and Treatment (EORTC) ().\nSubsequently, successive therapies with 3,000,000 IU interferon alpha three times weekly for 6 months, combined with 10 mg/m2 methotrexate (MTX) and 19 courses ECP including bexarotene showed no improvement.\nSix months prior to alloHSCT an antibody-conjugate treatment with brentuximab vedotin (BV) was administered. The patient received five courses, which led to an improvement of skin lesions and their reduction to about 30% of body surface. CT scans showed no further progression of the lymph nodes, the CD4:CD8 ratio was 76.7 with 63.5% Sézary cells in the flow-cytometry, confirming stable disease according to the EORTC and ISCL classification (, ).\nDue to preexisting comorbidities, we performed an alloHSCT from an unrelated HLA identical (10/10) and cytomegaly virus matched (recipient and donor IgG positive) 30 year old male donor with non-myeloablative conditioning (fludarabine 30 mg/m² body surface area on d-4 to -2 and total body irradiation with 2 Gy on day -1) followed by infusion of pb stem cells (). Immunosuppression consisted of cyclosporine A from d-1 (blood target level 200 ng/ml) and mycophenolate mofetil (3x1000 mg per day). The patient received a total of 6.6x106 CD34+ cells/kg body weight, 1.1x108 CD3+ cells/kg body weight and 0.2x108 CD16+ cells/kg body weight. In the absence of acute graft-versus-host disease (GvHD), mycophenolate-mofetil was tapered 500 mg every 14 days from day 40 and discontinued on day 74 while cyclosporine A was tapered from day +56 and discontinued on d+196.\nThe restaging on day +30 and +90 after alloHSCT showed residual infiltration of SS cells in the FACS analysis of the bone marrow (13 and 5% of all WBC, respectively) and chimerism of 70 and 93% on sorted CD3+ cells. The CT scan on day +90 after alloHSCT confirmed the persistent lymphadenopathy. On day + 172 after alloHCT a complete hematological remission (CR) with no infiltration of SS in bone marrow trephine biopsy, 100% chimerism on sorted CD3 positive cells from bone marrow aspirate and normal pb CD4:CD 8 ratio was documented, thus confirming graft-vs-Sézary effect (). The CT scan showed no lymph node enlargements. The inspection of the skin revealed no suspect lesions.\nAfter discontinuation of immunosuppression the patient developed chronic GvHD of the ocular mucosa and the liver according to national institutes of health (NIH) criteria () on day 277 and required immunosuppressive treatment with intermediate dosage of methylprednisolone, cyclosporine A and mycophenolic acid. The immunosuppression could have been slowly tapered to 720 mg mycophenolic acid b.i.d, 5 mg prednisolone and autologous plasma eye drops as previously described (, ). Furthermore, since September 2021 ruxolitinib was added to the GvHD treatment (). Currently, 1215 days after alloHSCT the patient is in continuous complete remission of SS, having active ocular chronic GvHD.","This man has been battling a rare type of skin cancer called Sézary Syndrome for a long time. Despite multiple rounds of chemotherapy and other treatments, the cancer continued to grow. After a period of improvement with a new antibody treatment, doctors decided to perform a stem cell transplant from an unrelated donor. This transplant helped to eliminate the cancer cells, but unfortunately, he developed chronic GvHD (graft-versus-host disease) affecting his eyes and liver. He is currently receiving treatment for this side effect and remains in remission from the Sézary Syndrome."
55,"The second case was that of a 93-year-old woman. Her diagnosis at admission included a gallstone, acute attack of chronic cholecystitis, biliary pancreatitis, septic shock, and multiple organ dysfunction syndrome. The surgeons performed endoscopic retrograde cholangiopancreatography to relieve the biliary obstruction. The growth of Escherichia coli (ESBL +) and Enterococcus faecium was observed in bile bacterial culture during ultrasound-guided cholecystostomy. Blood cultures were positive for Escherichia coli (ESBL +). Mechanical ventilation and CVVH were performed at admission. The initial antibiotic treatment regimen comprised 500 mg imipenem/cilastatin q12 h ivgtt and 1,250 mg vancomycin ivgtt. Starting on D2, the patient was administered 750 mg vancomycin QD ivgtt. On D5, the patient was treated with CVVH again because of oliguria and elevated creatinine. On D7, blood culture tests were negative, thus imipenem/cilastatin was switched to piperacillin-tazobactam, and vancomycin was continued. On D14, the drainage fluid culture was negative and the patient was transferred to the general surgery department for further treatment. On D21, antimicrobial treatment was discontinued.","A 93-year-old woman was admitted to the hospital with a severe gallbladder infection and inflammation of the pancreas, leading to septic shock. Doctors performed a procedure to clear the blockage in her bile ducts and identified bacteria in her bloodstream. She received strong antibiotics and support with dialysis to help her kidneys. After several weeks of treatment, her infection cleared, and she was transferred to general surgery for further care."
56,"A 5-year-old boy initially presented with headache, right eye pain, and vomiting, to a rural district hospital in the State of Sabah, East Malaysia on Borneo island. The boy also experienced difficulty in walking, blurring of vision, and fluctuating consciousness 1 month before admission. There was a significant (but unquantified) loss of weight and anorexia. He completed his bacille Calmette-Guerin (BCG) vaccination but missed all scheduled shots after his first birthday because of poor family support. On examination, a Glasgow Coma Scale (GCS) of 9 was documented (E2V2M5) with left lateral strabismus. The pupils were unequal (4 mm/3 mm) and sluggish. Power in all four limbs was 4/5 (Medical Research Council scale) with normal reflexes. Babinski was downgoing, and Kernig's sign was not elicited.\nThe boy was transferred to our center for further evaluation and treatment. Non-contrast CT brain showed acute hydrocephalus with cerebral edema. Subsequently, an external ventricular drain was inserted. Intra-operatively, the opening pressure was high, and outflowing cerebrospinal fluid (CSF) was clear and colorless.\nMagnetic resonance imaging of the brain revealed diffuse enhancing nodular leptomeningeal thickening, especially at the basal cisterns (). No intra-axial lesion was present. Small non-enhancing cystic lesions were seen along the leptomeningeal surface (), and no restricted diffusion was depicted. A diagnosis of tuberculous meningitis was considered, and an extensive TB workup was undertaken. The positive results from that battery of tests were a high erythrocyte sedimentation rate (ESR) of 90 mm/h and elevated CSF protein with normal CSF glucose levels. Otherwise, the Mantoux test was negative, and the blood, CSF, and CSF TB cultures showed no organism. The CSF for acid-fast bacilli as well as CSF GeneXpert were also negative. CSF latex agglutination was negative for streptococcus Group B, haemophilus influenzae, streptococcus pneumoniae, neisseria meningitidis, and escherichia coli. Both gastric lavage for acid fast bacilli stain and gastric lavage for GeneXpert were negative.\nSmear-negative TB was considered after reviewing the biochemical and imaging findings. Using the Composite Reference Standard (CRS) criteria, this child was classified as probable TB (). A standard anti-TB regimen was started consisting of isoniazid, rifampicin, pyrazinamide, and ethionamide. Intravenous dexamethasone was prescribed during the first 2 weeks of admission and was subsequently changed to oral administration.\nThe boy's condition improved considerably after 14 days, and he was well enough to undergo a comprehensive ophthalmology examination. He had a visual acuity of 1/60 in the right eye, and there was no light perception in the left eye. A positive right afferent pupillary defect was detected. Ophthalmoscopy showed normal macula bilaterally.\nBoth his parents were screened and found to be negative for TB. He was discharged well after a 4-week hospital stay.\nOne month later, he presented again with vomiting, unsteady gait, and anorexia. The follow-up MR brain study revealed exuberant nodular leptomeningeal enhancement that was more extensive than the initial study (). Also, the previously seen cystic lesions have increased in size and number (). MR spectroscopy (single voxel placed at the thickened tentorium cerebelli) showed a markedly elevated choline:creatine ratio of 4.34 and absence of a singlet peak at 3.8 ppm (). A biopsy sample was obtained and sent for histopathological examination (HPE). Transcranial approach at the right Keen's point was used to obtain the biopsy sample. The patient incidentally needed a revision of his external ventricular shunt at that time, so dural tissue was obtained via the Burr hole just prior to the shunt revision.\nHistopathological examination (HPE) revealed a moderately cellular neoplastic proliferation with background desmoplastic and myxoid changes seen from the hematoxylin and eosin (H&E) stain slides (). The cells were fairly monomorphic with mild nuclear pleomorphism displaying enlarged round to oval nuclei with fine chromatin and inconspicuous nucleoli (). Occasional mitosis was seen (). The Ki-67 proliferation rate was high, accounting for about 80% (). Immunohistochemical (IHC) studies showed that the tumor cells were positive for S100 and synaptophysin (). Given these findings, the boy was diagnosed with DL-GNT.\nA family conference was held to counsel the parents on possible treatment options. The parents decided to opt for palliative treatment. The patient succumbed to the illness 3 months later, 22 weeks in total, after diagnosis.","This 5-year-old boy was initially admitted with symptoms like headaches, eye pain, and difficulty walking, and he had lost weight. After further tests, including scans and spinal fluid analysis, doctors diagnosed him with a type of tuberculosis that affected his brain, causing swelling and fluid buildup. He received treatment for this infection and had a temporary tube placed in his brain to drain the excess fluid. Despite treatment, his condition worsened, and further tests revealed a more aggressive tumor in his brain, leading to a difficult decision about his care. Sadly, he passed away after several months of treatment."
57,"A 1.9-year-old girl was referred to our hospital on March, 2016 due to 4-day fever and cough, with no pertinent past medical history. Physical examination revealed temperature 37.4°C, heart rate 120 beats/min, respiration 50 times/min, blood pressure 90/60 mm Hg, and transcutaneous oxygen saturation 92% without oxygen administration, fatigue, and depressions in suprasternal fossa, supraclavicular fossa, and intercostal space. She developed hypoxemia, so the reservoir mask of 6 l/min was utilized for ventilatory support. The right lung showed diminished breath sounds. Cardiovascular, nervous system, extremities, antinuclear antibodies (ANAs), and extractable nuclear antigens (ENAs) examinations were normal. Routine blood tests showed the following results: hemoglobin (Hb) 118 g/l, white blood cell (WBC) 7.83 × 109/L, neutrophils (N) 53.3%, lymphocytes (L) 40.4%, and C-reactive protein (CRP) 156 mg/l. Arterial blood gas analysis revealed a pH of 7.48, partial pressure of carbon dioxide in artery (PaCO2) of 42 mm Hg, partial pressure of oxygen in artery (PaO2) of 50 mm Hg, base excess (BE) of 6.9 mmol/l, and oxygenation index of 238. Pulmonary CT suggested consolidation with atelectasis in the middle lobe of right lung ().\nElectrocardiogram indicated sinus rhythm with blunt T wave of part of the lead, visible in double peak. The patient received cephalothin for anti-infection at admission. On 2nd day, due to pneumonia complicated with atelectasis in her CT scan, the first fiberoptic bronchoscopy (FB) was used to relieve atelectasis and obtain respiratory samples for bacteriologic, cytologic, and histologic detection. On 3rd day, the titer of MP-immunoglobulin M (IgM) was 1:160 and then azithromycin and methylprednisolone were applied for anti-inflammation. On 5th day, due to persistent fever, aggravated cough, and lesions on chest radiography progressed after conventional anti-infective therapy, the second FB was performed and found plastic bronchitis in the airway (). Bronchoalveolar lavage (BAL) fluid (BALF) was negative in other microorganism, except MP-DNA up to 5.1 × 108 copies/ml. MP resistance mutation site 2063/2064 showed positive. No other etiological evidence was found in body fluid or secretions. On 9th day, the body temperature returned to normal level and the clinical symptoms and imaging improved.\nOn 14th day, the blood oxygen turned to normal and stopped oxygen inhalation. The titer of MP-IgM was increased to 1:10240. She was discharged on the 17th day. However, on 24th day, she had sudden high fever. Because she was still not getting better after 3-day conventional anti-infective therapy and persistent consolidations in X-ray, the third FB was used on 27th day on outpatient department and removed bronchial casts. Her temperature was normal on the next day and the chest X-ray on the 30th day had great improvement (, ). She did not present any discomfort within 60 days.","This 9-year-old girl was brought to the hospital because she had a fever and cough for four days. During her stay, she became very sick with low oxygen levels and a lung infection, requiring oxygen support and a procedure to clear her airways. Tests revealed she had a specific type of pneumonia caused by Mycoplasma pneumoniae, which was confirmed by finding the bacteria in her lung fluid. After receiving the right antibiotics and supportive care, she recovered and was able to go home healthy."
58,"A 2.4-year-old girl was hospitalized on October, 2015 due to 3-day persistent fever and cough, without underlying disease. Reservoir mask of 10 l/min was utilized for ventilatory support. Due to pulmonary CT suggested inflammatory consolidation with atelectasis, the first FB was used for treatment and etiological diagnosis. After 2 days of cephalothin and azithromycin treatment, she still had fever and cough and developed hypoxemia. Arterial blood gas revealed a pH of 7.44, PaCO2 of 45 mm Hg, PaO2 of 52 mm Hg, and oxygenation index of 247. Then, nasal high-flow oxygen of 10 l/min was adopted and methylprednisolone was applied for anti-inflammation. Physical examination revealed temperature 38.3°C, heart rate 135 beats/min, respiration 45 times/min, blood pressure 85/50 mm Hg, transcutaneous oxygen saturation 93% without oxygen administration, fatigue, flaring of nares, and decreased respiratory sound in the left lung. Cardiovascular, nervous system, extremities, ANA, and ENA examinations were normal. The routine blood tests indicated Hb 118 g/l, WBC 6.25 × 109/L, N 55.3%, L 37.6%, and CRP 26 mg/l. On day 4, pulmonary CT suggested inflammatory consolidation accompanied with left pleural effusion ().\nFiberoptic bronchoscopy and pathological results revealed fibrinoid formation in the left upper lobe and lower lobe, indicating plastic bronchitis (). BAL fluid was negative in etiological, except for MP-DNA and Epstein Barr (EB)-DNA up to 1 × 108 copies/ml and 4.8 × 105 copies/ml, respectively. MP resistance mutation site 2063/2064 was also positive. No other etiological evidence was found in body fluid and secretions. Hypokalemia (K 3.28 mmol/l) and dysfunction of blood coagulation () occurred during disease. On 5th day, chest radiography showed increased patchy shadows in the left lung (). Therefore, the second PB was performed to relieve bronchial casts. After administration with methylprednisolone of 10 mg/kg/d and gamma globulin of 2 g/kg, body temperature returned to normal and anoxia was improved on the 6th day. The methylprednisolone was applied for 11 days. She was discharged on the 12th day and the titer of MP-IgM was increased by eight times on the 14th day (). In the follow-up, she still had cough on the 18th day. On the 48th day, the chest X-ray turned to normal ().","A 2-year-old girl was hospitalized because of a persistent fever and cough that lasted for three days. Doctors initially tried antibiotics and other medications, but her condition didn't improve, and she became short of breath. After trying different oxygen treatments and medications, doctors discovered she had a serious lung infection and fluid buildup in her chest. With further treatment, including special breathing treatments and supportive care, her fever and breathing improved, and she was eventually able to go home after several weeks."
59,"A 4.3-year-old boy was admitted on April, 2016 due to 5-day fever and cough. He was in good health and had never been to hospital. Two days before admission, pulmonary CT from other hospital suggested inflammatory consolidation with atelectasis. Therefore, the first FB was performed to etiological diagnosis and atelectasis treatment on admission. FB revealed fibrinoid formation in the right upper lobe. After 2 days of treatment of cephalothin and azithromycin, the condition worsened and hypoxemia developed. Arterial blood gas revealed PaCO2 43 mm Hg, PaO2 56 mm Hg, and oxygenation index 266. Then, reservoir mask of 6 l/min was adopted for ventilatory support. Physical examination revealed temperature 38.0°C, heart rate 135 beats/min, respiration 32 times/min, blood pressure 90/60 mm Hg, and transcutaneous oxygen saturation 90% without oxygen administration, fatigue, and decreased respiratory sound in the right lung. Cardiovascular, nervous system, extremities, ANA, and ENA examinations were normal. Routine blood tests showed Hb 132 g/l, WBC 12.2 × 109/L, N 73.3%, and L 27.1%. The biochemical examination revealed a result of aspartate aminotransferase (AST) 2,031 U/L, alanine transaminase (ALT) 1,595 U/L, lactate dehydrogenase (LDH) 2,673 U/L, creatine kinase-MB (CK-MB) 41 U/L, triglyceride 1.33 mmol/l, procalcitonin (PCT) 1.56 ng/ml, CRP 38.8 mg/l, and ferritin 4,355 ng/ml. MP-DNA reaching 1 × 108 copies/ml in BAL fluid and MP resistance mutation site 2063/2064 were positive and MP-DNA of hydrothorax was 3.2 × 105 copies/ml.\nOn the 2nd day, pulmonary CT suggested substantial pulmonary consolidation in the upper and middle lobes of the right lung accompanied by significant effusion (). Due to persistent fever and significant elevation on blood inflammatory indicators, an additional methylprednisolone of 10 mg/kg/d was added for anti-inflammation and it was applied for 14 days. B-ultrasound showed that the maximum depth of pleural effusion was about 70 mm. Then, thoracentesis was performed and 300 ml of light yellow liquid was drained. Due to plastic casts in the airway and persistent consolidations in X-ray, FB was performed on the 3rd day and 6th day again. His temperature returned to normal and blood oxygen improved on 7th day. On 9th day, B-ultrasound showed no pleural effusion. The chest X-ray was normal and the titer of MP-IgM was increased by eight times on the 14th day. This patient had a fever for a total of 7 days after admission and discharged with azithromycin (oral) on the 15th day (). No other etiological evidence was found in body fluid and secretions. She was followed-up without any discomfort on the 22nd and 52nd day.","This 4-year-old boy was admitted to the hospital after a five-day fever and cough. Initial tests showed a lung infection with some fluid buildup, and he needed extra oxygen support. After several days of antibiotics and other treatments, his condition worsened, and doctors performed a procedure to help clear the infection. Eventually, his fever came down, and his breathing improved, allowing him to go home on antibiotics after a few weeks of monitoring."
60,"A 61-year-old male was diagnosed with de novo metastatic melanoma in January 2020 after presenting with sudden onset left upper limb dyspraxia and confusion. Comorbidities included hemochromatosis and a distant history of meningococcal meningitis. Magnetic resonance imaging (MRI) brain demonstrated a large right parietal lesion. Computed tomography (CT) and positron emission tomography (PET) scan revealed left upper and lower lobe lung lesions, solitary liver lesion, and base of skull lesion. Histopathology confirmed BRAF/NRAS wild-type metastatic melanoma. He proceeded with resection of the right parietal lobe metastases in February followed by ipilimumab (3 mg/kg)/nivolumab (1 mg/kg) commencing in March ().\nMRI brain on the April 3 demonstrated intracranial recurrence with PET/CT confirming stable extracranial disease. A redo craniotomy was performed on April 8, complicated by the development of cerebral abscess and ventriculitis requiring burr hole and drainage. Cultures confirmed corynebacterium acnes and he commenced intravenous (IV) Cephalothin for a total of 12 weeks. Six weeks following his last dose of immunotherapy and while on IV antibiotics for his cerebral abscess, the patient developed severe peripheral edema, dyspnea, and tachycardia. Electrocardiograph (ECG) demonstrated sinus tachycardia, left axis deviation, and right bundle branch block. Transthoracic echocardiogram (TTE) revealed a new circumferential pericardial effusion with early signs of tamponade. Serial troponins remained normal, and cardiac MRI showed no evidence of myocarditis. A diagnosis of ICI-induced pericarditis with associated pericardial effusion was made. The patient was commenced on aggressive diuresis, colchicine 500 mcg daily and ibuprofen 500 mg three times daily. The active decision to withhold high-dose corticosteroids was made given the patient’s concomitant cerebral abscess. He was monitored with weekly echocardiograms by the treating cardiologist with gradual resolution of the pericardial effusion over 4 weeks. Immunotherapy was discontinued. In June 2020, the patient had a further recurrence of brain metastases. A third resection followed by stereotactic radiosurgery to the cavity were completed at that time.\nSeven months following cessation of immunotherapy, the patient presented with sudden onset aphasia, left lower limb myoclonic jerks, and confusion. Further history revealed that the patient had developed subtle behavioral changes in the weeks prior. CT brain and angiogram showed no evidence of acute cerebrovascular event, infection, or intracranial disease progression. Laboratory results showed a normal CRP (0.7 m/L) and mild hyponatremia (129 mmol/L). An MRI brain revealed T2/FLAIR hyperintensity in the right mesotemporal lobe with differentials including encephalitis or postictal changes (). Electroencephalogram (EEG) demonstrated lateralizing periodic discharges from the right temporal region. Empirical acyclovir was commenced following a lumbar puncture that demonstrated a mild elevation of protein 0.62 g/L, normal white cell count, negative bacterial/fungal cultures, and negative viral PCR panel. Despite up titration of antiepileptics, the patient continued to deteriorate with increasing confusion, fluctuating level of consciousness, persistent dysphasia, and development of visual hallucinations. Autoimmune encephalitis and antineuronal antibody panels were normal. ICI-induced encephalitis was considered the most likely diagnosis and methylprednisolone 500 mg IV/day was initiated, continued for 3 days, and followed by 2 days of 250 mg IV/day. There was a rapid and remarkable improvement in symptoms following steroid administration. A repeat EEG showed resolution of lateralizing periodic discharges from the right temporal region. He was discharged on 80 mg oral prednisone, which was slowly weaned over 2 months.\nThe patient has continued on surveillance since cessation of immunotherapy in April 2020. His most recent imaging in March 2021 demonstrated an ongoing near-complete response of his metastatic melanoma. His pericarditis has not recurred with significant but partial neurological recovery from his grade 4 encephalitis.","This 61-year-old man was initially diagnosed with melanoma that had spread to his lungs and brain. He had a history of other health problems, including kidney issues and a previous infection. After surgery and immunotherapy, his melanoma improved, but then he developed a brain problem that caused confusion and difficulty speaking. Doctors determined he had encephalitis, likely caused by the immunotherapy, and treated it with steroids, which quickly improved his symptoms. Currently, his melanoma is responding well, and he is recovering from the neurological issues."
61,"A six-year-old, 9.7 kg, male Cavalier King Charles spaniel was referred to the Cardiology Unit of the Veterinary Teaching Hospital of the University of Bologna with a two-month history of severe exercise intolerance associated with a syncopal episode. Clinical signs had developed during a local wave of COVID-19 approximately two weeks after the family of its owner had manifested symptoms of this viral disease and their positivity to SARS-CoV-2 had been confirmed by the local Health authority. Despite the dog’s clinical condition, evaluation at our institution was postponed and performed only after two months from the occurrence of the aforementioned signs due to the COVID-19 illness and related quarantine of the owners. The dog had been previously evaluated by the primary veterinarian several times since he was a puppy, as regular examinations were performed approximately every six months. Previous medical history was unremarkable and no cardiac problems had been identified at earlier examinations. The patient was an indoor dog that was being fed a high-quality balanced commercial diet. He had no known exposure to toxic agents or medications and was current on vaccinations and parasite prevention.\nUpon presentation, cardiac auscultation revealed a grade II/VI left apical systolic murmur; the heart rate was 136 beats/min and the cardiac rhythm was regular. The femoral pulse was strong and synchronous with the heartbeat. Non-invasive systolic arterial blood pressure, assessed by a high-definition oscillometric device (petMAP graphic, Ramsey Medical, Inc., Tampa, USA), was 166 mmHg. Given the patient’s anxiety during physical examination, the pressure value was primarily interpreted as situational hypertension. Respiratory rate was mildly accelerated (44 breaths/min), likely due to the dog’s emotional stress, but lung auscultation was within normal limits. The remainder of the physical examination were unremarkable. Thoracic radiographs revealed mild generalised enlargement of the cardiac silhouette (vertebral heart scale 11.5, breed-specific reference interval 10.60 ± 0.50 []), with no obvious lung parenchymal abnormalities (). Sinus rhythm was observed on a six-lead surface electrocardiogram (Cube ECG, Cardioline S.p.A., Caverano, Italy) (). A transthoracic echocardiography was also performed by a board-certified cardiologist (GR) using an ultrasound unit (iE33 ultrasound system, Philips Healthcare, Monza, Italy) equipped with phased-array transducers (3–8 and 1–5 MHz) and continuous electrocardiographic tracing (, ). This showed LV volume overload and global systolic dysfunction without concomitant left atrial dilation (). Although the mitral valve leaflets were structurally and functionally normal, a mild mitral regurgitation with central jet was present. In light of the aforesaid findings, the valve insufficiency was hypothesized to be functional and due to the dilated cardiomyopathy (DCM) phenotype. No other echocardiographic abnormalities were identified.\nResults of routine blood work, including complete blood count, serum chemistry and coagulation profile (prothrombin time, activated partial thromboplastin time, fibrinogen and antithrombin), were unremarkable. In light of the DCM phenotype, further laboratory tests included a thyroid profile and assessment of the serum concentration of cardiac troponin I (cTnI, IMMULITE 20000, Siemens, Erlangen, Germany). The former test ruled out hypothyroidism as a possible cause of LV systolic dysfunction (thyroxine 29.5 nmol/L, hospital reference interval [HRI] 13–51 nmol/L; thyroid stimulating hormone 0.12 ng/mL, HRI 0.03–0.38 ng/mL), while the latter unveiled MI (0.19 ng/mL, HRI < 0.15 ng/mL) [,]. The dog was started on pimobendan (Vetmedin, Boehringer Ingelheim, Ingelheim amRhein, Germany) at a dose of 0.25 mg/kg orally every 12 h, and several blood samples were collected with the aim of submitting them for investigation of infections responsible for MI. Initially, serological tests for Borrelia burgdorferi, Dirofilaria immitis, Anaplasma phagocytophilum, Ehrlichia canis, Leishmania infantum, Toxoplasma gondii and Bartonella henselae infection were performed (SNAP 4Dx, IDEXX Laboratories, Inc., Westbrook, U.S.A.; MegaFLUO LEISH, Vetefarma S.r.l., Cuneo, Italy; MegaFLUO TOXOPLASMA Gondii, Vetefarma S.r.l., Cuneo, Italy; Indirect immunofluorescence performed as described by Fabbi et al. []), yielding negative results. Given the family history as well as the ongoing and still unexplained MI, further serum samples were subsequently submitted for SARS-CoV-2 investigation [,]. Different serological assays were performed (using samples collected on the same day) to detect antibodies against the S and the N proteins of SARS-CoV-2. More in detail, specific neutralizing antibodies against the receptor binding domain of the spike protein were determined in serum samples using the SARS-CoV-2 surrogate virus neutralisation test (sVNT, GenScript cPass™ SARS-CoV-2 Neutralisation Antibody Detection Kit, GenScript Biotech Co., Ltd., Leiden, Netherlands) following the manufacturer’s instructions (positivity: ≥30% inhibition). Furthermore, SARS-CoV-2 neutralization assay (VNT) was performed as described by Rijkers et al. [] (positivity: titers ≥1/10). Lastly, a commercial multispecies enzyme-linked immunosorbent assay (ELISA; EradikitTM COVID19-Multispecies, In3Diagnostic, Turin, Italy; positivity: >20%) was performed. Pending serological results, no other therapies were prescribed in addition to pimobendan, but exercise restriction was recommended. Within two weeks, results from serological assays became available. All tests yielded a positive result ().\nIn light of such findings, approximately two weeks from presentation, another control was performed to recheck the clinical, cardiological and serological condition of the dog, and to obtain nasopharyngeal and rectal swabs for SARS-CoV-2 genome detection (using a real-time polymerase chain reaction (PCR) targeting E gene, as previously described by Corman et al. []). The owner reported an improvement in exercise tolerance and no further syncopal episodes. Physical evaluation was unremarkable, with the only exception being the heart murmur, which maintained unchanged characteristics. Echocardiography demonstrated a mild improvement of LV systolic function, although a DCM phenotype and a functional mitral regurgitation were still present, thus explaining the persistence of the heart murmur (). Blood samples were collected to monitor the antibodies against SARS-CoV-2 as well as the serum concentration of cTnI. All serologic assays were still positive () and cTnI was still over the HRI (0.17 ng/mL). SARS-CoV-2 molecular analysis yielded a negative result. Therapy as well as instruction at home were unchanged, and another recheck was planned within three weeks.\nAt that control, although the clinical and echocardiographic findings were stable compared to the previous examination, and two serological tests were still positive (i.e., sVNT and VNT), cTnI was normalised (0.09 ng/mL) and ELISA yielded a negative result for SARS-CoV-2 (). The dog continued to receive pimobendan and be revaluated regularly over the following weeks, showing a good clinical condition, stable echocardiographic parameters and a cTnI within the HRI. He is still alive and doing well at the time of manuscript writing (9 months from the occurrence of clinical signs and 7 months from the first evaluation at our institution).","This six-year-old Cavalier King Charles spaniel was brought to the hospital because he was struggling to keep up with his usual activities, experiencing episodes of fainting, and had a concerning heart murmur. It turned out he had a viral infection (COVID-that likely triggered his symptoms, and he also developed a heart condition called dilated cardiomyopathy. Despite several tests and medications, including pimobendan, his heart condition remained stable, and he continues to be monitored closely. The dog’s COVID-19 infection was ruled out after repeated testing."
62,"In the first week of April 2021, one 11-month-old female PI calf (Brown breed) was identified in a herd of Apulia region, Italy, that consisted of 99 Alpine Brown cattle, all under the age of 1 year, including 91 lactating cows. The herd adheres to the compulsory eradication plans for tuberculosis, brucellosis and bovine leucosis and to the voluntary eradication plan for BVDV. The PI animals are destined to be slaughtered. The PI calf was taken to the Veterinary Hospital of the Department of Veterinary Medicine of the University of Bari, Italy, for a diagnostic confirmation of BVDV PI and for clinical and hematological assessments to be shown to students of the Veterinary Medicine degree course. A week before arrival, the calf was clinically examined on the farm of origin and subjected to virological and bacteriological investigations. To this purpose, nasal swabs (NSs) collected from both nasal cavities with a dry sterile swab, a fecal swab (FS) collected directly from the rectum with a similar dry sterile swab and EDTA-treated blood and serum samples were collected to monitor the health of the calf and the concomitance of other viral and/or bacterial infections. In particular, NS, FS and EDTA-treated blood were tested in RT-qPCR for BCoV, BVDV, bovine respiratory syncytial virus (BRSV), bovine parainfluenza virus (BPiV), bovine adenovirus (BAdV), bovine herpesvirus type 1 (BoHV-1), Mannheimia haemolytica, Pasteurella multocida, Histophilus somni and Mycoplasma bovis. The serum sample was tested for antibodies detection using the ELISA test (Svanovir® BVDV-Ab, Boehringer Ingelheim Svanova, Uppsala, Sweden) and the neutralization test for BVDV and BoHV-1, respectively. The collected samples were immediately transported on ice to the laboratory of Infectious Diseases of the Department of Veterinary Medicine of Bari (Italy) and stored at −80 °C (NS, FS) and at RT (blood samples) before processing.\nThe calf, which arrived on 12 April 2021 (T0), was immediately hospitalized in the Infection Diseases Unit of the Veterinary Hospital in a separate room and subjected to clinical examinations on the day of arrival and then two times a day during the entire period of hospitalization (Italian Ministry of Health authorization n. 0017484-DGSAF-MDS-P). To minimize the stress and discomfort, the calf was kept in a pen with straw bedding, was fed a commercial concentrate twice daily and had access to haylage and water ad libitum. The facility was closed for other animals and had restricted admission for people. A trained animal technician and a veterinarian monitored the calf at least two times a day. To enter the animal room area, the investigators undressed and put on work clothes and boots. The presence of the following clinical signs was noted down: rectal temperature, pain and depression, nasal discharge, polypnea, coughing and diarrhea. Two days after arrival (T1), the calf showed hyperthermia (39.8 °C) and, consequently, NS, FS, EDTA-treated blood and serum samples were collected to monitor its health status and to assess the presence of concomitant virological/bacteriological infections. The presence of BVDV was confirmed in the blood, NS and FS, and BCoV was contextually detected in the NS. As a consequence, stool samples, NSs, serum samples and EDTA-treated blood were collected weekly up to 60 days (T60) until BCoV test were negative.","An 11-month-old calf was found to have Bovine Viral Diarrhea Virus (BVDV) and Bovine Coronavirus (BCov) and was brought to the veterinary hospital for testing. The calf was examined and samples were collected to check for other infections, and the results confirmed the presence of BVDV and BCov. The calf was isolated and monitored closely, and samples were taken weekly to track the virus. After 60 days, the calf was no longer testing positive for the viruses."
63,"The first patient is a 53-year-old male with a long history of paroxysmal atrial fibrillation. He had some cardiovascular risk factors, including high blood pressure that had been well controlled for 5 years on a therapy with ACE inhibitor. He did not suffer from other relevant comorbidities, except for sleep apnea syndrome on home nocturnal ventilatory support. He had his first episode of paroxysmal atrial fibrillation six years before and he started oral anticoagulation with dabigatran (CHA2DS2-VASC score 1) and antiarrhythmic therapy, initially with Flecainide and then with Amiodarone. Despite this, he had been admitted several times to the emergency room due to irregular heartbeat episodes and other symptoms, such as palpitations and shortness of breath. The arrhythmic recurrences often required electrical or pharmacological cardioversions to control the symptoms. The patient was then referred to our clinic because of the gradual worsening of his symptoms. The arrhythmic episodes lasted several hours, with spontaneous resolution, crippling him in his daily life. Considering his young age, the absence of cardiac structural alterations, and the inefficacy of the rhythm control strategy, AF ablation was proposed to the patient. At admission into the ward, the patient was overweight (body mass index was 38 kg/m2) and the electrocardiogram showed a normal sinus rhythm, while the pre-procedure echocardiography revealed dilated left atrium (left atrial volume 43 mL/m2) and the ejection fraction of the left ventricle of 55%.\nWe started the procedure by performing a bipolar map of the right atrium with a ThermoCool SmartTouch™ SF (Biosense Webster, Johnson & Johnson Medical S.p.a., Irvine, CA, USA) ablation catheter. First, we delineated the inferior and superior vena cava, and the coronary sinus, and we tagged in yellow the His potential (A). Next, we carefully reconstructed the interatrial septum and, finally, to precisely define the fossa ovalis (FO), we set the color range on the bipolar map to 0.25–0.75 mV and we tagged in light blue all the fragmented and low voltage signals, which are typical of the FO area [] (A). The FO location was further confirmed by the ICE and the CartoSound® module (Biosense Webster, Johnson & Johnson Medical S.p.a., Irvine, CA, USA). A 10F Soundstar™ Ultrasound catheter was advanced in the right atrium and pointed at the FO. Thanks to the CartoSound® module, it was possible to mark the contours of the FO location and to visualize them on the 3D EAM previously obtained (B). In addition to the FO, using ICE and the CartoSound® module, it was possible to assess and to reconstruct the structures of the left atrium (left atrial appendage, pulmonary veins, and esophagus). Next, a single transseptal puncture was performed using the bidirectional guiding sheath VIZIGO™ (8.5F) and a transseptal needle (standard Brockenbrough needle 98 cm from Abbott, St. Paul, MN, USA). Since the sheath is visible on the Carto 3® EAM system, it was possible to place it in the superior vena cava (3 to 4 cm above the cavoatrial junction) without any use of fluoroscopy. At this point, the guidewire was removed and the transseptal needle with the stylet was gently inserted and advanced through VIZIGO™, avoiding pushing the needle over the tip of the sheath. In this step, the needle and the sheath were held in the fingers between 4 and 6 o’clock. The needle and the sheath were pulled back caudally, visualizing the VIZIGO™ sheath with the 3D EAM system until it reached the level of the coronary sinus and the tip of the dilator inside the sheath placed in the FO. From this location, the VIZIGO™ sheath was pushed and placed on the FO under the guidance of the EAM system and confirmed by ICE imaging (C). When the sheath was in the right position, the transseptal needle was advanced to perform the puncture. Live tracking of the distal part of the needle on the 3D EAM system was possible thanks to an Alligator clip lead wires (FIAB Spa, Florence, Italy) connected to the distal portion of the needle (see ). Both the transseptal sheath and the needle should be advanced for 1–2 cm in the left atrium (LA). Then, holding the needle, the sheath was advanced over the needle. Finally, the needle and the dilator were kept steady, and the sheath was advanced over the dilator. The transseptal sheath was deaired and a 0.032 inch guidewire was advanced toward the left superior pulmonary vein under ICE guidance.\nAfter a successful transseptal puncture, the ThermoCool SmartTouch™ SF catheter was advanced through the VIZIGO™ sheath into the left atrium to perform a fast anatomical mapping and PVI following the CLOSE protocol []. With the help of the VIZIGO™ steerable sheath, we performed ablations with good contact and stability, leading to fast and effective PVI. There were no complications during the entire procedure. The procedure time was 100 min, radiofrequency time was 20 min, and, finally, the fluoroscopy time was 00:00 min:s. The patient was followed up 3 months after the procedure with an outpatient visit and electrocardiographic-Holter monitoring, which did not register any AF episode. After a 9 months follow-up, the patient presents no more symptoms, is not taking any antiarrhythmic drugs, and does not have recurrences of AF. He is taking apixaban 5 mg twice a day and ACE inhibitor drug for blood pressure control.","This 53-year-old man has had irregular heartbeats for years, which have required him to be hospitalized several times. Despite taking medications to control his heart rhythm, he continued to experience episodes that needed electrical shocks to correct. To address this, doctors decided to perform an ablation procedure to permanently stop the irregular heartbeats. During the procedure, a catheter was carefully inserted into his heart to identify and destroy the areas causing the problem. The procedure was successful, and he has not had any further irregular heartbeats since. He is now taking medication to prevent blood clots and manage his blood pressure."
64,"A 46-year-old male was referred to our clinic due to a long history of persistent atrial fibrillation. He had arterial hypertension and diabetes mellitus under good pharmacological control. He also had microcythemia due to a thalassemic trait. The atrial fibrillation was discovered seven years earlier with an electrocardiogram performed during a routine medical examination. The patient was asymptomatic for palpitations, irregular heartbeat, or other cardiological symptoms. An echocardiogram was performed without showing any pathological feature and oral anticoagulation with Dabigatran (CHA2DS2-VASC score 2) was started. After four weeks of therapy, electrical cardioversion was performed with effectiveness in restoring sinus rhythm. An antiarrhythmic therapy with Flecainide 100 mg twice daily was also initiated. The patient did not undergo further medical checks during the following five years, when, during a cardiological visit, the recurrence of the arrhythmia was discovered. The patient mentioned reduction in daily normal activity due to asthenia. An echocardiography exam showed a left atrial indexed volume of 36 mL/m2 and a left ventricular ejection fraction of 47%. AF ablation through PVI was proposed. At admission, the electrocardiogram showed AF. To perform an accurate anatomical reconstruction of the right and left atrium and a high-density voltage map, the multi-electrode mapping (MEM) catheter Pentaray™ (Biosense Webster, Johnson & Johnson Medical S.p.a., Irvine, CA, USA) was used. As in the previous case, we started by mapping the right atrium to define the inferior and superior vena cava, the His location, and the coronary sinus (A). Next, a decapolar catheter was inserted in the coronary sinus under 3D EAM guidance. Again, we mapped the FO looking for fragmented and low-voltage signals. However, this time, using the Pentaray™ catheter, we were able to achieve a higher signal resolution and to easily discriminate between a low-voltage and fragmented signal area, although the patient was in AF. Moreover, using the Pentaray™ catheter, we were able to acquire a detailed map in a shorter time (5 min with Pentaray™ catheter vs. 10 min with Thermocool SmartTouch™ SF catheter in the previous case). Again, based on the 3D EAM and under ICE guidance, a single transseptal puncture was performed with the VIZIGO™ sheath and the transseptal needle, both visible on the 3D EAM system (B,C). The transseptal puncture procedure followed the same steps already described in the previous case (video of the puncture is available in the ). The Pentaray™ catheter was then advanced in the left atrium through the VIZIGO™ sheath and a detailed fast anatomical mapping of the chamber and the veins was obtained (). The VIZIGO™ steerable sheath was extremely useful to map all the left atrium structures and veins very quickly. Moreover, using ICE imaging and the CartoSound® module, we could identify important structures, such as the pulmonary veins (A) and the esophagus (B), and to co-register the ultrasound images with the fast anatomical map obtained with CARTO 3®. The Pentaray™ catheter was then replaced by the ThermoCool SmartTouch™ SF catheter to perform PVI following the CLOSE protocol []. Again, with the help of the VIZIGO™ steerable sheath, we performed ablations with good contact and stability, leading to a fast and effective PVI. As in the previous case, the entire procedure was performed without fluoroscopy and without complications (procedural time was 110 min, radiofrequency time was 19 min, and fluoroscopy time was 00:00 min:s). The patient was followed up 3 months after the procedure with an outpatient visit, echocardiogram, and electrocardiographic-Holter monitoring, which did not register any AF episode. The echocardiogram showed a normal left ventricular ejection fraction (65%) and the patient mentioned that there was not a reduction in normal daily activities. After 6 months follow-up, the patient is taking low dose of flecainide (50 mg twice per day), apixaban 5 mg twice per day and has not recurrences of AF.","This 46-year-old man has a history of irregular heartbeats (atrial fibrillation) that was discovered several years ago. He also has high blood pressure and a condition that affects his red blood cells. After successfully restoring his heart rhythm with cardioversion, he started taking medication to prevent future episodes. However, the irregular heartbeats returned, and after further testing, a procedure called ablation was performed to permanently correct the problem. This involved mapping his heart chambers and using heat to block the electrical signals causing the irregular beats, and he is now doing well with medication and follow-up monitoring."
65,"A 20-year-old Caucasian male (1.75 m tall and 76 kg (BMI 24.8)), was admitted to the medical department for persistent hyperpyrexia, severe sore throat, dyspnea, and impaired consciousness with stupor. Persistent symptoms started at home 4 days before and he assumed clarithromycin as empiric antibiotic therapy. The physical examination showed jaundice, dry mucous membranes, pharyngeal hyperemia in the tonsillar region and soft palate, and left laterocervical lymphadenopathy. He was tachypneic (respiratory rate of 30 breaths per minute) and the peripheral oxygen saturation (SpO2) in room air was 92%. The abdominal palpation revealed hepatosplenomegaly. The laboratory tests showed a white blood count (WBC) of 8000 cells/mcL with 74% neutrophils, thrombocytopenia (platelet count of 31,000/mcL), total bilirubin 5.8 mg/dL, C-Reactive Protein (CRP) 43 mg/L, creatinine 0.9 mg/dL, AST 150 UI/L, ALT 79 UI/L. The nasopharyngeal swab testing for SARS-CoV-2 was negative (RT-PCR). Blood cultures were carried out upon admission and a full-body computer tomography (CT) was performed on the second day of hospitalization. The CT showed ground glass bilateral pulmonary alterations, pericardial effusion, mediastinal lymphadenopathy, and hepatosplenomegaly ().\nThe neck CT scan with intravenous contrast evidenced a 5.4 cm retropharyngeal abscess with associated thrombosis of the left anterior jugular vein ().\nOn the second day of hospitalization, the microbiology laboratory communicated the early identification of Fusobacterium necrophorum grown in blood cultures by MALDI-TOF (Matrix Assisted Laser Desorption Ionization Time-of-Flight) spectrometry -Vitek ®MS Blood cultures performed at admission and on the second day and collected in standard anaerobic blood culture bottles were positive ().\nThe association of retropharyngeal abscess with thrombosis of the anterior jugular and involvement of other organs systemically and the microbiology led to the diagnosis of Lemierre’s syndrome. Despite empirical antibiotic therapy with piperacillin/tazobactam (18 g/day, continuous infusion), there was a rapid worsening of the clinical conditions with further impairment of the respiratory failure and severe hemodynamic alterations (mean arterial pressure (MAP) constantly <65 mmHg). This picture suggested a progression towards a septic shock status. Consequently, the patient was referred to the Intensive Care Unit (ICU).\nA multimodal approach was the core of the ICU management of Lemierre’s septic syndrome. It included source control, antibiotic therapy, anticoagulant therapy, hemodynamic support, and continuous renal replacement therapy (CRRT) ().\nThe source control was planned in a sterile environment. Under general anesthesia, the patient underwent an explorative puncture of the retropharyngeal abscess and surgical tracheostomy for airway protection. Unfortunately, although the procedure revealed purulent material, surgeons were unable to collect suitable samples for microbiological testing.\nAt ICU admission, the patient showed a clinical and radiological picture of acute lung injury and the need for deep sedation (propofol, remifentanil, and clonidine), and mechanical ventilation through protective ventilation (tidal volume 6 mL/kg and driving pressure < 15 cmH2O) and high FiO2 (80%). The sequential organ failure assessment (SOFA) score was 12, predicting a high mortality rate (50%) [].\nAbout antibiotic therapy, metronidazole (500 mg q8hr, intravenously) was added to piperacillin/tazobactam (started empirically at hospital admission). This antibiotic therapy was based on anecdotal clinical evidence because, due to the lack of cards for antimicrobial susceptibility testing of anaerobes in the laboratory, the antibiogram was not performed.\nHemodynamic support was based on the synergic association of norepinephrine and the non-adrenergic vasopressor argipressin (also known as arginine-vasopressin or anti-diuretic hormone) (EMPRESSIN® AOP Orphan Pharmaceutical Group) starting with norepinephrine at 0.3 mcg/kg/min and argipressin at 0.03 IU/min to maintain a MAP > 65 mmHg and lactic acid < 2 mmol/L. On day 2, the drug synergism allowed the rapid reduction of the norepinephrine and argipressin dosages (0.1 mcg/kg/min and 0.025 IU/min, respectively) ().\nOn the same day (day 2), there was an enhancement in the clinical conditions, and the biomarkers of inflammation and organ failure improved ().\nDespite the adequate MAP (>65 mmHg), from the 3rd to the 6th day of ICU admission, there was a new worsening of the clinical status. It featured anemia (worse value 6.8 g/dL) without evidence of a hemorrhagic source. The hemodynamic monitoring (Edwards EV1000™ device) showed a hyperdynamic septic state (Cardiac Output 15 L/min, Systemic Vascular Resistance 300 dynes/seconds/cm). Due to the worsening of the sepsis-related organ damage, on the 8th day, hydrocortisone (200 mg/day) was added to argipressin and norepinephrine. This approach led to a paramount hemodynamic improvement. From the 8th day of hospitalization, there was a progressive reduction of the dose of both vasoconstrictors until the suspension of norepinephrine, on day 9, and argipressin, on day 12 of ICU stay. Moreover, there was an important decrease in the Sequential Organ Failure Assessment (SOFA) score (from 12 on ICU admission to 7 on day 9).\nThe worsening of the clinical status and the anemia were associated with bilirubin and cytokines rise. The inflammatory response was treated by 4 cycles of continuous hemofiltration (from 4th to 7th day). At the end of the procedure, there was an unexpected rise in D-dimers and in bilirubin values, up to 15.3 mg/dL (10th day), as well as hyperpyrexia. Nevertheless, these findings improved within 24 h.\nHeparin therapy was administered throughout the entire stay according to thrombocytopenia (enoxaparin 6000 U/day at the beginning and 4000 U twice a day after normalization of the platelet count) monitoring D-dimer values and avoiding intravascular coagulopathy.\nOn the 9th day, a chest tube was placed for the drainage of lung pleural effusion and a new CT was carried out. It showed a persistence of the known injuries of the lungs, liver, and spleen despite the paramount improvements of laboratory and clinical values, as well as the normalization of hemodynamic parameters at monitoring. The patient was discharged to the ward on the 17th day to continue the antibiotic therapy and the rehabilitation program. After the discharge to the rehabilitation care unit, the young man was in good health condition. At the first follow up, performed 15 days after ICU admission, the CT scan showed better lungs ventilation, absence of retropharyngeal abscess, laterocervical lymphadenopathy, and internal jugular thrombosis. Hepatosplenomegaly and some cavitary lungs lesions were found. Another thoracic CT scan was performed after 3 months. It demonstrated an improvement of both the lung lesions and hepatosplenomegaly.","This 20-year-old man was admitted to the hospital after developing a severe infection that started with a sore throat and fever. Tests revealed a serious abscess in his neck, along with signs of infection in his liver and spleen, and a blood clot in a vein in his neck. He was treated with strong antibiotics and other medications to support his breathing and blood pressure. Despite the treatment, his condition worsened, and he needed a breathing tube and support from a dialysis machine. Doctors eventually performed a procedure to drain the abscess and improve his breathing. With continued treatment, including antibiotics, supportive care, and a gradual reduction in medications, his condition improved significantly. He was eventually able to be weaned off the breathing tube and other support systems and discharged home to continue his recovery. Follow-up scans showed improvement in the areas of infection, and he is now on a rehabilitation program to help him regain strength and function."
66,"A 34-year-old male presented with a 6 mm bluish nodule, slowly growing on his forehead. An excisional biopsy was performed, which revealed a pigmented lesion with rare mitotic figures and multiple microscopic satellites, extending into fat (Clark level V) to a depth of at least 4 mm (). Sentinel lymph node biopsy was negative for neoplasm. Immunohistochemical stains for Melan-A and HMB-45 were diffusely reactive and -catenin showed non-specific cytoplasmic staining. Ki-67 demonstrated a low proliferative index (<5% in tumor cells). Four-color in situ hybridization was performed to rule out melanoma which showed normal results. Fusion analysis for 104 using targeted RNA sequencing related genes did not reveal any gene rearrangements including PRKCA and PRKAR1A. Targeted mutation analysis for over 50 cancer-related genes showed GNA11 c.626A>T p.Q209L oncogenic mutation. Finally, whole-genome DNA methylation profiling and t-Distributed Stochastic Neighbor Embedding (t-SNE) cluster analysis were performed as described above. Genome-wide copy number profiles determined from the DNA methylation data failed to reveal significant copy cumber changes (A). t-SNE cluster analysis matched our case to the group of melanocytomas (B). Methylation profiling of tumors offers highly efficient and reliable information for classification of tumors and future studies aiming to explore the optimal use of this technique will warrant improved diagnostic and management approaches for pigmented lesions when there is a concern for malignancy.","A 34-year-old man had a small, growing bluish spot on his forehead that was removed for examination. The biopsy showed it was a type of pigmented skin lesion that had grown deep into the skin and spread to the surrounding fat. Tests ruled out cancer, but identified a specific genetic mutation that explains the growth of this unusual lesion. The doctor believes this finding will help in understanding and managing similar cases in the future."
67,"A 61-year-old woman with confirmed COVID-19 was admitted to a different hospital with a productive cough experienced for a few days. She required invasive assisted ventilation shortly after admission. Her medical history included rheumatoid arthritis, obstructive sleep apnea, and arterial hypertension. Because of progressive severe acute respiratory distress syndrome (ARDS), the patient was transferred to our tertiary care hospital for kinetic therapy (prone positioning for at least 12 h per day) and continuous renal replacement therapy (CRRT) due to oliguric acute kidney injury (AKI) (A). Because of progressive hypoxemia, venovenous ECMO therapy was initiated 13 days after admission to our hospital (A). Subsequently, nasopharyngeal swabs and tracheal aspirates tested negative for SARS-CoV-2. After tracheotomy and weaning, ECMO therapy and invasive assisted ventilation were no longer required, but the patient still needed intermittent renal replacement therapy (IRRT) (A).\nDuring the course of the disease, the patient developed laboratory signs of liver injury during ECMO therapy before the clinical appearance of jaundice with elevated bilirubin levels, but sustained synthetic liver function reflected by the international normalized ratio (INR) and serum albumin measurements (B–E). A diagnosis of SSC-CIP was confirmed by endoscopic retrograde cholangiopancreatography (ERCP), showing intraductal filling defects in the intrahepatic bile ducts due to biliary casts. In addition, the patient received drugs that have previously been associated with SSC, including amoxicillin-clavulanate, and ketamine sedation [,,]. Plasma levels of bilirubin and ammonia gradually increased after that, with stable liver synthesis reflected by normal values of the international normalized ratio (INR) without substituting coagulation factors (D,E).\nNevertheless, the patient developed progressive nausea, vomiting, weakness, and exhaustion as the disease progressed. Hepatic encephalopathy was treated with lactulose and rifaximin, but clinical symptoms worsened (A). Based on these observations, hemadsorption using the CytoSorb hemoperfusion device (CytoSorbents Europe, Berlin, Germany) was used in combination with IRRT (6 treatments within 7 days and 8–12 h per session). In this patient with SSC following ECMO therapy and severe COVID-19, hemadsorption successfully eliminated bilirubin, ammonia, and C-reactive protein (CRP) levels, while serum albumin levels remained stable (A). Direct comparison revealed that bilirubin was less efficiently eliminated by hemadsorption by 33% and 56% after 2 and 6 treatments, respectively (B) compared to ammonia by 65% and 74% after 2 and 6 treatments, respectively (C). During successful elimination of bilirubin and ammonia, clinical symptoms of nausea, vomiting, weakness, and exhaustion improved. Following a continuation of IRRT but termination of hemadsorption therapy, bilirubin (32.2 mg/dL) and ammonia levels (208 g/dL) increased again, worsening clinical symptoms within 20 days. The patient suddenly died due to cardiac arrhythmia before liver transplantation evaluation was initiated.","61-year-old woman admitted to the hospital with COVID-19 and a bad cough, requiring a breathing machine. She also had other health problems like arthritis and kidney issues, and her lungs became severely damaged. To help her breathe, doctors used a special positioning technique and kidney dialysis, and eventually, a heart and lung support machine. Despite these efforts, she developed complications including liver problems and difficulty digesting food, but a treatment called hemadsorption helped improve her condition temporarily before she sadly passed away."
68,"A patient of Ukrainian origin (UKR29) was born after the first normal pregnancy (39 week of gestation) from a healthy 27 year old mother and 32 year old father. At birth, the child was registered as a male. Birth weight was 3500 g and length was 53 cm. At the age of two months, the patient was examined due to hypospadias and bilateral cryptorchidism. At that time hormonal analysis was performed. At the age of 14 months a comprehensive examination, such as karyotyping, urological examination (including gonadal and pelvic ultrasound and MRI investigation) and hormonal analysis (including testosterone synthesis stimulation test) were performed. The patient’s psychological development was normal. Neither signs of Wilms’ Tumour nor renal anomalies were found in the patient.\nInformed consent was obtained from the patient’s parents. Ethical approval for this study was obtained from the Committee on Bioethics of the Institute of Molecular Biology and Genetics of National Academy of Sciences of Ukraine, protocol No. 2 (30 April 2013).","This young boy, born healthy with a good birth weight, had some issues discovered when he was two months old – he had hypospadias (a birth defect where the opening of the urethra isn't at the tip of the penis) and his testicles hadn’t dropped into his scrotum. To investigate these concerns, doctors performed several tests, including genetic testing and scans, and found no evidence of cancer or other kidney problems. His development was normal, and he’s growing up healthy. The parents gave their consent for the tests, and the study was approved by a research ethics committee."
69,"A 60-year-old female patient with a medical history of hypertension came to our attention because of several neurological deficits that had developed over the last few years, significantly impairing her daily life. Four years earlier, she developed sudden weakness and hypoesthesia of the right hand. The symptoms resolved in a few days and no specific diagnostic tests were performed. Two months later, she developed hypoesthesia and weakness of the right lower limb. On neurological examination at the time, she had spastic gait, ataxia, slight pronation of the right upper limb and bilateral Babinski sign. Brain MRI showed extensive white matter hyperintensities (WMHs), so leukodystrophy was suspected. However, these WMHs were located bilaterally in the corona radiata, basal ganglia, the anterior part of the temporal lobes and the medium cerebellar peduncle (A–D), and were highly suggestive of CADASIL. Genetic testing was performed, showing heterozygous mutation of the NOTCH3 gene (c.994 C<T; exon 6). The diagnosis of CADASIL was confirmed and antiplatelet prevention therapy was started. Since then, her clinical conditions remained stable, and the lesion load was unchanged at follow-up brain MRIs for 4 years until November 2020, when the patient was diagnosed with COVID-19 after a PCR nasal swab. The patient developed only mild respiratory symptoms, not requiring hospitalization or any specific treatment. Fifteen days after the COVID-19 diagnosis, she suddenly developed aphasia, agraphia and worsened right upper limb motor deficit, but she did not seek medical attention. Some days later, she reported these symptoms to her family medical doctor, and a new brain MRI was performed, showing a subacute ischemic area in the left corona radiata (E,F). Therapy with acetylsalicylic acid was switched to clopidogrel as secondary prevention, while her symptoms improved in the next few weeks. The patient underwent a carotid doppler ultrasound and an echocardiogram, which did not reveal any pathological changes. The review of the blood pressure log, both in-hospital and the personal one the patient had kept, excluded uncontrolled hypertension.","60-year-old woman with high blood pressure developed neurological problems over several years, including weakness and numbness in her hand and leg. Brain scans showed unusual white matter changes, leading to a diagnosis of CADASIL, a genetic condition. She recovered from COVID-19, but then experienced a stroke in her brain, causing speech and movement difficulties. After further testing, doctors found no problems with her blood pressure or blood vessels, and started her on a new medication to prevent future strokes."
70,"A 61-year-old male patient was referred for hearing rehabilitation with CI on the left side because of progressive asymmetric hearing loss (see ) and limited communication ability, restricting his professional performance as a dentist. WRS on the left side with a hearing aid was 20%, and on the right side, 50% (FMT at 65 dB SPL).\nContrast-enhanced cMRI revealed an intra- and extracanalicular VS (Samii T2) (A,B). Possible treatment options before cochlear implantation included tumor removal via a retrosigmoid approach or SRS as first-line therapy. The patient decided on the first option. Intraoperatively, the vestibulocochlear and facial nerves were preserved as the functionality was monitored with electrophysiologic monitoring (neuromonitoring). cMRI demonstrated a small residual IC VS 6 months postoperatively, and the patient decided to undergo SRS before the cochlear implantation. The SRS was performed during a single session with 13 Gy (70% Isodose; Dmax 18.6 Gy). The implantation was performed successfully six weeks later. The CI was placed more posterior than usual to minimize the artifacts in postoperative MRI scans []. Six months after CI surgery, the first postoperative MRI was performed using 1.5 Tesla MR with medium bandwidth (see C,D for post-CI MRI; the VS is marked with green arrows). A,B demonstrates the patient with the Rondo 2 speech processor (images used with the patient’s approval).\nOne month after implantation, aided WRS with CI on the left side (and masking of the right side) was 45% (FMT at 65 dB SPL) and six months later, 60%. Binaural hearing with CI on the left side and hearing aid on the right side resulted in an aided WRS of 90% (FMT) with CI only after two years.\nHearing in noise was measured with the aided Oldenburg Sentence test (OLSA). One year postoperatively, this test revealed scores of −1.1 dB signal-to-noise ratio (SNR) for the left side with CI (with masking of the right ear) and −3 dB with CI and hearing aid on the right side, indicating an improvement of hearing in noise with CI.","This 61-year-old man was referred for hearing rehabilitation because he had significant hearing loss that was affecting his ability to work as a dentist. Tests showed a tumor in his ear was causing the hearing loss. After considering surgery, he chose to have the tumor removed, and then a cochlear implant was placed in his left ear. Over time, the implant significantly improved his hearing, allowing him to communicate more easily and return to his profession."
71,"A 76-year-old male complained of bilateral progressive hearing loss for approximately 35 years (see ) and recurrent acute hearing loss on both sides. He reported no tinnitus or vertigo. The patient had a profound hearing loss on the left side with an aided WRS of 20% at 65 dB SPL (FMT) and 0% on the right side with bilateral hearing aids. The hearing nerve integrity was tested with an electrode in the external auditory meatus; the patient could hear humming when the amperage of 531 µA was applied.\nCranial MRI performed during evaluation for implantation revealed a multilocular schwannoma on the right side: small IC VS (T1) and a small intracochlear schwannoma (A,B). The case was discussed during the meeting of the Interdisciplinary Skull Base Board. The debated tumor treatment options included resection with a translabyrinthine approach, CyberKnife radiosurgery, or watch-and-scan. All three options were proposed and explained in detail to the patient. In addition, the patient was offered cochlear implantation on the contralateral ear with residual hearing. After presenting possible therapy options for tumor treatment and auditory rehabilitation with CI, the patient decided to treat both tumors using CyberKnife radiosurgery (13 Gy, 70% Isodose; Dmax 18.6 Gy) and opted out from cochlear implantation on the contralateral left ear. One and a half years after the CyberKnife treatment, following two cMRI examinations demonstrating stable tumor (D), the patient opted for cochlear implantation on the right side. Two years later, as he was very satisfied with the right ear’s auditory outcome, he opted for CI on the left ear. Twelve months postoperatively, the patient had an aided WRS of 35% (FMT) on the right side. He uses the CI over 10 h daily and has received the second CI two years after the first one. One year postoperatively, OLSA test revealed a 4.6 dB signal-to-noise ratio (SNR) with CI on the right side and hearing aid on the left side and 11.3 dB for the right side with CI (with masking of the left ear).","This 76-year-old man has had hearing loss for many years, and it’s gotten worse recently. Tests revealed two small tumors in his ears that were causing the hearing loss. He chose to have the tumors treated with radiation therapy, which worked well. After radiation, he received cochlear implants in both ears, which significantly improved his hearing and he now uses them for over 10 hours a day."
72,"A 57-year-old female patient presented with IC VS on the right side with profound hearing loss after SRS. At the age of 4, she had mumps resulting in a profound sensorineural hearing loss on the left side. An earlier CI evaluation revealed a negative promontory test on the left side. In 2010, she developed hearing loss on the right side, and IC VS was detected using cMRI. SRS was performed in 2019 in a different hospital (3 × 6 Gy) to stop tumor progression and prevent further hearing loss (A: pre-therapeutic PTA). Unfortunately, the hearing loss progressed (B), and by August 2020, aided WRS with a hearing aid was 0% on the right side. Therefore, after cMRI demonstrated a stable tumor, the patient decided on hearing rehabilitation with CI on the right side. The implantation was performed in our unit in November 2020 without complications. Two months after CI, aided PTA improved remarkably (C), and the patient understood 90% of the monosyllables at 65 dB (FMT), remaining on that level six months after implantation.\nHearing in noise was postoperatively measured with the aided Oldenburg Sentence test (OLSA). One year postoperatively, the patient scored 1.5 dB signal-to-noise ratio (SNR) with unilateral CI.","This 57-year-old woman has had significant hearing loss since childhood due to a tumor in her brain. She had surgery in 2019 to try to stop the tumor from growing and causing more hearing loss, but unfortunately, her hearing continued to decline. After further tests, she decided to try a cochlear implant (CI) in her right ear, which was successfully implanted in November Since the implant, her hearing has improved significantly, and she can now understand about 90% of what is said to her."
73,"A 74-year old, right-handed female presented with anxiety and depressive symptoms to the psychiatric ED at the University Hospital of Geneva (HUG, Switzerland) in 2012, after SA by abuse of acetaminophen. Although showing depressive symptoms, according to the Diagnostic and Statistical Manual of Mental Disorders (5th ed., DSM-V) [], the latter could not be classified as a major episode of depression (MDD). She had suffered a circumscribed ischemic stroke two years earlier, which had left her with incomplete Broca’s aphasia and dysprosody. By “incomplete” we mean two things here: (i) the fact that the severity of the speech impairment fluctuated over time, leaving the patient with better abilities on some days and worse on others, and (ii) the fact that the patient always retained some capability to express very simple words and phrases. However, due to the patient’s refusal to undergo testing with psychometric scales, we were unable to assess the severity of this deficit through the use of more objective means, such as battery scores.\nThe examining physicians, becoming aware of the specific symptoms, retrospectively examined her file (after the consent of the patient and later of her husband), which contained all the clinical, laboratory, and imaging elements that had been collected.\nThe patient had no family or personal history of psychiatric diseases, nor SI/SB, prior to the onset of her language impairments. Her only other somatic complaint was hypertension. Her family was very supportive and consisted of a husband, three children, and several grand-children. The patient was bilingual in Italian and French and had been working as a writer and translator.\nAfter hospitalization in a psychiatric unit, a cerebral MRI was performed, revealing a diffuse white matter high-signal hyper-intensity in the left posteroinferior portion of the frontal lobe, just anterior to motor cortex. Small white matter high-signal hyper-intensities were also located in the frontal lobe of the right hemisphere. A neuropsychological investigation was not possible because of the patient’s refusal.\nThe patient was started on psychotropic treatment with the antidepressant sertraline (increasing the dose gradually to 50 mg/day) and, with an anxiolytic purpose, quetiapine (12.5 mg × 3 per day), a drug that with higher posology is mainly prescribed to treat bipolar disorder and schizophrenia. She was treated by a multidisciplinary team of psychiatrists, neurologists, psychologists, and speech therapists. Although the patient showed gradual improvements, both with regard to the anxious and depressive symptomatologies, we observed marked deteriorations in her psychiatric (increased anxiety and depressive symptoms) and neurological patterns (greater difficulty in finding words and more incongruent prosody) whenever she was asked to describe her situation and emotions to the best of her abilities. We also observed a marked improvement in her language expression abilities once her anxiety and depressive symptoms were controlled. The patient described feeling powerless after the onset of her language deficit, which, having been a professional a professional in the field of language, represented an immense and burdensome impairment for her, eventually leading to her SI and SA. The multidisciplinary team worked to offer her other means of expression besides language, and to mobilize her emotions by employing a range of approaches (art therapy, music therapy, psychomotricity, garden therapy, and pet therapy). Therapists attempted to elicit the activation and expression of complex emotional responses by showing her photographs, both generic and of the patient’s family, by engaging the patient in small “role-playing games” that were interesting to the patient, and by encouraging her to use words in songs that she had memorized during her youth, and to which she was emotionally and affectively linked.\nWhile SI was fluctuant initially, after 6 months the patient no longer had any SI and showed an overall improvement. She was therefore discharged and referred to an outpatient facility where she continued treatment for about one year. Then, stating that she was feeling much better, the patient decided to no longer continue her treatment and also wanted to stop attending any sessions with both psychiatrist and speech therapists, contrary to medical advice at the time. Soon after, her anxious and depressive symptoms reappeared, her language expression deteriorated, and, 6 months after stopping her outpatient treatment, the patient performed suicide by drowning in a lake.","This 74-year-old woman came to the hospital after attempting suicide with acetaminophen. While she was feeling anxious and depressed, doctors couldn't officially diagnose a major depression episode due to her speech difficulties from a previous stroke. She had trouble finding words and expressing herself clearly, which made her feel powerless and contributed to her suicidal thoughts. Doctors treated her with medication and therapies like art and music to help manage her emotions and find new ways to communicate, but sadly, her symptoms returned and she eventually died by suicide."
74,"The mother, 34-years old, primigravida (G0P0), underwent all recommended tests. The first-trimester morphology scan revealed normal crown-rump length, visible nasal bone, and normal nuchal translucency value. Moreover, the double marker for chromosomal aneuploidies (13, 18, and 21) indicated a low-level risk. The TORCH IgM and IgG screening showed no acute or recent infection (negative IgM), and the IgG titer was high. The woman had not been previously exposed to harmful factors that would have justified placing the pregnancy in the high-risk category. The second-trimester morphology scan performed at 22 weeks confirmed the normal development of a female fetus.\nHowever, at 33 weeks of pregnancy, the first abnormal sign was noted. The amniotic fluid quantity started to increase, leading to the diagnosis of polyhydramnios. Another visible alteration was the shape and position of the lower fetal limbs, indicating minor clubfoot and altered fetal biophysical profile. By the time the pregnancy reached 36 weeks, the biophysical variables were severely modified. The fetal heart rate monitored using the non-stress test was worrying. There were significant decelerations, abnormal fetal movement, and poor muscular tonus. Additionally, the quantity of amniotic fluid continued to rise. Cumulatively, these observations led to the decision to deliver the baby prematurely via emergency C-section, 36 weeks into the pregnancy.\nThe C-section was uneventful, and the mother made a fast recovery, but the female newborn weighing 2200 g received an APGAR score of 3. Unfortunately, when thoroughly examined by our team, it was noticeable that the fetus’s movement, breathing, and swallowing capacity were impaired, and she was unable to sustain spontaneous breathing. The newborn was constantly and fully dependent on assisted mechanical ventilation. Her condition continued to deteriorate despite all the efforts. Unfortunately, at two months of age, the baby succumbed to respiratory failure and multiple associated complications.\nBased on the clinical signs and paraclinical tests, we were able to establish the following diagnostics: generalized congenital muscular atony, right diaphragmatic hernia, cerebral atrophy, neonatal anemia, bilateral varus equinus, neonatal hypocalcemia, prematurity and low birth weight, ostium secundum atrial heart defect, and tricuspid valve dysplasia.\nThoracic X-rays show reduced ribcage expansion of the right hemithorax, suggestive of right diaphragmatic hernia. The transfontanelar ultrasound and head CT showed moderate cerebral atrophy, mostly in the frontal lobe. The generalized and severe muscular hypotonia was investigated using a muscular biopsy that showed no significant alterations. Both tests for aminoacidopathies and spinal muscular atrophy were negative. Serum levels of creatine kinase (CK) and lactate dehydrogenase (LDH) were high but with a tendency to normalize. The karyotype showed a normal profile 46XX.\nGiven the multitude of heterogenic clinical symptoms, we suspected a genetic syndrome yet to be diagnosed, so we proceeded to perform an Array Comparative Genome Hybridization (aCGH) with Single Nucleotide Polymorphism (SNP). aCGH+SNP was conducted on a blade with 4 ∗ 180,000 (180 K) samples (110.112 CGH samples, 59.647 SNP samples, 3000 replicated samples and 8121 control samples) covering the entire human genome with a spatial resolution of ~25.3 kb DNA (G4890A, design ID: 029830, UCSC hg19, Agilent). The scans were interpreted with the CytoGenomics Agilent software, using standard interpretation parameters with a SureScan Microarray Scanner. The resulting profile was abnormal: there were three areas associated with loss of heterozygosity on chromosomes 1 (q25.1–q25.3) of 6115 kb, 5 (p15.2–p15.1) of 2589 kb and 8 (q11.21–q11.23) of 4830 kb, a duplication of 1104 kb on chromosome 10 in the position q11.22, and duplication of 1193 kb on chromosome 16 in the position p11.2p11.1.\nConsidering this abnormal genetic profile, the parental couple received genetic counseling. Furthermore, we continued to test both partners through Next Generation Sequencing (NGS) by Illumina.\nThe results confirmed the following abnormal genetic profile; TTN (NM_001267550.1, sequencing): heterozygous variant on Chr2(GRCh37):g.179479653G>C—TTN variant c.48681C>G p.(Tyr16227*)—exon 260, heterozygous variant on Chr2(GRCh37):g.179396832_179396833del—TTN variant c.104509_104510del p.(Leu34837Glufs*12)—exon 358 (TTN: NM_001267550.1—reference sequence). The TTN variant c.48681C>G p.(Tyr16227*) creates a premature stop codon. Sanger sequencing also confirmed this variant and was classified as likely pathogenic (class 2). The TTN variant c.104509_104510del p.(Leu34837Glufs*12) creates a shift in the reading frame starting at codon 34837. The new reading frame ends in a stop codon 11 positions downstream. This variant has been confirmed by Sanger sequencing, and it is also classified as likely pathogenic (class 2) ().\nIn light of the clinical outcomes, based on the previous unfortunate experience, the couple agreed to receive genetic counseling. The couple was advised to pursue in vitro fertilization (IVF) with preimplantation genetic testing (PGT-M). Considering the mother’s age and weight, her Anti-Müllerian hormone (AMH) serum level and antral follicle count, we used a short-antagonist ovarian stimulation (OS) protocol with 200 UI of FSH (follitropin beta) concomitantly with 150 UI of combined FSH and LH (menotropin). Ten days later, seven oocytes were retrieved through transvaginal, sonographically controlled follicle puncture. Five of them were injected through intracytoplasmic intracytoplasmatic sperm injection (ICSI), resulting in two blastocysts. The embryonic biopsy was performed on day 6 of the blastocyst stage for these two embryos ().\nThe amplification of the entire genome was performed using the SurePlex DNA Amplification System by Illumina Inc. 2018, California US. Using the BlueFuse Multi Analysis Software (Illumina Inc. 2018, San Diego, CA, USA), all 24 chromosomes were detected euploid for embryos. The identification of the mutation TTN gene on exon 358 (father’s mutation) and exon 260 (mother’s mutation) was performed only for euploid embryos using Sanger sequencing with specific primers on ABI 3500. PCR products for both embryos were purified and sequenced in both senses with a BigDye Terminator v3.1 Cycle Sequencing Kit by Thermo Fisher Scientific. Specific primers were manually designed according to both mutations and tested afterwards using blood samples from the parents. Both embryos tested by PGT-A were euploid. One of them was a carrier of the mother’s mutation c.48681C>G p.(Tyr16227), exon 260, and the other was a wild type (WT) for both mutations ().\nWe performed a frozen-thawed embryo transfer in the following cycle, transferring the WT euploid embryo after endometrial preparation with exogenous estrogen. The result was positive, and we confirmed the ongoing viable pregnancy via ultrasound 14 days after.\nThroughout the pregnancy, we performed the non-invasive double marker test (low-risk result) and fetal DNA analysis using maternal blood (low-risk result) and an invasive amniocentesis at 17 weeks of gestation, indicating a normal genetic profile. To test whether or not the second fetus presents a genetic abnormality, we extracted the DNA directly from the amniotic fluid. Targeted sequencing was performed on both DNA strands of the relevant TTN region. The reference sequence is TTN: NM_001267550.2. To exclude maternal cell contamination (MCC), we analyzed 15 STR autosomal markers plus amelogenin using the PowerPlex 16HS multiplex kit (Promega, Madison, Wisconsin, USA). Moreover, all the non-invasive ultrasound scans showed a normal growth rate and organ development. The evolution of the pregnancy was uneventful, and at 38 weeks, we carried out the C-section delivery of a healthy female baby of 2990 g, receiving an APGAR score of 9.","Summary: This baby girl was born prematurely at 36 weeks due to complications during her mother’s pregnancy, including fluid buildup around the baby and concerning heart rate patterns. Sadly, the baby struggled to breathe and passed away a few months later due to severe breathing problems. The doctors discovered a genetic condition affecting the TTN gene, which caused muscle weakness and other issues. Through genetic testing and careful planning, the parents were able to use preimplantation genetic testing (PGT) to select a healthy embryo for transfer, resulting in a successful pregnancy and the birth of a healthy baby girl at 38 weeks."
75,"A 40-year-old male engineer, former professional rugby player, was referred to our clinic with a left heel inflammatory pain that was worsening during jogging or trailing. The symptoms started seven months before and the patient presented to a regional local hospital for investigations where a plain radiography was performed and a simple bone cyst diagnosis was suspected. Patient was recommended a break from physical activity for six months and non-steroid anti-inflammatories drugs (NSAIDS) to ameliorate pain. The pain increased gradually and he started to complain of swelling. The pain was hardly controlled with NSAIDS and non-morphinic analgesics.\nOur clinical examination revealed a mild tenderness in the posterior foot, including the ankle and the heel, without evidence of a palpable mass. Ankle and subtalar joint mobilities were limited. The laboratory blood tests and urine analysis results were normal.\nRadiography revealed a benign-appearing bone lesion of 16 × 19 mm within the anteroinferior part of the calcaneum which was well defined, radiolucent, almost entirely homogeneous with a small central sclerotic focus-“Cockade sign” [,], describing the classical appearance of a calcaneal intraosseous lipoma (). We also performed and MRI exam that showed a focal lesion, hyperintense on both T1 and T2 weighted images, and isointense with fatty tissues (a,b). There was a discreet focal attenuation in the center of the lesion, on T2* sequence, suggestive for focal calcification (c). The MRI aspect corresponded to a Milgram type II intraosseous lipoma (predominantly fatty lesions with central necrosis/calcifications/ ossifications) [].\nSurgery was performed and a direct lateral approach to the calcaneum was chosen. The saphenous nerve and the long peroneus tendon were reclined superiorly and distally (a). A bone window was performed immediate distally to the lateral tubercle of the calcaneum. Aggressive curettage of the lesion was carried out and the intralesional samples were send to the histopathological exam (b). The cavity was filled with a medium viscosity poly (methyl methacrylate)-based bone cement with gentamycin. No perioperative complications occurred.\nThe histopathological exam () and immunohistochemistry (S100 and Vimentin positive) confirmed the diagnosis, a stage II Milgram intraosseous lipoma.\nPostoperatively, the pain subsided completely. The patient was discharged the second day after the surgery. Total weight bearing was allowed without crutches. No cast or brace was applied. Rehabilitation was conducted for two weeks in order to encourage full ankle mobilities.\nIn the follow-up, the patient was evaluated every six weeks during the first three months and every six months until the first two years after surgery. Starting from the first postoperative consultation at six weeks, the ankle joint mobilities were normal, the patient was painless, and radiological examination showed no modifications. At the time of the last follow-up at 24 months, no modification and no recurrence were observed (a,b) and the patient returned to work six weeks after the surgery.","A 40-year-old man with a history of rugby experienced persistent pain in his heel that worsened with activity. Initial tests suggested a bone cyst, but further imaging revealed a benign fatty tumor within his heel bone, called an intraosseous lipoma. Surgery was performed to remove the tumor, and it was confirmed through a tissue sample. Following the surgery, his pain completely disappeared, and he was able to return to work within six weeks."
76,"A 72-year-old white male with end-stage liver disease due to cryptogenic cirrhosis underwent a deceased-donor orthotopic liver transplantation (OLT) in 2010. The post-transplant immunosuppression (IS) regimen included tacrolimus 5 mg twice daily, prednisone 20 mg daily, and mycophenolate mofetil (MMF) 1000 mg twice daily.\nHe remained relatively well until seven years post-transplant when he presented with right foot pain and right lower extremity swelling for a duration of 4 weeks. Doppler ultrasound showed an acute right popliteal, tibial, and peroneal deep venous thrombosis for which he was prescribed apixaban. Two months after the initiation of anticoagulation therapy, he presented with a recurrence of right lower extremity swelling, increased pain and numbness, and a new weakening of the right foot. Further history revealed progressive fatigue, dyspnea, and a 30-pound weight loss over 3 months.\nA computed tomography (CT) of the chest showed scattered lung nodules with multiple areas of bilateral thoracic lymphadenopathy. A positron emission tomography (PET-CT) revealed the abnormal FDG uptake of numerous, sub-centimeter bilateral pulmonary nodules (max SUV 8.4) with mediastinal (1.6 cm, max SUV 13.4), bilateral hilar, right femoral, inguinal adenopathy, and abnormal FDG uptake in a soft tissue mass adjacent to the proximal right femur (5.8 × 3.9 cm2, max SUV 11.9) ().\nAn excisional biopsy of the soft tissue mass showed diffuse infiltration with atypical monomorphic lymphoid cells with large regions of necrosis (). By immunohistochemistry the tumor cells expressed CD3, CD4, CD30 (30%), and BCL-2, and were negative for CD5, CD8, CD10, CD20, CD21, TIA-1, perforin, T-cell receptor (TCR) gamma, and ALK-1. In situ hybridization for Epstein–Barr virus (EBV)-encoded RNA was negative and plasma EBV DNA was not detectable. The proliferation index estimated by Ki-67 staining was 70–80%. TCR gene rearrangement analysis showed a monoclonal band for both the TCR gamma and beta genes. A bone marrow biopsy showed normocellular bone marrow, with trilineage hematopoietic maturation, and no lymphoid aggregates or atypical lymphoid cells, but with a mononoclonal TCR rearrangement identical to that found in the soft tissue mass, consistent with a low-level involvement by T-cell lymphoma. He was diagnosed with stage IV CD30-positive T-cell PTLD, peripheral T-cell lymphoma (PTCL), which was not otherwise specified (NOS).\nAt the time of the PTLD diagnosis, IS therapy consisted only of tacrolimus 1 mg twice daily. After discussion with the transplant team, the tacrolimus dose was decreased to 0.5 mg twice daily, and CHOP-21 chemotherapy was initiated. However, based on the emerging ECHELON-2 outcomes with the addition of brentuximab vedotin (BV) to an anthracycline-based chemotherapy backbone in CD30-positive PTCL, after two cycles of CHOP, the patient’s chemotherapy regimen was changed to BV-CHP [,,]. BV was dosed at 1.8 mg/kg with CHP every 3 weeks with G-CSF support, according to the published ECHELON-2 regimen [,]. Cycle 3 (BV-CHP #1) was complicated by neutropenic sepsis, and BV was reduced to 1.3 mg/kg for cycles 4–6. Cycle 4 (BV-CHP #2) was again complicated by neutropenic sepsis, and cyclophosphamide and doxorubicin dosing were reduced by 25% for the remaining cycles 5 and 6. Interim PET-CT after cycle 4 showed a complete resolution of previously seen pulmonary metastases, a decrease in the mediastinal (1.3 cm, max SUV 9.3; from pre-treatment 1.6 cm, max SUV 13.4) and hilar adenopathy, and a near complete resolution of the soft tissue mass in the right anterior thigh (2.4 × 2.3 cm2, max SUV 1.68, from pre-treatment 5.8 × 3.9 cm2, max SUV 11.9), overall consistent with a partial response (PR). End-of-treatment PET-CT after cycle 6 showed stable disease in the mediastinal (1.3 cm, max SUV 10.25) and hilar adenopathy, and no new or recurrent adenopathy, with complete resolution of the previously seen right lower extremity soft tissue mass, consistent with ongoing PR (). Maintenance therapy with single-agent BV was discussed and started, but after one BV maintenance dose he was again admitted with a neutropenic febrile episode and the patient requested to stop all therapy, as he was satisfied with the achieved response.\nFollowing completion of front-line therapy, his course was complicated by elevations of alkaline phosphatase (peak 417 U/L 3 months post-treatment initiation, from pre-treatment 97 U/L) and AST (peak 75 U/L 11 months post-treatment initiation, from pre-treatment 33 U/L) levels. ALT remained within normal limits. A liver biopsy completed 8 months after treatment initiation showed minimal lobular inflammation, but no evidence of portal inflammation, bile duct injury, or endothelitis, as seen with acute cellular rejection, and no ductopenia, as seen with chronic rejection.\nDue to chemotherapy-induced thrombocytopenia and an episode of duodenal ulcer bleeding, his anticoagulation was briefly interrupted and then re-initiated after endoscopy clipping and the recovery of thrombocytopenia. His known DVTs were monitored with serial Doppler ultrasound, which demonstrated a chronically dilated, thrombosed right popliteal vein with post-thrombotic venous changes, representing chronic thrombosis despite being on anticoagulation. No additional complications were observed. Neutropenia and thrombocytopenia normalized 4 months after the last dose of BV, with mild persistent anemia (8–11 g/dL). He remains alive and well 25 months since his diagnosis without clinical or radiographic evidence of progression.","This 72-year-old man was diagnosed with a type of lymphoma, a cancer of the immune system, after a liver transplant several years ago. He developed new symptoms including swelling in his leg, pain, and numbness, along with fatigue and weight loss. Imaging tests revealed several areas of cancer in his lungs and leg, and a mass in his thigh. After a period of chemotherapy, the cancer responded well, and scans showed a significant reduction in the tumors. However, his liver enzymes have increased slightly since treatment, and he continues to have some blood clotting issues."
77,"A 15-year-old boy came to the emergency department (ED) with a two-day present-ation of abdominal pain, lack of appetite, and vomiting. On examination, the patient was found to have pain and tenderness on the right side of his abdomen. (Clinical, laboratory, radiological, and intraoperative details for all patients are described in .) SARS-CoV-2 was detected via the polymerase-chain-reaction (PCR) of his nasopharyngeal (NP) swab. Empiric intravenous (IV) antimicrobial treatment with cefotaxime and metronidazole was started and he was taken to the operating room for a laparoscopic appendectomy.","This 15-year-old boy went to the hospital because he was experiencing abdominal pain, vomiting, and a lack of appetite for two days. During the examination, doctors found tenderness on his right side, suggesting appendicitis. Tests confirmed he had COVID-19, and he underwent surgery to remove his appendix. After the surgery, he was treated with antibiotics and is now recovering at home."
78,"A 14-year-old boy presented to the ED. He had a 24-h history of nausea, diarrhoea, lack of appetite, and abdominal pain, mostly in the right iliac fossa. SARS-CoV-2 was detected via the PCR of his NP swab. On presentation to the ED, pain and tenderness on the right side of the abdomen were noted by examination. Abdominal ultrasound (US) showed findings consistent with acute complicated appendicitis. Empiric IV antimicrobial treatment with cefotaxime and metronidazole was begun and he was taken to the operating room for a laparoscopic appendectomy. An abdominal fluid culture revealed E. coli. The patient was admitted to the hospital during the first 24 h from the onset of symptoms, but the intraoperative findings of peritonitis and broad intra-abdominal inflammation may indicate that acute COVID-19 infection can speed up the disease course of acute appendicitis.","A 14-year-old boy came to the emergency room complaining of stomach pain, nausea, and diarrhea. Tests showed he had COVID-19 and that he had a serious infection called appendicitis, which required surgery to remove his appendix. During the surgery, doctors found additional infection in his abdomen. He was admitted to the hospital to recover and is being monitored closely."
79,"An otherwise healthy 15-year-old girl presented with a one-day history of generalised abdominal pain, nausea, and vomiting. An abdominal examination found pain and tenderness in the right lower quadrant. An abdominal US showed findings consistent with acute complicated appendicitis. A SARS-CoV-2 nucleic acid test was positive. The patient was initially treated conservatively for acute uncomplicated appendicitis with IV antimicrobial treatment (ampicillin plus metronidazole), but abdominal pain advanced, blood inflammation markers elevated, and therefore treatment was converted to surgery. It is possible this patient already had acute complicated appendicitis on ED admission.","Sixteen-year-old girl came to the hospital with a stomach ache, nausea, and vomiting. Tests showed she had a possible infection in her appendix, and a COVID-19 test came back positive. Initially, doctors tried to treat the problem with medicine, but her symptoms got worse, and she needed surgery to remove her appendix. It appears she may have already had a serious infection in her appendix when she first came to the hospital."
80,"A girl aged 12 years presented with fever, abdominal pain, and painful urination of one-day’s duration. The patient had tested positive for COVID-19 eight days before the onset of abdominal pain. SARS-CoV-2 was detected by the PCR of her NP swab. Per abdominal examination findings revealed superficial and deep tenderness in the right lower abdominal quadrant to palpation and localised tenderness to percussion. An abdominal US showed findings consistent with acute complicated appendicitis. IV antimicrobial treatment with cefotaxime and metronidazole was begun, and she was taken to the operating room for a laparoscopic appendectomy. An abdominal fluid culture revealed P.aeruginosa, Str.viridans, and Gemella morbillorum.","This 12-year-old girl came to the hospital with a fever, abdominal pain, and painful urination. She had recently tested positive for COVID-19, and her symptoms started after that. Tests showed she had a serious infection in her appendix, requiring surgery. During the surgery, doctors found other bacteria in her abdomen, and she was treated with antibiotics. She is now recovering well after her appendectomy."
81,"A girl aged 16 years presented with fever, abdominal pain in the epigastric and ileocecal region, nausea, lack of appetite, and vomiting of two days’ duration. Patient 5 had a recurrence of acute uncomplicated appendicitis. She had had the first episode two years previously, with acute uncomplicated appendicitis. She was treated conservatively with antibiotics; however, she was ultimately operated on laparoscopically. In her case, COVID-19 infection presumably exacerbated the course of appendicitis and resulted in abdominal pain that was a cause for diagnostic laparoscopy and further appendectomy. Unlike the four other cases in which the histology showed necrotic areas in the appendix wall, concluding that appendectomy was necessary (gangrenous appendicitis—see ), Patient 5’s surgery could have been avoided if symptoms had not persisted.","This 16-year-old girl came to the hospital with a fever, stomach pain, and vomiting that lasted for two days. She had a previous episode of appendicitis two years ago, which was treated with antibiotics and then surgery. Now, it seems that a recent COVID-19 infection worsened her appendicitis, leading to another bout of pain and requiring another surgery. Unlike other cases, her appendix didn't show signs of severe damage, so the doctors believe the COVID-19 infection played a significant role in her current symptoms."
82,"A girl aged five years presented with fever, abdominal pain, nausea and vomiting of one day’s duration. She had a recurrence of acute uncomplicated appendicitis. This girl had had her first episode two years previously, when she had acute appendicitis with an appendicular mass. She was treated conservatively with antibiotics; however, Patient 6 once again was treated non-surgically. In her case, the COVID-19 infection presumably exacerbated the course of appendicitis and resulted in abdominal pain.","A five-year-old girl came to the doctor because she had a fever, tummy ache, and was throwing up for one day. She had appendicitis before, two years ago, and it was treated with antibiotics. Now, she’s having another episode of appendicitis, likely made worse by a recent COVID-19 infection. Because of this, she will need to be treated with antibiotics again."
83,"We report the case of a 31-year-old Caucasian woman, gravida 3, para 1, who was referred after a second trimester fetal anatomy screening at 20 weeks gestational for a suspicion of a complex fetal cardiac malformation, for which several specialized opinions tried to reach consensus.\nThe obstetrical history of the patient includes a previous Caesarian section with a normal course of parturition and a spontaneous miscarriage. The current pregnancy presented a low risk for aneuploidy according to the performed cell-free fetal DNA test. The classical karyotype performed after the abortion did not reveal any chromosomal abnormalities.\nPrevious ultrasound evaluations were incongruent and reported the following findings:an isolated aortic arch anomaly (supposedly aneurysmal dilation from which the left common carotid artery emerges) and coarctation of the aorta with the anterograde flow; ventricular septal defect, coarctation of the aorta, and a vascular formation located superior from the aortic arch with the appearance of an arteriovenous fistula; aneurysmal dilation located above the pulmonary trunk bifurcation and a dilated left common carotid artery with a retrograde flow; minor ventricular septal defect with a normal ductus venosus triphasic flow.\nWe performed fetal echocardiography, which demonstrated a mild cardiomegaly with a left deviated 72-degree heart axis, normal aspect of the four-chamber view, a small membranous ventricular septal defect, and ductal aortic coarctation; the ductus venosus flow was normal (, and ). In addition, we identified an aneurysmal structure measuring 1.63/1.25/1.16 cm with turbulent Doppler flow, situated above the emergence of the pulmonary trunk and continued by a dilated vascular structure that bifurcates in the cervical region; the aneurysm seemed connected to the left pulmonary artery as well. A dilated left subclavian artery was also suspected (, and ).\nIn the context of complex cardio-vascular malformations, the patient requested the termination of the pregnancy by drug-induced abortion.\nThe hands-on dissection of the fetus revealed a set of abnormalities that could stand as an anatomical basis for what has been found during the ultrasound examination.\nThe first and the most pronounced aspect was the distention of the whole venous system of the neck and mediastinum. The specimen presented a linguo-facial vein that described a rather sinuous pathway alongside the inferior margin of the mandible (). Both the linguo-facial vein and the external jugular vein appeared with a markedly increased caliber, around 4–5 times larger than expected for this gestational age. Both left and right jugular veins and the right subclavian vein were assessed as three times larger than usual, respecting the normal relations to the neighboring structures ( and ).\nThe confluence between the left jugular and subclavian vein into the left brachiocephalic vein was observed to be very dilated to superior and inferior, extending above the superior margin of the omohyoid muscle as well as below the inferior concavity of the aortic arch. Moreover, on the inferior side of the enlarged brachiocephalic vein, a vessel could be observed descending lateral to the left vagus nerve and communicating with the left pulmonary artery. The left pulmonary artery was observed to be dilated as well, around twice as normal ().\nRegarding the great vessels of the heart, there are some anomalies to be discussed. A narrowing of the aortic arch was identified distally to the emergence of the left subclavian artery ( and ). A large, patent ductus arteriosus was found, ending right at the narrowing level observed in the aortic arch (ending right at the coarctation level) (). The left subclavian artery was dilated as well, sizing as much as the ascending aorta and the aortic arch, creating the illusion of a terminal branch rather than a lateral one ( and ).\nHeart analysis concluded no distinct changes in heart architectural formation for this gestational age. Atrioventricular and ventriculoarterial concordance was noted. Atria and ventricles were increased in relation to the mediastinum. Surprisingly for an aortic coarctation, the right atrium was not found to be enlarged.","This woman was referred for a second ultrasound due to concerns about a possible heart problem in her baby. The initial ultrasounds had shown some confusing findings, but a detailed fetal echocardiogram revealed several significant heart abnormalities, including a complex aortic arch, a narrowing of the aorta, and a connection between the pulmonary artery and a large vein in the baby’s neck. Because of these serious concerns, the patient chose to terminate the pregnancy."
84,"A 34-year-old woman (gravida 3, para 3) with three spontaneous vaginal deliveries was transferred to the Ulsan University Hospital from a local clinic due to severe abdominal pain accompanied by right flank pain. The patient had been previously healthy and had no specific medical or surgical history. She had an irregular menstruation cycle, and her last menstruation occurred five weeks and six days previously. The initial vital signs at the emergency room were stable; systolic and diastolic blood pressure were 114 mmHg and 68 mmHg, respectively. The initial pulse rate was 71 beats per minute. Whole abdominal tenderness with muscle guarding was noted on physical examination. Blood tests showed a low hemoglobin level (10.7 g/dL). A urinary pregnancy test was positive, and the serum β-HCG level was 7377.0 mIU/mL. Gynecological sonography found no evidence of an intrauterine pregnancy, except for normal bilateral adnexa with free fluid collection, suggestive of hemoperitoneum. After eight hours, the follow up blood test showed a lower hemoglobin level (8.6 g/dL). Two packs of packed red blood cells were transfused. We suspected a ruptured ectopic pregnancy through elevated serum β-HCG, but the ectopic mass could not be identified on pelvic ultrasound. Thus, we planned abdominopelvic computed tomography (APCT) to determine the cause of the right frank pain. Approximately 2 cm hypervascular mass in the subphrenic region, with a moderate amount of hemoperitoneum, was revealed (), which was thought to be the cause of the bleeding. Because of suspicions of a diaphragmatic ectopic pregnancy or other ruptured unknown hepatic mass, she was admitted for emergency surgery. Diagnostic laparoscopic surgery was performed in collaboration with a hepatobiliary surgeon and an obstetrician-gynecologist. On laparoscopy, about 400 mL of blood and clots were aspirated from the pelvic cavity, but both adnexa appeared normal. Approximately 20 × 10 cm tissue, suspected to be the placenta with a hematoma, had covered the diaphragm. After the removal of the placenta-like tissues, we found a 2 cm hypervascular mass attached to the diaphragm (). The mass was completely resected from the diaphragm and sent for histological examination. After the surgical mass removal, the patient was discharged without any postoperative complications, and the serum β-HCG level normalized within a month. The final pathologic diagnosis indicated that the mass was a product of conception, consistent with an ectopic pregnancy ().","This 34-year-old woman came to the hospital with severe abdominal and flank pain. She was found to be pregnant, but an ultrasound didn't show a pregnancy in her uterus. Blood tests revealed she was anemic, and a CT scan showed a large mass on her diaphragm that was causing bleeding. The mass was determined to be a retained placenta, and it was surgically removed. She is now recovering well and her pregnancy hormone levels have returned to normal."
85,"A 68-year-old male with a history of diabetes was admitted to our hospital with a two-week history of abdominal pain, jaundice, nausea, anorexia, and episodes of loose stools. Physical examination revealed right-sided abdominal tenderness. Laboratory examination revealed slightly higher bilirubin levels (0.4 mg/dL), but serum amylase and lipase levels, and complete blood count were all within the normal range. Abdominal computed tomography demonstrated a large cystic mass in the head of the pancreas, which measured 8.1 × 7.5 × 7.4 cm, and dilatation of the common bile duct, measuring 22 mm in diameter. There was also dilatation of the pancreatic duct, measuring 5 mm in diameter. The remainder of the pancreas was grossly unremarkable. Fine needle aspiration (FNA) was performed using endoscopic ultrasound (EUS). The EUS FNA fluid test showed a CEA level > 900 ng/mL, and fluid cytology was negative for malignancy or high-grade dysplasia. Endoscopic retrograde cholangiopancreatography (ERCP) was performed with biliary stent placement, which led to the resolution of his jaundice. An extended pylorus-sparing pancreaticoduodenectomy was performed. The operation was uneventful, and the patient was discharged 4 days after surgery.\nGross examination: The pancreatic head was entirely replaced by a mass lesion measuring 8.2 × 7.9 × 7.2 cm and was a unilocular cystic lesion containing gray-green turbid fluid with granular material. The cyst structure appeared to communicate with both the main and side duct branches. The cyst lining was gray-green to yellow, trabecular, and glistening to granular with few fibrous strands that arborized through the cystic structure and anchored at opposing sides of the cyst. Using a standard pancreatic cancer sampling protocol, paraffin-embedded sections of formalin-fixed tissue were studied by routine histology at the Indiana University Pathology Laboratory.\nMicroscopic examination: Histologically, the tumor showed two components composed of an epithelial component and a spindle cell component that were intimately intermingled together. The epithelial component had features ranging from well differentiated to moderately and poorly differentiated pancreatic ductal adenocarcinoma. The majority of the epithelial component was well differentiated with simple small to large ductal structures lined by a single layer of columnar to cuboidal cells, which had small and basally located nuclei with smooth and round nuclear contours and open chromatin. They had a moderate amount of eosinophilic cytoplasm without mucinous content (). The moderately differentiated component showed a more complex glandular structure with convoluted and interconnected ducts with a single layer of cells or a cribriform-type structure including multiple layers of cells with enlarged and irregular nuclei (). Some areas showed prototypical morphology of conventional pancreatic ductal carcinoma with small and angulated ducts infiltrating the desmoplastic stroma. The poorly differentiated epithelial component was small and focal. It showed vague and poorly formed ductal structures, or solid nests to small sheets of dispersed epithelioid cells with no ductal structures (). These cells had enlarged vesicular nuclei with irregular nuclear contours and conspicuous nucleoli. The spindle cell component was highly cellular with compact spindle cells, which showed hyperchromatic and elongated nuclei with scant cytoplasm. There was rare mitosis in the epithelial component, but the spindle cell component showed frequent mitosis with up to 12 mitoses per 10 high-power fields. Frequent apoptosis was also observed in spindle cell areas. Scattered necrotic areas were present in both components. There were no osteoclast-like giant cells or rhabdomyoblasts and no osteoid formation. There were foci of hemosiderin deposition, especially in the spindle cell areas surrounding the cystic lining. None of the ducts showed papillary or mucinous features. No areas subjacent to the epithelial component showed ovarian stroma-like features. All margins were negative for tumor. Twenty lymph nodes were present, all of which were negative for metastatic tumors. The pathologic staging was pT3pN0.\nImmunohistochemistry: Extensive immunohistochemical studies were performed at the Indiana University Pathology Laboratory due to the mixed features of the lesion (). The epithelial component was positive for markers of pancytokeratin AE1/AE3, epithelial membrane antigen (EMA), CK7, and CK19, and negative for MUC2, MUC5, MUC6, synaptophysin, and chromogranin. Spindle cells were negative for these markers. The spindle cells were diffusely positive for vimentin and DOG1 with patchy positivity for S100. Both epithelial and spindle tumor cells were negative for the estrogen receptor, CD10, inhibin, TLE1, SOX10, Melan A, HMB45, actin, desmin, myogenin, MyoD1, STAT6, and CD117. No nuclear staining was observed for β-catenin. CD163 highlighted cells with hemosiderin deposition, consistent with histiocytes. The tumor cells were negative for CD21 and CD35 expression. P53 showed a wild type staining pattern with no complete loss or overexpression in tumor cells of both components. Cyclin D1 showed patchy nuclear staining in the epithelial component but was negative in the spindle cell component. P16 was positive in the spindle cell component but negative in the epithelial component. The spindle cells demonstrated approximately 20% positivity of Ki-67 nuclear staining, while it showed only scant (about 2%) nuclear staining in the epithelial component (). Additional immunohistochemical staining for PDL-1 (SP142), MLH1, MSH2, MSH6, and PMS2 was performed at the Caris Life Science Laboratory (Phoenix, Arizona) and showed negativity (0%) for PDL-1 expression and intact protein expression of MLH1, MSH2, MSH6, and PMS2.\nMolecular study: Molecular analysis of the tumor tissue was first performed by Indiana University Molecular Pathology Laboratory and showed that the tumor was microsatellite stable with no mutation in BRAF, KRAS, and NRAS genes. Additionally, the tumor tissue was sent to the Caris Life Science Laboratory (Phoenix, AZ, USA) for next generation sequencing analysis of whole exome sequencing (WES). Direct sequence analysis was performed on genomic DNA using Illumina NovaSeq 6000 sequencers. Tumor mutation burden (TMB) was low and genomic loss of heterozygosity (LOH) was also low, with 10% of the tested genomic segments exhibiting LOH. The whole exome sequencing in our case showed no pathogenic alterations in the genes, such as BRAF, ATM, BRCA1, BRCA2, PALB2, SMAD4, NRG1, and NTRK1/2/3. However, the results for AXL1, HDAC1, MED12, NOTCH1, PIK3CB, POLD2, PRKACA, PTPN11, TERT, and XRCC1 were indeterminate because of the low coverage of exons in these genes.\nThe patient was followed up for three months after surgical resection. The last time he had an appointment for discussing the adjuvant chemotherapy. But he was then lost to follow up without receiving adjuvant chemotherapy.","Mr. Jones was admitted to the hospital after two weeks of experiencing abdominal pain, jaundice, nausea, and loose stools. Tests revealed a large, unusual growth in his pancreas that was blocking the flow of bile. To relieve the jaundice, a stent was placed in his bile duct. Surgery was performed to remove the growth, and the remaining pancreas was carefully preserved. The surgery was successful, and he was discharged home after four days."
86,"After a multidisciplinary evaluation, at the end of November 2019, a 13-year-old girl attended the Pain Therapy Clinic of the Ospedale Pediatrico Bambino Gesù in Rome, where acupuncture is also practiced as an analgesic technique. She reported pain in the left wrist and hand, with intensity 10 on the Numeric Pain Rating Scale (NRS), pulsating, and always present, thus preventing any movement. Marked hypersensitivity and allodynia were present at the level of the left fingers, wrist and hand. Pain was present in both flexion and extension of the wrist and caused a marked reduction in strength in the left wrist and hand, making a handshake impossible for the girl. There was no redness but swelling and sweating at the level of the fingers of the left hand. The pain was so intense that it interfered with her regular attendance at school, generating social withdrawal phenomena, which are unfortunately very frequent in patients with chronic pain.\nPain began three months before the consultation, after an accidental fall with trauma to the left wrist. It gradually increased and did not respond to either NSAIDs or limb immobilization. The diagnostic tests performed at the time (X-ray, Doppler ultrasound and magnetic resonance imaging) were negative, as were the blood chemistry tests ( and ).\nFrom the age of 8, the girl was followed by a pediatrician in our hospital for a history of cramps and pain in the lower limbs, especially in the ankle and left knee, both in the absence of trauma or caused by frequent falls. In the following years, clinicians found bilateral flatfoot (with subsequent surgery), mild ligamentous hyperlaxity and vitamin D deficiency. In addition, borderline cognitive level with motor coordination disorder and executive function deficit, as well as stuttering, were then diagnosed. Elements of anxiety emerged from the administration of Self Administered Psychiatric Scales for Children and Adolescents (SAFA-A, D and S) questionnaires, as highlighted in particular by the subscales “Separation anxiety” and “Generalized anxiety” [,]. Concern for one’s own health was observed, with experiences of herself as being ill; the scores indicate a propensity to somatize. There was a tone of mood oriented in a deflected sense, and insecurity. The results of the Lie scale were: 8; T: 65 [,]. In addition to pulsating headache with phono and photophobia, frontal epilepsy was also diagnosed, which could explain the frequent falls, and moreover had excluded hyperbaric oxygen therapy as a feasible regimen in this case (due to a cost/benefit evaluation, and increased exposure to oxygen toxicity during the treatment itself). Episodes of dizziness with difficulty in maintaining an upright position lasting a few hours were also observed, and on two occasions she also had an episode of unconsciousness lasting about 2 min.\nDuring the first visit, as a consequence of the mother’s need to have time to convince the recalcitrant daughter, in the meantime it was recommended to administer oral tramadol (100 mg/mL), 5 gtt in the morning and 5 gtt in order to reduce musculoskeletal pain. The reason for choosing a drug such as tramadol rather than any other pharmacological option lies in the fact that the pain was so intense that it affected the patient’s relationships []. We therefore opted for a drug that would have an immediate effect, so as to be able to undertake the acupuncture course. Subsequently, in the first two sessions, after careful disinfection of the skin with 2% chlorhexidine, and using the appropriate needles for length and diameter based on the type of acupuncture and the selected points, we used the following acupoints: TE 4 (Yang Pool), TE 5 (Waiguan), LI 5 (Yang Xi) and SI 4 (Wan Gu), all on the left side. These points were chosen on the basis of a pathology which, according to traditional Chinese medicine, was caused by cold wind. These points produced heat and dissipated the wind []. The needles were kept for 30′, with stimulation every 10′. In the third session, the girl reported a slight improvement in the painful symptoms but at the same time unbearable pain in the affected limb during the previous sessions due to the insertion and maintenance of the needles in the affected area. We decided to change strategy and to use abdominal acupuncture and stimulation of the points CV 4 (Guan Yuan), CV 12 (Zhong Wan), CV 16 (Zhong Ting), CV 17 (Shan Zong), ST 24 bilateral (Huaroumen) and KI 17 bilateral (Shang Qu), with appropriate needles and maintaining the same interspeed and time of stimulation. The needles were inserted to a depth of 0.2 cun. These new points (CV 4, CV 12, CV 16, CV 17 and KI 17), in addition to heating and dissipating the wind, re-established the correct circulation of qi []. In fact, used in combination, their purpose was to move the qi from the kidney to the extremity of the upper limb passing through the shoulder [,]. ST 24 was used to calm the patient and increase her compliance with acupuncture [].\nAuriculotherapy was also associated with Vaccaria seeds on the Wrist, Hand and Shenmen points, with the recommendation to stimulate them at home for 10 min, 4 times a day until the next session. Vaccaria seeds are used to stimulate certain points in auriculotherapy due to their almost spherical shape, and the absence of pharmacological properties []. The Shenmen point was chosen for its anxiolytic effect, given the patient’s psychological difficulties [].\nAfter eight sessions (two months) of abdominal acupuncture the pain completely disappeared (Numeric Pain Rating Scale value 0) and the girl regained full functional capacity of the arm and a normal life. Follow-up at three months, six months and one year demonstrated complete remission of symptoms, with constant values of NRS equal to 0. The reduction in pain, and subsequently its total disappearance, allowed the patient to resume normal school attendance, and therefore to resume a life of normal, balanced relationships.","The 13-year-old girl came to the hospital because she had severe pain in her wrist and hand that made it impossible to move. She had experienced this pain for three months after a fall, and it didn't respond to usual treatments. After a series of tests, doctors discovered she also had anxiety, a history of falls, and a tendency to feel unwell. To help with the pain, they tried acupuncture, focusing on specific points on her body, and also used auriculotherapy with Vaccaria seeds. After eight sessions, the pain completely disappeared, and she was able to return to school and a normal life."
87,"Case 1 was a 2-year-old boy who was admitted to the department of hemato-oncology due to pallor without respiratory symptoms or signs including no hemoptysis. Laboratory results revealed severe anemia, and his chest radiograph and chest computed tomography scans revealed pulmonary hemorrhage as the focus of bleeding (). The patient was diagnosed with IPH and treated with corticosteroids. His clinical course was uneventful and the corticosteroid dose was gradually tapered after the first month of treatment. However, he was re-admitted due to hemoptysis. Although he had no history of allergy and low levels of specific immunoglobulin (Ig)E to cow’s milk, Heiner syndrome was nevertheless suspected, and milk avoidance was recommended. The patient has been adhering to a strict milk restriction diet and has not had any further hemorrhagic events, and is not taking corticosteroids.","A 2-year-old boy was admitted to the hospital because he was very pale and had bleeding in his lungs. Tests showed he had a serious type of anemia and bleeding in his lungs. He was treated with medicine, and his condition improved, but he was readmitted with more bleeding. Doctors suspected a condition called Heiner syndrome and recommended he avoid milk, which he has been doing successfully."
88,"Case 2 was a 1-year-old girl who presented with recurrent hematemesis. She was diagnosed with IPH, and systemic corticosteroids and avoidance of cow’s milk were recommended based on our clinical experience with the first case. However, due to multiple episodes of accidental milk ingestion, she experienced repetitive pulmonary hemorrhage despite corticosteroid therapy. Given the exacerbation of clinical symptoms after milk exposure, she was diagnosed with Heiner syndrome. This case demonstrated the importance of corticosteroid therapy and strict milk restriction. At 2 years after diagnosis, the patient underwent an oral milk provocation test for 5 days, and she showed no symptoms or signs of hemorrhage.","A one-year-old girl was seen because she was vomiting blood repeatedly. Doctors diagnosed her with a condition called IPH, and they recommended stopping cow’s milk and giving her steroids. However, she continued to vomit blood even with these treatments because she accidentally drank milk. After further testing, she was diagnosed with Heiner syndrome, which caused her lung problems. Thankfully, after a milk test, she showed no symptoms, and her condition improved."
89,"Lastly, case 3 was a 2-year-old boy who presented with hemoptysis. Clinical investigations were performed to rule out pulmonary tuberculosis and other infectious causes, and these all came back negative. Two years later, he was hospitalized twice for pneumonia while living abroad and probably was accompanied by pulmonary hemorrhage due to first onset of anemia. Hemoptysis recurred at the age of 3; therefore, he underwent a comprehensive work-up including lung biopsy, which confirmed pulmonary hemosiderosis. Although the patient had no history of cow’s milk allergy, milk avoidance and systemic corticosteroids were initiated. Oral milk provocation was attempted 1 year later by introducing cow’s milk and dairy products such as cheese and ice cream every day for 1 week; this led to increased sputum and pulmonary infiltrates on the chest radiograph (). As a result, this patient was diagnosed with Heiner syndrome.","A 2-year-old boy was seen because he was coughing up blood. After testing ruled out other causes like tuberculosis, doctors found he had a condition called pulmonary hemosiderosis, where blood leaks from the lungs. He had previously had pneumonia while living abroad, which may have contributed to the problem. He was diagnosed with Heiner syndrome and started on a special diet avoiding milk and steroids to manage his symptoms."
90,"A 20-day-old girl was admitted to the neonatal intensive care unit with a chief complaint of poor oral intake through the emergency room. She was lethargic and did not suck well with swallowing only 10 to 20 mL of formula at a time in the last two days. However, the amount of urine did not decrease, and diapers were changed 10 to 14 times per day. Vomiting and diarrhea were not observed. She was born at 38+2 weeks of gestation with 3380 g (50th–75th percentile) via cesarean section. No abnormal findings were noted during the prenatal and immediate postnatal periods. She was the first child of healthy, nonconsanguineous Korean parents, and her family history was unremarkable. At admission, her weight was 3100 g (25th–50th percentile), length was 53 cm (50th–75th percentile), and head circumference was 36 cm (50th–75th percentile). Although vital signs were appropriate for her age (heart rate 150 beats/min, blood pressure 78/50 mmHg, respiratory rate 48 breaths/min, and body temperature 36.5 °C), her lips were dry, and the capillary refill time was prolonged to 5–6 s. Physical examination revealed both thumbs in palms, frontal bossing, prominent upper lip, high arched palate, sparse frontal scalp hair, and bilateral 5th finger clinodactyly. An initial capillary blood gas analysis showed severe metabolic acidosis (pH 7.16, pCO2 28.3 mmHg, pO2 42 mmHg, HCO3−—17.3 mmol/L, base excess—17.3 mmol/L). With an impression of dehydration, 20 mL/kg normal saline was infused intravenously for over 1 h before other laboratory results were obtained.\nThe laboratory tests at admission were as follows: serum sodium 113.3 mEq/L, serum potassium 8.79 mEq/L, serum chloride 90.8 mEq/L, total CO2 8.1 mEq/L, serum lactic acid 1.0 mmol/L, serum ketone body 24 µmol/L, blood glucose level 83 mg/dL, blood urea nitrogen 55.1 mg/dL, and serum creatinine 0.65 mg/dL. Her urinalysis revealed a specific gravity of 1.014 and pH 5.0 and was negative for white blood cells and red blood cells. Her spot urine sodium and potassium levels were 74 and 27.7 mEq/L, respectively. The serum and urine osmolality values were 232 and 229 mOsm/kg, respectively. All the results of the neonatal screening test were normal, which included TSH (1.2 mIU/L), 17-hydroxyprogesterone (1.6 ng/mL), total galactose (1.0 mg/dL), and mass spectrometry for amino acid, organic acid, fatty acid, purine, peroxisome, and carbohydrate metabolic disorders. The plasma ammonia level was within the normal limit as 97 µg/dL. The plasma renin activity and serum aldosterone level were markedly elevated to 142.0 ng/mL/h (normal range, 0.32–1.84 ng/mL/h) and 4560 ng/dL (normal range, 4.2–20.9 ng/dL), respectively. Renal ultrasonography revealed no abnormalities except mild hydronephrosis in the right kidney (). No abnormal findings were found in cardiac echocardiography or brain magnetic resonance imaging.\nTo correct severe hyponatremia, 60 mL of 3% sodium chloride was initially intravenously administered over 8 h. Her urine output on the first day of admission was 8.45 mL/kg/h. Hyponatremia and hyperkalemia were improved with intravenous fluid and oral sodium chloride supplementation (8 mEq/kg/day). The patient consumed an adequate amount of milk (170–200 mL/kg/day), and weight gain was appropriate (40–80 g/day) after oral sodium chloride supplementation.\nFor the genetic diagnosis of the patient, targeted exome sequencing (TES) was performed. Genomic DNA was extracted from proband blood. All exon regions of all human genes (~22,000) were captured by a Twist Human Core Exome Kit (Twist Bioscience, South San Francisco, CA, USA). The captured regions of the genome were sequenced using a NovaSeq 6000 sequencing machine (Illumina, San Diego, CA, USA). In TES, no other pathogenic/likely pathogenic single-nucleotide variants (SNVs) or small insertion and deletion variants associated with the clinical phenotypes were identified. However, her clinical phenotypes and biochemical results indicated PHA1. Therefore, we performed a chromosomal microarray (CMA) to identify deletion-encompassing genes responsible for PHA1. CMA (CytoScan Dx, Affymetrix Cytogenetics, Santa Clara, CA, USA) revealed a 203 kb heterozygous deletion at 4q31.23: arr[GRCh37] 4q31.23(148865586_149069090)x1 (). This deletion spans exons 7–9 of NR3C2 and exons 15–23 of the ARHGAP10 gene. Haploinsufficiency of the NR3C2 gene, which encodes the mineralocorticoid receptor, is responsible for ADPHA1. However, the details of the functional role of the ARHGAP10 gene in human disease remain unclear. Parental testing showed that the deletion was paternally inherited. Her father had no history of clinical PHA1 manifestation and had normal plasma electrolytes and serum aldosterone values with only slightly elevated plasma renin activity at testing.","Our little one was admitted to the hospital because she wasn't eating well and seemed very tired. She had low salt levels in her blood and was dehydrated, which is why we gave her fluids. Tests showed she had some unusual findings, including a slightly enlarged palate and some genetic changes that could explain her condition. After treatment, her salt levels improved, and she started gaining weight, and we are continuing to monitor her closely."
91,"A 75-year-old Caucasian woman with a history of well-controlled hypertension and hypercholesterolemia presents to the ED with complaints of a new-onset headache. She describes her headache as constant and refractory to over-the-counter pain relievers. Her physical examination is unremarkable. She has no ocular complaints, and no eye examination is performed. A non-contrast computed tomography (CT) scan of the brain is performed and reported to be normal. She is discharged with a prescription for Vicodin. Two weeks later, she returns to the ED with a worsened headache and blurred vision. The ophthalmologist on call is consulted by telephone. Visual acuity is noted to be 20/25 in both eyes (OU), pupils are round reactive to light, and no afferent pupillary defect is present. The patient has small pupils that precluded an easy view to the back of the eye with a direct ophthalmoscope. Attempts to check intraocular pressure are unsuccessful as the tonometer would not calibrate. A slit lamp examination is not done as the machine is not working. A CT and computed tomography angiogram (CTA) are performed at the recommendation of the tele-neurology doctor on call, both of which are normal. No labs are ordered. The patient is instructed to see the ophthalmologist in the morning. When the patient wakes up the next morning, her vision is worse. On examination in the ophthalmologist’s office, her visual acuity has decreased to 20/400 right eye (OD) and 20/25 left eye (OS).\nGiant cell arteritis (GCA) is a common disorder that presents to the ED and should be high on the differential for all elderly patients presenting with a headache, visual loss, or diplopia [,]. presents the most common presenting symptoms. Asking the right questions is crucial in preventing permanent blindness. On further questioning, the patient denied jaw claudication and temporal tenderness but did complain of ear pain and eye ache. Other historical clues that can be helpful include polymyalgia rheumatica, weight loss, fatigue, and abdominal pain due to mesenteric ischemia [,]. Laboratory evaluation should include the erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and complete blood count including platelet count [,]. A list of the most commonly abnormal lab values for the disease process are listed in . These laboratory tests are elevated at diagnosis in most patients and crucial to monitoring disease activity [,]. However, 20% of patients have normal laboratory testing []. CRP is much more sensitive than ESR, but the combination of all three is the most helpful and also guides management [,,]. Acute serum amyloid A (A-SAA) is less readily available but also highly sensitive []. Magnetic resonance imaging (MRI) with and without gadolinium of the orbits and temporal artery may be very helpful [,,,]. A CT scan does not pick up the vasculitis but an ultrasound of the temporal arteries may []. GCA can cause enhancement of the optic nerve or orbit on the MRI; it also can cause enhancement of the periosteum and temporalis muscle surrounding an occluded or partially occluded temporal artery [,]. If there is a history sufficiently suspicious for GCA (even if laboratory tests and imaging normal), the standard of care is to place the patient on 40 mg of prednisone (if no visual symptoms or signs) and refer for a temporal artery biopsy within two weeks [,,]. Rheumatology is then consulted, and the patient may be switched to a steroid sparing agent like methotrexate or tocilizumab (Actemra) as the prednisone is tapered slowly [,]. Monitoring always includes repeating laboratory values. If a patient has transient visual obscurations (graying or blacking out) or blurred vision due to choroidal nonperfusion or double vision, the prednisone dose should be at least 60 mg PO each morning with food [,]. If the ESR and CRP are very elevated, a significant thrombocytosis is present and/or the MRI shows extensive inflammation and/or the patient has already lost vision in one eye, the patient should be treated with high doses of IV steroids (methylprednisolone 250 mg q6 h) as an inpatient [,]. The characteristic severe visual damage is not reversible, but IV steroids usually prevent contralateral visual loss. Bilateral occipital lobe infarcts have been described. These patients can also have increased morbidity from stroke, myocardial infarctions, or aortic aneurysmal rupture if not treated [,,].","This 75-year-old woman came to the hospital because of a new headache that wasn't getting better. After some tests, including a CT scan, doctors found no obvious cause, but her vision started to get worse. Further investigation revealed she might have giant cell arteritis, a condition that can cause inflammation in the arteries and lead to vision loss. She was started on medication and closely monitored to prevent further vision problems."
92,"A 25-year-old woman with a past medical history of polysubstance abuse presents to the ED with a chief complaint of severe headaches that wake her from sleep and are present on awakening. She has tried NSAIDS without any improvement. She admits to alcohol, marijuana, and methamphetamine use and asks for Vicodin. Her physical examination is normal, and a non-contrast CT of the brain is normal. She is discharged with a limited supply of Vicodin and referred to outpatient neurology for migraine management. Her insurer is Medicaid, and she finds it difficult to visit a neurologist who will accept her insurance. She returns to the ED seven additional times with the same complaint. On her most recent visit, she complains of transient visual obscurations that gray out or black out her vision for seconds to minutes. She is again referred to Neurology and this time to Ophthalmology as well. Again, no one accepts her insurance and she presents to the ED for an eighth visit. On this visit, she complains of severe central visual loss bilaterally and on examination is unable to see more than the “big E” on the Snellen eye chart bilaterally. Her pupils are round but minimally reactive to light. No afferent pupillary defect (APD) is present. A fundoscopic exam is not obtained given that she is uncooperative (crying hysterically), there is no protocol for pupil dilation, and a non-mydriatic camera is unavailable. The ophthalmologist on call is slow to answer and the patient is admitted but unfortunately, the call group does not cover inpatients.\nWomen of childbearing age who are overweight are the population most at increased risk for idiopathic intracranial hypertension (high intracranial pressure with no specific cause) [,,,]. It can also occur in women of normal BMI as well as men [,,]. Exposure to steroids, doxycycline, or other medications can trigger this disorder [,,].\nEarly morning headaches should raise concern for increased intracranial pressure and/or an intracranial mass. The headaches often get worse when laying down (gravity dependent) and can be accompanied by transient visual loss when changing from lying to sitting or standing [,,]. Increased intracranial pressure (ICP) is serious and must be addressed. Patients who complain of early morning headaches should always have their eyes examined for papilledema whether or not they have visual symptoms [,,]. High Intracranial pressure (ICP) causes insidious visual field loss that begins in the periphery and is seldom noticed early on [,,,]. In some patients, diplopia also occurs due to the sixth cranial nerve being stretched across the petrous ridge [,].\nThe clinical history is critically important in this case too. Key symptoms are listed in . Patients should be asked about transient graying out or blacking out of vision especially when going from lying down or sitting to standing [,,]. This patient ideally would have been referred to ophthalmology at the initial visit and had a non-mydriatic photo taken of the optic nerve. Rather than a non-contrast CT scan, an MRI/MRV of the brain would have been the imaging study of choice [,]. Venous sinus thrombosis can cause increased ICP []. While in the ED, a lumbar puncture should be performed in the lateral decubitus position to document opening pressure but also cells, glucose, and protein [,,]. An elevated protein should prompt an MRI of the spine as a spinal tumor may be causing the increased ICP [,]. The differential diagnosis also includes indolent infectious diseases, such as tuberculosis and inflammatory entities including sarcoidosis [].\nThis is a very treatable pathophysiology, especially if identified early. Patients are placed on acetazolamide—Diamox Sequels provide extended release—and can be dosed at 500 mg PO BID [,]. When caught early, vision is preserved. Weight loss alone may recommended in mild cases with minor symptoms and preserved visual function (vision, color, visual field, and mild papilledema). Once the process has caused central visual loss, the prognosis is guarded and admission for acetazolamide, and lumboperitoneal or ventriculoperitoneal shunting is the standard of care [,,,]. Venous sinus stenting is also an option in select patients. Optic nerve sheath fenestration is also an option but most helpful before the loss of central vision [,,,].","This 25-year-old woman has been experiencing severe headaches that wake her up and are present when she wakes up, and recently started experiencing blackouts. She has a history of substance use and has tried over-the-counter medications without relief. After several visits to the emergency room, she began experiencing vision problems, including temporary graying out of her vision. Doctors suspect she may have increased pressure inside her skull, which can cause headaches and vision loss. She is now being closely monitored and will likely need further tests and treatment to determine the cause of her symptoms and preserve her vision."
93,"A 40-year-old woman presents to the ED with neck pain and non-specific neurologic symptoms including numbness, tingling, and headaches. She denies any other symptoms. A non-contrast CT of her brain is performed, which is normal. Tele-neurology is consulted, but her symptoms do not fit the stroke protocol, so no recommendations are made. The patient is discharged without any specific instructions for follow-up.\nFour weeks later she returns to the ED with bilateral visual loss. She first notices visual blurring several days prior to presentation. She denies any other neurologic symptoms, has no family history of vision problems, and is otherwise healthy on no medications. On examination she is unable to see anything on the eye chart but can appreciate light. Her pupils are round, reactive to light, and without an afferent pupillary defect. The anterior segment, IOP, and eye movements are normal. The ophthalmologist on call is contacted and recommends transfer to the university hospital 90 miles away. Tele-neurology is contacted, and they recommend a CT/CTA, which are both normal. They also recommend transfer to a university. A transfer is requested but all universities in the state were on diversion and refused transfer. Attempts to see the fundus with a direct ophthalmoscope are unsuccessful.\nDevastating unilateral or bilateral visual loss can occur due to a wide variety of causes. The differential diagnosis includes compressive, infectious, inflammatory, toxic, vascular, neoplastic, or hereditary causes [,,]. The initial evaluation in the ED can be very helpful in guiding therapy and preserving whatever vision is present. When a patient presents with visual blurring, the first step is to determine if the problem is in the retina or the optic nerve by taking a history and performing eye signs (i.e., vitals) including red desaturation, Amsler grid testing, and fundus photography [,]. The classic symptoms of retina vs optic nerve symptoms are presented in . Once it has been determined that it is an optic nerve issue, the age of the patient will guide the work-up even more than the appearance of the nerve.\nPrior to the advent of MR imaging, vague neurological symptoms were difficult to evaluate. Both multiple sclerosis and neuromyelitis optica have characteristic findings on MRI and lumbar puncture [,,]. Both are serious diseases that cause both visual and/or neurologic disabilities that can be permanent. However, treatment can be sight-saving as described in . presents the most common etiologies of bilateral vs unilateral visual loss. A non-contrast CT is an inadequate test for this population. If the testing is done prior to visual loss, the patient can be treated with IV steroids and referred for outpatient initiation of definitive therapy [,,,]. Distinguishing between MS-related optic neuritis and NMO-related optic neuritis is of prime importance because early initiation of effective immunosuppressive therapy is key to preventing relapses and permanent disability—see [,].","This woman came to the emergency room complaining of neck pain and strange feelings like numbness and headaches. After a normal brain scan, she returned a month later with sudden, severe vision loss in both eyes. Despite several tests, including CT scans and consultations with neurologists, the cause of her vision loss remained unclear. Doctors believe the vision loss may be due to a variety of causes, and they are continuing to investigate to determine the best course of treatment."
94,"A 50-year-old man presents with acute onset of double vision. His eye vitals are otherwise normal. He has a past medical history significant for diabetes, hypertension, and hypercholesterolemia. He denies headache or eye pain. The ophthalmologist on call was unable to be reached. The tele-neurologist recommended a non-contrast CT/CTA, which was reported to be normal. No additional testing was done, and the patient was discharged and told to follow-up with an ophthalmologist. One week later, the patient is found down and arrives at the ED in an ambulance. The patient never regains consciousness and passes away from a ruptured aneurysm.\nManaging double vision can prove equally as challenging as managing visual loss without an accurate ophthalmic examination. In a university-based ED setting, patients are typically seen in person by the ophthalmology residents on call, who are in turn supervised by a neuro-ophthalmologist. The neuro-ophthalmologist is then able to confirm a clinical diagnosis of a cranial nerve palsy or any other etiology of double vision. Depending on the diagnosis, the appropriate radiologic imaging protocol is followed and then interpreted by a neuro-radiologist. In the community-based ED setting, this stepwise evaluation and approach is not readily available. In this setting, a very helpful starting point is to take comprehensive external photos of the patient in the nine positions of gaze (i.e., straight ahead, up, down (with eyelids held up), left, right etc.). A list of the recommended diagnostic work up for common causes of double vision are presented in .","This man came to the hospital because he was experiencing double vision. He had a history of diabetes, high blood pressure, and high cholesterol. After some tests, including a CT scan, doctors couldn't find a cause for his vision problem. Sadly, he later collapsed and passed away due to a ruptured aneurysm."
95,"A 58-year-old Caucasian man did a video visit with his primary care physician, in which he complained of severe pain in the distribution of his herpes zoster that had occurred years before. No vesicles were visible. He was placed on nonsteroidal anti-inflammatory during the day and Tylenol with codeine at bedtime. Despite receiving the Pfizer COVID vaccination seven months earlier, he presented to the ED with a fever, fatigue, muscle aches, sinus congestion, and a cough. COVID PCR testing was positive, but chest X-ray was normal. A comprehensive metabolic panel and complete blood count were normal. He was discharged to quarantine at home. Two days later, the patient returned to the ED with acute loss of vision in both eyes to 20/400, no relative afferent pupillary defect was present, and fundus photography in the ED with non-mydriatic camera was normal. Additional laboratory assessments that were found to be abnormal included elevated erythrocyte sedimentation rate (40), C-reactive protein (33), and D dimers (2000). Chest CT revealed ground glass changes consistent with COVID-19; pulse ox revealed diminished saturation of 88%. A non-contrast head CT was normal, but an MRI of the brain and orbits revealed a large occipital stroke. The patient was admitted for Decadron, anticoagulation, and supplemental oxygen. Access to the monoclonal antibody was denied. The inflammatory markers and D dimer normalized, and pulmonary function improved. The visual loss was permanent.\nCOVID-19 (SARS-CoV-2) infections classically present with symptoms of fever, cough, fatigue, muscle aches, and neurologic alterations that result in loss of smell and taste [,,]. The neurologic and ocular manifestations are less well known, and the understanding of optimal management is in evolution. It has been postulated, however, that live virus can potentially be found in the tear film [,,,]. Additionally, the virus can travel via ACE2 receptors through intact ocular epithelium and the endothelial lining of organs [,]. Ocular symptoms can be as mild as hemorrhagic conjunctivitis to as vision-threatening as retinal vascular occlusions and posterior ischemic optic neuropathy as listed in [,]. Though the literature is limited, there have been several reported cases of the latter. In these cases, the typical presentation to the ED is with complaints of acute, painless, monocular, or binocular vision loss in the setting of a previous or recent diagnosis of COVID-19 (though this has also been reported in patients with a previous COVID-19 diagnosis > 3 months) []. Oftentimes, these patients have multiple chronic conditions that make them more susceptible to a more severe disease course. Positive patients with elevated inflammatory markers (IL-6, CRP, ESR, and fibrinogen) and d-dimer are at the highest risk for visual loss [,,]. Thus, it is very important for the clinician to have a high index of suspicion for the patient that presents with elevated markers. Given that COVID-19 lowers the threshold for thrombotic complication, especially in the chronically ill, Decadron and anti-coagulation may prevent visual loss in patients with cytokine storm and hypercoagulability [,]. In certain cases, this may mean expedited complete visual recovery whereas in other instances, vision may improve spontaneously over time if at all [].","A 58-year-old man came to the hospital with a fever, cough, and vision loss in both eyes, which he had developed after testing positive for COVID-Initial tests showed no signs of infection in his eyes, but further imaging revealed a stroke in his brain. Doctors treated him with medications to reduce inflammation and prevent blood clots, and his symptoms gradually improved. Although his vision loss was permanent, he was able to recover and was discharged home to continue monitoring his condition."
96,"We herein present a case that was recently managed at our institution, the Department of Surgery of the San Camillo Forlanini Hospital of Rome, Italy.\nA 53-year-old man with previous history of alcohol-related liver cirrhosis presents to our department for routine follow-up. His comorbidities include hypertension managed with oral antihypertensive drugs and diabetes mellitus type 2. He has no significant allergies and never underwent any surgical procedure. He brings an ultrasound, which shows a 4 cm heterogenous mass in segment 8. His alfafetoprotein level is elevated to 76 ng/mL. He has no symptoms and looks in good performance status. We scheduled him for a triphasic CT scan, which shows a lesion of 4.3 cm with brisk arterial contrast and venous washout. According to the LIRADS classification, this lesion could be considered a class 5 with diagnostic features of hepatocellular carcinoma. The patient was discussed in our multidisciplinary tumor board including hepatobiliary and transplant surgeons, hepatologists, radiologists, pathologists, oncologists, and interventional radiologists. The plan was to submit the patient to curative intent treatments given his early presentation according to the Barcelona Clinic Liver Cancer Staging System (BCLC), namely surgical resection or liver transplantation; radiofrequency ablation was excluded given the tumor’s dimensions. Given the good performance status, the position of the lesion (which was right below the Glissonian capsule) and the liver function of the patients, the MDT decided to schedule the patient for surgery. We therefore saw the patient in clinic and discussed the procedure. Informed consent was signed, and liver function was tested using ICG retention rate. We used 0.5 mg/Kg corresponding to 40 mg in this 80 kg patient. The DICOM data of the CT scan of the patient were then submitted to our radiologist who performed a 3D reconstruction of the patient’s anatomy and the relationship of the lesion with the major vessels. Furthermore, the exact dimensions of the portal territories for segment 8 were reconstructed and showed on the model. We normally aim at the narrowest but still oncologically safe resection possible. The surgery is then planned on the model, identifying the borders of the resection and the exact location of the Glissonian pedicle to tackle and the hepatic veins to skeletonize and cut. Once the preoperative surgical plan is discussed between the surgeons and the radiologists, the patient can be scheduled for surgery. This generally happens 2 weeks from the administration of ICG to achieve a complete washout of the dye by the normal parenchyma and a retention by the tumor that will then be showed intraoperatively using the narrow band camera. The patient is scheduled for a laparoscopic anatomical segment 8 resection. In our experience, we use the so called “French position” to operate laparoscopic cases, with the patient standing in between the legs of the patient and two assistants on each side. Two screens in the operating room are dedicated to the endoscopic vision, one screen is dedicated to the intraoperative ultrasound, while one dedicated screen allows to show the preoperative surgical planning and therefore guide the resection throughout the case. We use a five-trocar technique with 1 umbilical port and 4 ports on the subcostal line. One port is epigastric and is very important for the dissection of the hepatocaval confluence. An extra 5 mm access is used to perform an extracorporeal Pringle manuever. Open laparoscopy access is gained at the level of the umbilicus. After inserting all the trocars, the narrow band camera is used to identify the HCC on the hepatic dome on segment 8, which is shining green because of the ICG administered 2 weeks before. Intraoperative ultrasound and doppler are then performed to confirm the border of the resection. Pringle manuever is prepared. Dissection is started from the hepatocaval confluence to immediately identify the middle and right hepatic veins. For segment 8 resections, no extensive right lobe mobilization is necessary unless exposure is limited. We then start our parenchymal transection using a combination of energy-based device clamp-crushing technique, CUSA dissection and bipolar coagulation. We identify the middle hepatic vein at its origin, and we carry our parenchymal transection in a cranio-caudal fashion, sweeping the liver parenchyma from the vein. This avoids any tearing on small peripheral branches of the middle hepatic vein. Slowly progressing caudally, we encroach the Glissonian pedicle for segment 8, going to the lesion and vascularizing the tumor bearing area. We test the pedicle using a bulldog clamp and checking with the doppler the absence of flow in segment 8 and the presence of flow in the remnant liver. We then ask the anesthesiologist to inject 1 mL of ICG intravenously. We will then see all the liver shining green but not segment 8, which is our resection area. Guided by the ICG we will then carry out the anatomical resection. The Glissonian pedicle is stapled, and the resection is carried out dissecting the whole resection area from the middle and right hepatic veins. Once the resection is finished, the vascularization of the remnant liver is checked both with the ICG and the doppler. A drain is generally not placed unless there are specific issues during the procedure. The patient was placed on a fast-track protocol with early feeding and mobilization and was discharged home on postoperative day 4. Follow up is now more than 1 year and the patient is currently in good health status with no signs of recurrence.","A 53-year-old man with liver problems and diabetes was found to have a concerning growth in his liver. After careful evaluation, including scans and a tumor board discussion, doctors decided to operate to remove the growth. The surgery was performed laparoscopically, using advanced imaging and techniques to ensure a precise and safe removal. The patient is now recovering well and is being closely monitored for any signs of recurrence."
97,": A 60-year-old female was treated for NTG elsewhere but had been referred to our clinic with deterioration of the visual field. Her BCVA were 0.8 and 0.1, right and left, respectively, and she had normal intraocular pressure (IOP) (19 mmHg). Her parents were both treated for glaucoma, which could account for positive family history (treated with caution as we had not seen the parents). As both optic discs did not look glaucomatous, they looked a little pale but not excavated. The disc appearance did not match very advanced visual fields, so the patients’ drops were discontinued. The patient, seen 3 months later on follow-up visit, exhibited further deterioration of the visual fields with concomitant left vision loss. The BCVA had deteriorated to 0.2 and 0.02, right and left, respectively. An MR scan revealed olfactory groove meningioma that was successfully and subtotally removed by bilateral craniotomy. The BCVA improved to 0.9 and 0.9, right and left eye, respectively, and the VF improved significantly (RE completely, LE—significant loss remained).\nSummary: this is a case of rapid bilateral vision loss with regard to the BCVA and VF (too rapid for glaucoma), in addition, VF did not match optic disc appearance. Positive or “pseudo-positive” family history may be misleading, causing protracted, unnecessary topical treatment, especially in case of putative NTG.","This 60-year-old woman came to our clinic because her vision was getting worse. Initially, her vision loss was rapid and significant, and tests showed it wasn't due to glaucoma. An MRI scan revealed a small growth on the brain that was causing the problem, and it was successfully removed. After the growth was removed, her vision improved significantly."
98,": A 56-year-old male treated for POAG with high IOP (30–48 mmHg) for a couple of years but after initial success of drops, he was referred to the clinic due to high pressures (over 40 mmHg). His mother was blind due to glaucoma (confirmed). He underwent trabeculectomy in both eyes when BCVA was 0.5–1.0, but 3 years later, vision deteriorated in both eyes (especially in right eye) despite IOP being maintained around the low teens. VF loss observed over 3 years seemed to be consistent with glaucoma and the island of central vision was lost last. The rapid decrease in central visual acuity in the presence of low and stable IOP was the reason for neuroimaging. He had an MR scan done that revealed an intracranial meningioma that was totally resected by bilateral craniotomy. The right eye is blind and the left eye has some useful VF with BCVA around 0.1 and has been stable for 2 years now.\nSummary: this is true high tension primary glaucoma with a family history that progressed despite successful filtering surgeries. The true family history does not exclude intracranial malignancy, if the course of glaucoma is not typical (long-lasting deterioration after successful IOP drop and atypical pallor of the disc). It is difficult to determine the exact impact of high IOP vs. anterior visual pathway compression on vision loss in this patient. Additionally, disc pallor, a typical sign of compressive neuropathy, may be observed also in juvenile glaucomas or in cases with extremely high values of IOP.","This 56-year-old man has had glaucoma for several years, despite using eye drops to control his pressure. His vision has steadily worsened over time, especially in his right eye, even though his eye pressure remained low. An MRI scan revealed a growth on his brain that was removed, which is believed to be the cause of the vision loss. Currently, his right eye is blind, but his left eye’s vision has stabilized."
99,": An 82-year-old male was treated for NTG elsewhere but referred to our clinic for consultation and for left ptosis surgery. His BCVA were 1.0 and 1.0, right and left eye, respectively, and he had normal IOP (14 mmHg). On ophthalmoscopy, both discs look glaucomatous, but the left disc more advanced. Only the left eye exhibited VF changes typical for glaucoma that corresponded ideally with a retinal nerve fiber layer thickness defect in OCT examinations. NTG was stable but unilateral. An MR scan was performed that revealed picture of 4 × 4 mm pituitary microadenoma contacting the chiasm. In three years’ observation, visual field and the tumor size remain stable.\nSummary: this is the case of unilateral stable glaucoma with coexisting pituitary adenoma. It is unclear if the combination of glaucoma and microadenoma is pure coincidence, or if the microadenoma is responsible for the neuropathy.","This 82-year-old man came to our clinic for an evaluation of his glaucoma, a condition that affects the optic nerve and can lead to vision loss. He already had glaucoma in his left eye, and scans showed a small, non-cancerous growth in his pituitary gland pressing on the optic nerve. Fortunately, the tumor and his glaucoma have remained stable over the past three years, and he continues to have good vision in both eyes."
100,": A 65-year-old hyperopic female was referred to our clinic because she developed left eye pallor with consistent VF loss. Her BCVA was 1.0 with correction +4.5 DSph and 0.5+ with correction +4.5 DSph, right and left eye, respectively. The IOP was 15 and 16 mmHg, right and left eye, respectively. Her angle in gonioscopy was narrow (I/II deg.), but neither acute nor prodromal glaucoma were confirmed, which is why an MR was performed. It revealed a left optic nerve sheath meningioma measuring 11 × 12 × 7 mm involving optic nerve canal. The tumor was totally removed by left craniotomy and pathology confirmed a diagnosis of psammomatous meningioma. The patient is stable and continuously observed; the BCVA 2 years after surgery is the same, 1.0 and 0.4, right and left eye, respectively.\nSummary: this straightforward case of unilateral pallor of the optic disc justifies outright MR but, nevertheless, an MR may be retarded by the belief that a unilateral NTG could exist even without excavation, or by the suspicion of acute angle closure in the past. After acute angle closure in the disc, more pallor than cupping may be observed.","A 65-year-old woman came to the clinic because she noticed her vision was getting worse in one eye. Tests showed a small tumor on her optic nerve, which was causing the vision loss. The tumor was successfully removed with surgery, and her vision has remained stable since then. She is now being monitored regularly to ensure the tumor doesn’t return."
101,": A 70-year-old female was referred to our clinic because her NTG progressed. Her BCVA was 0.5 and 1.0, right and left eye, respectively. The IOP on glaucoma drops was 15 and 16 mm Hg, right and left eye, respectively. Both discs looked clearly glaucomatous with C/D = 0.8–0.9 with disc hemorrhage on the right side. However, the VF revealed bitemporal hemianopia hiding typical glaucomatous field loss. An MR scan was immediately performed and revealed pituitary macroadenoma (24 × 30 × 20 mm) affecting the chiasm. The tumor was removed by transsphenoidal resection. The VF improved very rapidly after surgery and has remained stable for 4 years.\nSummary: this is a typical case of pituitary macroadenoma affecting the chiasm with progressive VF loss and typical bitemporal hemianopia. Coexistence of true glaucoma is rare; interestingly, the progression of glaucoma was halted after the tumor was excised. The influence of the adenoma on the optic disc appearance is uncertain.","This 70-year-old woman came to the clinic because her glaucoma was getting worse. Her vision was decreasing, and the eye exam showed signs of glaucoma damage, including a loss of peripheral vision. An MRI scan revealed a large tumor in her pituitary gland that was pressing on her optic nerve. The tumor was successfully removed, and her vision has since improved significantly."
102,"A young man was killed by a 30-year-old man after they had consumed alcohol and cocaine. The murderer claimed he was not capable when he committed the crime because he suffered from an alcohol-/drug-caused behavioral impairment producing neurological damage, having regularly consumed alcohol and drugs since the beginning of adolescence. In detail, he reported to have started habitually consuming alcohol, cannabis, cocaine and amphetamine when he was a teenager. The defendant also claimed to be predisposed to anti-social behavior because of genetic factors. Indeed, his forensic consultant performed a genetic testing on him focused on three genes (MAOA, COMT, SLC6A4), finding that he was a carrier of the polymorphisms of 5-HTTLPR (fragment 44 bp–SS genotype) and COMT (Leu136Leu) in homozygosity.\nHence, the court requested a team of forensic experts to assess the capacity of the defendant, performing toxicology testing and a complete neuropsychiatric evaluation.\nToxicology testing was performed on urine (four days after the murder), saliva (two days after the murder), blood and pubic hair (10 days after the murder). In blood and saliva, it failed to find significant levels of drugs or alcohol, while in urine it detected benzoylecgoine (322 ng/mL). In the pubic hair, significant levels of cocaine (141 ng/mg), benzoylecgonine (21 ng/mg), and ethylglucuronide in concentration >30 pg/mg were found.\nA full clinical/neuropsychological examination was performed. No clinical signs of neurological impairment and no signs of alcohol-dependence were observed. A personality disorder not otherwise specified was diagnosed. 3-Tesla brain MRI and brain CT-PET were also performed. In MRI imaging, a decrease in cortical thickness with larger lateral ventricles, a statistically significant volumetric asymmetry of the amygdalae (the right amygdala was smaller than the left one) and a decreased volume of the right orbito-frontal cortex (OFC) (in comparison with the left one) were observed. No ischemic lesion or anomalies in the corona radiata and in the subtentorial/cerebellar area were found. PET-CT did not find any alteration of brain perfusion or metabolism.","This man died after a violent crime, and the person who committed it claimed they were unable to control their actions due to long-term alcohol and drug use. Genetic testing revealed that he carried certain gene variations that may have contributed to his impulsivity and difficulty regulating his behavior. Tests after the crime showed traces of cocaine in his system, but no significant levels of alcohol. Brain scans revealed some changes in the structure and function of his brain, particularly in areas related to decision-making and emotional control."
103,"A 25-year-old man abducted, raped and robbed two women under the influence of alcohol in six months. He reported that his father often physically and psychologically abused him and his mother during his childhood and that a teenager raped him when he was a child. He was unschooled and few years before the rapes he was convicted for having stabbed a man who had insulted him. After having been released, he committed several burglaries. Moreover, he reported to have frequently beaten his wife and to have often fantasized about raping women since he was very young, even if he knew rape was illegal. Finally, he reported to have begun to consume alcohol during his childhood, albeit he never became an alcoholic.\nHence, the court requested a forensic psychiatrist to assess the capacity of the defendant.\nA full clinical/neuropsychological examination was performed. No clinical/electroencephalographical signs of neurological impairment and no signs of alcohol-dependence were observed. An intelligence quotient (IQ) of 59 was found and an antisocial personality disorder was diagnosed. A genetic test focused on five genes (MAOA, COMT, SLC6A4, HTR1B, and DRD4) found a 3-repeat variable number of tandem repeats (VNTR) variant of MAOA and a TT genotype for the rs13212041 polymorphism of the HTR1B gene.","This man has a complex history involving abuse, trauma, and criminal behavior. He disclosed a difficult childhood with physical and emotional abuse from his parents, a childhood rape, and past offenses including assault and burglary. A forensic psychiatrist evaluated him and diagnosed him with antisocial personality disorder, along with a genetic predisposition to aggression. Tests showed no neurological problems or alcohol dependence, but revealed specific genetic variations that may contribute to his behavior."
104,"A 27-year-old woman was diagnosed to have a fetus with left-sided CDH in her routine antenatal ultrasound (at 20 weeks gestation). Based on antenatal fetal imaging, the liver was in its thorax, the left lung was not visible, the right lung measured 1.95 × 1.67 cm and the lung-to-head ratio (LHR) was 1.275 (observed/expected LHR 29–33% [,], qualitative lung index/QLI 0.499), and percent predicted lung volume (PPLV) on fetal MRI was 20.5, all of which indicated poor prognosis []. Additionally, the fetal echocardiogram was suggestive of hypoplastic left heart syndrome (HLHS). The prenatal screening included amniocentesis with 46 XX karyotype and normal alpha-fetoprotein levels. The pregnancy was also complicated by polyhydramnios.\nAn appropriate-for-gestational-age female infant was delivered by emergent cesarean section for fetal bradycardia after initial induction of labor at 39 weeks gestation. At delivery, she was apneic and floppy, and immediate cord clamping was performed. Her airway was intubated one min after birth, and a Replogle tube was placed to decompress her stomach. Her Apgar scores were 2, 5 and 8 at 1, 5 and 10 min, respectively. Her initial neonatal intensive care unit (NICU) course included gentle mechanical ventilation, followed by bedside surgical repair of CDH two weeks after birth. A postnatal echocardiogram confirmed small left-sided cardiac structures. She also had pulmonary hypertension (PHT) with supra-systemic pulmonary pressures that were managed with inhaled nitric oxide (iNO), milrinone infusion and sildenafil. She required a peripherally inserted central catheter (PICC) for parenteral nutrition and a gastrostomy tube placement to allow enteral feeding. Her respiratory support was gradually weaned to low flow nasal cannula at 0.5 L/min with 100% O2, received Palivizumab and was continued on oral sildenafil for mild residual PHT. Her microarray was normal. She was discharged home (located at a higher altitude) at 2.5 months of age, only to be readmitted two days later when she presented to the local emergency room with fussiness and emesis and developed respiratory failure, requiring endotracheal intubation. Her brain natriuretic peptide (BNP) was elevated at 4650 pg/mL and was diagnosed with pulmonary hypertensive crisis.\nDuring her second hospitalization in the NICU, she was extubated to nasal continuous positive airway pressure (CPAP) within three days after management with high settings on high-frequency oscillatory ventilation and iNO for hypoxic respiratory failure and PHT. However, she did not tolerate further wean in respiratory support, owing to worsening PHT. She had multiple episodes of PHT crisis with hypoxia and hypercarbia. To assess the V/Q status in her lungs, a nuclear medicine scintigraphy scan was performed at four months of age that showed 10% perfusion to the left lung in comparison to 90% to the right lung. A chest CT scan confirmed hypoplastic left lung. Cardiac catheterization at five months demonstrated worsening PHT, and she was restarted on iNO. Flexible bronchoscopy at five months showed gross narrowing of left mainstem bronchus and lobar bronchi. Due to continued PHT crises and an inability to remain extubated, she underwent a tracheostomy at 5.5 months. She had a BroviacTM (CR Bard, Salt Lake City, UT, USA) catheter placed for central venous access. She received several courses of dexamethasone, with minimal change in her respiratory status.\nAt six months of age, the under-perfused left lung was emphysematous with a hyperinflated left lower lobe that caused a mediastinal shift to the right side (a). This led to compression of the right lung, further compromising gas exchange. At this stage, due to the futility of all efforts to improve her hypoxic respiratory failure and the severe ventilation/perfusion mismatch (V/Q mismatch) in her left lung, a multidisciplinary discussion involving cardiology, pediatric surgery, pulmonology and neonatology was held to determine the next steps in her management. A trial on different ventilator modalities with higher positive end-expiratory pressure (PEEP) and another course of dexamethasone did not result in any improvement (b). Given her labile nature with significant PHT and oxygenation concerns, there was concern if she would benefit from a lobectomy given the high risk of a procedure.\nA decision was made to trial left main bronchial plugging by placing an inflatable 5Fr bronchial blocker to determine if improving V/Q mismatch by allowing better ventilation of the better perfused right lung segments may facilitate a decrease in respiratory support. We used two separate portable video laryngoscopes (VL), one equipped with a miller 1 blade and the other with a flexible fiberoptic bronchoscope (FFB). A 5 Fr Uniblocker (Fuji Systems, Tokyo, Japan) was guided through the glottic opening under visualization by the miller 1 blade VL. The bronchial blocker was guided into its final position and the balloon was inflated with the help of FFB. The position of the bronchial blocker was confirmed by a chest radiograph (a,b).\nThe left bronchial blocker placement resulted in an immediate improvement of her respiratory status with a decrease in oxygen requirement and improved ventilation with weaning of ventilator settings, and better inflation of the right lung (a,b). The adverse effects of the bronchial blocker placement included displacement, which required replacement under visualization by anesthesiology. Therefore, surgical exploration was subsequently performed by anterolateral thoracotomy, 80% of the emphysematous left upper lobe was resected (), a small area of the left pulmonary sequestration was also identified and a chest tube was placed. Physiological and blood gas changes are shown in . Respiratory support was weaned as tolerated, her chest X-ray improved (b) and she was discharged home two months after surgery.","This baby was born with a serious condition called left-sided CDH, where part of her liver was in her chest. She needed emergency surgery to repair this and also had a heart problem called HLHS. After a difficult start, she was treated with multiple medications and breathing support in the NICU. Despite these efforts, she continued to struggle with breathing problems and high blood pressure in her lungs. Eventually, doctors placed a device to help block off the poorly functioning lung and performed a lung surgery to improve her breathing. After the surgery, her breathing improved, and she was able to go home two months later."
105,"In July 2019, a 74-year-old male patient was admitted to the Infectious Disease Section of the Verona University Hospital for investigation; he had HBV in his blood with a titer of 26,100,000 IU/mL (cobas® HBV, Roche Molecular Diagnostics, Branchburg, NJ, USA) but exhibited normal liver function. Although he displayed the hepatitis B e antigen (HBeAg), he was negative for the HBsAg, but positive for the homologous anti-HBs (ADVIA Centaur HBV assays, Siemens Healthcare GmbH, Erlangen, Germany).\nIn August 2013, the patient received a kidney transplant for nephroangiosclerosis. At this time, the serologic screening for HBV had shown that he was HBsAg-negative, anti-HBs-positive (12 mIU/mL), HBeAg-negative and positive for antibodies to the HBeAg and to the hepatitis B core antigen (anti-HBc). No antibody markers of a hepatitis C and hepatitis D virus infection were detected; serum HBV DNA had not been determined. The indices of hepatic cytolysis were normal. The kidney donor was negative for HBV. Post-transplant, the patient received immunosuppressive induction with basiliximab, tacrolimus, mycophenolate and steroids, and was then included in the follow-up program as per protocol; the HBsAg remained negative throughout, accompanied by normal liver biochemistry.\nIn May 2019, the patient developed chronic myeloid leukemia (CML). Before treatment with imatinib mesylate, the patient repeated the HBV serology and the HBeAg was again detected in his blood in the absence of circulating HBsAg; further testing using a real-time PCR showed that he had HBV DNA in serum at a titer of 26,100,000 IU/mL. The patient was still anti-HBs-positive (15 mIU/mL) with normal liver enzymes. A diagnosis of HBVr was made and, in July 2019, the patient started entecavir (ETV) treatment at 0.5 mg/day, which was subsequently reduced in April 2020 to 0.5 mg/48 h because of renal function deterioration. The patient underwent a close follow-up (every 2 weeks) for the first 6 months of treatment and, after, with monthly scheduling. The therapy led to a consistent decrease in viremia, reaching a plateau of 3 Log10 HBV DNA reduction after 6 months of treatment (); however, viremia did not decrease further in the following months. No classic ETV-resistant mutations were observed in the HBV reverse transcriptase (rt) gene. However, we detected the mutation rtL269I, previously reported to confer a reduced susceptibility to ETV []. On January 2021, the patient switched to tenofovir alafenamide (TAF) treatment; HBV DNA rapidly declined and reached a value of 378 IU/mL at the last FU. The single ALT elevation (107 U/L) in the 2nd month of TAF treatment was documented in the course of an infection of the residual left limb requiring surgical toileting. Unfortunately, the patient died from coronary heart disease in September 2021.\nThe serum samples collected from July 2019 were tested for the HBsAg with the highly sensitive Lumipulse® G HBsAg-Quant assay (Fujirebio Inc., Tokyo, Japan) as well as for the HBcrAg (Lumipulse® G HBcrAg, Fujirebio Inc., Tokyo, Japan) []. The nucleotide sequences of the HBV S gene and the rt domain were determined using the Sanger sequencing method at BMR Genomics service (BMR Genomics, Padua, Italy) and analyzed using ClustalW2 software (; accessed on 23 December 2019 and on 6 December 2020) []. The HBV genotype was determined based on a reverse hybridization line probe assay (INNO-LiPA HBV Genotyping, Fujirebio Europe, Gent, Belgium) [].\nDespite its high analytical sensitivity for HBsAg mutants [], the HBsAg assay failed to detect a circulating HBs antigen. The HBV S gene sequence analysis identified four mutations within the a-determinant (aa 124–147) (P/T127H, Q129N, F/Y134H and G145R) and five additional mutations within the mayor hydrophilic region (MHR, aa 99–169) (T116N, P120S, C121Y, K/R160S and W165S) (). The values of the HBcrAg from baseline to the end of the follow-up were persistently above the upper limit threshold of the assay (>7.0 Log10 U/mL). Viral genotyping showed that the patient was infected with HBV genotype D.","This patient has a complex medical history, including a kidney transplant, chronic myeloid leukemia, and a long-standing infection with the hepatitis B virus (HBV). Initially, he was found to have a very high level of HBV in his blood, but without the virus being detectable in his body. He was treated with entecavir, which helped to lower the amount of the virus in his blood, but the virus remained active. Eventually, he switched to a different medication, tenofovir alafenamide, which was more effective in reducing the virus. Despite treatment, the patient ultimately passed away from a heart condition. Throughout his treatment, doctors carefully monitored his liver function and the virus, using various tests to track his progress."
106,"We report the case of a 4-year-old male child, admitted to our clinic for generalized seizures, which persisted in spite of anticonvulsant therapy (Diazepam), with no previous acute symptoms. His personal history revealed ureterovesical junction obstruction, mild hydronephrosis, and an episode of generalized seizures approximately 2 months before the current admission for which chronic therapy with sodium valproate (Depakine) was recommended. We must mention that the brain MRI performed at that time was normal. The family history showed the presence of ageusia and anosmia in both parents.\nAt the time of admission, the patient was intubated and mechanically ventilated and the clinical exam revealed only pallor.\nThe laboratory tests performed on the day of admission revealed anemia (Hemoglobin—Hb 9.98 g/dL, Hematocrit—Htc 28.54%), a severely increased number of monocytes (9624/µL), and a mildly increased C-reactive protein (CRP 7 mg/L). Taking into account the family history, a real-time polymerase chain reaction (RT-PCR) of the oropharyngeal swab was performed and it tested positive for SARS-CoV-2. Moreover, both parents were confirmed with this infection. Both urine and blood cultures were negative. The serology for viral hepatitis B and C, as well as antinuclear and anti-double-strained DNA antibodies were negative. We performed a thoracic computed tomography (CT), which showed consolidation in the lower lobe of the left lung associated with an opacity in the right apex, suggesting possible atelectasis ( and ). The cranial CT revealed no pathological findings. The patient was admitted to the intensive care unit with a diagnosis of COVID-19 in a severe form.\nWe initiated antibiotic treatment (ceftriaxone 800 mg twice a day and amikacin 100 mg twice a day), antiviral therapy (lopinavir/ritonavir 2.5 mL twice a day), corticosteroids (Dexamethasone 4 mg twice a day), anticoagulants (enoxaparin 0.2 mL in a single daily dose), and antipyretics (Paracetamol), and we continued the chronic anticonvulsant therapy with sodium valproate. The second RT-PCR performed on the third day of admission was also positive for SARS-CoV-2 infection. Unfortunately, the patient’s condition deteriorated progressively, and, after approximately 72 h of hospitalization, he developed desaturation and bradycardia. We repeated the laboratory parameters before the bradycardia event and we found leucopenia (leukocytes 3500/µL), neutropenia (neutrophils 1111/µL), mildly increased creatin kinase (280 U/L), a high ferritin level (121 ng/mL, normal ranges 7–84 ng/mL), hypoalbuminemia (3.29 g/dL), a prolonged time of prothrombin (22.3 s) and an increased international normalized ration (INR 1.74).\nDespite all efforts to resuscitate the patient, he died on the fourth day of admission.","This 4-year-old boy was admitted to the hospital because he was having seizures, even though he was taking medication for them. He also tested positive for COVID-19, and his parents also had the virus. Tests showed he was very weak and had a low red blood cell count, and his body was fighting a severe infection. Sadly, despite treatment, he didn't respond and passed away."
107,"A 43-year-old Caucasian male reported a 1-month history of spontaneous clear left side nipple discharge with a recent appearance of a homolateral painless breast swelling. There was no history of bloody discharge. Past medical history was pertinent for obesity class I (BMI: 33.3) and bilateral hypoacusia for otosclerosis. There was no family history for breast or ovarian cancer. His social history indicated no use of alcohol, but previous use (twelve years ago) of tobacco products.\nOn physical examination, he was an overweight Caucasian male with symmetrical breasts. On palpation, there was a bilateral pseudogynaecomastia with a smooth, ill-defined left breast thickening, especially at the union of the outer quadrants. With applied pressure, a minimal clear stream of discharge fluid was elicited from the left nipple and was felt to be localized to a single duct.\nDigital breast tomosynthesis (DBT) with synthesized reconstructed 2D images (s2D) was performed in medio-lateral-oblique (MLO) projections for each breast and in both cranio-caudal (CC) and latero-medial (LM) projections for the left breast. The s2D images showed a regular appearance of the breast buttons without gynaecomastia, and an area of asymmetrical density at the union of outer quadrants of the left breast that was better identified at the DBT images as an area of architectural distortion with scattered peripheral punctate calcifications, sparing the nipple-areolar complex. ().\nA breast ultrasound (US), performed on the same day, showed in correspondence of the mammographic findings, the presence of an ill-defined, hypoechoic area of acoustic shadowing with peripheral anechoic lacunae and a close small focal ductal ectasia. ()\nAccording to Breast Imaging Reporting and Data System (BI-RADS) [], these findings were classified as category 4b.\nAn US-guided Fine-Needle Aspiration Cytology (US-FNAC) was performed. Our laboratory received 4 microscope slides fixed in alcohol and coloured with Papanicolaou stain. Microscopic examination showed atypical ductal cells with poor myoepithelium arguing for several types of proliferative non-malignant lesions but also DCIS. According to the IAC Yokohama System for Reporting Breast Fine-Needle Aspiration Biopsy Cytopathology (1st Edition, 2020) [], these findings were classified as category C4 (). Since the Rapid On-Site Evaluation (ROSE) [] already showed this suspicious atypia, we contextually performed an US-guided biopsy (US-CNB) with a 16 Gauge (G) semi-automated core biopsy needle. Our laboratory received two specimens of breast tissue of 1.5 cm of maximum dimension who were submitted for routine processing. Microscopic examination showed breast parenchyma with fibrosis with a focal area of atypical ductal hyperplasia (ADH), p63 positive and with focal positivity for CK5/6. According to Guidelines for non-operative diagnostic procedures and reporting in breast cancer screening [], these findings were classified as category B3 ().\nAll the findings of cytology and histology in association with the radiological assessment underlined the importance of the triple test [] and recommended us to perform surgical excision of the suspected area. Therefore, after discussing with the multidisciplinary team (MDT), according to the patient’s consent, a left subcutaneous nipple-sparing mastectomy was performed without sentinel lymph node biopsy ().\nOur laboratory received a specimen designated left mastectomy, weighing 90 g (). It comprised oriented fibro-fatty tissue, 8 × 4.5 × 4 cm in aggregate, which on gross examination revealed an area of small cystic formations and a whitish lesion of 0.2 cm on which random samplings were performed. Microscopic examination showed an intraductal papilloma (IP) with ADH and a single focus of DCIS solid-cribriform type, respectively. Nuclear grade was 3 (p63+, CK5/6−) with negative surgical margins. No invasive cancer was present (). The specimen was sent for immunohistochemical examination of estrogen (ER) and progesterone (PR) receptor. The tissue was positive for both ER (with 70% of nuclei staining with strong intensity) and PR (80% of nuclei staining strongly) (). Venipuncture samples were subsequently sent to an external laboratory for genetic analysis, the results of which were negative for BRCA1 and 2, and other mutations ().\nAccording to these surprising results, after a clinically and radiologically evaluation of the left axillary lymph nodes, the MDT decided not to perform axillary dissection and to administer tamoxifen as approved adjuvant hormone treatment of men with ER-positive early stage breast cancer [].\nThe patient was noted to be doing well 6 months post-operatively and is still on follow-up at the time of writing.","This 43-year-old man has been experiencing a clear discharge from his left nipple and a growing lump in his breast for about a month. Tests, including mammograms and ultrasounds, revealed an area of concern in his breast with some unusual cells, leading to a diagnosis of atypical ductal hyperplasia and a small area of DCIS (ductal carcinoma in situ). Because of these findings, he underwent a surgery to remove the affected area of his breast, and the results showed a small intraductal papilloma with the same atypical cells. Finally, the patient was prescribed tamoxifen as an adjuvant treatment and is being closely monitored for any signs of recurrence."
108,"In December 2020, an 83-year-old woman presented to the Emergency Department of our hospital with a large ulcerated and necrotic bulging lesion on her forehead. Ill-defined, dusky erythematous plaques extended on the parietal and frontal areas of the scalp and the face. Violaceous-darkish nodules were also observed. Comorbidities included chronic obstructive pulmonary disease, hypertension, diabetes, and ischemic encephalopathy. The physical examination revealed bilateral cervical lymphadenopathy.\nThe patient’s relatives provided photographic documentation of the evolution. The lesion had emerged four months before admission as a 2 cm bruise-like patch on the forehead (a), before it rapidly developed into a large purplish plaque after 1 month (b), and then to the current presentation (c).\nThe second lockdown in Italy and the fear of the SARS-CoV-2 contagion had led the relatives to postpone the medical evaluation. A biopsy from a violaceous nodule showed a full dermal proliferation of irregular anastomosing vascular channels lined by single or double layers of enlarged endothelial cells, which permeated between collagen bundles, causing “collagen dissection” (a,b). The endothelial cells were large and pleomorphic, with vesicular nuclei and prominent nucleoli, and were immunoreactive for CD31, CD34 and ERG (c,d), with no observed HHV8 expression or MYC overexpression.\nThese data confirmed the diagnosis of angiosarcoma of the scalp. All routine investigations were normal. Total body computed tomography (CT) showed cervical lymphadenopathy without brain or visceral metastases. Although radiotherapy and electrochemotherapy were considered, they were not performed due to the patient’s advanced age, comorbidities, and tumor size. The patient was referred to palliative care.","An 83-year-old woman came to the hospital with a large, concerning growth on her forehead that had been growing rapidly over the past four months. Tests revealed that this growth is a type of cancer called angiosarcoma, which involves abnormal blood vessels in the skin. The patient had other health problems, including lung disease and diabetes, which made treatment more challenging. Because of her age and overall health, doctors decided not to pursue aggressive treatments like radiation, and instead focused on providing comfort care."
109,"We describe the case of an 18-year-old boy presented with ASD associated with a mild intellectual disability (patient 5 in the tables). Informed consent was obtained from all subjects involved in the study. Regarding the familial load, the paternal uncle presents an anxiety disorder treated with a selective serotonin reuptake inhibitor.\nThe proband is the first child of unrelated and healthy parents. He attended school with support, had good global functioning and social relationships with classmates, despite his social anxiety, and had progressive improvements in his social skills.\nAt the age of 13 years old, after his summer break, social isolation acutely worsened, associated with a confusional state, psychomotor agitation, speech impairment, visual hallucinations, cognitive regression, a loss of personal autonomy, and increased anxiety. Quetiapine up to 300 mg/day and alprazolam 0.50 mg/day were prescribed, with complete recovery. Cerebral MRI and metabolic tests were unremarkable. Array-CGH test was not significant, showing a duplication of the long arm of chromosome 6, inherited from the father.\nAt the age of 15 years old, the patient had another acute breakdown, which was treated with quetiapine 300 mg/day and had partial recovery (only affective symptoms partly improved) until one year later, when symptoms worsened, with disorganized thought, obsessive symptoms and rumination, catatonic behaviors, associated with asthenia, reduced autonomous mobility, persistent hyporeactivity to stimuli, stiffness in the limbs and hypomymia, apathy, and isolation. Upon initial evaluation in the psychiatric ward, physical examination was unremarkable. Quetiapine was replaced with aripiprazole, with gradual titration, starting with 2.5 mg/day and 2.5 mg increases every 4 days, up to 10 mg/day, with supplementary lorazepam, resulting in a transient improvement in the clinical picture. After 2 days, the boy showed signs of psychomotor retardation, hyperreactivity to stimuli, anorexia, and asthenia. Creatine kinase (CK) was in the normal range when he was discharged.\nAfter 7 days, given the worsening symptoms associated with increased obsessive thoughts, hyperthermia, and CK elevation, the boy was admitted in an emergency department and pharmacotherapy was immediately discontinued. The patient was hospitalized in an intensive care unit for 4 weeks, then in a pediatric ward for 1 week, and finally in our hospital for 10 days. During hospitalization, limb stiffness, perioral myokymia and myoclonus, facial amimia, uncoordinated movements of the tongue and difficulty swallowing, polypnea, tachycardia, and arterial hypertension were observed. Intravenous hydration, dantrolene, clonidine, intravenous benzodiazepines, and carvedilol were administered, followed by bromocriptine therapy and intravenous lorazepam 2 mg 5 times a day. A gradual improvement in vigilance, reduction of hypertonus, and resolution of hyperthermia were observed, with gradual motor improvement. After 8 days, blood results showed a reduction in CK (404 U/L, normal CK range 0–50 UI/mL) and a mild increase in liver enzymes (ALT 72 U/L).\nAfter being discharged from our hospital, the patient carried out monthly clinical and CPK controls, and after 6 months the patient had a general assessment in our hospital, with persisting control of previous clinical manifestations.","This 18-year-old boy has a genetic condition that affects his brain and causes difficulties with social anxiety and learning. He experienced several episodes of severe mental distress, including confusion, hallucinations, and catatonic behavior, which were treated with medication. After trying different medications, doctors found a combination of aripiprazole and lorazepam helped him improve, but he still had some challenges. Currently, he is being closely monitored and continues to receive treatment to manage his symptoms and maintain his overall well-being."
110,"A 63-year-old man was admitted to our Respiratory Disease Unit at the University Hospital—Ancona, for a 6-month exertional dyspnea and bilateral pleural effusion prevalent on the ride side, detected on chest computed tomography (CT).\nHe was former smoker without occupational exposure to asbestos. His medical history was remarkable for asymptomatic brain aneurysm, blood hypertension, multiple lumbar disc herniation. On admission to our unit, physical examination, oxygen saturation on room air, heart rate and blood pressure were normal, whilst breathing sound was suppressed at the third right lower lung fields.\nThe patient first underwent a repeated CT scan that allowed us to rule out a pulmonary embolism and confirmed moderate right pleural effusion with parietal and visceral pleural thickening, in the absence of significant parenchymal abnormalities (). Thoracic ultrasound (TUS) revealed hyperechogenic pleural fluid with atelectasis of basal segments of the right lower lobe (); at thoracentesis, fluid appeared cloudy and yellow coloured, and a physico-chemical exam was consistent with exudate and microbiological tests, including an acid-alcohol-fast bacilli (AAFB) search, were negative ().\nA subsequent medical thoracoscopy (MT) revealed the presence of yellow pleural fluid (overall 1800 mL removed) and parietal pleura hyperemia with fibrotic plaques (). Ten pleural biopsies were obtained by forceps on parietal pleura and histopathological examination documented a large lymphoplasmacytic infiltration, fibrosis, reactive mesothelial cells and vascular proliferation, in absence of neoplastic lesions or granulomas; the final diagnosis was suggestive for non-specific pleuritis (NSP).\nAn extensive diagnostic work-up, including echocardiogram, abdominal angiography CT scan, autoimmune, viral, and bacterial serology, failed to detect any potential known cause of NSP and blood tests were normal, except for a mild elevation of C-reactive protein. Thus, the patient was diagnosed with idiopathic NSP and therapy was started with steroids (Methylprednisolone 0.5 mg/kg, tapered in one month with clinical and radiological improvement.\nSix months later, the patient complained chest discomfort and mild dyspnea, and CT scan showed a relapse of small amount of right pleural effusion associated with diffuse pleural thickening; a PET-FDG showed slight captation on right basal parietal pleural without abnormal captations in other organs ().\nDue to the limited pleural effusion and the absence of sliding at TUS, we decided not to repeat thoracoscopy, but to refer the patient to the Thoracic Surgery Unit of Sant’ Andrea University Hospital (Rome) for a surgical pleural biopsy. Histological examination revealed a diffuse fibrosing pleuritis, with occasional hyaline features, fibrinous exudate, and a dense lymphocytic and plasma cell inflammation. Interestingly, inflammatory infiltrate was diffuse but with irregular distribution throughout the histological specimen. Immunoperoxidase stains showed a marked increase of IgG4 positive plasma cells (up to 50/HPF) with an IgG4+/IgG+ ratio >40% reaching the diagnostic threshold level for IgG4 disease []. High IgG4 serum levels were found (324 mg/dL) []. Based on these findings, the patient was diagnosed with IgG4-related pleuritis ().\nAfter exclusion of systemic involvement of IgG4 disease, steroid therapy was started (Prednisone 0.5 mg/kg/d for 2 weeks, tapering up to 2.5 mg/day as a maintenance dose for an overall period of 6 months) with a complete and stable resolution of pleural effusion, improvement of respiratory symptoms and a progressive reduction of IgG4 serum levels, returned within normal limits (64 mg/dL).","This 63-year-old man was admitted to the hospital after experiencing shortness of breath and fluid buildup in his lungs for several months. After several tests, including a fluid removal and biopsies, doctors determined he had non-specific pleuritis, an inflammation of the lining around the lungs with no clear cause. Further testing revealed an unusual pattern of antibodies in his blood, leading to a diagnosis of IgG4-related pleuritis. Treatment with steroids successfully cleared the fluid buildup and improved his breathing, and his antibody levels returned to normal."
111,"We report the case of a 2 months old female, presented for consultation due to the presence of a lump on her left thigh, with progressive and constant growth after birth. The lesion was first described on the prenatal ultrasound at 30 weeks of gestation as a pre-femoral soft tissue mass of 20/7 mm (). The patient was delivered by cesarean section due to fetal distress but was otherwise normal at birth. Development was normal, and there was no relevant family history. On clinical examination, there was a 25/10 mm nodule on the antero-intern side of the left thigh that was firm, mobile and within the deep layers. The overlying skin was normal. There were no other lesions elsewhere on the patient’s body.\nThe initial X-ray and ultrasound (US) showed a pre-femoral soft tissue mass that measured approximately 30/13 mm, with nonhomogeneous structure, hypoechoic areas, calcifications, and weak Doppler signal, being located anteriorly to the vascular elements of the thigh (A). Abdominal ultrasound was normal.\nMagnetic resonance imaging (MRI) showed a mass of 19.33/15.19/34 mm, with a nonspecific vascular involvement (B). In T1-weighted images, the MRI appearance consisted of a low signal. In T2-weighted fat-saturated images, a high signal intensity of the lesion was shown with nonhomogeneous contrast setting after intravascular contrast was administered, but with late homogenization, located on the antero-internal part of the left thigh with an important mass effect on the left vastus intermedius muscle. The lesion was considered to be probably a schwanoma of the left saphenous nerve.\nElective surgery was scheduled. An italic S-shaped incision on the antero-internal face of the left thigh was performed, from the crural arch distally extended for about 6 cm. A mass of approximately 4 cm × 1.5 cm × 1.5 cm was revealed, which included the entire thickness of the sartorius muscle (A,B). In the 1/3 medial part of the tumor, dissection was performed, isolating it from the femoral vasculo-nervous package without opening the sheath of the vasculo-nervous canal. The sartorius muscle was resected at a distance of about 2 cm distal and proximal to the tumor, with complete tumor resection (C). Hemostasis was performed and adjacent tissue approximated. The excised mass was sent for pathological analysis. The patient had a favorable surgical outcome and was discharged 3 days postoperatively. At one year after surgery follow up, the child had no recurrence.\nHistologically, the mass in the sartorius muscle was noted as a proliferation of tapered cells arranged in an irregular spiral pattern and crossed by thin-walled vessels. In the central region, biphasic proliferation consisting of nodules with necrosis and central calcifications was observed, and between the nodules, fusiform cell proliferates arranged in small intersecting bundles (A,B). Occasional micronuclei were evident. A pseudocapsule formed by a thin layer of connective tissue <1 mm covered the mass. Neoplastic proliferation encompassed residual skeletal muscle fibers in the center of the lesion. Extracapsular and peripheral scarce mature adipose tissue were seen with isolated large-caliber blood vessels. Immunostaining revealed the following results: vimentin positive, smooth muscle actin positive in nodules with necrosis and calcifications (miotic nodules), desmin focal positive, Ki67 low (about 5 positive cells per 100 tumor cells, suggesting low cell kinetics) (D–F).\nDiagnosis of IM of left sartorius muscle was made.","A two-month-old baby girl was seen because of a growing lump on her left thigh that doctors found during a prenatal ultrasound. After being born by C-section, she seemed healthy, but the lump continued to grow. Tests, including MRI, showed the lump was likely a benign tumor called a schwannoma, and surgery was performed to remove it completely. The surgery was successful, and the baby is doing well with no signs of the tumor returning after a year."
112,"Case 1 was a female child aged 7 years and 11 months. She had visited the hospital with a chief complaint of cold water pain in the anterior mandible. She had a history of trauma to the anterior primary teeth, including the lower right central incisor, right lateral incisor, and left lateral incisor, at 3 years of age. Hypomineralized areas, brownish-white in color, were observed on the labial side of her lower bilateral central incisors (). There was no past medical history. Genetic screening was not performed; the permanent tooth hypomineralization was thought to be caused by primary tooth trauma. The patient also complained of pain from air blowing and cold water, and the VAS value was 6.5. Immediately after the treatment to suppress the hypersensitivity, she no longer experienced pain with air or cold water, and VAS was zero. When patient came to the hospital one month later, her VAS score showed 4; therefore, the treatment was reapplied. After the fourth treatment, the hypersensitivity had not completely disappeared, and the VAS was 1. For the seventh treatment, patients’ VAS value of hypersensitivity pain were stable at 0.5–0. Furthermore, the surface of the brownish tooth had changed to appear almost cloudy after seventh treatment (). During the process of this treatment, discolored devitalized teeth, gingival inflammation and percussion pain did not appear. Digital analysis indicated a pre-treatment cloudiness of 6331 pixels, which was significantly reduced to 65 pixels after treatment (). In addition, the area of brown color decreased by approximately six-fold, from 12,898 to 2118 pixels. These results suggest that both cloudiness and brown color disorder were significantly improved.","This 7-year-old girl came to the hospital because she had painful sensitivity to cold water and air blowing in her mouth, especially around her front teeth. She had experienced trauma to her baby teeth in the past, which may have caused some weak spots in her teeth. The doctors treated her to reduce this sensitivity, and after several treatments, the pain significantly decreased and the discoloration of her teeth improved. The treatments helped to clear up the cloudiness and brown spots on her teeth, making them look much better."
113,"Case 2 was a male child aged 8 years and 7 months. He visited the hospital with a chief complaint of pain in the anterior maxilla following exposure to cold water. He had a history of trauma to the anterior primary teeth at the age of one year, with composite resin repair of a fracture in the crown of the upper right primary central incisor. There was no past medical history. An abnormal position of the upper right permanent central incisor and clouding of the labial surface were observed, which were likely due to trauma to the primary teeth (). Examination results indicated a VAS value of 6 for cold water and 7.5 for air blowing. Immediately after treatment, the patient no longer felt pain with air or cold water, VAS was zero. One month later, the VAS was 4 by cold water and 5 by air. The treatment was reapplied once monthly. The hypersensitivity had become acceptable to the patient and VAS was 2 after fourth treatment. During the seven treatments, the pain did not completely disappear, the VAS by cold water was 1–2, whereas the VAS by air was 2–4. While extensive clouding remained, the color tone was obscured and improved (). During the process of this treatment, discolored devitalized teeth, gingival inflammation and percussion pain did not appear. Digital analysis showed significantly reduced cloudiness from 27,886 pixels to 7904 pixels (). The hypomineralized tooth was mostly cloudy, with a narrow area with a brown color. However, this area significantly decreased after treatment (p < 0.03). This result indicated that not only strong cloudiness but also slight brown color were significantly improved.","This 8-year-old boy came to the hospital because he was experiencing pain in his upper teeth after being exposed to cold water. He had a previous tooth injury when he was a baby, and the dentist noticed that one of his permanent teeth was slightly misaligned. The dentist treated his teeth with a special procedure to reduce the sensitivity to cold and air, and over several months, the pain and cloudiness in his teeth improved significantly. While the teeth weren't completely normal, the discoloration and sensitivity were greatly reduced."
114,"Case 3 was a female child aged 8 years and 7 months. She had visited the hospital with a chief complaint of cold water pain in the left side of the maxilla. The left upper second primary molar was extracted because of apical periodontitis and root resorption due to severe caries, at 4 years of age. There was no past medical history. Dark brown hypomineralization was observed on the buccal tooth surface of the first premolars (). The patient also complained of pain from air blowing and cold water, and the VAS value was 4. Immediately after the treatment to suppress the hypersensitivity, her VAS was zero. When patient came to the hospital one month later, her VAS score showed 1; therefore, the treatment was reapplied. During the seventh treatment, the hypersensitivity improved, and the VAS was 0. The dark brownish tooth surface of the first premolars was changed to pale brown (). Digital analysis significantly reduced the area of the brownish tint from 4858 to 1755 (). On the other hand, cloudiness was not detected.","This 8-year-old girl came to the hospital because she had a painful tooth that needed to be removed. The tooth was taken out because it was badly decayed and infected. After the tooth was removed, she experienced sensitivity to cold and air, which was treated with special medicine. Over the next month, the sensitivity improved significantly, and the discoloration of her other teeth also faded."
115,"Case 4 was a male child aged 5 years and 9 months. He had visited the hospital with a chief complaint of cold water pain in the anterior mandible. There was no history of trauma and caries in the primary teeth and no other systemic history. The cause of hypomineralization in the permanent teeth was not determined. The brownish-white in color were observed on the labial side of his lower central incisors (). The patient also complained of pain from air blowing and cold water, and the VAS value was 3. Immediately after the treatment to suppress the hypersensitivity, his VAS was zero. After one month, VAS was reduced to 0.5 and VAS was zero after four treatments. Seven treatments improved the color of the hypomineralization (). Cloudiness areas improved from 6872 to 1903, and brown areas decreased significantly from 6595 to 1667 ().","A 5-year-old boy came to the hospital because he was experiencing pain in his lower front teeth when exposed to cold water or air. The cause of the pain and discoloration in his teeth was not found, but the treatments helped to reduce the pain and improve the appearance of the teeth. After several treatments, the discoloration in his teeth significantly improved, and the pain was completely gone. The dentist will continue to monitor his teeth to ensure the problem doesn't return."
116,"A 54-year old woman underwent a fine-needle aspiration biopsy (FNAB) for a 2.3 cm rapidly growing thyroid nodule (). The cytological examination showed both solid groups and discohesive oxyphilic cells (Hürthle cells) in a background featuring lymphocytes. Based on these features, the FNAB was diagnosed as a low-risk indeterminate lesion (AUS/FLUS). Five months later, the nodule grew to 3.6 cm, and thus another FNAB was performed; a diagnosis of suspicious for malignancy was rendered. The patient underwent a total thyroidectomy (nodule 4.1 × 3.4 cm) with cervical lymph node dissection, and a removal of the internal right jugular vein that was invaded by the tumor. Microscopically, a Hürthle cell carcinoma with foci of paucicellular anaplastic cancer was diagnosed (Stage IVB; cT3b cN0 Mx/pT4b pN0 M0). In particular, large epithelial cells featuring granular eosinophilic cytoplasms, hyperchromatic nuclei with evident nucleoli were arranged in a solid and trabecular pattern alternated with scattered anaplastic spindle cells and necrotic areas. Immunohistochemical stainings for pancytokeratin and PAX8 were positive in both these components. Conversely, TTF1 was expressed by Hürthle cells only. Thyroglobulin (Tg) immunostaining was negative in both Hürthle and anaplastic spindle cells ().\nTwo years later, because of the appearance of a hacking cough, a 18-fluorodeoxyglucose (18-FDG) positron emission tomography (PET) scan was performed and revealed several millimetric lung hypermetabolic areas. Over time, the serum Tg under LT4-suppressive therapy had increased from 0.15 to 19 ng/mL. Two months later, a computed tomography (CT) scan revealed the presence of multiple lung lesions, in particular one in the medium lobe invading the airways (21 mm diameter), a second in the right inferior lobe (4 mm diameter) and a third in the left lung (9 mm diameter). A transbronchial biopsy of the largest lesion was performed, and the histology was consistent with a thyroid cancer metastasis.\nOn January 2019, the patient came to our observation, 18FDG-PET/CT showed a further increase in the number, size and FDG uptake of the lung lesions, and the appearance of multiple lymphadenopathies in the neck and mediastinum. In particular, the lesion in the right inferior lobe had grown to 8.5 cm, with a SUV max of 50. The histology of the tumor has been reviewed by two independent pathologists who confirmed the diagnosis of ATC. The molecular test performed on the primary thyroid tumor for BRAF, NTRK and ALK mutations was negative, and chemotherapy with cisplatin plus doxorubicin was given. After three courses of treatment the disease was stable, but after three further courses the disease progressed. The patient was then given four courses of paclitaxel; however, the disease continued to progress. Immunohistochemistry for PD-L1 performed on the primary tumor was negative, but the presence of tumor-infiltrating lymphocytes (TIL) was noticed. The primary tumor was also tested for microsatellite instability-MSI using a panel of five markers (BAT25, BAT26, D2S123, D5S346 and D17S2720), and the tumor was instable for the presence of mutations of BAT-26, D2S123 and D17S2720. Based on these results, on January 2020 a treatment with lenvatinib (24 mg daily) in combination with pembrolizumab (200 mg every 21 days) was started as a salvage therapy. After five months of treatment, a partial response was achieved with a reduction by 76% of the lung lesion in the medium lobe from 8.5 cm to 2 cm and at 18FDG PET/CT a drop in SUVmax from 50 to 4.7. The treatment was continued, but lenvatinib was progressively reduced to 10 mg daily due to grade 3 diarrhea, vomiting and weight loss. After 12 months, the administration of pembrolizumab was delayed every six weeks. The lenvatinib treatment has been withdrawn several times, and the patient compliance was low. In spite of that, after 18 months of treatment all lung lesions continued to respond to therapy (PR with a reduction by 50% of the sum of the diameters of the target lesions by RECIST) (). Unfortunately, a pleural nontarget lesion showed a progression, over four months, as demonstrated by the 18FDG-PET/CT (10 mm vs. 7 mm, a 43% increase by RECIST and a SUV max of 25.8 vs. 6.5) (). The pleural lesion is being treated with stereotactic radiotherapy (42 Gy in seven fractions). The patient is still alive, and the treatment with lenvatinib and pembrolizumab is still ongoing.","This patient was initially diagnosed with a potentially cancerous growth in her thyroid after two FNAB tests showed concerning results. Eventually, a surgery to remove the thyroid and surrounding lymph nodes confirmed a diagnosis of aggressive Hürthle cell carcinoma that had spread to her lungs. Despite treatment with chemotherapy, the cancer continued to grow in her lungs, but a new combination of drugs, lenvatinib and pembrolizumab, led to a partial response. While the lung lesions have shrunk, a new lesion has appeared in the chest, and the patient is currently receiving radiation therapy to treat it. The patient is still receiving ongoing treatment and monitoring to manage the cancer."
117,"An 11-year-old male came to our observation for his first dental visit. His medical history was negative. No symptoms were reported by the patient or his parents. The face was symmetric and no swelling of the cervical lymph nodes was observed. Intraorally, the dentition of the permanent teeth was completed, except for the third mandibular molars and the second and third maxillary molars. Bucco-lingual expansion of the jaw bones was not evident. An orthopantomogram was performed to assess the development of third molars []. Unexpectedly, the analysis revealed an intraosseous doughnut-like lesion radiopaque at the periphery and radiolucent in the center associated with the left mandibular third molar germ (a). Additional dental abnormalities were not observed. The maximum diameter of the lesion was 5.7 mm. Based on these findings, developmental abnormalities of the third molar (e.g., dilated odontoma) and odontogenic (e.g., cementoblastoma) and non-odontogenic (e.g., osteoblastoma or osteoid osteoma) tumors were considered for differential diagnoses. To better characterize the lesion, a computed tomography (CT) scan was required. The analysis established bone integrity around the lesion and its independence from the local neuro-vascular structures. In addition, it revealed, on the sagittal projection, a small gap in the proximity of the buccal surface of the mandible (b). As the most significant clinical concern related to this condition is the risk of developing pulpal necrosis, it was decided to extract the germ of the third molar and the underlying lesion. To do this, under local anesthesia, a mucoperiosteal flap was raised posterior to the mandibular right second molar. The vestibular cortical plate was removed, exposing the ovoid mass, which was removed with the germ of the mandibular tooth. The surgical flap was repositioned and sutured. Healing was uneventful. The excised lesion appeared as an empty hard spherical mass virtually devoid of content (). It was routinely processed for paraffin embedding after fixation and decalcification. Histologically (a,b), the outer hard tissue was dentin. The inner part of dentin was in continuity with basophil calcified material, which in turn was focally in contact with the bone-like matrix. The basophil calcified material focally presented a rod-like structure consistent with enamel (b, insert). More centrally, the lesion was composed of fibro-vascular tissue. The pathologic findings were considered as consistent with a dilated odontoma.","During his first dental visit, an 11-year-old boy had an unusual finding on an X-ray – a small, unusual growth around one of his lower wisdom teeth. The growth appeared to be within the bone itself and was carefully examined to rule out any serious problems. To remove the growth and prevent future issues like tooth decay, the dentist carefully extracted the wisdom tooth and the growth along with it. The removed tissue was examined under a microscope and determined to be a dilated odontoma, a common and harmless type of dental growth. The boy’s healing was smooth, and he is now back to his regular dental care."
118,"A 61-year-old woman was referred to our clinic complaining of an isolated, sudden, and painless visual loss in her right eye, within 24 h following a 2 h airplane flight (at 30,000 feet) from Paris to Madrid. Her medical history showed well-controlled hypercholesterolemia.\nTwenty-four hours later, best-corrected visual acuity (BCVA) was 20/200 in her right eye (RE) and 20/20 in her left eye (LE). There was a relative afferent pupillary defect (RAPD), color vison deficiency, and an inferior hemifield and temporal-superior quadrant scotoma (A) in the RE; funduscopic examination revealed a 360° swelling of the right optic disc, with superonasal flame-shaped hemorrhaging, venous congestion, and tortuosity. The LE was normal, with a cup-to-disc ratio of less than 0.1, suggesting “disc-at-risk”. Fundus fluorescein angiography (FFA) of the RE showed late optic-disc staining (). Cardiac and carotid Doppler ultrasound, autoimmune, and hypercoagulability tests were normal, with the exception of slightly raised serum cholesterol levels. Cranial computed tomography (CT) revealed previously unknown white matter lesions (). NA-AION associated with cerebral SVD was diagnosed.\nAfter one year of treatment with aspirin (100 mg daily), the patient developed visual disturbances in her LE, occurring during a 10 days drive in the French Alps, with a daily accumulated altitude of 1500 m. BCVA was 20/200 in her RE and 20/40 in her LE. Examination revealed edematous and flame-shaped retinal hemorrhaging at the border of the left ONH, vascular tortuosity, fluorescein leakage (during FFA), and severe widespread visual field loss with central-sparing (B), suggesting a NA-AION in the LE.\nAt the time of publication, BCVA had decreased to 20/200 in both eyes, fundus examination showed bilateral optic disc atrophy, and spectral domain optical coherence tomography had confirmed a marked decrease in peripapillary retinal nerve fiber layer thickness in both eyes ().","Patient-Friendly Summary: This 61-year-old woman experienced sudden, painless vision loss in one eye after a long flight. Examination revealed significant swelling and damage to her optic nerve, and doctors diagnosed her with a condition called NA-AION (Neuro-attributable Angle-Closure Idiopathic Optic Neuropathy). Despite treatment, she later developed similar vision problems in her other eye. Ultimately, both eyes experienced optic nerve damage, leading to decreased vision."
119,"Female, 83 years old (y/o), with hypertension, hypercholesterolemia, carotid vasculopathy (type III, AHA) and history of (h/o) smoking affected by degenerative aortic stenosis, underwent valvular replacement with a St. Jude 21 mm mechanical prosthesis in 2000. Twenty years later, she was hospitalized for respiratory distress. TTE showed left ventricle (LV) dysfunction with severe prosthetic valve stenosis (aortic acceleration time (AAT): 140 ms, transaortic maximum speed: 4.8 m/s, maximum/median gradient: 90/52 mm Hg, indexed effective orifice area (EOA): 0.3 cm2/mq, EF: 35%). TEE showed hypomobility of the anterior leaflet. Due to the shielding from the prosthesis, it was unclear if there was a thrombus or a pannus (). As it is possible to differentiate between a pannus and a thrombus due to their different radiological density (HU > 145 and > 90, respectively) [], MDCT was performed, and it showed that the anterior aortic leaflet was stuck and surrounded by hypodense tissue (Hounsfield units (HU): 203.8) interposed between native and prosthetic annuli (effective orifice area (EOA): 45 mm2, EOA/0.15) indicating a pannus (a–c). This information was of utmost importance as instead of staring anticoagulant treatment, the patient directly underwent repeat surgical repair with a bioprosthesis. The diagnosis of pannus was confirmed by pathology.","This 83-year-old woman has a history of heart problems, including a valve replacement in Recently, she was hospitalized because of difficulty breathing, and tests revealed that her heart valve was severely narrowed. Imaging showed a growth of tissue around the valve, called a pannus, which was preventing the valve from working properly. Because of this, she needed another surgery to replace the valve with a new bioprosthetic valve."
120,"Female, 44 y/o, affected by mitral valve (MV) dysplasia (parachute valve with double medioposterior papillary muscle) and subaortic stenosis caused by a fibromuscular ring, underwent subaortic membrane resection and septal myectomy in 1989. Due to worsening exertional dyspnea and persistence of subaortic stenosis, a St. Jude Regent 17 mm was implanted in 2006 (40 y/o) with improvement of her physical condition. In the last 2 years, TTE detected a progressive increase of the intraventricular gradient with LV hypertrophy (maximum speed, 4.1 m/s, maximum/median gradient: 64/39 mm Hg). TEE performed in May 2020 showed normal excursion of the prosthesis’ leaflets and confirmed severe subaortic stenosis (speed: 5.5 m/s, maximum/median gradient: 120/63 mm Hg) (). New subaortic membrane formation (SAM) was suspected but not clearly detected by TEE. MDCT provided accurate 3D reconstructions of the LV outlet tract (LVOT) with a better topographic assessment of the new SAM and its surrounding structures. The SAM was located 7 mm below the aortic prosthetic annulus, with the maximum thickness of 5 mm and hemicircumferential extension along the interventricular septal surface. This information was crucial to guide surgical excision of the SAM (a,b).","This 44-year-old woman had a heart valve problem since 1989 and had a new valve implanted in 2006, which helped her breathing. However, recent tests showed that the pressure in her heart was increasing again, and a new membrane was forming below her valve. A special scan (MDCT) showed exactly where this new membrane was located and how thick it was, which helped the doctors plan a surgical procedure to remove it. The surgery will help improve her heart function and reduce her symptoms."
121,"Male, 80 y/o, with a metabolic syndrome. He underwent thromboendarterectomy because of right internal carotid artery serrate stenosis. Due to bivasal critical coronary stenosis (anterior descending (DA) and left circumflex (LCx)) and severe degenerative aortic stenosis, he underwent coronary artery bypass graft (CABG: left internal mammary artery (LIMA-IVA)) and aortic bioprosthesis implantation (Intuity 25 mm) in 2019. Ten months after surgery, he started developing intermittent fever with serial hemocultures growing Enterococcus faecalis. TTE detected paravalvular regurgitation (PVR) with focal hyperechogenic thickening of the leaflets. Diagnosis of endocarditis was made, and antibiotic treatment was started (meropenem shifted to ampicillin and ceftriaxone according to the antibiogram). TEE showed a pulsatile perivalvular pseudoaneurysm in the mitroaortic intervalvular fibrosa (). MDCT was performed a few hours later, confirming the presence of a pseudoaneurysm with the maximum axial size of 15 × 10 × 30 mm communicating with LVOT through a 5 mm window, and also detected a periaortic abscess in the anterolateral side of the vessel with longitudinal extension of 4 cm, which was only poorly detected by TEE (a–c).","This 80-year-old man has a history of heart problems and had successful surgery in 2019 to improve his heart health. Recently, he developed a fever and infection, which doctors identified as endocarditis (an infection on the heart valves). Tests showed a leak around the new valve and a pocket of infection near his aorta. He is now receiving antibiotics and further imaging to monitor the infection and the leak."
122,"Male, 69 y/o, with hypertension, hypercholesterolemia and previous myocardial infarction. He was affected by severe degenerative aortic stenosis and underwent trans-catheter aortic valve replacement (TAVR) with LOTUS Edge 27 mm in April 2020. TTE performed a few days after the TAV implantation detected an increased transprosthesis gradient (maximum/median gradient, 78/52 mm Hg) in the absence of fever or positive hemoculture. TEE showed hypomobility of the noncoronary cusp of the bioprosthesis (). Valve’s thrombosis was suspected and heparin administration was started. MDCT detected a paravalvular leak caused by misfolding of the prosthesis’ frame; the suspicion of valve thrombosis was also confirmed by the finding of two hypodense appositions at the lower edge of the valve. The patient underwent balloon valvuloplasty with complete resolution of the valvular dysfunction (a–c).","This 69-year-old man has a history of heart problems, including a previous heart attack. He had a new valve put in place in April 2020, but recently, the valve wasn't working as well as it should. Tests showed a leak around the valve and a possible blood clot, and the doctors treated it with medication and a procedure to smooth out the valve. The leak has now been fixed, and the valve is functioning properly again."
123,"A 45-year-old, multiparous, overweight female with a history of OHP use for 13 years (levonorgestrel 0.15 mg and estradiol 0.03 mg daily) consulted the emergency room of our institution following a one-week clinical course of worsening dyspnea, general malaise, headache, and ageusia. At admission, the patient reported dyspnea at rest, associated with intermittent retrosternal oppressive chest pain radiating to the back. The physical exam revealed pulmonary aggregates on auscultation, and her vital signs showed tachypnea, tachycardia, and desaturation. Oxygen therapy was started, requiring a non-rebreathing mask at 12 L/min to maintain adequate oxygen saturation. RT-PCR test for SARS-CoV-2 was indicated. Arterial blood gases analysis showed a PAO2/FIO2 ratio of 56, and the patient was then transferred to the respiratory intensive care unit (ICU).\nHer COVID-19 diagnosis was confirmed with the positive results of the RT-PCR test for SARS-CoV-2 (50 copies of RNA/reaction). Laboratory test results showed positive severity predictors, including an elevation of D-dimer (>20 mg/L), troponin I (0.150 ng/mL), ferritin (2934 ng/mL), and lactate dehydrogenase (879 U/L) levels. Other admission paraclinical tests showed leukocytosis, neutrophilia, lymphopenia, mild thrombocytopenia, and elevation of transaminases more than three times the laboratory upper limit. Because of the risk of bacterial pneumonia co-infection, ampicillin-sulbactam was started as empiric antibiotic treatment.\nDue to significant elevation of the D-dimer, a CT pulmonary angiography (CTPA) was taken according to the YEARS protocol. The results of the CTPA showed a massive PTE with compromise to the posterior basal segmental artery of the left lower lobe, inferior lingula, and apical-posterior segment of the left upper lobe. An echocardiogram was performed, showing right ventricular dysfunction. Systemic thrombolysis with r-tPA (alteplase) 100 mg infusion over 2 h was administered according to the European Society of Cardiology guidelines.\nAfter treatment, the patient showed an improvement in her hemodynamic and ventilatory patterns. Nonetheless, during the hospital stay, the patient displayed additional complications: (1) septic shock secondary to a hospital-acquired infection requiring broad-spectrum antibiotics and hemodynamic support therapy, (2) respiratory failure with invasive mechanical ventilation support requirement and subsequent tracheostomy, and (3) severe anemia with the need for blood transfusions.\nThe patient had an adequate response to treatment with satisfactory clinical evolution, successful extubation, and transfer to the general hospital floor. The patient was evaluated by the OB-GYN attending physician, who contraindicated the further use of estrogenic hormonal contraceptives. Finally, the patient was discharged from our institution with indefinite anticoagulation therapy with a factor-XA inhibitor. The entire hospital stay course is represented in .","This 45-year-old woman came to the hospital because she was having trouble breathing, chest pain, and feeling unwell for a week. She was diagnosed with COVID-19 and also developed a serious blood clot in her lungs, which caused her heart to weaken. She received treatment with blood thinners and antibiotics, but also developed a lung infection that required a tracheostomy. Despite these complications, she improved and was eventually discharged home with ongoing blood thinners and advised against using hormonal birth control."
124,"A 74-year-old man, ASA physical class III (163 cm, 73 kg, BMI 27.4), was scheduled for tumor-wide excision, mandibulotomy, tracheostomy, and free flap reconstruction because of mouth floor squamous cell carcinoma. His medical history included hypertension and previous cystolitholapaxy for bilateral ureteral stones. The patient was taking losartan and hydrochlorothiazide and denied any drug allergies. A pre-operative chest radiograph (10 days before the surgery) showed a normal picture and an echocardiogram indicated normal left ventricular function. A mild productive cough was noted.\nA standard monitoring set-up (electrocardiogram, blood pressure, and SpO2) was implemented before induction of anesthesia. Pre-operative vital signs were within normal range (heart rate, 74 bpm; blood pressure, 168/85 mmHg; respiration rate, 18 times per minute; and an oxygen saturation of 94% on room air). Following pre-oxygenation, general anesthesia was induced with remifentanil (target-controlled infusion: 3 ng/mL), lidocaine (20 mg), propofol (180 mg), and succinylcholine (80 mg). Oral tracheal intubation with a 7.5 mm endotracheal tube was performed using a video-assisted intubating stylet (Trachway®, Markstein Sichtec Medical Corp, Taichung, Taiwan). Airway secretion was found during the tracheal intubation procedure. Mechanical ventilation was set at a volume-controlled mode with the following settings: tidal volume (500 mL), respiratory rate (10 times per minute), and positive end-expiratory pressure (PEEP; 4 cmH2O). Sevoflurane at an end-expiration concentration of 2% and cis-atracurium were used for the maintenance of anesthesia. An arterial line was established through a radial artery for continuous beat-to-beat monitoring.\nA cuffed 8.0 sized tracheostomy tube (Rota-TrachTM, Vitaltec, Taichung, Taiwan, ID 8.0 mm, OD 11.0 mm, TL 76 mm) was placed after an uneventful tracheostomy procedure. The endotracheal tube was then withdrawn without incident and no secretion impaction inside the tube was noted. However, SpO2 dropped from 100% to 96% during the course. An immediate recruitment maneuver with FiO2 1.0 was performed. When the tracheostomy was completed and ready for the second stage of the neck dissection procedure, the patient’s SpO2 continued to drop down from 96% to 89%. Peak airway pressure increased from 22 to 29 cmH2O, and EtCO2 decreased from 42 mmHg to 37 mmHg. Blood pressure was within normal range. Pure oxygen was given, and arterial blood gas (ABG) was checked, which revealed a low PaO2 (140 mmHg under FiO2 1.0).\nMeanwhile, physical examinations revealed that chest wall movement was normal at the left side but significantly suppressed at the right side. Lung auscultation revealed significantly diminished breath sounds over the right chest with a mixture of coarse crackles and rhonchi. Malposition of the tracheostomy tube with one-lung ventilation was immediately excluded. Since the pre-operative chest radiograph taken 10 days before was normal and the patient did not present any infection signs (fever, leukocytosis, etc.), pneumothorax was an initial impression ready to be excluded. A bedside lung POCUS with a curved transducer (2.5 to 7.5 MHz) was immediately used to evaluate the lung images.\nThe images of the lung POCUS showed decreasing lung sliding over the right upper lung field and a reduced sandy seashore sign with intermittent lung pulse on motion mode (M-mode; A). B-line was not observed within the 2nd and 5th intercostal space. Right mainstem intubation by the tracheostomy tube was excluded by fiberoptic bronchoscopy exam (the tip was within the trachea and above the carina). While advancing the bronchoscope past the right main bronchus, however, copious sticky yellowish sputum was encountered in the bronchial branches, especially at the right upper bronchus (B). A portable chest radiography was taken and showed partial atelectasis over the right upper lobe and increased interstitial lung density, which were compatible with the ultrasound findings (C). After adequate suctioning and recruitment maneuvers, an ABG analysis was repeated (PaO2 115 mmHg under FiO2 0.5). Because of suspected pneumonia and the planned long duration of the operation, the surgery was then deferred. Sputum culture was collected and empirical antimicrobial treatment with piperacillin and tazobactam was initiated. Haemophilus influenza was isolated from the sputum culture. After a 10-day antibiotic treatment course, a repeat chest radiograph was clear, and the patient received the pre-planned surgery uneventfully.","This 74-year-old man was preparing for a complex surgery to remove a mouth cancer, which required removing part of his jaw and placing a tube in his throat. Before the surgery, he received medications to help him relax and was closely monitored with equipment to track his heart rate, blood pressure, and oxygen levels. During the surgery, doctors noticed that he wasn't getting enough oxygen, and they used special techniques to help him breathe, including increasing the amount of oxygen he was receiving. After further investigation, they discovered he had pneumonia and postponed the surgery until he was healthy enough to proceed."
125,"Case 1: The first patient was a 79-year-old female individual with a history of hypertension, heart failure, and middle cerebral artery infarction. Blood pressure control and cardiac function were in good condition before surgery, and no neurological complications were observed. The patient’s pulmonary function test result was normal, although her chest X-ray revealed pneumonia in the right middle lobe, for which she had been treated. The patient underwent total hip arthroplasty under general anesthesia. Before the general anesthesia, monitoring using several modalities was instituted, including electrocardiography, a noninvasive blood pressure monitor, pulse oximeter, and bispectral index (BIS) monitor. The BIS was maintained at 40–60. Anesthesia was induced with propofol (2 mg/kg) and rocuronium (0.8 mg/kg), and intra-arterial cannulation was performed for continuous blood pressure monitoring. Approximately 20 min into the surgery, the patient’s oxygen (O2) saturation level dropped from 93.1% to 83.1%. While being ventilated at a fraction of inspired oxygen (FiO2) of 0.4, her arterial blood gas showed that the partial pressure of oxygen (PaO2) dropped from 161.6 to 51.2. We increased the positive end expiratory pressure (PEEP) to 10 cm H2O and FiO2 to 1.0 and performed a recruitment maneuver; however, her O2 saturation level increased only temporarily and dropped again to 81%. Upon suspecting atelectasis due to a collapsed lung, we reversed muscle relaxation and induced spontaneous respiration. The O2 saturation level recovered to 90%, and we continued the surgery with spontaneous respiration. After surgery, the patient’s O2 saturation level recovered to the preoperative state of 98%.","This 79-year-old woman had some existing health problems, including high blood pressure and heart failure, but was otherwise doing well before her hip surgery. During the surgery, her oxygen levels dropped significantly, leading doctors to suspect a collapsed lung and take steps to improve her breathing. They successfully resolved the problem and continued the surgery with her breathing on her own. Thankfully, her oxygen levels returned to normal after the surgery, and she is recovering well."
126,"Case 2: The second patient was an 89-year-old male individual with a history of hypertension and delirium. Before surgery, his blood pressure was well controlled, and although he was taking dementia medicine, the patient was able to follow commands well. His pulmonary function test results indicated an obstructive pattern. Total hip arthroplasty was performed using the same anesthetic regimen used for the first patient. While ventilating at an FiO2 of 0.4, the patient showed an onset of hypoxia, with O2 saturation level dropping from 100% to 80% and PaO2 dropping from 129 to 53.0. This patient also showed an improvement of O2 saturation level from 81% to 88% after recovering spontaneous respiration by administering a muscle relaxant-reversing agent. His O2 saturation level improved to 90% with continuous positive airway pressure. Similar to the first patient, the second patient’s O2 saturation level improved to 98% after surgery.\nNeither patient developed any respiratory complications after surgery. The first patient had no notable findings on the postoperative chest X-ray, whereas the second patient showed subsegmental atelectasis on the right middle lobe compared with the preoperative findings ().","This 89-year-old man had a history of high blood pressure and confusion, but his blood pressure was well-managed before surgery. He had some breathing problems, and his surgery required careful monitoring of his oxygen levels. During the surgery, his oxygen levels dropped, but they improved with the help of a muscle relaxant-reversing agent and continuous positive airway pressure. After surgery, his oxygen levels returned to normal, and he didn't experience any other breathing problems."
127,"The patient is a 91-year-old Caucasian man with a past medical history of coronary artery disease, congestive heart failure, atrial fibrillation, hypertension, interstitial lung disease, and obstructive sleep apnea presented with a 2-week history of productive cough, fever, shortness of breath and generalized malaise. On presentation, vitals showed blood pressure of 77/35 mmHg, heart rate of 122 bpm, respiratory rate of 38 bpm, a temperature of 102 F, and oxygen saturation of 98% on 15 L of oxygen. The patient was diaphoretic, with decreased breath sounds in the right lung field, and on palpation of the abdomen, there was right upper quadrant fullness.\nInitial laboratory studies showed elevated white blood cells (WBC) 22.6 × 109/L with neutrophilia, bicarbonate 21 mmol/L, lactic acid 6.5 mmol/L, anion gap 17, ALT 71 IU/L, AST 69 IU/L, and ALP 450 IU/L. ECG showed atrial fibrillation with a rapid ventricular response. CXR showed acute right pleural effusion (). The patient was intubated for respiratory failure. He was also started on antibiotics (piperacillin-tazobactam and azithromycin) and intravenous normal saline with no improvement in blood pressure. The patient was then started on intravenous vasopressor support with norepinephrine and vasopressin and admitted to the intensive care unit (ICU).\nDue to the right upper quadrant fullness, elevated liver enzymes and fever, an abdominal ultrasound was performed, which showed an acute complex heterogeneous hypoechoic 8 × 7 × 6 cm mass-like lesion in the right hepatic lobe ().\nTo better characterize the lesion, a CT abdomen was done. The CT showed a complex low-density right hepatic lobe subcapsular lesion measuring 13 × 8 × 7 cm, directly abutting the right anterior diaphragm, along with diffuse gross gallbladder wall thickening with cholelithiasis and a moderate right pleural effusion ().\nThe patient underwent chest tube placement with the removal of 1600 cc of cloudy light-brown-colored fluid. Pleural fluid analysis was consistent with empyema, with WBC of 70,800 cells/mcL (61% neutrophils), glucose less than 10 mg/dL, LDH of 4821 IU/L, pH of 7.0, protein of 3.6 gm/dL, and Gram stain showing Gram-positive cocci in chains. Cytology was negative for malignant cells but showed severe acute inflammation and rare mesothelial cells. The blood culture on admission grew beta-hemolytic streptococci. The pleural fluid culture grew Streptococcus anginosus. On day two of hospitalization, the patient was scheduled for CT percutaneous drainage of liver abscess. However, the CT revealed a significant decrease in the size of the right subdiaphragmatic perihepatic collection to 1.5 cm in greatest thickness ().\nDue to the improvement of the hepatic collection, the CT-guided drainage of the collection was not performed. Following the persistence of pleural effusion and decreased effluent from the chest tube, an intrapleural thrombolytic was instilled via chest tube with the removal of 1.5 L exudative fluid. Repeat CXR showed improvement in the right pleural effusion (), and the patient was successfully extubated on day three of hospitalization.\nHis hospital course was subsequently complicated by new onset and worsening respiratory distress on day eight of hospitalization requiring re-intubation. Repeat CT chest showed diffuse narrowing of the left mainstem bronchus, occlusion of right posterior segmental bronchi with atelectasis, complete collapse of the left lower lobe due to occlusion of left lower lobe central bronchi with trace right pleural effusion ().\nEmergent flexible fiberoptic bronchoscopy was done and showed copious thick mucopurulent secretions that were suctioned to clear. The bronchoalveolar lavage grew Candida albicans, which was not thought to be a pathogen. After two weeks of hospitalization and the inability to wean the patient from the ventilator, the patient was terminally extubated, and care was focused on comfort.","This 91-year-old man was admitted to the hospital with a serious infection, including a fever, cough, and shortness of breath. His blood tests and imaging showed a large collection in his liver and fluid buildup around his lung, along with signs of pneumonia. After receiving antibiotics and supportive care, the liver collection improved, and the fluid around his lung cleared up, allowing him to be successfully weaned off the ventilator. However, he developed new breathing problems and was ultimately cared for comfortably at home."
128,"A 93-year-old patient visited our hospital with hypokalaemia, malnutrition, and decreased renal function detected by a family physician.\nFive years before her visit to the hospital, she had undergone bowel resection several times (). As a result, she had been suffering from diarrhoea for about three months, thought to be caused by SBS. The diarrhoea improved spontaneously and she had no abdominal symptoms. Then, one year before admission, watery diarrhoea appeared, and although antidiarrhoeal medication was prescribed, there was little improvement.\nHer past history included colonic perforation, abdominal wall hernia with strangulated ileus, and resection of about 2 m 30 cm (59.1 inches) of the terminal ileum (). Five years prior to this admission, she was diagnosed with strangulated ileus, and the small intestine was resected, 7 cm from the terminal ileum and 50 cm from the ligament of Treitz (). At presentation, the patient’s blood pressure was 95/67 mmHg, heart rate was 59 beats per minute, SpO2 as 95%, and her temperature was 36.6 °C. On physical examination, normal breath sounds and heart sounds with mild systolic murmurs were observed. The abdomen was flat and soft. Murphy’s sign was negative, and there was no costovertebral angle tenderness. Lower leg oedema was observed. The results of blood tests were as follows: white blood cell count 15.30 × 103/μ (neutrophils 78.3%, lymphocytes 15.5%, monocytes 5.6%, eosinophils 0.4%, basophils 0.2%), red blood cell count 3.34 × 106/μ, hemoglobin 11.3 g/dL, hematocrit 33.2%, platelet count 27.9 × 104/μ, total bilirubin 1.6 mg/dL, aspartate aminotransferase (serum glutamic-oxaloacetic transaminase) 48 IU/L, alanine aminotransferase (serum glutamic-pyruvic transaminase) 37 IU/L, total protein 5.2 g/dL, albumin 2.5 g/dL, blood urea nitrogen 18.7 mg/dL, creatine 1.13 mg/dL, Na 143 mEq/L, K 1.7 mEq/L, Cl 99 mEq/L, Ca 5.0 mg/dL, P 2.8 mg/dL, Mg 0.8 mg/dL, and estimated glomerular filtration rate 34.1 mL/min/L. An abdominal computed tomography showed mild oedema of the colon, and no other obvious abnormalities (). No obvious organic abnormalities were noted on imaging, but the bowel was shortened because of repeated bowel resections. Colonic specimens showed only nonspecific inflammatory findings (a,b). We believe that this inflammatory finding was due to an imbalance between the protective and aggressive factors of the intestinal tract due to ageing.\nAfter hospitalization, her electrolyte imbalance and renal function were improved by an infusion of magnesium and potassium, but the diarrhea persisted. A colonoscopy was performed and pathological specimens were taken. However, the colonoscopy, which was performed to evaluate diarrhea, did not reveal any specific findings. In addition, stool culture and histopathological examination did not reveal the exact cause. Taken together, this was likely to be de-adaptation of SBS, as a part of the clinical course. Since diarrhea and electrolyte abnormalities continued, we concluded that her bowel could not absorb nutrients, and therefore switched to central venous (CV) nutrition (delivered via a CV port), which improved her diarrhea. She was discharged in good general condition about one month after creation of the CV port.","This 93-year-old woman was admitted to the hospital because of low potassium levels, poor nutrition, and reduced kidney function, which were noticed by her family doctor. She has a history of several bowel surgeries and ongoing diarrhea, likely due to a long-standing digestive issue. After receiving treatment for her electrolyte imbalance and kidney function, she was unable to absorb nutrients and started receiving nutrition directly into her bloodstream. Ultimately, she was discharged home with improved nutrition and continued monitoring."
129,"A 29-year-old male, HIV-positive since 2015, severely immunosuppressed that was lost to follow-up before starting ART. He presented in March 2019 at the emergency room (ER) with a one-day history of fever, shortness of breath and cough without providing information about his HIV status. Initial assessment showed polypnea of 30 cycles per minute (cpm), hypoxia, fever (39 °C), elevated C-Reactive Protein (CRP) and bilateral middle and lower zone air space opacities on chest X-ray. He was admitted to the ward and started empirical treatment for community acquired pneumonia (CAP). Two days later, he was transferred to the ICU with aggravated tachypnea (50 cpm), severe hypoxemia (paO2 49 mmHg) despite oxygen supplementation and pneumomediastinum, bilateral pneumothorax and diffuse ground-glass opacities on thoracic-CT scan (a). The CD4+ lymphocyte count was 6/mm3 and the HIV-viral load was 18,200 copies/mL. All other microbiologic tests were negative. Treatment was then switched empirically to trimethoprim-sulfamethoxazole (TMP-SMX) 15 mg/kg of TMP each day in 3 takes plus corticosteroids for a presumed diagnosis of PJP. Later the diagnosis was confirmed by positive immunofluorescence as Pneumocystis jirovecii (P. jirovecii) in bronchoalveolar fluid (BAL).\nDue to refractory hypoxemia and given the high probability of barotrauma, the patient was started on venovenous-ECMO(VV-ECMO) without prior tracheal intubation. He later needed intubation due to poor bronchial clearance of secretions and completed a 14 days-period of protective IMV in an attempt to reduce extra corporeal support. He completed 21 days of therapy with TMP-SMX plus corticosteroids according to recommended PJP treatment dosage (prednisolone 40 mg two times day for 5 days, then 40 mg each day for 5 days and after that 20 mg each day for 11 days). ART was started 15 days after the ICU admission, with a significant reduction in the viral load one month later (151 copies/mL). ECMO and protective IMV were maintained for 40 days, followed by 19 days of weaning off. The pneumomediastinum and bilateral pneumothorax were managed conservatively, and a new CT-scan performed 50 days later showed great improvement (b). He was transferred to the ward after 69 days of ICU stay showing signs of significant myopathy. Three months after discharge, he was revaluated at outpatient care as fully recovered and with CD4+ lymphocyte count improvement (49/mm3).","69 days ago, a 29-year-old man with HIV, who had missed previous medical appointments, was admitted to the hospital with a serious lung infection. He was struggling to breathe and had a high fever, and doctors initially thought it was pneumonia. After further tests, they discovered he had a rare lung infection called P. jirovecii, and he needed a breathing machine to help him breathe. He received treatment and his condition improved, and he was eventually able to go home, with his HIV levels significantly reduced."
130,"A 64-year-old woman with a history of hypertension, dyslipidemia and chronic pulmonary disease presented at the ER with fever, shortness of breath and a worsening cough despite a previous complete course of antibiotics for presumed CAP. She was hypoxic, with isolated elevation of CRP and diffuse ground-glass opacities on thoracic CT-scan (a). Her status deteriorated despite antibiotics and oxygen supplementation in the Intermediate Care Unit, so she was transferred to the ICU and intubated. Three days after IMV and prone positioning, she was connected to VV-ECMO due to refractory respiratory acidemia. Anti-HIV testing was positive. Immune and viral study revealed severe immunosuppression (9 CD4+/mm3) and high serum viral load (4.050.000 copies/mL) and TMP-SMX plus corticosteroids were started for presumed PJP, at the recommended PJP treatment dosage. Diagnosis was confirmed by positive immunofluorescence for P. jirovecii in BAL.\nECMO was discontinued after 10 days. During the weaning off invasive ventilation, there was recrudescence of ARDS with increased ventilatory parameters and need for prone positioning. Nosocomial infection was considered, broad spectrum antibiotics were started and bronchofibroscopy repeated, with persistently positive immunofluorescence for P. jirovecii and a positive polymerase chain reaction (PCR) for cytomegalovirus in BAL. She completed a total of 33 days of treatment with TMP-SMX and 21 days of ganciclovir with respiratory improvement and started ART. She was extubated after 83 days and was transferred to the ward after three months of ICU stay for muscular rehabilitation, without other dysfunctions.\nFollow-up imaging can be seen in b. She was transferred to a rehabilitation unit with a residual need of oxygen support (2 L per minute), from which she recovered after some months of pulmonary rehabilitation.","This 64-year-old woman was admitted to the hospital with a bad cough and shortness of breath, even after taking antibiotics. Tests showed she had lung damage and was struggling to breathe, and she needed to be put on a breathing machine. It was later discovered she had HIV and a serious lung infection called PJP, which required intensive treatment and support. After several months of treatment and rehabilitation, she was able to breathe on her own and was discharged home with a small amount of oxygen."
131,"A 53-year-old woman, with no relevant medical history so far, was brought to the ER due to a two-month history of progressive psychomotor slowness and confusion, which had worsened in the week before. At physical examination, she was agitated and febrile. Head CT scan showed some intra-axial lesions in the left frontal and temporal lobes. The cerebral spinal fluid (CSF) had mild pleocytosis and moderately elevated proteins. The serology for HIV was positive, and the nucleic acid test of the CSF was positive for toxoplasma gondii. She was admitted in the ICU with a de novo diagnosis of HIV infection, with severe immunosuppression (CD4+ count 28 cells/mm3), clinically manifested as cerebral toxoplasmosis. On day 3, she began coughing, with respiratory hypoxemic insufficiency and bilateral diffuse glass opacities on chest-CT scan (a). The presumptive diagnosis of PJP was posteriorly confirmed with both direct dye-examination and PCR positive for P. jirovecii in BAL. She was treated with TMP-SMX for both PJP and cerebral toxoplasmosis. Following one week of appropriate medical treatment, the patient had a favorable response, and was discharged to the ward for further care.\nAt the end of the month, she was readmitted to the ICU because of respiratory failure and elevated lactate. Respiratory secretions and gastric aspirate were both negative for tuberculosis. Other microbiology tests (including blood serologies for other common opportunistic agents) were also negative. She repeated chest-CT, and had severe deterioration in the lung opacities, with bilateral consolidation described as possible ARDS and/or nosocomial infection. As she showed no signs of clinical improvement despite corticosteroids and High Flow Oxygen Therapy (HFOT), she was intubated, had a repeat bronchofibroscopy and started broad spectrum antibiotics.\nThe patient developed septic shock and ARDS with refractory hypoxemia and she was put on VV-ECMO. The indirect immunofluorescence was positive for P. jirovecii in BAL. She completed 21 days of treatment for PJP and 7 days of piperacillin- tazobactam, with respiratory improvement. ECMO was stopped after 12 days.\nPersistent fever and elevated inflammatory markers ensued, with isolation of multidrug-resistant Pseudomonas aeruginosa in respiratory secretions. Chest X-ray confirmed lobar nosocomial pneumonia. She started a targeted antibiotic course with cefepime, with good clinical, analytical, and radiological response. Roughly one week later, she was extubated to non-mechanical ventilation, and rapidly weaned off respiratory support to no oxygen supplementation. The evolution in her condition can be seen at the images in b.\nShe was discharged to the ward after one month of ICU stay for muscular rehabilitation, already on antiretroviral therapy and free of acute infectious complications.","This 53-year-old woman was admitted to the hospital after experiencing a gradual decline in her thinking and movement over two months. Tests revealed she had a new infection with HIV and toxoplasmosis, which had caused swelling in her brain. She then developed a lung infection, leading to respiratory failure and the need for a breathing machine. After several weeks of treatment and supportive care, she made a full recovery and was discharged home, continuing with HIV treatment and physical therapy."
132,"A 36-year-old male, overweight and with HIV infection diagnosed in 2009, with poor adherence to appointments and complete discontinuation of ART in the three months before admission.\nThe patient presented at the ER with a 3-week history of worsening cough, dyspnea, and fever. Initial assessment showed hypoxia, fever (39 °C), elevated CRP, 6 CD4+ lymphocytes/mm3 and several ground glass opacities on thoracic CT-scan (a). He started empirical treatment with TMP-SMX plus corticosteroids at the recommended PJP treatment dosage and was admitted to the ward. The need for oxygen support increased in the next few hours and the patient responded poorly to HFOT. Twenty-four hours later he was admitted to the ICU and VV-ECMO was started. No tracheal intubation was performed. PJP was confirmed by positive immunofluorescence in BAL.\nAfter 9 days of ECMO support the patient became delirious and agitated, which caused flow problems in the extracorporeal circuit and eventually led to the need for sedation and subsequent intubation. He completed 21 days of treatment, initially with TMP-SMX, then changed to atovaquone plus primaquine due to hematologic toxicity. ECMO support was maintained for 26 days.\nHe was transferred to the ward for rehabilitation after 37 days of ICU stay, and already on ART. The follow-up CT-scan can be seen in b.\nAll four patients are being followed and regularly observed as part of our Infectious Diseases program and are functional and radiologically recovered, a summary of the patients’ characteristics and evolution is presented in .","This 36-year-old man with HIV and a history of not taking his medication well came to the hospital with a serious lung infection. He had a bad cough, fever, and difficulty breathing, and tests showed damage to his lungs. He was treated with a special machine to help him breathe and antibiotics, but his condition worsened, requiring him to be intubated and receive ECMO support for several weeks. After a long recovery, he was able to be weaned off the machine and antibiotics, and he is now starting HIV treatment again and is being closely monitored."
133,"Case History: A 52-year-old white male inmate with a history of non-steroidal anti-inflammatory drugs (NSAIDs) therapy and enalapril therapy for hypertension was admitted to the emergency room for repeated lipothymia in the absence of sweating, with hematemesis from the previous evening and melaena from three days before. The patient was hemodynamically unstable with acute anemia. The hemoglobin value upon admission was 6g/dL, while the procalcitonin in the blood was not evaluated. Therefore, a computed tomography (CT) scan of the abdomen was performed, which revealed a narrow lumen of the second portion of the duodenum; furthermore, the esophagus-gastro-duodenoscopy (EGDS) examination revealed multiple sub-centimeter lymph node formations in the stomach with normodistended walls due to insufflation, and fundus and gastric bodies occupied by food residues and clots; at the level of the first duodenum, there was an ulcerated lesion covered by a large clot. After a worsening of the condition, the patient was transferred to Intensive Care, was intubated and underwent therapy to restore hemodynamic balance. On the fifth day, the hemodynamics were unstable, and the anemia persisted. An emergency gastroscopy was performed in resuscitation, which revealed the absence of blood in the esophagus, stomach, and duodenum, and ulcerative lesion of the duodenal bulb with circumferential extension to the intestinal wall. Conditions precipitated due to common complications of hypovolemia. Hemorrhagic shock and peritonitis due to enterobiasis were assessed as causes of death. After 72 h, an autopsy was performed in accordance with the recommendations on the harmonization of forensic autopsy rules of the Committee of Ministers of the Council of Europe (1999) and according to the commonly accepted criteria for sudden cardiac death (SCD). Femoral blood was analyzed for alcohol (ethanol) and volatiles by head-space gas chromatography coupled with a flame ionization detector (GC/HS-FID). All post-mortem specimens were screened for the presence of the main different classes of drugs (pharmaceuticals and illegal drugs), using immunological or chromatographic methods as appropriate. A systematic toxicological analysis (STA) was performed by the LC-MS/MS system (API 3200 triple quadrupole ABI-SCIEX) in multiple reaction monitoring (MRM) mode.","This 52-year-old man was rushed to the hospital after experiencing dizziness, vomiting blood, and black stools. He was very weak and had low blood counts. Tests revealed a serious problem in his stomach and duodenum, with a large ulcer and swollen lymph nodes. Despite treatment, his condition worsened, and he needed to be put on a breathing machine. Ultimately, the cause of death was determined to be a combination of hemorrhagic shock and peritonitis, and a thorough investigation was conducted to rule out other causes."
134,"A nine day old male newborn was admitted to our hospital due to fever and poor general condition. The pregnancy was complicated by threatened miscarriage and placental abruption. He was born at 36 weeks + 1 day of GA by spontaneous delivery. Perinatal cardiotocographic monitoring was negative. Neonate blood gas analyses and cardiorespiratory adaption were normal, and the Apgar score was 7 and 8 at 1′ and 5′ minutes, respectively. Birth weight was 2950 g. The subsequent early postnatal period was complicated by transient hypoglycemia; neonatal clinical assessment was normal, postnatal weight loss was within normality range (<10%), and the neonate was discharged on the fourth day of life. The mother was tested for SARS-CoV-2 at admission in the obstetric ward with a negative result and a positive result at discharge, without any symptoms. At day nine, the baby developed fever (38 °C) and poor feeding.\nThe nasopharyngeal swab, tested for SARS-CoV-2 by qualitative realtime PCR (AllplexTM SARS-CoV-2 Assay, Seegene), was positive; thus, he was admitted to our COVID-19 center. In the subsequent 24 h, he developed progressive respiratory failure and diarrhea with enterorrhagia and was admitted to the PICU. Surgical evaluation with abdominal X-ray and ultrasound excluded the suspicion of volvulus or necrotizing enterocolitis; echocardiography and electrocardiogram were normal although the N-terminal prohormone of brain natriuretic peptide (NT-proBNP) and Troponin T (TnT) were elevated ().\nThe baby was supported with noninvasive ventilation, and treatment with antibiotic wide coverage (ampicillin plus gentamycin plus metronidazole) was started. In the subsequent days, the neurological condition deteriorated with impaired consciousness and pathological spontaneous motricity, and we observed worsening of respiratory failure leading to intubation and mechanical ventilation on day three. The chest radiograph and CT scan showed a picture of bilateral interstitial pneumonia with an extensive area of atelectasis in the basal site.\nOn the nasopharyngeal swabs (NPS) and bronchoalveolar lavage (BAL), SARS-CoV-2 viral load was assessed by quantitative realtime PCR (Quanty COVID-19 assay, Clonit Srl, Milan, Italy), revealing a high viral load at admission ().\nSARS-CoV-2 variant determination was performed by realtime PCR single-nucleotide-polymorphism (SNP) detection approach (COVID-19 Variant Catcher, Clonit Srl, Milan, Italy, CE IVD). N501Y mutation and HV69-70 deletion, suggesting VOC 202012/01 Lineage B.1.1.7 variant (also known as UK variant), were detected. Thus, we started anti SARS-CoV-2 hyperimmune plasma day three and four, plus dexamethasone 0.15 mg/kg/die and remdesivir 2.5 mg/kg day 5 and 1.25 mg/kg for nine more days.\nBecause of the neurological impairment, a lumbar puncture was performed and was normal, with negative microbiological results including SARS-CoV-2. The metabolic test on blood and liquor were normal. Immunological screening showed no abnormalities. The EEG showed hypovolted brain electrical activity with a discontinuous pattern; no electrographic or electroclinical seizure was recorded. The cerebral CT scan was normal; the brain MRI showed deep medullary vein thrombosis associated with cytotoxic edema in the deep periventricular white matter (fan-shaped configuration) (, and ), thus, treatment with enoxaparin 100 UI/kg q 12 was started.\nThe clinical condition progressively improved; after thirteen days the baby was extubated and in four more days became oxygen independent, able to feed on the breast, with neurological and EEG improvement. Extended thrombophilic screening including homocysteine, anticardiolipin antibodies, antiphospholipid antibodies, anti-beta-2-glycoprotein antibodies, protein S, protein C, activated protein C resistance, lupus anticoagulant, factor XIII, von Willebrand factor antigen, Factor V Leyden (G1691A) mutation, factor II prothrombin (G20210A), MTHFR (C677T) mutation all were normal. Genetic investigation of CFTR (Cystic Fibrosis Transmembrane conductance Regulator) showed normal results. After 39 days, the nasopharyngeal swab for SARS-CoV became negative, and the baby was discharged after 42 days. At the five month follow-up visit, the baby was doing well, without any clinical problems. A T1-weighted MRI showed significant reduction in linear hyperintense lesions, normal intensity of periventricular white matter, and enlargement of lateral ventricles (, and ).","This nine-day-old baby was admitted to the hospital because he had a fever and wasn’t doing well. He was born prematurely and had some complications during pregnancy, and later tested positive for COVID-As his condition worsened, he needed breathing support and antibiotics, and doctors found a blood clot in his brain. After receiving treatment, including immune therapy and medication, he gradually improved and was eventually able to go home after 42 days. At his follow-up visit at five months, he was doing well and the brain scans showed a significant reduction in the areas of concern."
135,"A 5-year-old boy with a molecularly confirmed diagnosis of DMD was referred for further clinical evaluation because of ID, ASD, joint hyperlaxity, and morphogenetic anomalies. A history of epilepsy with tonic–clonic seizures, photosensitivity, and moderate elevation in serum creatinine phosphokinase (CPK) levels following physical exercise was reported in his mother. He was born from non-consanguineous parents after an uneventful dizygotic twin pregnancy. A cesarean section was performed at 35 weeks of gestation due to a twin pregnancy. His birth weight was 2080 g (50th centile), and his Apgar scores were 81 and 95. His parents reported initial concerns during the child’s first year of life. A lack of eye contact, visual tracking, and social interest were noted from early on, associated with delayed milestones. He reached head control at 3 months and could not roll over and sit up without support till the age of 14 months; he walked independently at 4 years of age. On EEG, focal spikes over the frontal region and the left temporal region and generalized spike-and-wave complexes during sleep were detected in the absence of overt epilepsy. A brain MRI showed normal results. Following the detection of an increased CPK level (13,000 UI/L) and elevated liver enzymes (AST 272 U/L, ALT 388 U/L), muscular dystrophy was suspected at the age of 4 years and the child underwent the genetic analysis of the dystrophin gene. The deletion of exons 46-51 of the gene was detected through MLPA, consistent with the diagnosis of DMD. The mother was found to be a heterozygous carrier, as expected from her increased CPK level. When evaluated at the age of 5 years, the child presented with severe developmental delays and autistic features, including poor eye contact, the absence of protodeclarative pointing, attention deficit, and inadequate social-communicative abilities. He could not follow simple instructions and a cognitive test could not be performed. Joint hyperlaxity and peculiar facial traits were noted, including a high forehead, epicanthic folds, deep-set eyes, an elongated face, and large ears. Notwithstanding the reported high rate of cognitive impairment and neurobehavioral abnormalities in DMD, the complexity of the whole clinical phenotype of our patient—in particular, the association of motor delay and severe ID with ASD and the distinctive facial dysmorphisms—led us to hypothesize comorbidity with an additional genetic condition, namely, with FXS. Following array-CGH analysis, which detected no additional CNVs, except for the deletion formerly identified in the dystrophin gene, the child underwent FMR1 molecular analysis. He was found to be a mosaic carrier of a large premutation (PM) with 170 CGGs and of an MFM with an expansion above 200 CGG triplets, confirming the clinical hypothesis of FXS (). His unaffected mother was heterozygous for a normal allele with 20 CGGs and a premutation allele with 80 triplets. The boy is currently receiving a rehabilitation program with slight improvement shown, especially in his motor skills.","This 5-year-old boy has a genetic condition called Duchenne Muscular Dystrophy (DMD), which causes muscle weakness and affects his development. He also has autism and significant delays in learning and social skills, and his parents suspect he may also have Fragile X Syndrome (FXS). Tests confirmed the DMD diagnosis, and he also has a genetic variation associated with FXS. He is currently receiving physical therapy and showing some improvement in his motor skills."
136,"This 7-year-old girl is the only child of non-consanguineous parents. She was born at 39 weeks of gestational age via an urgent cesarean section due to maternal premature rupture of the membranes (PROM). At birth, she presented respiratory distress and her Apgar scores were 51 and 85. Her birth weight was 3550 g (75th centile), her length was 53 cm (90th centile), and her OFC 36.5 cm (around 98th centile). Soon after birth, she developed spontaneous tremors of the upper limbs, axial hypotonia, and apnea episodes treated with phenobarbital and oxygen, respectively. A brain ultrasound and MRI were normal. EEG displayed continuous activity, with occasional sharp elements in the right temporo-occipital area. Audiometric and fundus oculi examinations were both normal. She reached head control at 3.5 months, sitting position at 10 months, and non-autonomous standing station at 15 months. Up to 15 months, she presented difficulties in handling objects with coarse grip. Language was poor with very few words developed at 13 months. Psychomotor delay was accompanied by macrocephaly: until 4 months of age, OFC was at the 98th centile and from 8 to 10 months it was abundantly above the 98th centile. Upon physical examination, she presented with a broad and rounded forehead, a small nose with saddle root and anteverted nostrils, a reverse epicanthus, sparse eyebrows in the medial portion, fetal finger pads, ligamentous hyperlaxity, and a sandal gap with prominent heel (). Upon clinical evaluation performed at 4 years and 5 months of age, the young girl pronounced few simple words, walked with a broad-based gait and showed a lack of sphincter control. Negative results came from the direct nucleotide sequencing analysis of the following genes: lamin A/C, SEPN1, NFIX, EZH2, NSD1, SETD2, COL6A1, COL6A2, and COL6A3. Array-CGH revealed a “likely benign” 9q21.31 duplication of approximately 200 kb, with no associated genes accessed on 18 January 2021 and accessed on 18 January 2021). Parental origin was not investigated. Molecular analysis for FXS revealed heterozygosity for a normal allele of 29 CGG repeats and a series of expanded alleles in the range of PM and FM (between 73 and >200 CGGs). After the diagnosis of FXS in the proband was established, the parents were also examined with the following results: her father carried the 29 CGG allele, while her mother had a normal allele of 23 triplets and a PM of 121–131 CGG triplets. As the diagnosis of fragile X syndrome could not explain all the clinical findings of the proband, a trio WES was undertaken and revealed the presence in the proband of a de novo heterozygous variant c.592G>A p.(Glu198Lys) in the PPP2R5D gene (NM_006245.3). This variant is reported in HGMD (Human Gene Mutation Database; CM153575) [] and never in GnomAD (Genome Aggregation Database). The same variant was previously reported de novo in patients affected by moderate/severe ID [,].","This 7-year-old girl was born early after her mother’s water broke and had some breathing problems at birth. She has developed some delays in reaching milestones like sitting and walking, and she has difficulty with fine motor skills. Her brain scans were normal, but she has unusual features like a large forehead and loose joints. Genetic testing revealed she has a new variation in a gene called PPP2R5D, which is associated with a condition called fragile X syndrome, and this is likely the cause of her developmental challenges."
137,"Patient 3 is a 33-year-old man who is the second child of non-consanguineous parents. He started walking and saying his first words at the age of 2. At the age of 10 years he suffered his first seizure episodes and when he was 22 years old he had a coma episode following a severe seizure crisis. Brain angio-MRI showed temporo-mesial sclerosis, left A1 segment agenesis with origin of the left anterior vertebral artery from the right circle, asymmetry of the supratentorial ventricular system due to the prevalence of the right trigone, and an occipital horn. He is currently still undergoing anticonvulsive treatment with valproic acid, oxcarbazepine, and topiramate. His character is calm, with a few nervous jerks (due to seizure medication). Presently, he attends a day center and practices sport (judo) and recreational activities (dancing). Family history revealed two further male patients (both sons of a maternal cousin) affected by ID of an unknown cause and diagnosis. Physical examination showed an elongated face, high forehead, wide and anteverted ears, a long and flat philtrum, midface hypoplasia, joint hyperlaxity, and hypotonia. A molecular analysis of the FMR1 gene and array-CGH were performed, revealing an FMR1 MFM allele (>200 CGGs) (A). Following this result, his mother was tested and found to be heterozygous for a normal allele of 30 CGG triplets and a PM allele with 79 CGGs. She underwent menopause at 39 years and had a spontaneous fracture of the femur at 50 years. Computerized bone mineralometry showed severe osteoporosis. No history of seizures was reported in the mother. The proband’s sister was found to carry a PM of the FMR1 gene and she had a first unaffected daughter and a second son affected by FXS. Additionally, array-CGH revealed a chromosome 2p25.3 deletion spanning around 500 kb, again derived from his premutated mother (B). The deleted region on chromosome 2 was included between positions 1,145,059 and 1,670,349 (according to Genome Browser Assembly hg19, GRCh37, February 2009) and encompasses the SNTG2, TPO, and PXDN genes, and possibly MYT1L. There were no specific probes in this last locus at the 44 K resolution of the employed array. Due to the association of the MYT1L gene with ID and seizures and since the clinical presentation of the proband was complicated by severe convulsions, we quantified the MYT1L transcript levels in the proband and his mother in order to verify if there was a “positional effect” of the microdeletion on chromosome 2. The results of real-time PCR revealed a decreased level of MYT1L transcript in the proband when compared to his mother and neurotypical controls (C).","This 33-year-old man has a history of seizures and a coma episode, which led to scans revealing changes in his brain structure. He’s currently taking medication to control his seizures and has some physical characteristics like a flat face and joint looseness. Genetic testing showed he has a genetic condition related to Fragile X Syndrome, inherited from his mother, and a deletion on chromosome 2 that may be contributing to his symptoms. Further testing is being done to understand the impact of this deletion on his brain."
138,"A 59-year-old Lithuanian male presented to our department due to deterioration of cognitive functions that had been observed for 2–3 years and gotten worse over the past three days. The patient could not perform some simple tasks in everyday life and lost his previous interests. He maintained some independence though, such as being able to go to the supermarket and do housework unsupervised. Past medical history was significant for dyslipidaemia, arterial hypertension, and stroke at the age of 36 with mild right hemiparesis. He also experienced several episodes of aphasia, which could be considered as transient ischemic attacks (TIAs). The patient had a history of smoking for a long time. He was born full-term and healthy; his parents, four siblings, and two offspring did not have any relevant health problems and no hereditary diseases were identified among family members. On neurological examination, mild bilateral dysmetria was observed and the mental examination revealed executive dysfunction and pronounced cognitive slowing. Mini–Mental State Examination (MMSE) score was 25, Frontal Assessment Battery (FAB) score was 5, phonemic fluency (words beginning with P) was 4 in one minute, and semantic fluency (animals) was 3 in one minute. Laboratory blood tests revealed significant dyslipidaemia (total cholesterol level—7.55 mmol/L, low-density lipoprotein level—5.82 mmol/L). Cerebrospinal fluid analysis was unremarkable. Low grade bilateral internal and external carotid artery stenosis was detected on carotid ultrasound. Brain magnetic resonance imaging (MRI) revealed communicating hydrocephalus, most likely due to brain atrophy and secondary brain changes, with no obvious cause of obstruction in the ventricles (Huckman index was equal to 66; the width of the third ventricle was equal to 10 mm), and extensive leukoencephalopathy, Fazekas scale score 2–3, lacunar lesions in the dorsal part of pons, thalamus bilaterally, and right cerebellar hemisphere ().\nSince the patient developed early onset progressive dementia, had a stroke at a young age, several TIAs, and brain MRI was significant for extensive leukoencephalopathy, genetic testing by next generation sequencing for inherited cerebral small vessel disease was performed. On follow-up, the patient began manifesting positive psychiatric symptoms (hallucinations, delusions, anxiety) at the age of 60 that required several hospitalisations to the psychiatric ward. Cognitive functions further deteriorated from baseline MMSE score of 25 to 14 in 3 years, and the patient gradually became fully dependent in daily life. He also developed bladder and bowel incontinence and gait apraxia at the age of 62. In parallel, brain MRI showed evolution of findings: communicating hydrocephalus and leukoencephalopathy were progressing over time (HI was equal to 82, the width of the third ventricle was equal to 11 mm, Fazekas scale score 3), and new lacunar ischemic lesions and hemosiderin deposits appeared ().","This 59-year-old man has been experiencing a gradual decline in his thinking and memory for the past few years, making everyday tasks more difficult. He has a history of stroke, TIAs, and high cholesterol, which may have contributed to changes in his brain. Brain scans showed significant damage and shrinking of the brain tissue, along with fluid buildup, and further testing is being done to determine the cause. As his condition has worsened, he has also started experiencing mental health symptoms and is now largely dependent on others for care."
139,"Patient 2.II.1 (a right) is a male diagnosed at the age of 34 years old. He presented with high serum ferritin levels (but <1000 µg/L) and high serum iron. In addition, he had hypogonadotropic hypogonadism treated with testosterone and moderate hepatic steatosis. As expected for an iron overload disease, the hepcidin levels of the patient were low (0.1919 ng/mL). One year later, serum ferritin levels peaked to 3942 µg/L. Magnetic resonance shows no evidence of iron overload in the heart while in the liver revealed increased iron concentration of 47 µmol/g indicative of hepatic iron overload (normal values <36 µmol/g). Iron chelation with Desferoxamine was used as the main therapeutic treatment. Initially, phlebotomies were performed in combination with iron chelation but had to be stopped due to intolerance. Iron chelation treatment ended in 2020 and the patient is now asymptomatic. The patient will continue with maintenance therapy.\nPatients A.II.1 and A.II.2 (b upper first panel) are two male brothers of Asian origin diagnosed with HH at 35 and 37 years old respectively. Both presented with high levels of serum ferritin and iron, while in both patients, the hepcidin levels were 0.2395 and 0.0111 ng/mL respectively. Hepatic magnetic resonance showed a severe hepatic iron overload (282.97 µmol Fe/g and 265 µmol Fe/g). The treatment option for both patients consisted of weekly phlebotomies in combination with iron chelation (Desferoxamine). A.II.1 proband started the phlebotomies in January 2019 (weekly) and the Desferoxamine treatment in May 2019. In February 2021, after 100 phlebotomies and approximately 22 g of iron removal the ferritin levels dropped to normal levels, but transferrin saturation remained high. A.II.2 proband started the phlebotomies in July 2017 (once a month) and the Desferoxamine treatment in January 2018 (initial dose of 1080 mg/day that eventually was increased to 2160 mg/day in May 2018). At the last data available (February 2021), the patient accumulated a total of 46 phlebotomies that removed a total amount of 9 g of iron and resulted in the normalization of ferritin and transferrin saturation parameters. The iron depletion was partially confirmed by the last hepatic magnetic resonance performed to the A.II.2 patient that showed a moderate iron overload (70.33 µmol Fe/g).","This note describes two men, including the patient, who have a condition called hereditary hemochromatosis, which causes their bodies to absorb too much iron. Both men had high levels of iron in their blood and livers, leading to organ damage. They were treated with regular blood removal (phlebotomy) and iron-chelating medication to remove the excess iron. After several years of treatment, the patient’s iron levels returned to normal, and he is now doing well."
140,"Patient B.II.1 (b upper second panel) is a male of 46 years old diagnosed in 2012 with hemochromatosis that presented with hyperferritinemia and severe hepatic iron accumulation (300 µmol Fe/g) detected by hepatic magnetic resonance. The patient also suffers from dyslipidemia and internal hemorrhoids. The patient does not consume alcohol and is an ex-smoker as of May 2014. Genetic analysis shows that this patient is a carrier for the Cys282Tyr mutation in the HFE gene. Secondary to the hemochromatosis, the patient presents with severe chronic arthropathy in feet, spine (spondylarthrosis) and hands. The treatment initially was monthly erythroapheresis (later, the rate of erythroapheresis was reduced to once every two months). In January 2015, phlebotomies were introduced as part of the treatment. In May 2017, the hepatic magnetic resonance showed no sign of hepatic iron overload.","This 46-year-old man has a condition called hemochromatosis, which causes too much iron to build up in his body. This has led to problems with his liver and joints, causing pain in his feet, spine, and hands. He’s a carrier of a specific gene mutation that contributes to this condition. After years of treatment with blood removal and iron-binding therapy, recent scans show his liver is no longer overloaded with iron."
141,"A 65-year-old woman with a noncontributory medical history was referred to the Oral Surgery Unit, Policlinico Umberto I, “Sapienza” University of Rome, Italy, to undergo surgical reconstructive therapy peri-implantitis lesion localized around the mandibular left distal implant ( and ). The patient’s written detailed informed consent was obtained for the diagnostic and therapeutic approach and the use of the documentation for research purposes and publishing.\nThe procedure involved the prosthetic superstructure removal, oral and buccal full-thickness mucoperiosteal flaps incision, surface debridement and decontamination, and guided bone regeneration of an infra-bony defect using a mineralized dehydrated bone allograft and resorbable membrane in the non-submerged mode of wound healing [].\nDuring open-flap debridement of the infected implant surface with sodium bicarbonate air powder abrasion (PROPHYflex™ 3 with periotip, KaVo, Biberach, Germany) (), rapid onset swelling arose on the left cheek as well as in the periorbital space. The procedure was stopped immediately and the surgical area was rinsed with sterile saline solution to remove all residual bicarbonate particles. Before repositioning and suturing the flap, intra- and extra-oral inspection and palpation of the face and neck were performed to determine the spread and extension of entrapped air. Extra-oral examination revealed slight asymmetry of the face and complete left eyelid ptosis due to swelling of the left periorbital space and cheek ().\nA crackling sensation with no tenderness was detectable on palpation of the subcutaneous tissue in the swelling area. Visual acuity, light reflex, and extraocular movements were intact. Intraoral examination showed no swelling or crepitus in the mandibular region because air, spreading upwards alongside the buccinator muscle insertion, was entrapped into the upper and middle loose spaces of the face. The patient complained of experiencing only slight discomfort but no pain and no difficulty swallowing, breathing, or speaking. Therefore, computed tomography was deemed unnecessary to avoid undue radiation exposure. Subcutaneous emphysema diagnosis was based on the sudden onset during air-powder debridement of soft tissue swelling associated with crepitus in the absence of erythema, oedema, significant pain, or lymphadenopathy.\nIn the lack of signs or symptoms of serious complications, close observation was performed. The patient was reassured that the swelling should reduce spontaneously in 2–3 days and subside within 7–10 days with no complications or morbidity. After an adequate observation period, the patient was discharged with a prescription for 875 mg of amoxicillin plus 125 mg of clavulanic acid (Augmentin; GlaxoSmithKline, London, UK) twice daily and 250 mg of metronidazole (Flagyl; Zambon, Milan, Italy) three times daily for ten days. The antibiotic protocol was adopted to prevent the potential aerobic and anaerobic polymicrobial infection due to the dissemination in subcutaneous tissues from peri-implantitis lesion microbiota []. Furthermore, to reduce the probability of complications, the patient was advised to avoid coughing, sneezing, and nose-blowing, which could increase intraoral pressure.\nFollow-up visits were scheduled every two days to monitor the progressive swelling reduction and complete resolution, which was obtained spontaneously after a week without any complications.","This 65-year-old woman needed surgery to address an infection around an implant in her jaw. During the procedure, a sudden swelling appeared in her face and around her eye, requiring the surgery to be stopped immediately. The swelling was caused by air getting trapped in the tissues, and thankfully, it resolved on its own without any serious complications. She was given antibiotics and advised to avoid activities that could increase pressure in her mouth, and her swelling completely disappeared within a week."
142,"A 23-year-old man was admitted to our cardiomyopathy clinic for repetitive ventricular ectopic beats. He was hemodynamically stable with no other relevant symptoms. He never experienced syncope and was unaware of any case of cardiomyopathy or sudden cardiac death in his family. Remarkably, his medical history included an episode of acute myocarditis one year before. At that time, he was admitted to the emergency department of a different hospital with chest pain, troponin rise, and T wave inversion in the inferolateral leads on ECG (). An urgent coronary angiogram revealed normal coronary arteries. Then, a cardiac magnetic resonance (CMR) was performed, showing a non-dilated left ventricle (LV) with low-normal ejection fraction (EF), as well as normal RV dimensions and function. T2-weighted images highlighted the presence of mid-wall myocardial edema involving the interventricular septum, where mid-wall late gadolinium enhancement (LGE) was also noted on post-contrast images (). Endomyocardial biopsy was proposed, though the patient did not provide informed consent. The patient was discharged with a diagnosis of acute myocarditis, with a recommendation for close clinical follow-up. When re-assessing the patients at his 1-year follow-up, echocardiography showed an initial reduction of LV EF, with an area of hypo-akinesia involving the lateral wall, and preserved RV dimensions and function. A new CMR study was performed, which confirmed the mildly reduced LV EF with no evidence of myocardial edema. Post-contrast images, however, revealed a diffuse circumferential subepicardial LGE involvement of the LV myocardium ().\nTo exclude a left-dominant variant of arrhythmogenic cardiomyopathy, in which this LGE pattern has been reported with CMR, genetic testing and accurate family screening were then performed. His 56-year-old mother and 30-year-old sister, both asymptomatic, were also found to have inverted T waves in the inferolateral leads on ECG and a mildly reduced LV EF on echocardiogram. Performing CMR on those two subjects, a pattern of LGE very similar to the one detected in the proband was detected (). No relevant clinical findings were identified by exploring the paternal side of the family. To our knowledge, this was the only documented case of a “myocarditis-like” onset of arrhythmogenic cardiomyopathy among the family members.\nMolecular testing was carried out by analyzing a panel of target genes through an NGS-based procedure. The MAF threshold was set to 5% using Illumina Variant Interpreter Software. Genetic testing identified a heterozygous variant in DSP (c.5428C>T, p.Gln1810Ter).\nAccording to the American College of Medical Genetics (ACMG), the variant was classified as likely pathogenic (class IV).\nThe same mutation was found in the patient’s relatives with a positive phenotype (), and a diagnosis of familiar left-dominant arrhythmogenic cardiomyopathy was finally made. The patient, as well as his mother and sister, started therapy with β-blocker drugs, and the proband also received an implantable cardioverter-defibrillator.","A 23-year-old man was admitted after experiencing irregular heartbeats. He had a previous episode of inflammation in his heart muscle a year ago, but tests at that time showed no blockages in his arteries. Follow-up tests revealed changes in his heart muscle that suggest a condition called arrhythmogenic cardiomyopathy, which can cause irregular heartbeats and potentially lead to sudden cardiac arrest. Genetic testing identified a specific gene mutation that explains this condition, and it was found in his mother and sister, leading to a diagnosis of inherited cardiomyopathy. The patient and his family are now being monitored closely and will receive medication and a device to help prevent dangerous heart rhythms."
143,"An 8-year-old Caucasian girl was referred to our clinic for joint hyperlaxity, skin hyperextensibility and delayed wound healing. She was the second child of non-consanguineous parents, born preterm (29 weeks + 6 days) with an urgent Cesarean section due to maternal pre-eclampsia and placental abruption. Birth weight was low but appropriate for gestational age (930 g; 11th centile), and prematurity requested prompt admission to the neonatal intensive care unit. Twelve hours after birth, she experienced small bowel perforation due to meconium ileus, which required resection surgery and subsequent ileostomy without local complications. In the subsequent weeks, bilateral retinal detachment likely due to the retinopathy of prematurity was also diagnosed and promptly treated with laser photocoagulation and subsequent vitrectomy at 2 months of age. Additionally, she was diagnosed with bilateral cataract presumably secondary to prematurity. For this complication, she underwent surgery by the age of 18 months and 3 years to the left and right eye, respectively. The ophthalmologic prognosis was complicated by high-grade myopia and visual deficit. According to the last evaluation, she had a visual acuity of 3/10 in the left eye and a partial blindness in the right one (she only perceives lights), treated with daily topic ocular β-blockers. At the age of 7, she had a right traumatic femoral bone fracture after a minor trauma (a fall from a chair), requiring surgical treatment.\nOn examination, the girl was found to be overweight (weight 75–90th centile; BMI 75th centile—CDC charts [], with generalized joint hypermobility (Beighton score: 9/9) (a), skin hyperextensibility, multiple atrophic and post-surgical dystrophic scars (b), multiple ecchymoses in her lower limbs, absence of lingual frenulum, mild right-convex thoracic scoliosis, bilateral genu valgum-recurvatum, cubitus valgus with elbows hyperextensibility and bilateral pes planus. She had no strength deficit, with global hypotonia but normal muscular trophism and deep tendon reflexes. However, she had a global hypotonia. Intellectual abilities were normal. She was able to walk, with the help of a crutch. On both legs, soft, velvety skin and subcutaneous tissues had been long misdiagnosed as a mild lymphedema. Parents also reported easy bruising for their daughter. None of her family members presented with similar signs or symptoms. Chest, spine and limb radiography confirmed the orthopedic abnormalities. Global respiratory function with spirometry, abdominal and supra-aortic trunk ultrasounds and video-electroencephalography all resulted normal. A comprehensive cardiovascular evaluation evidenced a mild mitral valve insufficiency without clinical relevance. No other vascular or lymphatic anomalies were detected. Multidisciplinary follow up, including pediatric, oculistic, psychiatric and cardiovascular evaluations, has been continued for 5 years. She reports no pain. She attends school with good cognitive and social skills and weekly swimming sessions. Support insoles were prescribed without a clear clinical improvement.","This 8-year-old girl has a number of medical challenges due to being born prematurely. She had a difficult start in life with a bowel problem, retinal detachment, and cataracts, all requiring surgery. She also has very loose joints and stretchy skin, which makes her prone to bruising and injuries. Despite these challenges, she is doing well in school and enjoys swimming, and her doctors are continuing to monitor her health."
144,"A 12-year-old girl developed abdominal pain and reported frequent bloody stools for over a month. She had been diagnosed with moderate left-sided UC at nine years of age. Remission was initially induced with prednisolone, and she remained in remission with azathioprine due to mesalazine intolerance. She experienced moon face and increased appetite as side effects while taking prednisolone. She was later diagnosed with a UC relapse based on colonoscopic findings of marked erythema and the absence of vascular pattern. Because of the side effects of previous prednisolone therapy, the patient and her guardian declined further steroid therapy. We decided to induce remission with GMA. However, securing two blood vessels for GMA was expected to be difficult because of the patient's small anthropometric measurement (height: 134.9 cm, weight: 31.7 kg). Therefore, we elected to perform GMA with the single-needle method. She underwent GMA once per week for 10 weeks. A 17-gauge dialysis puncture needle (outer diameter: 1.4 mm, length: 25 mm) was inserted into the right elbow (). The dialysis console processed a blood flow rate of 40 mL/min (total blood volume: 1,800 mL). In this case, the treatment time was 90 minutes. No decrease in blood pressure was observed during this procedure. Heparin was used as an anticoagulant. All 10 GMA treatments were completed without puncture failure or poor blood removal. Additionally, no side effects were observed. However, the patient did not attain remission with GMA. After an unsuccessful attempt of oral tacrolimus therapy, remission could be achieved and has maintained with infliximab (5 mg/kg, every 8 weeks) for 10 months.","This 12-year-old girl has a history of inflammation in her colon (UC) that she was managing with medication. Recently, her UC flared up again, and she started experiencing bloody stools and abdominal pain. To help her get better, doctors decided to use a special treatment called GMA, which involved a small needle insertion to deliver medication directly into her bloodstream. After completing 10 GMA treatments, she was able to achieve remission with infliximab, a different medication. She is now doing well and has been able to maintain her remission for the past 10 months."
145,"A 60-year-old female presented to our tertiary medical center for a second opinion regarding the incidental pathology finding of stage III nonmucinous appendiceal adenocarcinoma after an emergent appendectomy for perforated appendicitis at an outside hospital four months prior. Her initial pathology revealed primary nonmucinous, moderately differentiated, stage III, pT4pN1aM0, appendiceal adenocarcinoma, involving 1 of 3 periappendiceal lymph nodes with extensive lymphovascular space invasion. Mismatch repair protein was intact. She completed staging computed tomography (CT) and colonoscopy. On imaging, there was no evidence of distant metastasis, but a small right ovarian cyst and calcification of the gallbladder wall were noted (). The ovarian cyst had been evaluated intraoperatively at the index operation by a gynecologist, and it was deemed that no intervention was needed at that time. Completion right hemicolectomy and possible right oophorectomy followed by adjuvant FOLFOX (folinic acid, fluorouracil, and oxaliplatin) were recommended. However, she opted to forgo any treatment at that time. The patient was asymptomatic in the interim. The patient represented to clinic with CT findings of growth in the right ovarian cyst, from 4 to 11 cm, with a new 6 cm complex cystic/solid mass along the left pelvic sidewall (). On presentation, she complained of lower abdominal fullness and cramping with intermittent bloating and early satiety. Her exam was mostly unremarkable except for the fullness in bilateral adnexa.\nHer case was presented at the multidisciplinary tumor board. At that time, her pathology was also reviewed (). We recommended completion right hemicolectomy as well as resection of adnexal masses, which were concerning for malignancy. We also discussed the possibility of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy if peritoneal metastasis was discovered on exploration. In addition, she was recommended to undergo cholecystectomy at the same time.\nIntraoperatively, the patient was found to have diffuse carcinomatosis. Cytoreductive surgery included right hemicolectomy, cholecystectomy, and total abdominal hysterectomy and bilateral salpingo-oophorectomy with en bloc resection of the adnexal masses. This was followed by HIPEC with mitomycin C. The peritoneal carcinomatosis index (PCI) was 20, and the completeness of cytoreduction score (CC) was 1 due to subcentimeter implants on the small bowel serosa from the jejunum to the terminal ileum. The patient had an uneventful postoperative recovery and was discharged on postoperative day 6.\nSurprisingly, pathology revealed primary gallbladder adenocarcinoma, moderately to poorly differentiated, arising in a background of high grade biliary intraepithelial neoplasm and porcelain gallbladder. The carcinoma extended through the visceral peritoneum onto the serosal surface and into the pericystic soft tissue on the hepatic bed surface (). All tumor deposits collected from the operation were consistent with metastasis from the biliary origin.\nWith this new finding, her case was rediscussed at tumor board. The original appendiceal specimen slides were reviewed and found to be similar histologically to the gallbladder adenocarcinoma (). The possibility of synchronous gallbladder and appendiceal primaries was discussed but given the morphological resemblance between the two and the pattern of spread, primary gallbladder adenocarcinoma with carcinomatosis was the most likely diagnosis.\nThe patient completed four cycles of gemcitabine and cisplatin before switching to FOLFOX after surveillance imaging demonstrated disease progression. Shortly after receiving the first cycle of FOLFOX, she presented with an acute abdomen secondary to perforated viscus and underwent emergent laparotomy. She was subsequently transitioned to hospice.","A 60-year-old woman was initially diagnosed with cancer in her appendix after an emergency surgery. Further tests revealed that the cancer had spread to nearby lymph nodes and other areas, but no distant organs. She then developed a large growth in her ovary and a new mass in her pelvis, leading to a more extensive surgery that included removing her gallbladder, uterus, and ovaries. During the surgery, doctors discovered that she also had cancer in her gallbladder, and after further treatment, she ultimately passed away."
146,"The female patient was 4 years old when she came to our attention. She was brought to the practice by her parents because she complained of hypersensitivity to heat and cold, even during normal breathing. The patient had never undergone a dental examination or treatment.","This 4-year-old girl is experiencing unusual sensitivity to temperature changes, feeling too hot or too cold even when she’s just breathing. Her parents brought her to the doctor because of this, and she has never had a dental checkup or treatment. We will need to investigate the cause of these temperature sensitivities and ensure she receives the necessary dental care. We will be conducting a thorough examination to determine the best course of action for her health."
147,"A 33-year-old female living in Fresno, California presented to the hospital with progressively worsening diplopia and headache for 5 days. Chart review showed that she had been diagnosed with coccidioidal meningitis three years ago when she presented with similar headaches and reduced visual acuity. Computed tomography (CT) of the head at the time showed hydrocephalus. CSF opening pressure was 52 cm H2O. Coccidioides complement fixation titer of the CSF was positive at 1:16. She was started on oral fluconazole 1000 mg daily for adequate CNS penetration and a ventriculoperitoneal shunt was placed at that time. She was eventually discharged home but lost to follow-up.\nIn the Emergency Department on Day 0, her vital signs were stable within normal range. Physical exam was benign, other than oblique diplopia. CT of the head showed hydrocephalus and a right posterior parietal ventriculoperitoneal shunt tube (). Lumbar puncture was performed on Day 1. Opening pressure was 17 cm H2O. CSF analysis revealed leukocytes of 51/uL with 69% lymphocyte predominance, glucose 23 mg/dl, protein 324 mg/dl. CSF studies showed positive Coccidioides complement fixation at 1:32 and VDRL 1:32. Fungal culture of CSF was negative. RPR titer was 1:32. Upon further investigation, the patient had been diagnosed with syphilis about 2 years ago when she presented to an Emergency Room with vaginal pain and swelling. RPR at that time was positive at 1:16, but the patient had already left the Emergency Department and did not receive any treatment. The Department of Public Health also confirmed that she had never received appropriate treatment for syphilis.\nShe was started on Fluconazole 1000 mg daily to treat CNS infection with Coccidioides as well as Penicillin G 4 million units IV every 4 hours for 14 days to treat neurosyphilis. Unfortunately, her mental status continued to decline requiring intubation for airway protection. On Day 20, she underwent external ventricular drain (EVD) placement due to worsening hydrocephalus on repeat imaging. Subsequent imaging on Day 22 showed worsening ventriculomegaly with bifrontal hemorrhage despite EVD placement (). Due to lack of neurological improvement and poor prognosis, her family elected to transition to comfort-focused care, and she was discharged on a hospice on Day 30.","This 33-year-old woman returned to the hospital with a return of headaches and double vision, symptoms similar to those she experienced three years ago when she was diagnosed with coccidioidal meningitis. Tests showed she had a history of syphilis that she hadn't been treated for, which may have contributed to her current condition. Despite treatment with antifungal and antibiotic medications, her condition worsened, and she needed a tube to help with breathing and her brain fluid pressure. Ultimately, her family chose to focus on comfort care, and she was transferred to a hospice facility."
148,"A 25-year-old man presented to our epilepsy center for evaluation of seizures. He was born at term without any developmental delays and had no risk factors for epilepsy including traumatic brain injury, brain surgery, febrile seizures, central nervous system infections, or family history of seizures and no significant past medical or psychiatric comorbidities. Three years prior to his presentation he had his first seizure. He did not remember the event, but while attending basic training in the Army, he was reportedly found in the shower confused by his fellow soldiers. There was no tongue bite or urinary incontinence, but he was disoriented afterward for much of that day. He had another episode within the same month while he was performing physical training exercises, whereby he collapsed and remained confused for hours, but no report of witnessed convulsions. An evaluation at that time was unrevealing. He had 12 episodes in the next 3 years. They were all similar, some associated with lateral tongue laceration suffered during the event. He was seizure-free for 6 months and then began to have spells at least monthly. He denied an aura or premonition preceding his seizures. His wife reported at night that he would “cry” at the onset and then appears to have clonic jerking bilaterally and symmetrically, up to 3 minutes in duration. He was reported to be distressed for a few minutes after the episodes. Brain MRI was reportedly normal and EEG abnormal, but the reports were unavailable. He had been taking levetiracetam 3000 mg daily with topiramate 50 mg daily. He had also tried valproic acid but reportedly had abnormal labatory studies so this was discontinued. At his appointment, it was determined that he would continue his current regimen of levetiracetam, and topiramate was increased to 100 mg total daily. A presumptive diagnosis of epilepsy was made upon clinical grounds though the classification included focal epilepsy localized to the frontal head region or genetic generalized epilepsy manifest as recurrent nocturnal generalized tonic-clonic seizures. At his follow up appointment, a high-resolution 3-T brain MRI was performed and was normal without intracranial abnormalities. EEG demonstrated 3–4 Hz generalized polyspike-and-wave discharges supporting a clinical diagnosis of genetic generalized epilepsy. The patient and his wife had recorded a video of his habitual seizures, which was reviewed an epileptologists (WOT). As noted in the video, he appears agitated and combative and is thrashing his extremities in a non-rhythmic and discontinuous manner with side to side head movements with eyes closed (). He and his wife were clear that this was the semiology of his typical seizure. The side to side head movements, eye closure, and discontinuous nonrhythmic hypermotor activity suggested FS . He was subsequently admitted to the epilepsy monitoring unit for LTVEM for differential diagnosis and classification of recurrent events. During the admission, EEG redemonstrated interictal generalized spike and polyspike and slow wave complexes noted previously. He had one seizure with clinical semiology suggesting a focal to bilateral tonic-clonic seizure due to head version, yet lateralized and focal seizures are known to occur in genetic generalized epilepsies . Despite the appearance of focal features, the ictal EEG demonstrated a generalized seizure onset. Immediately following a definitive diagnosis of epilepsy with electroclinical support from a electroclinical bilateral tonic-clonic seizure, he exhibited the exact same post-ictal behavior that was witnessed in clinic while reviewing the smartphone video. This behavior observed on the smartphone video was therefore able to be linked to his habitual postictal state with violent thrashing that simulated a FS (). In discussion with the patient and his wife, the difference between his seizure and a postictal state with confusion and combativeness was underscored to define a sequence of events rather than separate events. LTVEM was therefore able to establish a diagnosis of genetic genealized epilepsy despite the history suggesting focal epilepsy and the smartphone video suggesting a FS.","This 25-year-old man has been experiencing seizures for three years, starting after a confusing event during his military training. He has had multiple seizures since then, sometimes with injuries like a cut tongue. He’s been trying different medications, but they haven’t fully controlled his seizures. After further testing, including a brain MRI and EEG, doctors determined he has a genetic form of epilepsy, and they are now monitoring him to get a more precise diagnosis and plan for ongoing treatment."
149,"A 60-year-old Japanese male patient without any past medical history presented with dyspnea for 5 days in June 2019 (before the COVID-19 outbreak). He had no history of cigarette smoking, alcohol consumption, or sick contacts. He had a frequent cough, tachypnea (40 breaths per minute), low-grade fever (37.2°C), and hypoxemia (PaO2, 50.2 mm Hg on room air). He did not have wheezes or lung crackles and abnormal heart sounds on auscultation. Edema, skin rash, muscle weakness, myalgia, and arthralgia were absent. Blood tests revealed leukocytosis (10,300 cells/μl with 76% neutrophils, 2.0% eosinophils, and 14.0% lymphocytes) with high C-reactive protein levels (5.27 mg/dl). He had normal liver and renal function tests (aspartate aminotransferase 25 IU/L, normal <38 IU/L; alanine aminotransferase 30 IU/L, normal <40 IU/L; blood urea nitrogen 13.6 mg/dl, normal <20 mg/dl; and creatinine 1.04 mg/dl, normal <1.10 mg/dl) and no elevation of creatinine kinase (155 IU/L, normal <170 IU/L). Autoimmune screening did not identify any abnormalities, including anticyclic citrullinated peptide, anti-nuclear antibodies, anti-double-stranded DNA antibodies, anti-proteinase 3 (PR3) antibodies, anti-myeloperoxidase (MPO) antibodies, anti-Scl-70 antibodies, anti-Sjögren's syndrome-related antigen A (SSA/Ro52) antibodies, anti-aminoacyl-transfer RNA synthetase (ARS) antibodies, anti-Jo-1 antibodies, and anti-melanoma differentiation-associated gene 5 (MDA5) antibodies. Chest X-ray and computed tomography (CT) scan showed diffuse ground-glass opacification and consolidation in bilateral lung fields (Figure ). On the day of admission, the patient's condition deteriorated rapidly and he received noninvasive intermittent positive pressure ventilation (NPPV). The diagnosis of rapidly progressive interstitial lung disease with autoimmune disorders, such as severe inflammatory myopathy-related interstitial lung disease, could not be ruled out. Based on the diagnosis of ARDS of unknown etiology (PaO2/FiO2 235 with a positive end-expiratory pressure of 5 cmH2O), high-dose (1000 mg/day) intravenous (IV) methylprednisolone therapy was initiated. Empiric antibiotics (IV piperacillin-tazobactam and levofloxacin) were also given, although blood culture and urinary pneumococcal and Legionella antigen tests were negative. After 3 days of steroid pulse therapy, the patient improved dramatically and was weaned from NPPV and, thereafter, from oxygen support. The dose of IV methylprednisolone was reduced to half every 3 days and later it was switched to oral prednisolone (60 mg/day), which was also gradually reduced. Chest CT scan taken on the 13th day of admission revealed almost complete disappearance of abnormal shadows from the lung field (Figure ). He was discharged without dyspnea on the 26th day of admission. The steroid was tapered down gradually and discontinued 7 months after discharge. Although the patient had been asymptomatic for a while, he had a relapse of dyspnea in 10 months later after the first onset of ARDS. Upon the second admission, he had hypoxemia (PaO2 64 mm Hg) on O2 5L/min via face mask and started receiving NPPV therapy. Chest CT scan showed a mixture of diffuse ground-glass opacification and consolidation similar to roentgenological patterns observed previously (Figure ). The findings of physical examination and blood tests were not significantly different from the previous admission except that he had grasping pain in both thighs, proximal muscle weakness in extremities, and elevation of serum creatinine kinase (1741 IU/L). He had no skin eruptions, such as nail-bed telangiectasia, heliotrope rash, Gottron's papules, Raynaud's phenomenon, and hyperkeratotic lesions on his fingers (mechanic's hands). The short-tau inversion recovery sequence (STIR) of magnetic resonance imaging (MRI) showed inflammatory changes in both hamstring muscles (Figure ). However, the Euroline myositis line blot assay showed negative results for either myositis-specific antibodies (Jo-1, PL-7, PL-12, EJ, SRP, Mi-2, MDA5, and TIF1-γ) or myositis-associated antibodies (Ku, PM-Scl100, Scl-70, and SSA/Ro52). From these findings, the diagnosis of ARDS that relapsed along with an initial manifestation of seronegative PM was made. After 3 days of high-dose (1,000 mg daily) IV methylprednisolone therapy, the patient's dyspnea and muscle weakness improved dramatically and NPPV therapy was discontinued. Additionally, the diffuse abnormal shadows on the chest CT scan (Figure ) and the high signal on STIR MRI of the hamstring muscles (Figure ) disappeared. The dose of IV methylprednisolone was gradually reduced to 40 mg/day prednisolone, when the patient was discharged without respiratory and muscular symptoms on the 26th day of the second admission (Figure ). The steroid was tapered down gradually and discontinued 6 months after discharge.","The patient was admitted to the hospital because he was having trouble breathing and a persistent cough. Tests showed inflammation in his lungs and elevated levels of inflammation in his blood, but no specific cause was found. He was treated with steroids and antibiotics, and his breathing improved significantly. After a period of recovery, he was discharged home and continues to be monitored. He experienced a relapse of his symptoms several months later, and was admitted again for further treatment, which also led to a successful recovery."
150,"A 70-year-old Caucasian woman with medical history significant for stage III chronic kidney disease, transitional cell ureteral cancer status post-left-sided nephroureterectomy, and three-year history of Waldenstrom's macroglobulinemia (WM) presented with complaints of right-sided weakness associated with paresthesias, dysarthria, and blurry vision of three weeks duration. Magnetic resonance (MRI) imaging of the brain demonstrated an enhancing, hypercellular mass centered in the left thalamus with additional foci of signal abnormality and enhancement in the cortex of the left frontal lobe and subcortical white matter (Figure ). These findings were concerning for an intracranial neoplastic process, especially given her history of WM.\nRegarding her oncological history, she was initially diagnosed with WM at the age of 67 after workup for complaints of chronic fatigue revealed elevated IgM levels (3370 mg/dl) as well as serum hyperviscosity. Bone marrow biopsy showed a low-grade B-cell lymphoma with plasmacytic differentiation and 60%–70% bone marrow involvement. Neoplastic cells were found to be lambda restricted and negative for CD5, CD10, and CD23 by flow cytometry. An increased number of lambda predominant cells were confirmed by flow cytometry and CD138 immunostaining. The patient was started on first-line therapy with the Bruton tyrosine kinase inhibitor ibrutinib; however, due to worsening adverse effects after 6 months of therapy she transitioned to rituximab, an anti-CD20 monoclonal antibody. Unfortunately, the patient was found to have worsening IgM levels and serum viscosity while on rituximab monotherapy over the next 6 months. Thus, she was restarted on ibrutinib while continuing rituximab every 3 months and had significant improvement on this combination of therapy.\nShe completed two years of maintenance rituximab and reduced-dose ibrutinib (140 mg) at time of presentation with the most recent IgM levels of 299 mg/dl prior to the onset of her previously mentioned neurological symptoms. Given her MRI findings, computed tomography (CT) imaging of the head, chest, abdomen, and pelvis was completed, which revealed multiple intracranial lesions but no evidence of lymphadenopathy or neoplastic process elsewhere. She further underwent lumbar puncture for cerebral spinal fluid (CSF) analysis with flow cytometry showing mostly T cells without evidence of B-cell non-Hodgkin lymphoma. As there remained high suspicion for central nervous system (CNS) lymphoma, the patient ultimately had a left parietal stereotactic brain biopsy with pathology findings of diffuse aggressive B-cell non-Hodgkin lymphoma (Figure ). Immunohistochemical studies were positive for CD20, CD23, BCL-6, MUM1, and LE1 (Figure ) with approximately 80% of cells expressing Ki-67 proliferation antigen (Figure ). Fluorescent in situ hybridization (FISH) analysis was negative for c-MYC, BCL-6, or BCL2 gene rearrangements. Lastly, mutation testing using next-generation sequencing returned positive for MYD88 L265P mutation.\nThe patient was placed on oral dexamethasone 4 mg four times daily with noticeable improvement in her speech and mobility. Due to the patient's poor renal function, she was not a candidate for induction therapy with methotrexate. Thus, she began treatment with whole brain radiation therapy (WBRT) to 30.6 Gy while continuing systemic treatment with ibrutinib. A repeat MRI of the brain two months later demonstrated near resolution of the patient's lymphoma with findings of only a few small foci of nonspecific enhancement adjacent to the biopsy cavity within the left thalamus (Figure ). There was no evidence of intracranial mass effect, midline shift, or abnormal extra-axial collection.","This 70-year-old woman has a complex medical history including kidney disease, cancer, and a blood disorder. She recently started experiencing weakness, difficulty speaking, and blurry vision. After scans revealed a growth in her brain, a biopsy confirmed it was a type of lymphoma. She is now receiving radiation therapy and taking medication to help control the cancer, and her symptoms have improved."
151,"The case study is devoted to investigating of penile pain in a 41-year-old married man. According to medical evaluation, the pain extended to the perineal and inguinal regions and it was reported to be more acute during erection. The patient was referred by urologist for sonographic evaluation of penis and testes. The pain had started 3 days before the urologist examination, following his first full erection for intercourse, after his positive COVID-19 polymerase chain reaction (PCR) test.\nThe patient did not have any other urologic symptoms such as discharge, hematuria, or dysuria. He denied any trauma to the penis, previous pelvic tumor, pelvic surgery and history of recent immobilization. He did not use vasoconstrictive drugs. The patient reported positive nosopharyngeal swab test for COVID-19 three weeks earlier. He had mild symptoms of COVID-19 infection including muscle pain, fever, cough, and fatigue. He had received conservative treatment and had not taken any anti-coagulants, antivirals, and corticosteroids. His medical history did not show any significant underlying disease and any risk factor for cardiovascular disease. He also did not have history of previous deep vein thrombosis. In physical examination of the penis and testes, no pathologic finding was detected such as skin tissue changes, discoloration, edema, tenderness, or palpable nodularity.\nUltrasound evaluation showed thrombosis of deep dorsal penile vein while the superficial dorsal penile vein, iliac veins, and inferior vena cava were intact (Figures ,,).\nLaboratory tests revealed slightly increased D-dimer level(may be due to inflammatory process of COCID-19 infection), normal levels of fibrinogen, anti-thrombin III, protein S, Protein C, anti-cardiolipin antibodies and normal count of platelets and white blood cell counts. Also Tests were negative for anti-phospholipid-IgG, IgM, and lupus anti-coagulant (Table ).\nImmediately after sonographic diagnose of deep dorsal penile vein thrombosis, the Rivaroxaban treatment was started with the dosage of 15 mg twice a day. Two months after starting the treatment, patient's symptoms were completely disappeared and he had no penile pain during erection and sexual disturbances anymore. Ultrasound evaluation revealed no evidence of acute deep dorsal penile vein thrombosis. Old partial thrombosis at the proximal part of the vein was seen. A little pain at the site of the partial thrombosis with the pressure of the ultrasound probe was noted (Figures ,,).","A 41-year-old man was experiencing pain in his penis and groin, especially during erections, and it started after he tested positive for COVID-After a urologist evaluation, a scan revealed a blood clot in a vein inside his penis. He started taking medication to dissolve the clot, and his pain completely disappeared after two months. Follow-up scans showed the clot was mostly gone, but a small area of lingering pain remained."
152,"A 45-year-old male was admitted to the emergency department with postural instability and dysarthria. To lessen his instability and avoid to fall, the patient widened his support polygon. He had also reported dyspnea at effort, which occurred 3 days prior to his admission. He had a history of rheumatic mitral stenosis, since 2005, for which he benefited from a percutaneous mitral dilation in the same year. He also reported a Penicillin allergy.\nInitial examination found the patient conscious. His heart rate was 125 b/m, blood pressure was 135/85 mm Hg. He was polypneic and orthopneic with a respiratory rate of 28 breaths/min, an O2 saturation of 96% on ambient air with the presence of bilateral crackles. He had a fever measured at 39.5C. Cardiac\nauscultation revealed a low-pitched diastolic rumble, well heard at the apex. The neurologic examination revealed unsteady gait and the patient was unable to perform Romberg's test.\nThe ECG showed coarse-mesh atrial fibrillation with an average ventricular rate of 90 cycles per minute (). No abnormalities were detected on the chest x-ray. Transthoracic echocardiogram (TTE) found rheumatic changes of the mitral valve including: commissural fusion and thickening, producing “dog leg deformity” of the anterior mitral leaflet (-A). The mitral valve area was 0,8 cm2 (-B) and the pressure gradient across the mitral valve was 22 mmhg (-E). We noted a mobile vegetation measuring 11,7 mm of length, located in the posterior leaflet of the mitral valve (-C). The left atrium was dilated at 47 cm2 while size and function of the left ventricle was normal. Pulmonary arterial systolic pressure (PASP) was important (-F) and the filling pressures of the left ventricle were elevated. In addition to that, we reported moderate aortic stenosis and regurgitation and mitral regurgitation at grade A.\nThree sets of blood cultures were made, coming back negative as well as the HACEK organism's serologies (Haemophilus; Actinobacillus; Cardiobacterium; Eikenella; and Kingella). It was decided to perform a brain MRI based on the clinical findings. It showed a recent ischemic stroke involving the right peduncular territory associated with chronic lacunar infarcts ().\nSigns of inflammatory response were noted, high white blood cell count at 14 770/mm3 in addition to C reactive protein (CRP) at 67 mg/l, Ferritin was elevated at 839 μg/l. The kidney function was normal. Further investigations were realized to precise the extension of infective endocarditis, all coming back normal. No point of entry for the pathogen was detected.\nThe patient was admitted to the cardiology unit. The diagnosis of infective endocarditis was made, based on the modified Duke criteria (1 major clinical criteria +3 minors clinical criteria). Given the negative blood culture endocarditis and the history of penicillin allergy, it was decided to start an antibiotic therapy combining: Vancomycin at 30mg/kg/day for 6 weeks (Vancomycin serum concentrations of 15–20 mg/L were aimed) and Gentamicin at 3mg/kg/day for 2 weeks, in addition to intravenous diuretics (80 mg of Furosemide as bolus followed by a maintenance dose of 40mg/12hours) with a strict control of kalaemia. Finally, for his supraventricular arrythmia, Enoxaparin was started at a curative dose (100 UI/kg/12h). The tolerability was good and no adverse events were reported.\nWithin 4 days, fever disappeared. The postural instability and the dysarthria were less important than in his admission. Routine TTEs showed a regression of the vegetation and a normalization of the left ventricular pressures. The patient was satisfied after the improvement of his clinical condition. He was addressed after that to the cardiovascular surgery department for a surgical treatment of his valve disease.","This 45-year-old man was admitted to the hospital because he was unsteady on his feet and had trouble speaking clearly. He also had difficulty breathing and a fever. Tests revealed he had an infection in his heart, which caused inflammation and a stroke. The doctors found a growth on his heart valve and started him on strong antibiotics and medications to help with his breathing and heart rhythm. After a few days, he started to feel better, and his doctors discussed surgical options for his heart valve."
153,"A 29-year-old female patient, gravida 1, para 1, with no significant pathological history, had presented herself in consultation complaining of a left cervical swelling that had been evolving for 11 months in a context of general state conservation. The mass was increased in size rapidly after the end of the breastfeeding period (three months). The clinical examination at admission found normal vital signs, and Body mass index of 26.3 kg/m2. Family history was unremarkable for cancer. The patient was used a combined oral contraceptive for menstrual regulation.\nThe cervical examination showed a postero-lateral mass of the left neck measuring 6 cm in diameter, painless, of firm consistency, unilobed, soft, adherent to the superficial and deep plane. There was no palpable cervical lymphadenopathy or inflammatory signs of the adjacent skin.\nA cervical magnetic resonance imaging (MRI) was performed, revealing a mass of the left posterior cervical soft parts, at the expense of the trapezium muscle, tissue, oval, of regular contours, well-defined, measuring 41 × 68 × 81 mm, enhanced after injection of Gadolinium, with multiple homolateral supraclavicular and lateral cervical lymph nodes (). The monotest, in the absence of an IRD tuberculin skin intradermoreaction, was negative.\nA biopsy of the mass with Tru-cut® was performed, concluding a desmoid tumor. Abdominopelvic and thoracic computed tomography, indicated for staging, showed the absence of other progressive lesions elsewhere.\nIn view of these clinical, radiological and pathological findings, a large resection of the tumor was indicated. The patient was installed in the supine position. Access to the posterior neck area was via a direct surgical approach (Fusiform incision). The mass was found to originate from the trapezius muscle without local infiltration of surrounding structures. A sharp dissection over the mass of the tumor away from the muscle was not possible. Complete excision of the mass was accomplished involving the fascia and trapezius muscle (). A close suction drain was placed. The safety margins are macroscopically healthy with a resection at 1.5 cm macroscopic distance from the palpable area of the tumor. The post-operative outcomes were simple without any complications and the drain was removed on the second postoperative day. The patient was discharged from the hospital on postoperative day 5, and the functional result was considered satisfactory without any impairment noted.\nThe pathology examination of the surgical specimen showed proliferation of spindle-shaped cells arranged in long fascicles in a collagenous stroma. This proliferation infiltrates the striated muscle, with negative excision margins. An immunohistochemistry staining was performed, demonstrating cytoplasmic labeling with anti AML antibody, and nuclear labeling of tumor cells with anti-beta-catenin antibody. This pathological and immunohistochemical aspect highlights the diagnosis of a desmoid tumor ().\nThe case was analyzed by a multidisciplinary committee, and it was decided to follow the patient without any adjuvant treatment given its long-term side-effects and the safety margins are healthy. After a three month, the clinical and radiological follow-up examinations were unremarkable. The outcome was favorable without local or distant tumor recurrence.","A 29-year-old woman came to the hospital because of a growing lump in her neck that had been bothering her for several months. Tests, including an MRI and a biopsy, revealed that the lump was a type of tumor called a desmoid tumor, which originated in her muscle. The tumor was completely removed through surgery, and the margins were clear, meaning no cancer cells were found. The patient is now being monitored regularly to ensure the tumor stays gone, and she is doing well with no complications."
154,"The participant in this study was a 24-year-old man who was in a motor vehicle accident that led to a severe TBI two years ago. According to the report of the spiral brain CT scan, the primary lesion was located in the left frontotemporal area due to contusion, and a few lacunar infarcts were seen in the left basal ganglia. Before the accident, he was an active member of a music band and was involved in bodybuilding activities. He was hospitalized for 48 days after the accident. Following discharge from the hospital, he received regular rehabilitation, including electrical stimulation of the wrist and knee extensors and ankle dorsiflexors, resistance training, and aerobic and endurance conditioning (e.g., walking on treadmill and stationary bike). At the time of the first visit to the research clinic, he could not independently walk or stand up from a chair and was using a wheeled walker for mobility and an ankle foot orthosis to prevent drop foot.\nThe clinical examination was performed by an experienced physical therapist. The participant had right (RT) hemiplegia with full and strong grasping and gripping but without the ability to write. Other impairments were aphasia, bradykinesia, and dyscoordination of movements of RT upper (i.e., finger to nose) and lower (i.e., heel to shin) extremities. Also, deep tendon reflexes were increased with no spasticity in his muscles. He was dependent in some activities of daily living (ADL) (e.g., dressing, toilet use, and feeding).","This 24-year-old man suffered a serious brain injury in a car accident two years ago, resulting in weakness on his left side and some cognitive difficulties. Before the accident, he was an active musician and bodybuilder. He has been receiving physical therapy to help him regain movement and independence. Currently, he uses a walker and ankle brace and needs assistance with many daily tasks, but he is making progress with his rehabilitation."
155,"A 53-year-old man presented with a pruritic rash on the trunk as well as on the upper and lower extremities. Examination was notable for lichenified papules throughout the trunk and extremities, most notably on the back. Biopsies of the rash showed mild spongiosis with an underlying superficial and deep perivascular infiltrate (). Due to failure of topical halobetasol, topical tacrolimus, oral antihistamines, prednisone, and mycophenolate mofetil, dupilumab was initiated at standard dosing. Within 3 months, the patient noticed a dramatic improvement of his rash and pruritus, complaining only of mild pruritus between injections and minimal residual post-inflammatory hyperpigmented macules. Dupilumab was stopped after 1 year due to insurance reasons, and the initial pruritic rash returned. After insurance reapproval, dupilumab was restarted with complete resolution of his rash and pruritus.","This 53-year-old man had a persistent, itchy rash on his body that didn't respond to several different treatments. Doctors eventually found that Dupilumab, an injectable medication, was very effective at clearing up the rash and relieving the itching. After a year, the rash returned when Dupilumab was stopped, but it was quickly resolved when the medication was restarted. He is now experiencing complete relief from his rash and itching."
156,"A 48-year-old woman presented with a 5-year history of intense pruritus and rash significantly impacting her daily life. Examination showed few excoriated papules and subtle lichenification on the upper back, elbows, dorsal forearms, thighs, and fingers. Biopsy revealed mild epidermal spongiosis with a perivascular lymphocytic infiltrate containing rare eosinophils, consistent with DHR (). After failing multiple therapies including topical betamethasone, topical tacrolimus, and oral mycophenolate mofetil, dupilumab was initiated with improvement in severity and duration of flares within the first 6 months. Due to slight progression of her baseline blurry vision and headaches, the dose was decreased to 200 mg every 2 weeks. The patient experienced subsequent flaring of her rash, so the dose was increased back to 300 mg every 2 weeks with resolution of her pruritus and rash and no further exacerbation of her ocular symptoms. Ultimately her ocular symptoms were evaluated by an ophthalmologist and deemed to not be consistent with dupilumab-induced conjunctivitis nor glaucoma.","This 48-year-old woman has struggled for five years with intense itching and a rash that has made daily life difficult. Tests showed a specific type of skin inflammation, and after trying several medications, Dupilumab was found to be effective in reducing her symptoms. Initially, the dose was adjusted to manage some side effects, but it was later increased to fully control her itching and rash. After further evaluation by an eye doctor, it was determined that the eye symptoms were not related to the Dupilumab medication."
157,"A healthy 43-year-old woman presented with a 1-year history of a pruritic rash affecting her legs and abdomen. On exam, the patient was noted to have erythematous, blanchable papules coalescing into small plaques on her abdomen and distal part of the legs. Biopsy of the rash revealed an unremarkable epidermis and superficial perivascular lymphocytes with abundant interstitial eosinophils consistent with DHR. Patch testing was performed, which was 2+ for nickel sulfate and 1+ for p-tert-butylphenol formaldehyde resin, but the rash was persistent even with allergen avoidance. After failing multiple topical regimens, including triamcinolone and clobetasol, as well as oral prednisone, the patient was initiated on mycophenolate mofetil therapy, with excellent control but poor gastrointestinal tolerance. Her rash subsequently recurred, so dupilumab was started at standard dosing, and 5 months after starting dupilumab the patient's rash and pruritus had resolved without any side effects.","A 43-year-old woman has been struggling with a persistent itchy rash on her legs and abdomen for the past year. Tests showed the rash was likely due to allergies to nickel and a preservative ingredient. Despite trying several creams and pills, the rash didn't improve. Eventually, she found relief with an injectable medication called dupilumab, which completely cleared up her rash and itching without any side effects."
158,"A 68-year-old man presented with a 6-month history of a pruritic rash that began on his back and legs and spread to his knees, elbows, shoulders, and chest. Patch testing showed 1+ positivity for both sodium laurel sulfate and benzaprene #4, which were deemed not clinically relevant. On examination, he had scattered erythematous scaly patches on the upper chest, shoulders, and back with overlying excoriation. Biopsy of the right shoulder showed an unremarkable epidermis and a sparse perivascular and interstitial mixed infiltrate containing scattered interstitial eosinophils, consistent with a DHR (). Oral prednisone initially cleared the rash, but it recurred on discontinuation. The rash was also recalcitrant to trials of topical steroids, oral antihistamines, and topical tacrolimus; therefore, he was transitioned to dupilumab at standard dosing. After 3 months, the patient reported complete clearing of the rash and pruritus. He did note occasional eye dryness, which was well-managed with artificial tears.","68-year-old man came to the doctor because of a persistent itchy rash that had spread over his body for six months. Tests showed he was reacting to two ingredients in soaps and detergents. After trying several treatments, including steroids and antihistamines, a new medication called dupilumab helped clear the rash completely. He continues to take dupilumab and uses eye drops to manage some dryness. After three months, the rash was gone, and he felt much better."
159,"A 75-year-old man presented with a 1-year history of recurrent diffuse, pruritic rash. Examination revealed a generalized eruption of erythematous papules with minimal scale on the extremities and trunk particularly the flanks. Initial differential diagnosis included hypersensitivity dermatitis, contact dermatitis, non-bullous pemphigoid, atopic dermatitis, and Grover disease. A biopsy was performed on the left part of the chest and revealed a predominantly perivascular inflammatory infiltrate with occasional eosinophils consistent with DHR. A direct immunofluorescence test was negative. The patient failed multiple therapies, including topical triamcinolone, clobetasol, and hydroxyzine. Oral prednisone helped but was discontinued due to steroid-induced diabetes. The patient was started on dupilumab 300 mg injections every 14 days and within 4 months, his dermatitis and pruritus resolved. Due to cost, the injections were spaced to every 30 days, and he continued to experience resolution of his symptoms without any side effect from the medication.","This 75-year-old man has been struggling with a persistent, itchy rash on his arms and chest for the past year. After examining the rash and taking a biopsy, doctors determined it was a specific type of inflammatory skin condition called DHR. Despite trying several creams and medications, the rash didn't improve until he started taking Dupilumab injections, which successfully cleared up his symptoms. He continues to take the injections every 30 days and is experiencing a full resolution of his rash and itching."
160,"A 78-year-old man presented with a 5-year history of extreme pruritus. No significant dermatitis was observed aside from faint pink patches on the upper chest and lower back with mild lichenification. Patch testing was performed and revealed 1+ positivity to potassium dichromate, but no culprit allergens were identified. A biopsy taken from the right part of the chest revealed mild acanthosis and minimal spongiosis with a perivascular lymphocytic infiltrate containing rare eosinophils in the presence of a negative direct immunofluorescence test, consistent with a DHR. The patient failed multiple therapies including topical corticosteroids, antihistamines, doxepin, narrow-band UV-B light, doxepin, gabapentin, butorphanol, and aprepitant. Azathioprine was poorly tolerated due to fatigue. The patient was then started on dupilumab with dramatic improvement in his pruritus and a 50%-60% reduction of the rash within the first 2 months with no associated side effects. His condition remains stable on this medication.\nAdditional clinical information is summarized in .","This 78-year-old man has struggled with extremely itchy skin for five years. Tests showed a specific cause for his itching, and while other treatments hadn't worked, he recently started a new medication called dupilumab, which has significantly reduced his itching and rash. The medication has been well-tolerated, and his condition is now stable. He is continuing to take dupilumab to manage his symptoms."
161,"A 31-year-old gravida 4 para 0 African American woman at 22-weeks gestation presented with vaginal bleeding to an outside hospital. Her obstetric history was significant for 2 therapeutic abortions and 1 spontaneous abortion. During prenatal care, the fetus was noted to have a unilateral dysplastic kidney. The patient’s family history was significant for two family members with cervical cancer and two family members with endometrial cancer. She was placed on bedrest with inpatient admission. 48 h after admission she developed pelvic pain and uterine contractions. She expelled a mass vaginally measuring 11 × 9 × 5 cm with no fetal contents. The pathology from the outside facility showed a showed a highly cellular tumor composed of spindle-shaped cells and bizarre multinucleated giant cells with focal myxomatous change with mitotic count is greater than 50 per 10 high power field (Positive for CD10, SMA, ER, PR, EMA) with the differential diagnosis including endometrial stromal sarcoma and undifferentiated uterine sarcoma. The patient underwent examination under anesthesia, demonstrating a 3 × 3 cm defect of the posterior vaginal wall with active bleeding, which was sutured for hemostasis. The cervix was long, closed, and high with no evidence of bleeding and the fetus was intact with normal heart tones. She was discharged in stable condition.\nAt 27-weeks, the patient was referred to our institution for maternal-fetal medicine, and gynecology oncology consultation. On initial evaluation, she reported no prior abnormal gynecology history and no history of infertility issues. She denied dyspareunia and bulk symptoms prior to or during pregnancy, and her periods before pregnancy were normal. A pap smear at 27-weeks gestation was normal and negative for human papilloma virus. Her intake physical exam at our institution revealed a normal pelvic exam with no evidence of residual vaginal mass, and an ultrasound revealed no intrauterine myomas.\nGiven the differential diagnosis included an endometrial stromal sarcoma, an undifferentiated uterine sarcoma, and a primary vaginal sarcoma, the patient underwent magnetic resonance imaging (MRI) without contrast of the chest, abdomen, and pelvis at 27 weeks. MRI findings were notable for a peri-centimeter cyst in the cervix consistent with a Nabothian cyst, a multiloculated cystic structure in the fetal abdomen consistent with dysplastic kidney, and an anterior placenta. There was no evidence of metastatic disease.\nDue to the inability to ascertain whether the cancer had originated in the uterus, cervix or vagina, the patient desired definitive management for possible uterine sarcoma. In consultation with maternal fetal medicine and gynecology oncology specialists, the decision was made to proceed with a cesarean hysterectomy at 36 weeks. Betamethasone 12 mg intramuscularly was given for two doses 24 h apart with the first dose at 35 weeks and 6 days gestational age.\nAt 36 weeks and 1 day, she underwent planned exam under anesthesia of the vaginal canal with cesarean hysterectomy, bilateral salpingectomy, and peritoneal biopsies. She delivered a male infant weighing 2520 g with Apgars of 9 and 9. Operative findings were notable for normal appearing pelvic organs. There were no palpable or visibly enlarged pelvic/periaortic lymph nodes or evidence of metastatic disease throughout the peritoneal cavity. The neonate was admitted to the NICU post-delivery for prematurity. Neonatal US confirmed a right multi-cystic dysplastic kidney. He was transferred out of NICU on day 2 of life, and discharged home with his mother.\nThe final surgical pathology report was benign: no malignant or neoplastic cells were seen. The patient was seen at her 6-week postpartum visit and was doing well with a normal pelvic exam. At a follow-up surveillance visit 3 months postpartum, she was noted to have a 1 cm posterior vaginal wall lesion; biopsy results showed a high-grade sarcoma. The tumor was estrogen receptor and progesterone receptor positive. Computer tomography (CT) with contrast of the chest, abdomen, and pelvis showed no evidence of metastatic disease. The patient underwent an upper vaginectomy and proctoscopy. Operative findings were notable for 1.5 cm polypoid lesion in the posterior vagina wall 5 cm distal to the posterior vaginal apex. A 1 cm surgical margin was obtained circumferentially around this lesion where feasible, and surgical pathology showed high-grade sarcoma () of the polyp lesion with negative surgical margins. The tissue removed from this excision showed residual sarcoma, morphologically similar to the original pathology from the outside hospital pathology. All other vaginal biopsies were also negative for malignancy. Marker seeds were placed in the proximal and distal margins of the areas where the sarcoma was excised. She was treated adjuvant high dose rate vaginal brachytherapy using a multi-channel vaginal cylinder. She received a biologically equivalent 2 Gy dose (EQD2) of 45 Gy to the whole length of the vagina and 60 Gy to the post-operative bed (). A CT scan 36 months after surgery continue to show no evidence of disease. She remains disease free 58 months after completion of vaginal brachytherapy.","This woman was diagnosed with a rare type of cancer in her uterus during her pregnancy. Initially, she experienced bleeding and a large mass was removed, but the pathology revealed it was a cancerous tumor. To ensure the cancer didn't spread, she underwent a planned hysterectomy and salpingectomy. Her baby was born healthy and transferred to the NICU for a few days. After the surgery, she received radiation therapy to destroy any remaining cancer cells. Thankfully, follow-up scans have shown no signs of the cancer returning, and she remains cancer-free five years later."
162,"A 55-year-old male with a history of type 1 diabetes mellitus (T1DM) and unspecified autoimmune disease who presented with acute onset of confusion as well as concrete visual hallucinations and behavioral change. There were no reports of any headache, fever, or stroke-like symptoms. His only outpatient medications were insulin and low-dose steroids.\nThe patient was initially admitted to an outside hospital where magnetic resonance imaging (MRI) of the brain revealed multifocal areas of restricted diffusion with areas of corresponding T2 hyperintensities on fluid-attenuated inversion recovery (FLAIR) sequences (Figure ). There was a concern for stroke in multiple vascular territories with concern for vasculitis. Initial workup was unremarkable, and the patient was started on methylprednisolone for presumed primary central nervous system (CNS) vasculitis. He was transferred to our institution for further management by the Neurology service.\nHis initial neurologic exam was notable for encephalopathy, manifesting as inattention, disorientation to place and time, and stupor. He was only able to follow simple appendicular commands. Cranial nerve exam revealed left lower facial droop. He had full strength in bilateral upper extremities and 4/5 strength in bilateral lower extremities. Initial differential diagnosis included autoimmune vasculopathies, primary CNS vasculitis, and infectious meningoencephalitis given his mental status changes, reported visual hallucinations, and multifocal strokes.\nSteroids were initially held on admission to our institution until further workup could be performed. Extensive rheumatologic labs were ordered, and only rheumatoid factor and anti-CCP were found to be mildly elevated. A contrast-enhanced MRI of the brain demonstrated evolving areas of restricted diffusion with multifocal new areas of restricted diffusion in multiple vascular territories (Figure ). There was also incomplete suppression of CSF signal on FLAIR with multiple areas of abnormal leptomeningeal enhancement, suggestive of a superimposed inflammatory process affecting the meninges (Figures , ). In addition, there was abnormal vessel wall thickening and enhancement, particularly involving the intracranial carotid arteries as well as anterior cerebral arteries (ACA) and middle cerebral arteries (MCA) (Figure ). Computed tomography angiography (CTA) of the head also demonstrated areas of vessel irregularity and multifocal areas of narrowing, particularly involving the ACA (Figure ). On further review of the initial lab work and neuroimaging, it was felt that a primary CNS vasculitis was unlikely. Rather, findings were more suspicious for a meningoencephalitis complicated by acute stroke.\nA lumbar puncture (LP) was subsequently performed, which demonstrated a lymphocytic pleocytosis (WBC 25 K/cumm, 80% lymphocytes), normal CSF glucose of 64 mg/dl, elevated protein of 104 mg/dl, and increased opening pressure of 28 cm H20. Infectious studies (including Syphilis screen, bacterial cultures, fungal cultures, HSV PCR, and flow cytometry) and inflammatory markers (including oligoclonal bands and IgG index) were unremarkable; however, India ink was performed on the CSF, which revealed a small number of encapsulated yeasts. He was subsequently diagnosed with cryptococcal meningoencephalitis and started on the appropriate anti-fungal regimen with amphotericin B and flucytosine.\nHe required daily LPs to ensure opening pressure remained less than 20 cm H2O. The patient was treated with four weeks of amphotericin B and flucytosine followed by eight weeks of fluconazole. Prior to discharge to an inpatient rehabilitation facility, the patient’s mental status was notably improved and near his neurologic baseline.","65-year-old man was admitted to the hospital after experiencing confusion, hallucinations, and changes in behavior. Initial tests showed signs of stroke and inflammation in the brain, but further investigation revealed he had a serious fungal infection called cryptococcal meningitis. He received treatment with strong antifungal medications and close monitoring, and his condition gradually improved. He was eventually transferred to a rehabilitation facility to help him regain his strength and function."
163,"A 35-year-old male with a history of hyperlipidemia and seizure disorder presented to an outside hospital following a breakthrough seizure, where he was incidentally also found to have punctate areas of acute cerebral infarcts in multiple vascular territories. Additional workup revealed the presence of a left atrial thrombus and newly diagnosed atrial fibrillation. He was ultimately discharged to home on apixaban. The patient then re-presented a month later for evaluation of transient diplopia, expressive aphasia, daily right temporal headaches, and right facial and left leg weakness. MRI of the brain showed new areas of diffusion restriction in the left cerebellar hemisphere and left medial occipital lobe (Figure ). CTA showed no signs of carotid occlusion or stenosis. The etiology of his multifocal strokes was thought to be related to his newly diagnosed atrial fibrillation and left atrial thrombus.\nThe patient was then transferred to our hospital for further evaluation. His initial NIH stroke scale was 8 (primary deficits were including unilateral facial palsy, bilateral lower extremity pronator drift and ataxia). Stroke labs, including lipid panel and hemoglobin A1C, were unremarkable. MRI of the brain with contrast showed a new infarct in the splenium of the corpus callosum in addition to prominent generalized meningeal enhancement (Figure ). MRI of the spine with contrast showed possible meningeal enhancement as well as punctate areas of encephalomalacia in the C3-4, C7, and T3 spinal levels. A bedside LP revealed a mildly elevated opening pressure of 24 cm H20, lymphocytic pleocytosis (WBC 150 K/cumm, 61% lymphocytes), protein 170 mg/dl, hypoglycorrhachia of 15 mg/dl, and presence of cryptococcal antigen. Other notable CSF labs included the presence of 11 oligoclonal bands. He was diagnosed with cryptococcal meningoencephalitis and started on a four-week course of amphotericin B and flucytosine. A repeat LP after several days of treatment showed a normal opening pressure of 14 cm H20, mildly improved pleocytosis (WBC 130 K/cumm, 84% lymphocytes), protein 172 mg/dl, and glucose 14 mg/dl. He did not require any additional lumbar punctures, and his symptoms (including headaches and left lower extremity weakness) gradually improved. The patient was discharged to an inpatient rehabilitation facility prior to returning home.\nThough our patient in Case 1 had a history of an unknown autoimmune disease, our patient in Case 2 had no history of autoimmune disease or other existing immunodeficiency. Both patients developed multiple cerebral infarcts in multiple vascular territories in the setting of cryptococcal meningoencephalitis, though our patient in Case 2 also had recently diagnosed with atrial fibrillation which further confounds the underlying etiology of his strokes.","This 35-year-old man experienced a stroke while being treated for another medical condition, and then developed new neurological symptoms. After being discharged, he returned to the hospital with headaches, vision problems, and weakness on one side of his body. Brain scans revealed new strokes and signs of inflammation in his brain and spine, leading to a diagnosis of cryptococcal meningitis. He began treatment with antifungal medications, and his symptoms gradually improved, eventually leading to his discharge to a rehabilitation facility."
164,"A 71-year-old male presented with a two-week history of painless right submandibular swelling that was not associated with fever. The patient had underlying hypertension and diabetes mellitus that were regularly treated.\nOn examination, a right submandibular swelling with normal overlying skin measuring 6 × 5 cm that was non-tender, mobile, and firm in consistency was noted (Figure , ). The swelling is ballotable by bimanual palpation. There was no other swelling palpable in the neck region. Intraorally, pus was noted at the Wharton’s duct orifice, and no sialolith was palpable.\nPreoperative blood investigations (complete blood count, serum urea and electrolytes, and serum uric acid), electrocardiography, and chest radiographs were normal. Computed tomography (CT) of the neck was performed as part of the preoperative assessment, which showed opacity in the right submandibular gland and duct (Figure , , ). A diagnosis of right submandibular stone was made. The patient subsequently underwent excision of the right submandibular gland under general anesthesia. Intraoperatively, the right submandibular gland was indurated (Figure ). During the excision, the surgeon noted another firm bulge along the submandibular duct that turned out to be a few smaller pieces of stones within the duct (Figure ). The size of the largest stone was 25 mm. Postoperative recovery was uneventful. Histopathology examination revealed severe acute-on-chronic sialadenitis with multiple calculi.","This 71-year-old man came to the doctor because of a painless lump in his jaw that had been growing for two weeks. The examination revealed a firm lump in his jaw, and tests showed it was a stone in his salivary gland. The stone was removed successfully, and the pathology report showed it was inflamed. He is recovering well after the surgery."
165,"A seven-year-old otherwise healthy female sustained bilateral elbow trauma after a fall with outstretched elbows and landing with force on the floor (kindergarten facility at the climbing frame).\nThe neurovascular status of both upper extremities was intact upon the arrival of the patient to the Trauma Unit. Clinical examination revealed loss of any active movement in both elbow joints in every plane. The joint was locked in a relatively extended position with the forearm neutral to a slightly supinated position. The patient had no sign of swelling or hematoma. Clinical suspicion was guided to a complex elbow injury, possibly with the participation of various bony structures.\nA gross estimation of the patient's potential hyperlaxity was performed except for the elbow joints using the Beighton scale without significant clinical findings []. Neurovascular status of the upper limbs was thoroughly re-examined, but no sign of neural or vascular impairment or compromise was found.\nPlain radiographs with standard projections (anteroposterior [AP] and lateral views) confirmed posterolateral elbow dislocation bilaterally with no signs of evident fractures. Identification of the bony structures was performed, and meticulous control and confirmation of the secondary ossification centers expected for the patient's age was done to exclude any secondary damage (Figures -).\nIn the emergency department, the upper limbs were immobilized in a provisional plaster with the elbows in a light hyperextension and neutral rotation of the forearms to reduce any movement and relieve the pain. The reduction was achieved under sedation in the operating theater and muscle relaxation with the patient in the beach chair position and with access to fluoroscopy during the whole procedure. The maneuver included gentle manipulation of the joints by slightly rotating, distracting and giving a flexion jerk to the joint. The audible and palpable ""click"" sign and the complete restoration of the arch of motion with the appropriate imaging confirmed the reduction as well as achievement of ligamentotaxis. Postoperatively, the patient retained both elbows in a functional position, stabilized with dorsal braces and collar and cuff to suspend the extremities (Figures -).\nRemoval of any support took place in three weeks post-reduction, followed by mild kinesiotherapy and gradual return to moderate athletic activities. The patient achieved a full range of motion with no pain and excellent ligament stability. At the six-week follow-up, new radiographs were obtained, with normal findings.","This seven-year-old girl fell at school and hurt both of her elbows. Her elbows became locked, and doctors carefully examined her arms to make sure there were no problems with the nerves or blood vessels. X-rays showed that her elbows were dislocated, but thankfully, no bones were broken. The doctor gently put her elbows back into place and stabilized them with a cast, and she is now recovering with physical therapy to regain full movement and strength."
166,"We present the case of a 42-year-old man who presented to the emergency department with a complaint of abdominal pain and diarrhea for 3 days. The abdominal pain started in the periumbilical region and was shifted to the right lower quadrant of the abdomen. The pain started gradually and had been progressing in severity. He described the pain as a stabbing in nature. It was exacerbated by movement and food intake. The pain was partially relieved by oral analgesic medications like paracetamol. The pain was associated with low-grade fever and decreased appetite. The patient also complained of diarrhea with five bowel motions/day. The stools were watery with no mucus or blood. He reported that diarrhea developed after he received an oral antibiotic therapy (cefuroxime) for a recent upper respiratory tract infection.\nThe past medical history of the patient was remarkable for diabetes mellitus that was well-controlled with oral antidiabetic agents. He did not undergo any previous abdominal surgeries. He had a smoking history of 15 pack-years. He had never drunk alcohol before. He worked as a taxi driver. The family history was unremarkable for any inherited gastrointestinal disorders.\nUpon examination, the patient appeared sick. He was not pale, jaundiced, or cyanosed. Vital signs revealed tachycardia (115 bpm), low-grade fever (37.5℃), normal respiratory rate (14 bpm), and maintained blood pressure (122/80 mmHg). The oxygen saturation was 99% on room air. Abdominal examination revealed a soft abdomen with diffuse tenderness. However, the tenderness was more pronounced in the right iliac fossa with a positive rebound sign. Further, the Rovsing sign was positive. Initial laboratory investigation revealed elevated leukocyte count and elevated inflammatory markers, including erythrocyte sedimentation rate and C-reactive protein. The renal and hepatic profiles were within the normal limits (Table ).\nIn light of the aforementioned clinical information, the patient was diagnosed as having acute appendicitis. A CT scan with intravenous contrast was performed to confirm the diagnosis. The scan demonstrated colonic wall thickening with edematous haustral folds suggestive of pseudomembranous colitis. Further, an endoluminal lesion was observed in the cecum with an average size of 6 cm. The lesion was well-defined and had a homogenous fat density with no solid component. The mass was causing a partial colonic obstruction. Such findings conferred the diagnosis of cecal lipoma (Figure ).\nThe patient was prepared for an emergency laparoscopy for further evaluation and management. The operation was done under general anesthesia and the patient was placed in the supine position. Limited segmental rection of the cecum with appendectomy was performed. The estimated blood loss was 10 mL and the total operative time was 100 minutes. The patient tolerated the operation with no complications. He had an uneventful recovery. Histopathological examination of the resected sample revealed the diagnosis of cecal lipoma and the associated acute appendicitis. The patient was discharged on the fifth postoperative day. After 3 months of follow-up, the patient remained asymptomatic with no active issues.","A 42-year-old man came to the hospital with abdominal pain and diarrhea that had been getting worse over three days. The pain started in his belly and moved to the lower right side, and he had a fever and felt unwell. Tests showed he likely had a lipoma (a fatty growth) in his cecum, which was causing a blockage and inflammation, leading to appendicitis. He underwent surgery to remove the lipoma and his appendix, and he recovered well and was discharged after five days."
167,"A 29-year-old, non-lactating, and non-gravid woman presented with a complaint of a lump in her right breast. The patient also complained of low-grade fever and unilateral pain in breast tissue. The general physical examination showed a one-centimeter erythematous and tender mass in the right breast tissue. There was no nipple discharge, axillary lymphadenopathy, or external draining sinuses. The primary care physician evaluated the patient and called for a USG for the assessment of the affected breast, which demonstrated an ill-defined lesion with thin fluid streaks in the lower outer quadrant, suggesting an inflammatory lesion (Figure ).\nAt the same time, cystic fluid from her breast was aspirated, and the bacterial culture test showed no growth of any microorganisms after 48 hours. The cytology revealed predominantly neutrophils and degenerating cells in a hemorrhagic background, which suggested an acute suppurative inflammatory process of the affected breast as shown in Figure .\nBased on the clinical features, imaging findings, and cytology reports, a presumptive diagnosis of acute mastitis with underlying bacterial abscess was established. Subsequently, she was being prescribed a course of antibiotics and antipyretics for one week. However, instead of improving, her condition worsened over time. Therefore, the physician decided to repeat the ultrasound-guided fine-needle aspiration and cytology (FNAC). The sonomammogram of the right breast revealed an ill-demarcated hypoechoic irregular lesion involving the parenchyma of the right breast in the outer lower and adjoining upper quadrants. The lesion measured 34.4 millimeters on a long axis with no fluid components as shown in Figure .\nThe repeated cytological examination also showed cellular clusters comprising histiocytes and epithelioid-like cells. Hence, based on these findings, the treating physician concluded that the ongoing clinical picture was more suggestive of tuberculosis of the breast. Therefore, after discussing with the patient, a therapeutic trial of an anti-tuberculosis therapy (ATT) was prescribed for four weeks. However, instead of any clinical improvement, her condition worsened over time and she had to visit her physician again just after three weeks. Her re-assessment by sonomammography was performed, which indicated an ill-demarcated hypoechoic irregular lesion, measuring 44.7×35.4 millimeters in the outer lower and adjoining upper quadrants of the right breast with thin fluid streaks as shown in Figure .\nThe sonologist concluded that these findings were consistent with an infectious or inflammatory lesion. Afterward, the primary care physician referred her to a teaching hospital for a surgical consult. So, re-evaluation of the patient was performed by a detailed history, thorough general physical examination, and routine laboratory investigations. It was then planned to perform incision and drainage of pus collection and excisional tissue biopsy after surgical resection of the mass. The excisional biopsy material was sent for histopathology and pussy discharge for acid-fast bacilli (AFB) smear. After surgery, she received broad-spectrum antibiotics (ceftriaxone and moxifloxacin) for two weeks. The subsequent histopathology showed areas of acute and chronic inflammation along with predominant lobulocentric granulomatous inflammation as shown in Figure .\nThese findings were suggestive of idiopathic granulomatous mastitis as an etiology. Concurrently, the AFB smear also came out to be negative. Hence, a final diagnosis of idiopathic granulomatous mastitis was established. Afterward, corticosteroids (prednisolone) were also added to the drug dosage regimen of this patient. The patient was followed up after one month, and during this period her symptoms had started to resolve. The medical team agreed to advise her to follow up until the complete resolution of her disease, and she was followed up monthly for the next three months. During these follow-up visits, we evaluated the patient for improvement in the clinical symptoms. Finally, a USG scan was performed, which showed complete resolution of the lesion with no abnormal findings. Table describes the timeline of all these events.","A 29-year-old woman came to the doctor complaining of a lump in her breast, along with fever and pain. Tests showed the lump was likely caused by an infection and inflammation in her breast tissue, but the culture didn't show any bacteria. After further tests, the doctor suspected tuberculosis of the breast, and started her on a course of anti-tuberculosis medication. Unfortunately, her condition worsened, and further tests revealed she had idiopathic granulomatous mastitis, a chronic inflammatory condition. Finally, she was treated with corticosteroids and followed up closely until her symptoms resolved."
168,"A 58-year-old man, with no relevant medical history, presented with a history of an enlarging painless mass at his right groin region for the past three months (Figure ). The patient did not have any other complaints or symptoms. Physical examination revealed a firm, skin-colored and mobile tumor with well-defined margins (5 cm largest diameter). There were no palpable adenomegalies.\nThe patient was referred to the General Surgery department by a urologist, with suspicion of a soft-tissue tumor. An MRI described a “focal subcutaneous lesion with nodular morphology of 4.7 cm and no malignancy features”. Based on clinical and image findings, it was decided to perform an excisional biopsy.\nDespite the apparent benign characteristics, the lesion was surgically removed along with the surrounding adipose tissue, preserving the margins. There were no complications related to the procedure.\nGrossly, it was a subcutaneous nodular non-capsulated solid lesion, multilobulated, well-circumscribed, greyish-yellowish, without necrotic areas (Figure ). Microscopically, a variable amount of atypical bland spindle cells and mature adipocytes were seen, with multinucleated floret-like cells in a myxoid stroma with ropey collagen bundle cells. Sclerosing areas were not disclosed (Figure ). On immunohistochemistry, the tumor was stained for CD34, S100, and MDM2 (focal-weak), whereas CDK4 expression was absent (Figure ). Based on these findings, an atypical pleomorphic lipomatous tumor was diagnosed.","A 58-year-old man found a growing lump in his groin that he noticed over the past three months. After a physical exam and some imaging tests, doctors decided to remove the lump for further examination. The lump was surgically removed and examined under a microscope, revealing a type of fat tumor with some unusual cells. The final diagnosis was an atypical pleomorphic lipomatous tumor, which is considered a benign growth."
169,"A 15-year-old female patient was referred by a pediatric cardiologist to our pediatric cardiac surgery clinic with a confirmed diagnosis of CCL syndrome since birth by a dermatologist. Her cardiovascular symptoms started one month before the presentation with a history of recurrent episodes of shortness of breath, palpitations, and chest pain. The severity of the symptoms has increased in the past few weeks. At the time of referral, she was on furosemide 10 mg twice daily and enalapril 10 mg once daily. Her parents are phenotypically normal. All her siblings, five brothers and two sisters, are free from the disorder. Also, the patient has a remarkable family history, as her cousin is a 20-year-old male with the same disorder. There is consanguinity between parents in the family. On general examination, she had a senile appearance with generalized inelastic, loose, and sagging skin. Vital signs revealed a heart rate of 114 beats per minute, respiratory rate of 20 breaths per minute, blood pressure of 123/73 mmHg, oxygen saturation (SpO2) of 100% in room air, and temperature of 36 °C. On cardiac examination, the precordium was hyperactive, the first and second heart sounds were obscured, and pansystolic murmur grade III/VI radiating to the axilla was detected. The hematological studies were within normal limits. Electrocardiogram (ECG) showed sinus tachycardia with right atrial enlargement and right ventricular hypertrophy (Figure ). Chest x-ray showed cardiomegaly with subsegmental atelectasis (Figure ). For more assessment and operative plan, transesophageal echocardiogram (TEE) revealed severe mitral and tricuspid valve prolapse with malcoaptation causing severe regurgitation of both valves with pulmonary hypertension and severe dilatation of both right and left atria (Figures -). After the patient’s condition was discussed in the heart team meeting, the plan was set for mitral and tricuspid valve repair versus replacement, depending on the intraoperative findings. Also, the patient was planned to be counseled by a medical geneticist. The case was discussed with the patient and her family as they were involved in the clinical decision.\nProcedure\nUnder general anesthesia, midline sternotomy was carried out, and the thymus was resected due to its enormous size. Standard cannulation was accomplished through the ascending aorta and superior and inferior vena cava with snugging around each cannula. Consequently, standard cardioplegic arrest with full flow of cardiopulmonary bypass (CPB) was achieved as the patient was cooled down to a temperature of 30 °C. The aortic valve was immediately examined after the aorta was transversely opened, and it had some significant enlargement and dilatation of the leaflet, but the valve was manually competent; thence, no intervention was done to the aortic valve. Both atria were significantly enlarged; thereupon, right and left atrial appendages were resected in combination with atrioplasty (Figures , ). The left atrium was opened, the mitral valve was examined and showed significant myxomatous changes of both anterior and posterior leaflets, and the valve was irreparable. Hence, the decision was made intraoperatively to replace the mitral valve with a 33-mm St. Jude Medical Epic porcine valve prosthesis (Figure ). After this, through an incision into the right atrium, the tricuspid valve was found to have a cleft at the septal leaflet and the anterior leaflet with significant dilation of the annulus. Accordingly, commissural tricuspid annuloplasty was performed. The tricuspid valve test rendered a competent valve. After completing the procedure, the patient was fully rewarmed and weaned off CPB. Intraoperative TEE showed trace tricuspid regurgitation, and the prosthetic valve was well seated at the mitral position. With these findings, the patient was decannulated, chest tubes and pacer wire were inserted, and closure was performed. She was transferred to the cardiac surgery intensive care unit (CSICU) in a stable condition. During the first 15 hours in the CSICU, the chest tubes drained blood in a total of 1,450 mL (right lower pleura), 300 mL (mediastinum), and 1,330 mL (left lower pleura). Afterward, the patient received five units of packed red blood cells (PRBCs), seven units of fresh frozen plasma (FFP), two units of cryoprecipitated antihemophilic factor (Cryo), two units of platelets, and two doses of intravenous protamine sulfate (50 mg per dose). The bleeding was then controlled (Table ). The patient was shifted from the CSICU to the ward on postoperative day 3 (POD 3). All chest drains were removed subsequently, and laboratory work was within normal limits. Predischarge transthoracic echocardiogram (TTE) showed no significant changes, and the patient was planned to be discharged home on POD 6 in satisfactory condition.","This 15-year-old girl has a rare heart condition called CCL syndrome that she’s had since birth. She’s been experiencing shortness of breath, chest pain, and palpitations, and these symptoms have gotten worse recently. Tests revealed that her heart valves were severely damaged, and her heart was enlarged, requiring surgery to repair the valves and reduce the size of her heart. During the surgery, the thymus gland was removed, and the mitral valve was replaced with a new valve. The surgery was successful, and she is now recovering well in the hospital."
170,"An 11-year-old boy was diagnosed with Crohn’s disease at the age of nine years. Since then, he was on a regular infliximab transfusion regimen at monthly intervals at a dose of 5 mg/kg for maintenance of remission of disease as symptoms relapse by the end of each month. He presented to the Maternity and Children Hospital in Al-Ahsa, Eastern Province in Saudi Arabia, complaining of palpitation for one year. The palpitations were intermittent at the beginning of the year but then worsen progressively over the last month. They were associated with easy fatigability and chest discomfort, with no history of cyanosis or chest pain. There was no history of a similar condition or history of cardiac disease or sudden death in the family. In addition, these symptoms occur with the manifestation of tachycardia during infliximab transfusion with no respiratory or mucocutaneous involvement or other signs of anaphylaxis. This transfusion reaction is managed by slowing the transfusion rate and premedication with steroids and antihistamines.\nUpon examination of the child, he appeared pale, underweight (with weight of 18 kg below the third centile) but not distressed. He had sinus tachycardia (150-160 beats/min) with maintained blood pressure (98/59 mmHg). chest examination revealed hyperdynamic precordium with pan-systolic murmur grade III out of VI at the apex with radiation to left mid-axillary line with no thrill. Rest of the examinations were unremarkable. Laboratory investigations showed microcytic hypochromic anemia related to the drop of iron profile and thrombocytosis, with positive anti-Saccharomyces cerevisiae antibodies for Crohn’s disease and negative antibodies for infliximab (Table ). ECG and Holter 24 hours monitor were done and showed intermittent sinus tachycardia with no dysrhythmias (Figure ). Echocardiography showed dilated left ventricle with ejection fraction of 21% and fraction of shorting of 10% associated with severe mitral regurgitation (Figure and Video ). The patient was diagnosed with acute heart failure secondary to dilated cardiomyopathy. He started on anti-failure medications controlling three parameters pre-load, after-load, and enhancing myocardial contractility by diuretics furosemide (1 mg/kg/dose twice a day initially), spironolactone (1 mg/kg/dose twice a day), enalapril (0.35 mg/kg/day divided three times a day) and digitoxin (2.5 mcg/kg/day once daily), respectively. Aspirin was added as an anticoagulant. In addition, the patient started on intravenous iron to improve anemia which was not responding to oral iron supplementations or dietary management. The patient was discharged home with the previous plan and kept on regular follow-up with pediatric cardiology with no need for cardiac surgery at the present time. In addition, he kept on regular follow up with pediatric hematology to assess the improvement in hemoglobin and response parental iron. Finally, the patient referred to a tertiary hospital for further follow-up with pediatric gastroenterology and the possibility of starting a different anti-tumor necrosis factor agent ustekinumab.","This 11-year-old boy has Crohn’s disease and has been taking medication to manage it. Recently, he started experiencing heart palpitations, tiredness, and chest discomfort. Tests revealed he has a weakened heart and fluid buildup, leading to heart failure. He’s now receiving medication to help his heart and anemia, and will continue to be monitored closely by specialists."
171,"In September 2020, a 42-year-old male was referred to our Department of Internal Medicine because of a finding in a chest X-ray. The patient was healthy with no previous hospitalizations and worked as a nurse at our institution. He was not taking any medications, had no smoking history, and was presenting no malignancy-related symptoms (fatigue, unintended weight loss, or changes in bowel habits). He had seen an anesthesiologist before being seen by a surgeon due to an inguinal hernia, a minor condition. The anesthesiologist noticed the pulmonary lesion. A chest X-ray showed a solitary pulmonary nodule in the right mid-lung that was 2 cm in diameter (Figure ). The patient was asymptomatic, as mentioned above, and had a previous chest X-ray that was normal.\nIn a physical examination, his temperature was 36.7°C, blood pressure was 138/78 mm Hg, heart rate was 76 beats per minute, and oxygen saturation was 98% in room air. In auscultation, heart and lung sounds were normal. Both oropharyngeal and abdominal examinations were normal, and he had no periodontal disease. The patient was admitted to the hospital for further investigation.\nBlood tests were normal, with a white blood cell count of 9,430 leucocytes/mm3 with 63% neutrophils, hemoglobin level of 153 g/L, and platelet count of 205 × 109/L. C-reactive protein was 83 mg/dL (normal range: <5 mg/dL). A reverse-transcription polymerase chain reaction (RT-PCR) test was negative on hospitalization day 1 (Table ). As mentioned, a chest X-ray showed a solitary pulmonary nodule in the right upper lobe.\nOur patient presented with a solitary pulmonary nodule, which raised the suspicion of a primary lung tumor or metastasis of unknown origin. For further radiological characterization and assessment of the pulmonary node, the patient underwent a thoracic CT scan, which revealed ground glass opacities that suggested either inflammatory or infectious conditions (Figure ), similar to atypical pneumonia. A second RT-PCR was performed on hospitalization day 3, and the result was positive. Based on these data, we established a diagnosis of COVID-19 pneumonia in an asymptomatic patient. The first test for the qualitative detection of IgG against SARS-CoV-2 (a chemiluminescent microparticle immunoassay) was negative on hospitalization day 3.\nOn day 5, the examination was normal, the patient’s oxygen saturation was 98%-99% in room air, and laboratory blood tests were normal, so we discharged the patient with no treatment, but he was attended in a follow-up in our outpatient clinic with a new chest X-ray and new laboratory blood tests on day 10 after discharge. The chest X-ray showed complete resolution of pneumonia, and laboratory blood tests were normal, with C-reactive protein < 5 mg/dL and a positive test for IgG against SARS-CoV-2.","A 42-year-old nurse was referred to the hospital after a chest X-ray revealed a small spot in his lung. He was otherwise healthy and had no symptoms, but the spot raised concerns about a possible tumor. Further tests, including a CT scan, showed signs of pneumonia, and a COVID-19 test confirmed the diagnosis. Fortunately, the pneumonia resolved completely, and he was discharged home with no treatment after a follow-up visit confirmed his recovery."
172,"A female patient aged 27 years presented with a slow-growing abdominal lump of nine months duration. There were no other symptoms except for fullness of the abdomen after taking food and weight loss. Clinical examination revealed large, non-tender, soft cystic swelling occupying the entire upper abdomen.\nOn radiological investigations, ultrasonogram (USG)-complex cystic mass with internal septation was present. Contrast-enhanced computed tomography (CECT) abdomen showed a large multiseptated cystic lesion occupying almost the entire abdomen and adhered to the pancreas with mass effect. The lesion was well defined, lobulated, hypodense in nature, and associated with main pancreatic duct dilatation. There was also portal vein thrombosis with portal cavernoma on the CECT abdomen (Figure ).\nUSG-guided FNAC revealed straw-colored aspirate with mature lymphocytes; no atypical cells were noted. Cyst amylase and carcinoembryonic antigen (CEA) were within the normal range (Figure ).\nAfter complete preoperative workup, the patient underwent surgical exploration. On exploration, there was a large multiloculated cystic lesion extending through gastrocolic omentum, pushing the stomach up and transverse colon downwards with hundreds of cystic spaces containing lymphatic fluid (Figure ).\nCysts were decompressed, and the entire lesion was excised. The anterior surface of the pancreas was forming the base of the lesion. Postoperatively patient had continued lymphatic discharge from the abdominal drain, and the patient was discharged with it (Figure ).\nFinal histopathological examination showed unremarkable pancreatic tissue with attached lesion consisting of dilated lymphatic spaces with lymphatic follicles in the wall, with congested blood vessels and fibrocollagenous tissue with chronic inflammatory infiltrate. Cytocentrifuged smears from fluid show lymphocytes, and no malignant or atypical cell was seen. The patient was followed regularly in outpatient department, and drain output gradually decreased over two weeks and was removed (Figure ).","A 27-year-old woman saw a doctor because she found a large, growing lump in her abdomen that had been bothering her for nine months. Tests, including ultrasound and CT scans, showed a large, complex cyst attached to her pancreas, which was causing her symptoms and pushing on nearby organs. Surgery was performed to remove the entire cyst, and the doctors found it was filled with lymphatic fluid. After surgery, the patient continued to drain fluid from her abdomen, but this gradually decreased over two weeks, and she was eventually discharged home. The final examination of the removed cyst confirmed that it was a benign lymphatic cyst with no signs of cancer."
173,"A 58-year-old woman with a past medical history of diabetes and hypertension presented to our clinic for evaluation of an enlarging left breast mass that she first noticed three years ago. She stated that the mass started to progressively increase in size in the past few months. She also reported a weight loss of 34 pounds over the last nine months. On physical exam, she had a palpable breast mass of approximately 3 centimeters in the tail of the left breast. Rest of physical exam was unremarkable. Review of labs showed WBC of 8.8/mL with absolute lymphocyte count of 4400/mL, hemoglobin of 12g/dl and platelet count of 316K. Prior mammograms and ultrasounds had revealed stable intramammary lymph nodes at the site of the present lesion on the left breast over the last three years with no suspicious calcifications or architectural distortions. There were no palpable ipsilateral or contralateral axillary lymphadenopathy. The mass was subsequently percutaneously biopsied. Histopathology showed diffuse atypical small lymphocytic cells (Figure ). Immunohistochemical staining revealed neoplastic lymphoma cells positive for CD20 (Figure ), CD5 (Figure ), CD23 (Figure ), PAX5, CD4, BCL2 and negative for CD3, CD10, BCL1, and BCL6. The final pathologic diagnosis was consistent with primary small lymphocytic lymphoma of the breast rather than chronic lymphocytic leukemia. This was unexpected. Bone marrow biopsy was done and histopathology revealed diffuse involvement with small lymphocytic lymphoma (Figure ). Fluorescence in situ hybridization (FISH) studies revealed trisomy 12 cytogenetic abnormality. Computed tomography (CT) imaging revealed extensive mediastinal lymphadenopathy (Figure ) and retroperitoneal lymphadenopathy (Figure ). The final clinicopathologic diagnosis was small lymphocytic lymphoma (Stage 4) with breast and bone marrow involvement. She was subsequently treated with the FCR regimen comprising of fludarabine, cyclophosphamide and rituximab which resulted in clinical and radiologic remission. The breast mass was no longer palpable and repeat CT scans did not reveal any evidence of any pathologic lymphadenopathy.","A 58-year-old woman with diabetes and high blood pressure came to the clinic because she noticed a growing lump in her breast over the past few months, along with unintentional weight loss. After a biopsy, doctors found that the lump was a type of lymphoma, a cancer of the immune system, that had spread to her lymph nodes in the chest and bone marrow. She was treated with chemotherapy, which successfully shrunk the cancer and led to a complete remission. Follow-up scans showed no signs of the lymphoma remaining, and the breast lump disappeared."
174,"This is a case of 41-month-old infant girl who presented with weight loss and intractable diarrhea associated with oral feeding. She is a product of full-term pregnancy, delivered through spontaneous vaginal delivery, with a birth weight of 2 kg, and she did not require admission to the neonatal intensive care unit (NICU). The pregnancy was uneventful. Parents are first-degree cousins, and the patient has two older healthy siblings.\nAt the age of six months, she presented to another hospital afebrile with a loss of weight and had watery, non-bloody diarrhea, six to seven times per day. Both endoscopy and biopsy from the duodenum were normal according to the mother. The patient was initially misdiagnosed with cow milk protein allergy, so hypoallergenic formula was given but there was no improvement, then after two months, she was switched to amino acid-based infant formula 200 ml five times per day. In the beginning, there was an improvement, but with time, she stopped gaining weight again. At the age of 10 months, she came to the gastroenterology and genetics teams at our facility with chronic diarrhea, poor growth, and abnormal hair. Her weight and height were 5.30 kg (<3rd percentile) and 61 cm (<3rd percentile), respectively. The diagnosis of THES was confirmed by whole exons sequence (WES) analysis, which identified the homozygous variant (c.1201G > A) p. (Glu401Lys) in the SKIV2L gene. Upon literature review, we did not find the mentioned variant mutation in any previous literature (Table ).\nAt 12 months of age, she was admitted for dehydration and nasogastric tube (NGT) feeding due to poor weight gain. Her body measurements upon admission were 5.64 kg (<3rd percentile) for the weight, and her height was 63 cm (<3rd percentile). Upon examination, she had some dysmorphic features such as a depressed nasal bridge, broad forehead, low set ears, and scanty dry hair. During her admission, she was having watery diarrhea with mucus two to three times a day. She was managed with intravenous fluid and the clinical nutritionist prepared a high-calorie formula. The nutrition therapy plan was to provide 180 ml of hypoallergenic baby formula (0.67 kcal/ml) every four hours orally, as much as she can tolerate, and if she did not complete her meal, give the rest through the NGT. This plan provided her with 192 ml/kg fluids and 135 kcal/kg/day energy. Her laboratory workup results were sodium 138 mmol/l, potassium 3 mmol/l, chloride 113 mmol/l, aspartate aminotransferase (AST) 40 units/l, alanine aminotransferase (ALT) 30 units/l, and gamma-glutamyl transferase (GGT) 32 units/l. Her immunoglobulins workup showed low immunoglobulin E <25 au/ml, and normal immunoglobulin G and A levels. She was improving and gained 100 grams in two days. On the seventh day of admission, she was able to tolerate oral feeding, so the patient was discharged on the same plan and to add 1 ml of medium-chain triglycerides oil every other day.\nAt the age of 21 months, the mother reported in a follow-up visit that her daughter’s weight has been improved and reached 7.8 kg, but still under the third percentile. The patient was continued on hypoallergenic baby formulas and a regular diet. At the age of 41 months, the patient presented with progressive bullous itchy skin rash, and fluid-filled vesicles on erythematous background with red erosions and fissures all over the body but sparing abdomen and back. It was associated with cough, rhinorrhea, fever, and decreased urine output and oral intake, but without gastrointestinal complications. The diagnosis of bullous impetigo was made, and the patient was admitted. Amoxicillin, clavulanate, and diphenhydramine were administered intravenously with topical clindamycin and tretinoin cream. The patient showed clinical improvement in three days. The patient was discharged on oral Augmentin, Mupirocin, and Loratadine (Figure ).\nOn follow-up evaluation at the age of 41 months, her weight and height were 10 kg (<3rd percentile) and 85 cm (<3rd percentile), respectively. She was able to tolerate normal oral feeding. She did not show any signs of intellectual disability, and she is developmentally up to her age now. She is active and plays with her cousins. She can speak and understand both Arabic and English, and according to the mother she is starting to memorize songs.","This 41-month-old girl has had ongoing problems with diarrhea and not gaining enough weight. She was initially thought to have a milk allergy, but that didn’t help. Genetic testing revealed a rare condition called THES, which affects how her body digests food. Because of this, she needed special formulas and extra support to get enough nutrition. Currently, she is doing much better with a special diet and is gaining weight, but she still needs to be monitored closely."
175,"A 49-year-old female patient was admitted with complaints of fever, abdominal bloating, and losing weight for one year. She was diagnosed with cirrhosis and was treated at a local hospital. Three months ago, the patient deteriorated; thus, peripheral blood test, bone marrow (BM) aspiration, and bone marrow biopsy were performed. The results showed lymphocytosis in the marrow. Therefore, the patient was referred to our center. On physical examination, she had a fever (about 38°C), mild pallor, swollen legs, mild hepatomegaly, and huge splenomegaly. There was no purpura and no peripheral lymphadenopathy. There were no clinical infections and no joint damage.\nComplete hemogram revealed hemoglobin of 117 g/L, platelet count of 82 G/L, total leukocyte count of 12.63 G/L with 65% lymphocytes, and 30% neutrophils. The peripheral blood smear showed lymphocytosis and thrombocytopenia (Figure ). The lymphocytes were predominantly large lymphocytes, which were having abundant cytoplasm containing coarse azurophilic granules and clumped chromatin. Her biochemical examination was fairly normal. Serological examination revealed no evidence of HIV, HBV, HCV, EBV, CMV, or dengue infection. The results of cultures of fungi and bacteria in blood were negative. The ultrasound of the abdomen confirmed mild hepatomegaly and huge splenomegaly.\nBone marrow imprint smears showed 33% lymphocytes (lymphocytosis) (Figure ). The lymphocytes displayed a medium to large size with a moderate amount of cytoplasm containing numerous azurophilic granules and a round nucleus with clumped chromatin. Bone marrow biopsy displayed an increasing level of cell density and lymphocytic infiltration in hematopoietic compartments with nonuniform size and similar morphology lymphocytes seen in peripheral smear (Figure ). The erythroid, myeloid, and megakaryocytic series were suppressed.\nCytogenetics revealed a normal karyotype. Flow cytometric analysis of the bone marrow showed that 49.5% of cells were of lymphoid origin. These lymphoid cells were positive for T-cell markers, including CD2, CD8, CyCD3, CD5, CD7, CD56, CD16, and TCR gamma delta. The cells expressed CD4-. These findings were suggestive of T-cell large granular lymphocytic leukemia.\nAfter being treated with methotrexate for one month, the clinical condition was ameliorated. The patient had no fever and splenomegaly regression, and the peripheral blood revealed an increasing proportion of neutrophils (65%). Therefore, the patient was given outpatient treatment and kept being examined monthly.","A 49-year-old woman was admitted to our hospital after experiencing a year of fever, weight loss, and abdominal bloating, which led to a diagnosis of cirrhosis. Further tests revealed an unusual increase in lymphocytes in her bone marrow, prompting a referral to our center for further investigation. The tests showed she had T-cell large granular lymphocytic leukemia, a rare type of blood cancer. After treatment with medication, her condition improved, and she was able to return home for ongoing monitoring."
176,"A 34-year-old man with no previous history of chronic illness and a non-smoker presented with a history of headache, fatigue, diarrhea, vomiting, and insomnia for three days. During the initial examination, he was conscious and alert. His blood pressure (BP) was 111/71, pulse rate (PR) 40, respiration rate (RR) 14/min, body temperature 36.7, and oxygen saturation (SpO2) 96% under ambient oxygen conditions. The patient had a clear chest, without any crepitating sounds in the cardiovascular system (CVS; S1+S2+0). An abdominal exam showed a soft and lax abdomen, and both lower limbs were normal. The status of the central nervous system (CNS) was normal, all cranial nerves were intact, and chest X-ray and chest CT scans were performed (Figures -). EKG showed sinus rhythm, first-degree heart block with prolonged QT interval, and bigeminy (Figure ). Echo revealed a normal echo study (Figure ). General clinical and blood parameters of the patients are shown in Table . Due to the COVID-19 pandemic, all patients reporting to the hospital with fever were routinely tested with the PCR test for COVID-19. Also, a nasopharyngeal swab was tested by RT-PCR and proved to be positive for SARS-CoV-2.","6 This 34-year-old man came to the hospital because he’s been experiencing headaches, fatigue, diarrhea, vomiting, and trouble sleeping for the past three days. His vital signs were normal, and a physical exam didn’t reveal any obvious problems. Tests showed he has COVID-19, and an EKG revealed a slight heart rhythm irregularity. He is being monitored and treated for COVID-19 and his symptoms."
177,"Another 34-year-old male patient who smoked visited the hospital with a history of fever, runny nose, and diarrhea for seven days. There was no complaint of shortness of breath or chest pains. The patient was tested for central nervous system (CNS) response and reflexes and was alert and conscious. Examination of clinical vitals parameters was performed and recorded as BP: 126/76; PR: 43; RR: 21/min; body temperature: 36.6°C; oxygen saturation (SpO2): 98%; CVS: S1+S2+0; chest bilateral vesicular breathing, and no lower limbs edema. EKG reports showed sinus bradycardia with a prolonged PR interval and QT interval; a U wave was observed in V1 (Figure ). Chest X-ray and chest CT showed unremarkable findings (Figures -). Echocardiography also revealed a normal echo study (Figure ). General clinical and blood parameters of the patients are provided in Table .\nPatient 2 was suspected of COVID-19 due to a non-symptomatic fever. The nasal swab of Patient 2 was tested by the same procedure as for Patient 1 and was SARS-CoV-2 positive.\nTreatment\nBoth patients were admitted to the isolation room and treated conservatively without hydroxychloroquine and azithromycin. They were administered 1 mg IV atropine and showed a transient change from bradyarrhythmia to sinus rhythm.","A 34-year-old man with a history of smoking came to the hospital because he had a fever, runny nose, and diarrhea for a week. Tests showed he had COVID-19, despite not feeling very sick. His heart rate was unusually slow, and an EKG revealed some abnormalities. He was treated with medication to help his heart rhythm and kept in isolation, and his condition stabilized."
178,"A 50-year-old Indian gentleman, a known case of diabetes and an active smoker, presented with a right eye painless inferior visual field defect upon waking up from sleep. At presentation, his right and left eyes' visual acuity (VA) were 6/24 and 6/9, respectively. Anterior segment examination was unremarkable in both eyes. Fundoscopy revealed swollen right optic disc with peripapillary splinter hemorrhage (Figure ). Humphrey visual field (HVF) showed right inferior altitudinal scotoma. Computed tomography of the brain and orbit proceeded to rule out compressive lesions. Thus, a diagnosis of right eye NAION was made. Three months later, he complained of a worsening visual field of the right eye. VA remained static with the right eye (VA 6/24) and left eye (VA 6/9). Examination showed right eye relative afferent pupillary defect (RAPD) with impaired red saturation and light brightness. His right optic disc was pale; however, the left was hyperemic and swollen with peripapillary splinter hemorrhage (Figure ). HVF showed right eye tunnel vision while the left eye displayed inferior arcuate scotoma (Figure ). The patient was admitted for further investigations and was co-managed by the neuro-medical team. The visual evoked potential test was suggestive of right optic neuropathy. Serum glucose and serum hemoglobin A1c (HbA1c) levels were elevated, measuring 13.9 mmol/L and 9.1%, respectively. Serum total cholesterol (4.6 mmol/L) and low-density lipoprotein (LDL) cholesterol (2.1 mmol/L) were normal, but triglyceride level was high (3.3 mmol/L). Full blood count, erythrocyte sedimentation rate (2 mm/hour), and C-reactive protein (0.7 mg/dL) were all within normal limits. Serum anti-aquaporin-4, anti-nuclear antibodies, rheumatoid factor, and infective screening tests were negative. However, magnetic resonance imaging revealed suspicious enhancement of both intra-orbital optic nerves suggestive of bilateral optic neuritis. Diagnosis of bilateral atypical optic neuritis was made. The patient was loaded with intravenous methylprednisolone 1 g/day for five days and subsequently oral steroid in tapering doses along with topical brimonidine tartrate 0.2%. At the end of one month, his VA remained static and fundoscopy showed left eye resolving optic disc swelling. Despite that, his left eye's visual field progressively deteriorated to inferior altitudinal scotoma (Figure ). Subsequently, we performed a lumbar puncture test, and the result was unremarkable. Repeated MRI of the spine and brain showed no focal enhancing lesion. Upon further history taking, the patient revealed that he had a history of taking phosphodiesterase type 5 (PDE5) inhibitor (tadalafil) on and off over the past year. Hence, we diagnosed him with bilateral sequential NAION, and he was co-managed with the endocrine team to optimize his diabetic status. His subsequent visual field remained static with right eye tunnel vision and left eye inferior altitudinal scotoma (Figure ).","A 50-year-old man with diabetes and a history of smoking experienced a loss of vision in his right eye, particularly in the lower part of his vision. Tests revealed swelling around his optic nerve and a shrinking of his vision field, leading to a diagnosis of a condition called NAION. Later, his vision in the right eye continued to worsen, and imaging showed inflammation in both eyes. After treatment with steroids, his left eye’s vision also deteriorated, and further investigation revealed that he may have developed NAION in both eyes, possibly linked to a medication he was taking."
179,"We present the case of a 30-month-old male who was brought to the family medicine clinic with a complaint of abdominal bloating and persistent diarrhea after every feeding for four months. His stools were foul-smelling and occurred more than four times a day. The diarrhea was associated with a failure to gain weight. The parents reported that the child has a normal appetite with no history of vomiting or feeding intolerance. There was no history of fever, night sweats, rash, cough, or joint pain. The child did not have any recent sick contact. The patient was seen by several general practitioners for the same complaint, but no diagnosis was obtained.\nThe patient had an unremarkable past medical history. He did not have any previous hospital admissions. He had no history of previous surgeries. He does not take any medications and was not known to have any food or drug allergies. Regarding the perinatal history, the child was full term with a birth weight of 3.5 kg. The labor and delivery were unremarkable for any complications. The child was up to date with his vaccination schedule. Regarding the developmental history, the child reached the developmental milestones at the appropriate ages, and there was no parental concern regarding his development. The social history was noncontributory. There was no history of diseases running in the family. The child was not born of a consanguineous marriage.\nUpon examination, the child was awake and alert and did not appear sick. No dysmorphic features were noted. The patient was below the second standard deviations for weight and height. He appeared pale, and there was no scleral icterus. His vital signs were as follows: heart rate of 90 bpm, blood pressure of 80/52 mmHg, respiratory rate of 22 bpm, and temperature of 36.8°C. Abdominal examination revealed a soft and non-tender abdomen with no organomegaly and had normal bowel sounds. Neurological examination, including hearing and vision tests, was normal. No evidence of muscle wasting was noted. The cardiorespiratory examination was unremarkable. The initial laboratory investigation revealed a hemoglobin level of 14.1 g/dL, leukocyte count of 5100/µL, and platelet count of 370,000/µL. Liver enzymes were mildly elevated. The total protein was 2.9 g/dL. His random glucose level was normal. Stool analysis, including routine culture, ova, and parasite testing, was negative. Stool testing for reducing substances yielded negative results. The remainder of the laboratory evaluations is summarized in Table .\nBecause of the clinical and laboratory findings, the patient was referred to the pediatric gastroenterology center for further investigation. The patient underwent upper endoscopy, which showed no abnormal gross findings. However, mild duodenal inflammation was evident in the histopathological examination of the obtained specimen. The patient underwent a 72-hour fecal fat collection, which indicated the presence of fat malabsorption. Subsequently, the fecal pancreatic elastase level was measured as 20 fecal μg/g. Such findings were consistent with exocrine pancreatic dysfunction. To exclude the possibility of cystic fibrosis as the underlying etiology of malabsorption, a chloride sweat test was performed and yielded negative results.\nA dedicated abdominal computed tomography scan to evaluate the pancreas for any structural abnormalities was performed. The scan demonstrated complete replacement of the pancreatic parenchyma by fatty tissue (Figure ). The suggested differential diagnoses by the radiologist for the fatty replacement of the pancreas included cystic fibrosis, Shwachman-Diamond syndrome, and Johanson-Blizzard syndrome. The blood sample of the patient was sent for genetic analysis. The diagnosis of Shwachman-Diamond syndrome was established as the analysis revealed a mutation in the SBDS gene (Table ). The detected mutation was previously reported in the literature [].\nThe patient was treated with pancreatic enzyme replacement therapy. After two months of follow-up, the parents reported that the patient had significant improvement in diarrhea. A weight gain was observed. Since the patient did not have any history of bleeding or recurrent infections, treatment with granulocyte colony-stimulating factor was not offered.","This 30-month-old boy has been experiencing frequent, smelly diarrhea after every feeding, along with a struggle to gain weight. He’s been seen by several doctors, but no one could figure out what was wrong. After further tests, including a stool analysis and imaging scans, doctors discovered he has Shwachman-Diamond syndrome, a condition that affects his pancreas and causes problems with digestion. He is now being treated with pancreatic enzymes, and his diarrhea and weight gain have improved."
180,"A 27-year-old male patient was admitted to a high-complexity institution due to a five-day clinical picture consisting of additive, symmetrical polyarticular pain, located in the wrists, knees, and left shoulder without other associated symptoms. The joint pain was continuous, permanent, and of great intensity that caused limitation of movement, mainly in the hands. In the systems review, no urinary or gastrointestinal symptoms were documented before the onset of joint symptoms. As the only relevant antecedent, he referred mild respiratory infection by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) confirmed by reverse transcription-polymerase chain reaction the month before hospitalization. The initial clinical examination confirmed the presence of bilateral carpal synovitis, left suprapatellar synovitis, and synovitis of the sternoclavicular joints (Figures , , ).\nMagnetic resonance imaging of the different compromised anatomical compartments was performed, presenting as additional findings the presence of bursitis of the lateral collateral ligament of the left lower limb and a peritendinous inflammatory process of the triangular fibrocartilage in the right wrist. Mild lymphopenia associated with a marked elevation of acute-phase reactants was documented in laboratory studies. Differential diagnoses of infectious etiology were sought, including a positive fourth-generation HIV enzyme-linked immunosorbent assay with a subsequent CD4 count of 98 cells/mm3 and a viral load of 459,000 copies/mL. Treatment with prednisolone at a dose of 1 mg/kg and sulfasalazine (1 g per day) was initiated and no significant improvement in joint symptoms was noted despite sequential changes from disease-modifying antirheumatic drugs (DMARDs) such as methotrexate and leflunomide. Subsequently, the patient achieved a complete joint response when antiretroviral therapy (abacavir, dolutegravir/lamivudine) was initiated, thereby achieving a response to therapy resulting in gradual reduction of glucocorticoid doses and tapering to maintenance therapy solely with sulfasalazine. The description of the most representative laboratory studies upon admission and during hospitalization is shown in Table .","This 27-year-old man was admitted to the hospital because he had intense, persistent pain in his wrists, knees, and shoulder for five days. He had recently recovered from COVID-19, and the pain was unusual for him. Tests revealed inflammation in his joints and some other abnormalities, including a possible HIV infection. After starting treatment for his HIV, his joint pain completely disappeared, and he was able to stop taking high doses of medication."
181,"A 43-year-old-male with no known medical problems presented from an outside hospital with concern for intraabdominal hemorrhage. He was in an MVC three weeks prior and presented with complaints of right upper quadrant abdominal pain, left-sided chest pain, nausea, and vomiting. A CT scan showed fluid in the lesser sac, suggestive of pancreatic hemorrhage, so he was transferred to the University of Kentucky Medical Center for further management (Figure ).\nInterventional radiology was consulted, and celiac angiogram showed an arterio-portal fistula in the liver which was embolized with 900 um particles Embozene (Palo Alto, CA: Varian Medical Systems, Inc.), as well as a few tiny rounded foci of contrast enhancement in the spleen which was managed conservatively without embolization (Figure ). Following the procedure, the patient developed an elevated white blood cell (WBC) count and increased abdominal distension. A repeat CT scan four days later showed an increase in the size of the lesser sac hematoma and a pseudoaneurysm in the anterior spleen (Figure ). Surgery was discussed with the patient, which he elected against and preferred to leave the hospital.\nHe returned to the hospital two weeks later with decreased appetite, melena, hematochezia, abdominal pain, and weakness. He was found to be anemic with a hemoglobin of 6.4 g/dL (down from 7.8 g/dL). CT scan and angiographic images revealed worsening diffuse multifocal parenchymal perfusion abnormalities and innumerable punctate foci of contrast pooling in the spleen, compatible with high-grade splenic injury and the Seurat spleen angiographic sign (Figures , ).\nCoil embolization of the splenic artery was performed with postembolization angiography showing no anterograde flow through the splenic artery, significantly decreased opacification of the previously noted intraparenchymal pseudoaneurysms, and opacification of the spleen via collaterals (Figure ). CT scan one month later revealed normal splenic parenchyma, with a persistent hematoma anterior and superior to the spleen (Figure ).\nThe patient continued to have a complicated hospital course and was found to have multiple myeloma confirmed with a bone marrow biopsy specimen. He, unfortunately, passed away just over a month later due to complications from multiple myeloma.","This 43-year-old man was admitted to the hospital after a car accident three weeks prior, experiencing abdominal pain and chest pain. Initial tests revealed a bleeding problem in his abdomen, and doctors performed a procedure to stop the bleeding. However, the bleeding continued, and he developed a serious splenic injury. After further treatment, the bleeding stopped, but he was diagnosed with multiple myeloma, a type of cancer, and sadly passed away a month later."
182,"A 15-year-old girl presented with chronic complaints of nasal obstruction and hyposmia on the left side. The patient had nasal obstruction symptoms for 6 months; before which she was asymptomatic. She was taking self-administered medications without relief. She had no history of maxillo-facial surgery or trauma in the past. She had no relevant family history or congenital anomalies. Upon clinical examination of the nose, there was a bump along the floor of the left nasal cavity, and her intraoral dentition appeared normal.\nA plain radiograph of the paranasal sinus and nasal cavity was performed which showed a radiopaque focus in the left nasal cavity (Figure ).\nThe CT scan of paranasal sinuses showed a tooth-like bony structure with a pulp cavity in the hard palate extending into the left inferior nasal cavity and a deviated nasal septum with convexity to the left (Figures -); a shape resembling a canine with a relatively smaller size.\nOrthopantomogram showed a tooth-like radiopaque structure (white arrow) (Figure ). A three-dimensional computed tomography (3D CT) scan showed a tooth-like structure in the left nasal cavity (Figure ).\nThis intranasal tooth was supernumerary. Mild mucosal thickening was noted in the bilateral maxillary and sphenoid sinuses. No tooth-like structures were found on the right side. All other teeth appeared normal. The patient had complete resolution of nasal obstruction and hyposmia following endoscopic removal of the ectopic intranasal tooth.","This 15-year-old girl had been experiencing a stuffy nose and difficulty smelling on one side for six months, but she hadn’t found any relief with over-the-counter medications. A doctor found a small, unusual tooth-like bone growing inside her nose, which was causing the blockage. CT scans confirmed the presence of this supernumerary tooth, and it was successfully removed with a simple procedure. After the tooth was removed, her nasal congestion and smell problems disappeared completely."
183,"A 56-year-old woman underwent laparoscopic bilateral salpingo-oophorectomy by the gynecological team. The patient has a history of total abdominal hysterectomy 20 years prior for endometriosis. Therefore, adhesions were obscuring the anatomy of the left ureter during her later surgery. In the early postoperative days, there was mild pain at the left iliac fossa and was managed with paracetamol and oral morphine. In the early period, there was no flank pain and no costovertebral angle tenderness. One week later, the patient presented to the emergency department with severe left iliac fossa pain associated with nausea and vomiting. Her investigations were within normal range, stable renal function, and normal inflammatory markers. Contrast CT showed extravasation of the contrast at the level of the left renal pelvis (Figure ) and ureteric dilatation down to the pelvic ureter (Figure ).\nConsidering the recent pelvic surgery, immediate diagnosis of left ureteric iatrogenic injury was concluded, and the patient was managed initially with urgent CT-guided nephrostomy insertion. The patient was in pain and an adequate nephrostogram was not performed during nephrostomy insertion. Therefore, three weeks posttraumatic injury, the patient underwent a proper antegrade nephrostogram. The contrast extravasated into the peritoneal cavity and there was no contrast passing into the distal left ureter (Figures , ). Therefore, a diagnosis of complete transection of ureteric injury was suspected. The nephrostomy was left in situ and the patient was planned for reconstructive surgery.\nEleven weeks posttraumatic injury, the patient was admitted for her planned surgery. Prior to the surgery, left retrograde study was performed and surprisingly the contrast went up to the left kidney with only annular stricture at the level of the injury (Figures , ). Therefore, a decision was taken for ureteroscopic dilatation of the stricture. The ureteroscopy showed a small annular stricture, less than 0.5 cm, that was passed easily over the safety guidewire and a ureteric stent, size 8 French, was inserted successfully.","This 56-year-old woman had surgery to remove her ovaries and fallopian tubes, following a previous hysterectomy. During a later surgery, adhesions caused difficulty in identifying the ureter, a tube that carries urine from the kidney to the bladder. A few weeks after the surgery, she developed severe pain in her lower left side, along with nausea and vomiting, and scans revealed a tear in the ureter. To drain the urine, a temporary tube was placed, but further tests showed the tear had completely closed. Finally, a procedure to widen the narrowed area of the ureter was successful, and a stent was placed to keep it open, allowing for a planned reconstructive surgery."
184,"A 76-year-old female with a past medical history of hypertension presented to the emergency department with persistent forehead swelling two months after recovering from COVID-19 infection. The patient was diagnosed with COVID-19 pneumonia in Egypt and completed a six-week course of dexamethasone. Per family, she also received multiple antibiotics during this time, the names and courses of which were unknown. She subsequently developed progressively tender frontal scalp swelling. Initial evaluation with otorhinolaryngology (ENT) in Egypt showed mucor-like organisms on pathology. She then came to the United States and was admitted for further evaluation. Her CT head showed evidence of multifocal sinusitis with breach of the inner and outer tables of the right frontal sinus with dural enhancement (Figure ). The CT scan also confirmed dehiscence of the inner table (Figure ). She was then empirically started on a course of Amphotericin for post-COVID mucormycosis. The patient underwent surgical debridement of significant underlying purulence with intraoperative cultures growing extended-spectrum beta-lactamase-producing Escherichia coli and methicillin-resistant Staphylococcus aureus. Due to these culture results, the patient was initiated on vancomycin and meropenem with minimal improvement in edema. She underwent repeat surgical debridement two weeks later with cultures redemonstrating previously identified bacteria with rare Pseudomonas aeruginosa growth as well. The patient endorsed moderate improvement in frontal scalp tenderness following this debridement. She was thus discharged home on a six-week course of IV vancomycin through a peripherally inserted central catheter, PO levofloxacin, and PO posaconazole, with a plan to continue the latter until fungal cultures were finalized. Two weeks later, she presented to the outpatient clinic with new-onset generalized weakness. Initial workup revealed profound hypokalemia (potassium of 1.5 mEq/L; normal range 3.4-5.3 mEq/L) and acute kidney injury (creatinine of 3.03 mg/dL from baseline of 1.20 mg/dL; normal range 0.65-1.00 mg/dL) likely due to vancomycin-related renal derangements and potassium wasting due to posaconazole. The patient was admitted for fluid resuscitation and switched from vancomycin to daptomycin upon discharge to prevent further renal impairment. As all her fungal cultures were negative and no fungal forms were seen on pathology, posaconazole was discontinued. The patient was able to complete her course of treatment, though it was complicated by mild creatine kinase elevation due to daptomycin.\nRepeat MRI at the end of her antibiotic course demonstrated decreased soft tissue thickening along the anterior skull base and superior nasal cavity with improvement in mucosal thickening involving maxillary sinuses, sphenoid sinuses, and residual ethmoid air cells (Figure ). However, two weeks later, the patient complained of persistent frontal tenderness and was noted to have new periorbital swelling. Due to frontal sinus sequestrum formation and worsening of symptoms soon after antibiotics were stopped, the patient was recommended to undergo craniectomy to achieve definitive source control. She also had to be restarted on daptomycin and meropenem while awaiting surgery.","68-year-old woman with a history of COVID-19 pneumonia developed a painful swelling on her forehead after recovering from the infection. Tests revealed a serious sinus infection with fungal growth, leading to multiple surgeries and antibiotics. Unfortunately, the antibiotics caused kidney problems and low potassium levels, requiring a change in medication. After completing treatment, she experienced new weakness and swelling, and further tests showed a sinus problem that needed surgery to fix."
185,"A 29-year-old male was brought to the emergency department following a car accident. It was reported that the wheel of a 5-ton water tanker rolled over his lower body as he was stepping into a car. Workup revealed a Glasgow Coma Scale (GCS) score of 15 with a pulse rate of 80 beats per minute and blood pressure of 90/60 mmHg. There was profuse bleeding due to a perineum injury. No fractures of the skull, cervical spine, and pelvis were detected in the X-rays. Urinary catheterization was performed without difficulty and there was no hematuria. No internal bleeding was seen during the abdominal ultrasound. The patient was immediately taken to the operating theatre for injury assessment after the replacement of fluid and blood.\nOperative Findings and Procedure\nWhile the patient was placed in the lithotomy position, the anterior displacement of the anus could be seen with a circular skin defect surrounding the region. The anus looked normal. There was a remaining 1 cm of normal perianal skin with no actual skin loss. Digital and proctoscopy examination showed an intact anorectum and its surrounding sphincters. Whereas exploring the wound revealed complete disruption of the posterior pelvic floor with lateral extensions into both gluteal regions and the right thigh. In addition, a hand could be passed behind the rectum and into the sacral promontory.\nThe case was associated with extensive venous bleeding with the absence of major arterial tears. It was managed by the insertion of multiple packs along with partial closure of the skin defect. A small incision was made to perform a defunctioning sigmoid colostomy. No intra-abdominal bleeding was reported. The packs were removed two days later without any bleeding complications. A total of 22 units of blood were required during the first 48 hours of admission. Figures , show the site of injury before and after treatment, respectively.\nProgress\nContinuous follow-up examinations were done during the following months of the accident. Seven examinations under anesthesia were performed. The skin was not closed completely at the site of the procedure. Consequently, it was decided not to reallocate the anorectum to a further posterior direction and the wound was left open. Initially, infection of deep injury recess was found and debridement and daily cleaning with betadine was performed. In later stages, as the wound became clean, saline dressings were substituted and the wound healed by granulation.\nThe defect was reduced to an area of 4 x 2 cm after four months of the procedure and was contracted towards the coccyx. Anterior to this, the anus felt normal on digital examination with a good sphincter tone and squeeze.\nPhysiological tests were done to test the sphincter function and sensation. Manometry showed the resting sphincter pressure of around 30 cmH2O. This increase in squeeze pressure was due to a combination of contraction of the puborectalis and gluteal muscles. The presence of rectal sensation was noted but not quantified. Defecography was assessed using a defecation proctogram. It revealed a normal anorectum that was displaced towards the pubic bone. Furthermore, although he was able to evacuate barium paste from the rectum, the anorectal angle did not widen during straining at the angle of 90°. Anyhow, there was no abnormal descent of the rectum on straining.\nFive months after the surgical procedure, the perineal wound healed, and the colostomy was closed. The patient was able to pursue normal defecation without any episodes of major or minor incontinence. In the following follow-up visit, the patient expressed satisfactory defecation and micturition without incontinence.","A 29-year-old man was brought to the hospital after a serious car accident where a water tanker wheel rolled over his body. Doctors found significant damage to his pelvic floor and performed surgery to repair the injury, including creating a temporary colostomy. The surgery involved removing damaged tissue and closing the wound, which gradually healed over several months. After months of follow-up and physical therapy, the wound closed completely, and he was able to return to normal bowel and bladder function."
186,"A 44-year-old female patient had a car accident and she was thrown out from the car landing on a hard rock on her buttocks. In the district hospital, she was found to have a partial laceration of the posterior perineum surrounding an intact anorectum. The bladder, urethra, and pelvis were not injured. A sigmoid colostomy was performed and then she was transferred to a tertiary hospital.\nShe had a crescent-shaped wound surrounding the anus from the three to nine o’clock position. In addition, the anus was displaced forward towards the vagina. Digital and proctoscopy examinations showed an intact anorectum and surrounding sphincters.\nProgress\nIn this case, the patient's wound was subject to identical conservative management procedures as in the previously mentioned case. However, the patient was discharged from the hospital earlier as it was determined that she had adequate support from a daughter who has adequate experience in nursing. As the patient's wound was clean and in its proliferation stage of healing, the patient was instructed to perform daily irrigation using handheld bidets. She was scheduled for follow-up appointments every three weeks, which she attended punctually. Consequently, the proper management of the wound caused it to be superficial and reduced to 1 x 2 cm after five months of the procedure, with no infections.\nThe defecation portogram performed in a follow-up appointment revealed an anteriorly displaced anus with an anorectal angle of 70°; this angle only widened to 90° on straining, and while evacuation occurred, it was incomplete. However, no atypical rectum descent was noticed, and the patient reported complete evacuation in the toilet after the examination.\nSeven months post-injury, the colostomy was closed, and five days after stoma closure, the patient reported normal bowel movement with no incontinence experienced and was subsequently discharged.\nFifteen months post-injury, a follow-up report noted complete healing of the wound with adequate defecation. However, the patient reported the occasional need to use glycerin suppositories. Moreover, the patient's regime of treatment resulted in there being no fecal impaction. Figures , show the site of injury before and after treatment, respectively.","This woman was injured in a car accident, sustaining a cut to her buttock and an abnormal displacement of her anus. To help her heal, doctors performed a temporary colostomy and provided careful wound care, including daily irrigation. After five months, the wound healed completely, and the colostomy was closed, allowing her to return to normal bowel function. Although she occasionally needed glycerin suppositories, she was able to manage her bowel movements effectively and was discharged with no further complications."
187,"A 25-year-old woman was admitted due to complaining of difficulty in swallowing. She was the youngest of the five siblings, also a non-smoker and non-drinker. She was a child of a consanguineous marriage as her father and mother are cousins. Furthermore, when she was five years old, she was diagnosed with Fanconi anemia. To confirm the diagnosis of FA, we communicated with the related university hospital for the records of the patients 20 years ago. They approved the diagnosis of FA with some peripheric blood sample studies without giving details. They had offered bone marrow transplantation, which the parents had not approved of. She had no major symptoms for 20 years, and the disease was under control. But the patient did not go to regular hospital check-ups. The patient's first notable characteristic was growth retardation manifested by short stature, microcephaly, and microphthalmia. Afterward, she had swallowing difficulty for the last two years and had lost around 10 kg in the last six months.\nThe patient was referred to the gastroenterology department due to swallowing problems. In the endoscopic examination, stenosis was observed in the hypopharynx that restricts the passage of the scope follows through. Positron emission tomography (PET-CT) scan showed multiple lymphadenopathies in the bilateral deep cervical lymph nodes (standardized uptake value [SUV] max: 8.8), and prominent pathological 18F-Florodeoksiglukoz (FDG) involvement beginning from the right-side oropharynx to the proximal esophagus (SUV max: 8.5) (Figure ). The patient underwent endoscopy, tumoral formation in the hypopharynx leading to only 3 mm passage opening was detected. As a result of punch biopsy and pathological examination, squamous cell carcinoma of the hypopharynx was revealed.\nThe patient was diagnosed with T3N2cM0 hypopharyngeal cancer, and due to locally advanced disease, surgery was not considered; finally, she opted for curative radiotherapy (RT) with concomitant cetuximab chemotherapy. The RT of the patient was planned on a helical Tomotherapy system (Figure ), including primary tumour and bilateral neck lymph nodes. At the beginning of RT, the complete blood count was near-normal, with only mild leukopenia (white blood count is 3,8x10^3cells/uL). Cetuximab loading dose could not be administered due to urgent treatment needs. Consequently, the first cetuximab dose 350 mg/m2 with RT was initiated. The remaining maintenance dosage was scheduled as 250 mg/m2 but could not be administered due to a sudden drop in blood count (white blood count to 0.48x10³cells/uL) after four days of initial cetuximab and 10 days of RT. Her treatment was terminated due to progressive cytopenia (Table ), after receiving 21.2 Gy in 10 days for primary lesion. A peripheral blood smear was investigated, and it was concordant with severe pancytopenia as no atypical cells with rare leukocytes and platelets. To secure hematological recovery, G-CSF was initiated, erythrocyte and platelet transfusion was administered when necessary. Despite the daily G-CSF utilization, there was no significant change in blood counts. Moreover, the patient's dysphagia was worsened and a nasogastric tube was placed for feeding, in addition to parenteral nutritional support. Despite receiving nasal oxygen, the patient's condition worsened, on the 30th day of the start of radiotherapy, and her oxygen saturation regressed, and she was intubated. The patient died 40 days after radiotherapy begin due to respiratory failure and sepsis.","This 25-year-old woman was admitted because she was having trouble swallowing and had lost a significant amount of weight. She has a history of Fanconi anemia, a rare genetic condition, and a family history of consanguinity. Tests revealed a cancerous tumor in her throat that had spread to nearby lymph nodes, and she ultimately passed away after receiving radiation treatment. Despite the treatment, her condition worsened, leading to respiratory failure and sepsis."
188,"A four-year-old, previously healthy boy weighing 24 Kg, from Dhaka, was admitted with a four-day history of a high continued fever. He also complained of suffering abdominal pain, loose motions, and emesis for two days. He had no history of flu-like symptoms, cough, or respiratory distress in the past month. There was a history of close contact with a COVID-19 patient (within one month of the illness). He had no history of dengue. On examination, he was found febrile (temperature 102°F), tachypneic, and tachycardic with unrecordable blood pressure. Auscultation of lungs revealed bilateral crepitations with good air entry. The abdomen was distended, flanks were full with mild, diffuse abdominal tenderness. Initial investigations showed dengue NS1 Ag positive and rt-PCR for SARS-CoV-2 negative, thrombocytopenia, positive C-reactive protein, altered coagulation profiles (Table ).\nThe chest X-ray (CXR) initially revealed bilateral pulmonary infiltrations. He was treated for dengue shock syndrome with plasma leakage with intravenous (IV) ceftriaxone, inotropes, and colloids. On the 5th and 6th day of fever, he developed petechial rashes on both extremities, cheilosis, and an erythematous rash over the trunk (Figure ).\nThe patient also developed a cough and respiratory distress, oxygen saturation (SpO2) by pulse oximeter was found to be 96% with 5L/min oxygen through a face mask. Repeat CXR revealed bilateral inflammatory lesions with pleural effusion. The ECG was normal. However, echocardiography revealed dilated coronary arteries, left main coronary artery (LMCA, +3.0 standard deviation, SD), left coronary artery (LCA, +2.5 SD) with the loss of distal tapering and mild left ventricular (LV) dysfunction (ejection fraction [EF] 52%) (Figure ).\nInitial serum ferritin, N-terminal-pro hormone B-type natriuretic peptide (NT-pro-BNP), D-dimer, serum troponin I were markedly raised (as shown in Table ). The anti-SARS-CoV-2 antibody (IgG) test came back positive on the seventh day of fever. A pediatric cardiologist was consulted, and we administered one dose of intravenous immunoglobulin (IVIG) 2 g/Kg, along with enoxaparin 2 mg/Kg for five days. Thereafter, oral prednisolone and aspirin were added. Meanwhile, the patient became afebrile, and the rash faded. His general condition improved with a gradual return of appetite. Three days after the IVIG, repeat tests showed decreasing serum ferritin, NT-pro-BNP, and serum troponin I. The patient was transferred to the ward after 12 days in the pediatric intensive care unit (PICU) thereafter discharged after five days with the necessary advice and follow-up appointment.","This four-year-old boy from Dhaka was admitted with a high fever, stomach problems, and a cough. Initial tests showed he had dengue fever and was also infected with COVID-19, but the COVID-19 test was negative. He also developed some rashes and breathing difficulties, and an X-ray of his lungs showed inflammation. Further tests revealed problems with his heart, and he received treatment for these issues, including IVIG and steroids. After several days, he improved and was discharged home with follow-up care."
189,"A previously healthy 12-year-old girl from Dhaka, weighing 55 Kg, was admitted with a four-day history of high intermittent fever, headache, arthralgia, and generalized body aches. She had an erythematous rash on her trunk, cough, and respiratory distress. She was initially admitted to another hospital, but her condition deteriorated, and she developed shock and was referred to our PICU. She had complained of flu-like symptoms within two weeks of the presenting illness. She had a history of one episode of dengue fever three years earlier but no contact history with a COVID-19 patient.\nOn admission, the patient was conscious, febrile (temperature 104°F), tachypneic, tachycardic with unrecordable blood pressure, and prolonged capillary refill time. SpO2 by pulse oximeter was 93% in room air and 96% with 2L/min oxygen via nasal cannula. Auscultation of lungs revealed bilateral diminished air entry with coarse crepitations, pleural rub. The abdomen was distended, full flanks with diffuse mild tenderness. She tested dengue NS1 Ag positive on the second day of fever. Management for dengue shock syndrome with plasma leakage was initiated with inotropes, IV furosemide, and albumin along with IV ceftriaxone and amikacin. She was given oxygen 3L/min via a face mask. The initial investigations showed thrombocytopenia, mild hypoalbuminemia, hypocalcemia, positive C-reactive protein, altered liver function tests with coagulopathy (as given in Table ). Her chest X-ray showed bilateral pleural effusions with pneumonitis (Figure ).\nA bedside echocardiogram revealed a prominent LMCA (+2.54 SD), minimal pericardial effusion, mild pleural effusion, and good biventricular function (EF 77%). Her NT-pro-BNP, serum ferritin, D-dimer were significantly high but serum troponin I was normal. At this time, the patient had mucocutaneous involvement with respiratory symptoms. Her anti-SARS-CoV-2 IgG came back positive. After reviewing the laboratory reports and echocardiographic features, we consulted the pediatric cardiologist. She was treated with one dose of IVIG 2g/Kg and started on oral prednisolone and aspirin. The patient was transferred to the main ward when she began to show clinical and laboratory improvements. She was soon discharged with the necessary advice and follow-up arrangements.","A 12-year-old girl from Dhaka was admitted to the hospital with a high fever, headache, and body aches, along with a rash and difficulty breathing. She initially felt like she had the flu, and tests confirmed she had dengue fever, which led to a dangerous drop in blood pressure and shock. Doctors treated her for dengue shock syndrome with medications and oxygen, and found fluid was leaking from her lungs. After receiving IVIG and other treatments, she started to improve and was eventually discharged home with follow-up care. She also tested positive for COVID-19, which likely contributed to her symptoms."
190,"A three-year-old girl from Dhaka, previously healthy and thriving, weighing 16 Kg, was admitted to PICU with the complaint of five days of high continued fever with diffuse, central abdominal pain, emesis, and diarrhea for the last two days. She had experienced two episodes of melena and hematemesis. She had a history of contact with a COVID-19 positive patient in the last month but had no symptoms before the present illness. There was no previous history of dengue. She had been treated in another hospital for shock, and her dengue NS1 Ag was positive on the second day of fever. On admission, the patient was febrile, with narrow pulse pressure. Her blood pressure (BP) was 60/45 mmHg with tachycardia, she had tachypnea with a SpO2 of 90% by pulse oximeter in room air, a low volume pulse, and cold extremities. Auscultation of lungs revealed diminished breath sound with crepitations bilaterally. The abdomen was distended and tender. She was diagnosed with dengue shock syndrome with plasma leakage. She was treated with inotropes, IV furosemide, colloids, and levofloxacin. She was given 5L/min oxygen via a face mask. Her initial investigations revealed thrombocytopenia, hypoalbuminemia, hypocalcemia, positive C-reactive protein, mildly raised serum procalcitonin, altered liver function, and coagulopathy (as shown in Table ). Her CXR revealed bilateral pleural effusions with pneumonitis. Echocardiography showed prominent dilated coronaries, LMCA (+2.5 SD), LAD (+2.0 SD) with loss of distal tapering and perivascular brightness, bilateral pleural effusions, a mildly dilated left ventricle with mild LV dysfunction (EF 57%) (Figure ).\nCardiac enzymes showed raised serum troponin I, NT-pro-BNP with raised serum ferritin and marked increased D-dimer level. The rt-PCR for SARS-CoV-2 Ag came back negative; however, the anti-SARS-CoV-2 antibody (IgG) was sent and came positive. By the sixth day of fever, the patient had conjunctival congestion and an erythematous rash on her trunk, which gradually faded (Figure ).\nAfter consultation with cardiologists, one dose of IVIG 2g/Kg was given; thereafter, oral prednisolone and aspirin were added. Three days after the IVIG, repeat tests showed decreased serum troponin I and NT-pro-BNP. As her general condition had improved, the patient was transferred to the pediatric ward after seven days of PICU support. Thereafter she was discharged with advice and a follow-up appointment.","A three-year-old girl from Dhaka was admitted to the hospital with a high fever, abdominal pain, and vomiting. She had also experienced bleeding in her stomach and was found to have a positive dengue test, leading to a diagnosis of dengue shock syndrome. The doctors treated her with medications and oxygen to support her breathing and blood pressure. During her stay, tests revealed other problems, including low platelet count and fluid leaking from her lungs. She also tested positive for antibodies against COVID-19, but the virus itself was not detected. As her condition improved, she was transferred to a regular pediatric ward and eventually discharged home."
191,"A previously healthy nine-year-old boy, weighing 37 Kg, and hailing from Dhaka, was admitted to PICU with a high, continued fever that had lasted four days. He suffered with a headache and generalized body aches for two days and diffuse abdominal cramps with diarrhea and emesis for one day. He had a history of an upper respiratory tract infection three weeks earlier but had not done an rt-PCR for SARS-CoV-2 Ag during that time. He had no obvious contact history with a COVID-19 patient and no history of dengue infection. He tested positive for dengue NS1 Ag on the third day of fever and was admitted to another hospital. After deteriorating, the patient was referred to our PICU. On admission, he had unrecordable BP, tachycardia, tachypnea, cold extremities, breath sound was absent on right side, diminished on the left chest wall with coarse crepitations, SpO2 89% in room air by a pulse oximeter. The abdomen was distended, full flanks with diffuse abdominal tenderness. He was resuscitated, and management for dengue shock syndrome with plasma leakage was started. He was given 5L/min oxygen via a face mask, IV inotropes, albumin, colloids, furosemide, imipenem, and amikacin. His blood test results included anemia, thrombocytopenia, hypoalbuminemia, marked hypocalcemia, positive C-reactive protein, raised blood urea level, mild alteration of liver functions with coagulopathy (as given in Table ). A stool occult blood test was positive. His CXR revealed pleural effusions and pneumonitis. His ECG showed sinus tachycardia. The echocardiogram performed on the second day of admission revealed minimal pericardial effusion, bilateral pleural effusions, mild LV dysfunction (EF 59%). In addition, he had high serum ferritin, D-dimer along with a slightly raised NT-pro-BNP, normal serum troponin I. Clinically, he developed an erythematous rash on his trunk with conjunctival congestion on the seventh day of fever, which warranted further anti-SARS-CoV-2 antibody testing. This came back positive on the eighth day of fever. After consultation with pediatric cardiologists, the patient was started on IV methylprednisolone at a dose of 30 mg/Kg for three days. On the ninth day of illness, the patient developed respiratory distress and became confused and aggressive. His blood pressure was still fluctuating. His SpO2 was about 88% with 7L/min oxygen (2L/min via nasal cannula, 5L/min through face mask). He was placed on mechanical ventilation. Repeat CXR showed persistent pulmonary infiltrations with pleural effusion (Figure ).\nA brain MRI revealed no obvious neurological abnormalities. Repeat echocardiography revealed dilated coronaries, LMCA (+2.6 SD), LAD (+2.5 SD), with loss of distal tapering, bilateral pleural effusion, mild LV dysfunction (EF 51%) (Figure ).\nAfter consulting with a pediatric neurologist and cardiologist, one dose of 2 g/Kg IVIG was given. Methylprednisolone and aspirin were added, and we closely monitored his platelet count. From the second day on ventilatory support, he began to improve and was gradually weaned from the ventilator. After 10 days of illness, his neurological complaints started resolving, repeat blood tests results were improved with gradual remission of pleural effusion. The patient was transferred to the pediatric ward after 12 days of PICU admission. He was discharged on request after five days with the necessary advice and follow-ups.","This nine-year-old boy from Dhaka was admitted to the intensive care unit (PICU) with a high fever, headache, and stomach problems. He tested positive for dengue fever and was initially treated at another hospital, but his condition worsened, requiring our care. During his stay, he developed breathing difficulties, fluid buildup in his lungs, and other complications, including a rash and heart problems. After receiving supportive care, including medications and oxygen, he gradually improved and was eventually discharged home after 12 days."
192,"A nine-year-old, previously healthy, developmentally well male child, weighing 31 Kg, and hailing from Dhaka, was admitted to the PICU. He complained of high, irregular fever over the previous four days and generalized body aches, headache, abdominal cramps with emesis for the previous two days. He had a history of dengue infection two years ago and positive contact history with a COVID-19 patient one month before the illness. He tested positive for dengue NS1 Ag on the second day of fever and was admitted to another hospital. Due to respiratory distress and fluctuating blood pressure, he was referred to our PICU. On admission, the patient had low mean pressure, tachypnea, tachycardia with a cold periphery. His breath sound was diminished bilaterally and course crepitations were heard on auscultation. He was febrile with erythematous, petechial rashes on both lower limbs, and gum bleeding. His rt-PCR for SARS-CoV-2 was negative, and blood and urine cultures yielded no growth. Initial investigations revealed, thrombocytopenia, marked hypoalbuminemia, hypocalcemia, altered liver functions, coagulopathy, positive C-reactive protein, negative procalcitonin, and slightly increased blood urea. His serum ferritin, D-dimer, serum troponin I, and NT-pro-BNP were very high (as given in Table ). The CXR revealed bilateral pleural effusions with pneumonitis (Figure ).\nThe ECG showed sinus tachycardia. Management of dengue shock syndrome with plasma leakage was started with IV inotropes, albumin, calcium gluconate, antibiotics (imipenem, levofloxacin), and furosemide. An echocardiogram was done and showed a mild pericardial effusion, prominent dilated coronaries, LMCA (+3.0 SD), LAD (+2.5 SD), bilateral pleural effusion, fair LV function. The test for SARS-CoV-2 antibody (IgG) came back positive. After evaluating his condition and consultation with pediatric cardiologists, a single 2g/Kg dose of IVIG was administered, followed by methylprednisolone, aspirin, and clopidogrel. His general condition gradually improved with improving laboratory reports. Serial CXRs showed gradually decreasing pleural effusions. He was transferred to the pediatric ward 10 days after PICU admission and was discharged with the necessary advice and follow-up plans.","A nine-year-old boy from Dhaka was admitted to the hospital with a high fever, body aches, and stomach problems. He had a previous dengue infection and had been exposed to COVID-Tests showed he had dengue fever and some other concerning issues, including low blood counts and fluid leaking from his lungs. After receiving treatment with medications and fluids, his condition improved, and he was eventually discharged home with follow-up care."
193,"A 67-year-old man with chief complaints of macrohematuria and an abnormally low hemoglobin level (4.8 g/dL; normal range, 13.7-16.8 g/dL) was referred to our hospital. His performance status was zero and none of any co-morbidities were identified. Whole-body computed tomography (CT) revealed left hydronephrosis, a bladder tumor on the right lateral wall, and right external iliac LN involvement (Figure ). CT revealed a bladder tumor with invasion of surrounding fibroadipose tissue on the right lateral wall (Figure ). Transurethral resection of the bladder tumor was performed after blood transfusion; histopathological diagnosis revealed high-grade UC of the bladder with muscle layer invasion. BCa was classified as clinical T3bN1M0 according to the staging system defined in the American Joint Committee on Cancer Staging Manual []. His estimated glomerular filtration rate (eGFR) was 64.57 mL/min and his renal function was maintained at a normal eGFR level during the medication for BCa. He received two combined courses of gemcitabine and cisplatin (GC; 1,000 mg/m2 gemcitabine on days 1, 8, and 15, and 70 mg/m2 cisplatin on day 2) every 21 days. To monitor the treatment effect on BCa, the patient underwent whole-body CT and pelvic MRI after every two courses of systemic therapy.\nAfter two courses with GC, CT revealed left external iliac LN involvement as a new lesion, although the BCa and right external iliac LN decreased in size. Disease progression was diagnosed according to the Response Evaluation Criteria in Solid Tumors guidelines, version 1.1 [] (Figure ). It was difficult to explain why the left external LN has enlarged even though other lesions showed a positive effect after GCarbo. One possibility could be that the UC being a heterogeneous tumor, may have unique properties in this case. As a second-line treatment, pembrolizumab (200 mg) was administered every three weeks. Grade 4 macrohematuria according to the Clavien-Dindo classification [] occurred two weeks after the administration of pembrolizumab; thus, he immediately underwent endoscopic hemostasis for the bleeding site after blood transfusion. The break-through bleeding occurred from the surface of the BCa. Thereafter, the patient received two more courses of pembrolizumab. CT findings after the administration of pembrolizumab revealed shrinking of the BCa, positive LNs, and right kidney (Figure -). Three weeks after the final dose of pembrolizumab, open RC with bilateral pelvic lymphadenectomy and bilateral ureterocutaneostomy were performed because of the possibility of palliative surgical treatment. There was firm adhesion between the right lateral wall of the urinary bladder and the pelvic floor. The adhesion around the bilateral pelvic lymph node was not identified. There have been no surgery-related complications. The pathological examination of the surgical specimen by two pathologists at our institution indicated UC, with a pathological stage of Tis without lymphovascular invasion, a negative surgical margin, and negative LN involvement (Figure -). The patient was followed up for 30 months without evidence of local recurrence or distant metastasis.","67-year-old man was referred to the hospital because of blood in his urine and low blood counts. Tests showed a tumor in his bladder and spread to nearby lymph nodes. The tumor was removed, and he received chemotherapy, but the cancer spread to more lymph nodes. Eventually, he underwent surgery to remove the bladder and surrounding tissues, and he is now being followed closely to ensure the cancer doesn't return."
194,"A 46-year-old African male with a past medical history of essential HTN presented to the emergency department with a five days history of cough, shortness of breath, diarrhea, muscle cramping, fatigue, poor oral intake and decreased urinary output. The patient was tested positive for COVID-19 one day prior to admission. On further examination, the patient was febrile, hemodynamically stable with a blood pressure of 125/57 mmHg with a mean arterial pressure of 77 mmHg, heart rate of 83 bpm, respiratory rate of 24 per minute, and oxygen saturation was 93%. The patient was adequately oxygenated on a 2 L nasal cannula. Initial lab report revealed 133 meq/L of Na, chloride 88 meq/L, potassium 6.3 meq/L, calcium 8 meq/L, creatinine 23 mg/dL, BUN 195 mg/dL, creatinine kinase 1,200 U/L, lactate dehydrogenase (LDH) 212 U/L, C-reactive protein 126.6, and elevated D-dimer 4,433 (Table ). Arterial blood gas showed bicarbonate 6 meq/L, CO2 6 mmol/L, anion gap 41 mmol/L, PH 7.17. His liver function panel was normal. Urinary analysis showed protein >600 mg/dL, blood 1+, creatinine 404.6 mg/dL, and urine protein electrophoresis was 1,735 mg.\nEKG findings were non-significant for hyperkalemia, Chest x-ray was negative for the acute process of viral infection (Figure ). Since the patient was dehydrated he was started on 2 L of normal saline followed by 1 g of calcium gluconate. Repeat potassium was 7.7 meq/L. Therefore, the patient was admitted to the COVID ICU followed by a nephrology consultation. Since remdisivir is not a good drug of choice in renal dysfunction, 6 mg of decadron was started and sodium bicarbonate drip was started at 150 mL/hr. Additional investigation including color Doppler ultrasound of lower extremities was done due to high D-dimer and was negative for deep vein thrombosis. Ultrasound of kidney and urinary bladder was also negative for hydronephrosis. Despite the lack of improvement in supportive treatment, the patient was scheduled for hemodialysis. Subsequently his renal function and electrolytes status improved. Creatinine decreased to 3.1 mg/dL, creatinine kinase 895 IU/L, and the anion gap trended to 15. On discharge, potassium was 4.4 meq/L, creatinine was 2.9 mg/dL and he was sent home on room air with four more days of decadron and pulmonary rehabilitation exercise was done to enhance the recovery from COVID-19 pneumonia.","This 46-year-old man was admitted to the hospital after experiencing a cough, shortness of breath, diarrhea, and fatigue, along with a positive COVID-19 test. He was dehydrated and had dangerously high levels of potassium, which were treated with fluids and calcium. After receiving treatment, his kidney function improved, and he was discharged home on medication and a plan for pulmonary rehabilitation."
195,"An 83-year-old female with a history of cold agglutinin hemolytic anemia requiring transfusions, chronic anemia with hemoglobin levels between 7-8 g/dl, hypothyroidism, hypertension, deep vein thrombosis in bilateral lower extremities, and chronic lymphedema, presented with shortness of breath, cough, weakness, lightheadedness, acrocyanosis or darkening of the fingers and toes (Figure ), jaundice, and darkening of her urine. She was found to have a productive cough with yellow sputum and worsening shortness of breath for the last four days, along with progressively worsening orthopnea that required sleeping upright. However, the patient denied chest pain, worsening leg swelling, paroxysmal nocturnal dyspnea, and wheezing.\nOn presentation, the patient was afebrile, normotensive, with a normal heart rate, but had severe hypoxia with a saturation of 88% on room air and 95% with 2 liters of oxygen administered via nasal cannula. Throughout hospitalization, her temperature ranged between 96.7 and 98.6 °F (36-37 ℃). Physical examination was remarkable for mucosal pallor, acrocyanosis, icterus of the facial skin, diffuse bilateral rhonchi on lung auscultation, and pitting edema in bilateral lower extremities below the knee. Chest X-ray showed an enlarged cardiac silhouette, perihilar vascular fullness, and bilateral interstitial prominence likely indicative of pulmonary vascular congestion. A cardiac echocardiogram found the ejection fraction to be 60-65% with mild left ventricular wall thickness and grade I diastolic dysfunction. However, B-type natriuretic peptide (BNP) levels were only mildly elevated at 571 pg/mL. Moreover, nasal swab testing for SARS-CoV-2 was positive but was negative for influenza A, influenza B, and respiratory syncytial virus.\nComplete blood count revealed a high white blood count of 26.8 thousand/uL (normal range: 4.0-10.8 thousand/uL), low hemoglobin of 5.8 g/dl (12.0-16.0 g/dL), low haptoglobin of 1 mg/dL (41-165 mg/dL), and high erythrocyte sedimentation rate of 110 mm/hr (0-29 mm/hr). Serum chemistry testing found hyperkalemia of 6.7 mEq/L (3.5-5.1 mEq/L), elevated blood urea nitrogen of 34 mg/dl (8-25 mg/dl) with normal creatinine of 0.6 mg/dl (0.6-1.3 mg/dl), and severely elevated total bilirubin of 10.8 mg/dL (0.1-1.4 mg/dL). Blood culture collected from two different sites revealed no growth. Direct Coombs test was positive, and direct Coombs C3D test was also found to be positive during this admission. Testing from prior admissions had determined a cold agglutinin titer of 1:512. Other relevant testing included negative findings on hepatitis B virus IgM core antibodies, hepatitis B virus surface antigen, and hepatitis C virus antibody.\nTo treat the symptomatic anemia, the patient was transfused with two units of crossmatch least incompatible warmed blood on day one and one unit of the same on day two of admission. The SARS-CoV-2 pneumonia was treated with oral remdesivir for five days and intravenous dexamethasone 6 mg for seven days. The patient was also given intravenous 40 mg furosemide for pulmonary congestion, and empiric ceftriaxone and azithromycin for pneumonia for three days. Post-transfusions, the patient’s day-three hemoglobin was 10.2 g/dL, which dropped in the next few days but stabilized at her baseline of 7-8 g/dL by discharge on day 10. By day three, the patient’s hyperkalemia had improved without intervention; the furosemide was discontinued since the patient was euvolemic on examination, and the azithromycin and ceftriaxone were stopped due to low concern for concomitant bacterial pneumonia. The patient remained in stable condition in the designated isolation room through the rest of her hospital stay, and after 10 days since her initial positive coronavirus 2019 (COVID-19) test, the isolation protocol was lifted. She reported feeling like she was “regaining her strength”. Yet, her oxygen levels persisted to desaturate with exertion and an ambulatory pulse oximetry examination revealed that she required 3 L of oxygen on discharge. The patient was recommended to be discharged to a sub-acute rehabilitation center.\nTwo days post-discharge, the patient had another drop in her hemoglobin to 6.3 g/dL and required admission to a cancer institute where she was hospitalized for eight days, requiring five units of packed red blood cells and 60 mg of methylprednisolone IV daily. She had no signs and symptoms of bleeding during her admission. Her hemoglobin level improved to 9.2 g/dl and remained stable prior to discharge. On evaluation by an oncologist, weekly treatment with rituximab was recommended post-discharge for four weeks. On discharge, she was also prescribed prednisone 40 mg for seven days followed by a taper to 20 mg for another seven days and a follow-up appointment with oncology.","This 83-year-old woman was admitted to the hospital because she was struggling to breathe, had a fever, and was feeling weak. She has a history of several health problems, including anemia, heart issues, and a previous blood clot. Tests showed she had a lung infection related to COVID-19, along with a severe drop in her red blood cell count, which led to her needing multiple blood transfusions. She was treated with medications and oxygen to help her breathe and stabilize her condition. After a period of treatment and recovery, she was discharged to a rehabilitation center to continue regaining her strength and will continue to receive treatment from an oncologist."
196,"A 32-year-old male patient with no significant past medical history other than COVID-19 infection, months prior to admission, presented to ED complaining of dysphagia. Symptoms began about 1 week prior to presentation with difficulty swallowing liquids that progressed to involve solids as well. Dysphagia was described by the patient as a choking sensation and that he feels the food getting stuck in his chest. Dysphagia is partially relieved with belching. The patient also reported pyrosis and occasional vomiting. Denied nausea, abdominal pain or any change in bowel habits. On review of systems, the patient endorsed subacute cough for 3-4 weeks duration. The cough was mainly nonproductive and has been worsening since onset. It was associated with shortness of breath. Shortness of breath occurred mainly with exertion and while talking. The patient denied hemoptysis, fevers, chills, night sweats, weight loss, myalgia and arthralgia.\nOn presentation, the patient was afebrile, HR: 94, RR: 19, O2sat: 100% RA and BP: 129/81. Examination revealed mild wheezes over lung bases bilaterally and no palpable lymphadenopathy. The remainder of the physical examination was unremarkable. Complete blood count and comprehensive metabolic panel were within normal range. Angiotensin-converting enzyme level was elevated at 81 U/L (Normal range 9 - 67 U/L). HIV Ag/Ab screening test was negative as well as COVID-19 PCR test.\nCT-chest with contrast (Figure ) showed prominent mediastinal and bilateral hilar adenopathy, multiple pulmonary nodules, mild interlobular septal thickening, suggesting interstitial pulmonary edema and peripheral left lower lobe ground-glass opacities, which could be pulmonary edema or infection. Esophagram (Figure ) showed findings compatible with extrinsic mass effect involving the middle esophagus, in keeping with bulky mediastinal lymphadenopathy noted on CT chest.\nOn the third day of admission, the patient underwent Endobronchial Ultrasound Bronchoscopy (EBUS) with transbronchial needle aspiration. Samples were sent for cytology, pathology and flow cytometry. Flow cytometry was negative for monoclonal B-cells and immuno-phenotypically abnormal T-cells. The pathology report showed abundant non-necrotizing granulomas, mature lymphocytes and endobronchial cells in all biopsied samples. Grocott methenamine silver (GMS) and AFB stains were negative for microorganisms. Fungus and AFB cultures were negative as well.\nThe patient was tolerating PO intake and was discharged from the hospital. The patient followed up with the pulmonology clinic one week after discharge for biopsy results and was started on oral prednisone 40 mg qDay. The patient respiratory symptoms did improve after three months of steroid therapy. Nonetheless, dysphagia persisted. Due to the persistence of dysphagia and as evidence of esophageal compression does not rule out infiltrative involvement. The patient underwent EGD with dilation followed by manometry studies a few months after discharge. The mucosa was grossly and histologically normal. Manometry studies were also unremarkable. The patient reported partial improvement of dysphagia after dilation. The patient is still following up with pulmonology and gastroenterology clinics for further evaluation and management.","A 32-year-old man came to the hospital because he was having trouble swallowing, which made him feel like he was choking. He also had a persistent cough and shortness of breath. Tests showed swelling in his chest and a possible mass pressing on his esophagus, and the cause was determined to be a collection of granulomas. After starting medication, his cough improved, but he still had difficulty swallowing, so he underwent further tests and treatment to address this ongoing issue."
197,"A two-year-old female, previously healthy and normally developing, presented with a six-week history of macrocephaly and truncal and peripheral ataxia. An eye examination showed a lack of papilledema but was suspicious for mild peripheral loss of vision. Her past medical history was unremarkable. She did not have diencephalic syndrome at presentation. An urgent brain MRI demonstrated the presence of a large lobulated multicompartmental supra-sellar mass centered within the hypothalamus/optic chiasm (5.4cm x 3.4cm x 5cm) (Figure ), with extension into the surrounding structures and mass effect on the midbrain and third ventricle causing obstructive hydrocephalus. She initially underwent an endoscopic biopsy and septostomy, along with a right-sided ventriculoperitoneal (VP) shunt to manage her hydrocephalus. The pathology of the lesion confirmed the diagnosis of a low-grade glioma (LGG) that was BRAF-V600E negative on immunohistochemistry but positive on next-generation sequencing (Figure ). She was started on chemotherapy with vincristine and carboplatin, but unfortunately she had rapid tumor progression with worsening hydrocephalus six weeks into chemotherapy. This progression caused the patient to develop further complications, including progressive right-sided hemiparesis, bitemporal hemianopia, central hypothyroidism, and feeding difficulties requiring a gastrostomy tube and placement of a second VP shunt. After extensive discussions and mutual expert consensus, her chemotherapy was stopped, and she was started on the novel targeted agent dabrafenib (5.25mg/kg/day). By three months of starting dabrafenib, the size of her tumor decreased by more than 70% (2.5cm x 3.5cm x 2.7cm), with continued decline until plateauing after two years of therapy (Figure ). Prior to dabrafenib, the patient had marked motor and speech impairments but is now able to perform all age-appropriate developmental skills independently. She no longer requires tube feeding and has been growing well. Her bitemporal hemianopia has improved markedly. Her visual acuity is the only symptom that has not shown significant recovery, as she continues to have low but stable visual acuity bilaterally. Our patient is currently five years old, has been on dabrafenib for 30 months, and, to date, she has not experienced any side effects while on targeted therapy. Our plan is to continue on dabrafenib as long as the patient is tolerating the therapy well with no recurrence or progression of the tumor.\nConsent was obtained from the parents for sharing of the clinical information and participation in the ORCYD (Oncology Repository for Children and Young Adults) biomarker study. Samples (both blood and CSF) were collected every three months after starting targeted therapy with dabrafenib. Cell-free DNA (cfDNA) from CSF and plasma isolated from blood collected in DNA BCT tubes (Streck, La Vista, NE) were extracted using the QIAamp® Circulating Nucleic Acid kit (Qiagen, Hilden, Germany). Samples were quantified with Qubit™ and the 1X dsDNA high sensitivity (HS) kit (ThermoFisher, Waltham, MA); 1-ul aliquots were loaded on HS DNA chips and run on a Bioanalyzer (Agilent, Santa Clara, CA) to check for cfDNA profile. Samples were pre-amplified with the SsoAdvance™ PreAmp Supermix (Bio-Rad, Hercules, CA) for 10 cycles (annealing temperature = 58°C) following manufacturer's instructions. Diluted pre-amplified samples were mixed with 2X droplet digital polymerase chain reaction (ddPCR) probe mix then used to generate droplets on a QX200 DG (Bio-Rad). Droplets were then submitted to PCR amplification: 95°C for 10 minutes, 94°C for 30 seconds, 56°C for 1 minute (45 cycles), and 98°C for 10 minutes. Droplets were then read on a QX200 Droplet Reader, and data were analyzed using QuantaSoft™ version 1.7 software (Bio-Rad). The primers/probes sequences used for pre-amplification and ddPCR steps are given below:\n5’-TCTTCATGAAGACCTCACAGTAA-3’ (BRAF_Fw76pb)\n5’-ATGGGACCCACTCCATC-3’ (BRAF_R-long/short)\nWild-type probe: /5HEX/AGATTT+C+A+CTG+T+AGC/3IABkFQ/\nMutant probe: /56-FAM/AGATTT+C+A+CTG+T+AGC/3IABkFQ/\nThree months after starting dabrafenib, the patient was enrolled in the ORCYD biomarker study to test her plasma and CSF for the presence of the BRAF-V600E mutation and to correlate the assay results with the MRI changes observed while on dabrafenib therapy. Samples were collected from the patient (peripheral blood and CSF obtained by lumbar puncture) while sedated for follow-up MRIs over the course of a year. However, the BRAF-V600E mutation was not detected in any of the samples from either plasma or CSF (Figure ).","This two-year-old girl was initially diagnosed with a fast-growing brain tumor that was causing her to grow unusually quickly and have difficulty with movement and vision. After trying different treatments, a new medication called dabrafenib helped to shrink the tumor significantly, and she started to improve. She is now doing very well, able to do all the things expected for her age, and no longer needs feeding tubes or extra shunts. While her vision hasn't fully recovered, she is continuing to thrive on dabrafenib and is being closely monitored to ensure the tumor stays under control."
198,"A 75-year-old male presented to our hospital with worsening mental status. Gait instability and expressive aphasia were noted on the physical examination. He was otherwise hemodynamically stable, with unremarkable laboratory studies and a negative urine drug screen. Computed tomography (CT) of the head without contrast showed a large right frontoparietal lesion crossing midline with surrounding vasogenic edema (Figure ). Further characterization with magnetic resonance imaging (MRI) showed a 5.5-cm intra-axial mass within the deep white matter of the right frontal lobe, with gadolinium enhancement and prominent FLAIR (fluid-attenuated inversion recovery) signal concerning malignancy.\nLater, the patient was started on dexamethasone, and a frontal stereotactic brain biopsy was performed. The initial pathology report showed a B-cell lymphoma with cells positive for CD20 immunostain (Figures -). However, due to its complexity, the sample was sent to an outside institution for a second opinion, which found the sample to be positive for the MYD88L265P mutation. Due to the strong association between this mutation and WM, quantitative serum immunoglobulins were measured, which showed an elevated IgM monoclonal paraprotein, confirming the diagnosis of WM.\nThe patient was started on treatment with rituximab, methylprednisolone, carfilzomib, and ibrutinib. The patient showed a good response by MRI at three months, and he was transitioned to oral ibrutinib only. Surveillance MRI at one year post-diagnosis showed patchy right frontal lobe enhancement indicating disease progression, although the patient had no clinical symptoms; he was then restarted on the initial chemo-immunotherapy protocol. However, ibrutinib was later held due to thrombocytopenia. Soon after, the patient presented to the hospital again with cognitive dysfunction, leg weakness, and falls, with MRI showing significant disease progression. At this point, his treatment was changed to palliative chemotherapy with high-dose methotrexate and rituximab. His disease responded avidly to this regimen, and he is currently completing a one-year course of treatment, with no radiologic or clinical evidence of recurrence (Figure ).","A 75-year-old man was admitted to the hospital because he was confused and having trouble speaking and walking. Tests showed a large growth in his brain, which was initially thought to be cancer, but was later identified as a type of lymphoma. After trying different treatments, the lymphoma responded well, and his condition improved significantly. However, the lymphoma later returned, and the doctors switched to a palliative treatment plan to manage his symptoms and improve his quality of life."
199,"A 79-year-old male with a past medical history of chronic infection of a left knee prosthesis, hypertension, and chronic kidney disease stage 3A presented to the wound care clinic after two days of subjective fever that partially improved with acetaminophen. He denied any associated symptoms. The patient had recently undergone multiple left knee revisions and received several antibiotics in an attempt to treat the draining chronic left knee infection (Table ).\nHe developed severe allergic reactions (urticarial rashes and angioedema) to cephalexin and ciprofloxacin trimethoprim/sulfamethoxazole. Subsequently, 11 days prior to presentation, he was started on ertapenem and daptomycin. Moreover, before intravenous daptomycin and ertapenem were started, he had not received additional antibiotics for over a month. The patient had no previous history of pulmonary diseases and he denied any exposure to pulmonary irritants. Upon admission, the patient had a Hickman catheter for long-term antibiotic use without signs of acute inflammation. He had wheezing throughout the bilateral lung fields but no crackles. A draining tract with serosanguinous fluid drainage was observed on his left knee. His physical exam was otherwise unremarkable. The initial laboratory work demonstrated moderate anemia, normal white blood cells but with bandemia, and an elevated erythrocyte sedimentation rate and C-reactive protein (Table ).\nA chest x-ray showed new diffuse interstitial opacities (Figure ). A single anteroposterior portable chest X-ray was obtained on admission. Compared to a chest X-ray nine months prior, there were new diffuse branching interstitial opacities extending outward from hila associated with additional circular interstitial opacities. These were likely representing peribronchovascular interstitial thickening. There may be a trace of left pleural effusion without right pleural effusion. Additionally, the aorta was atherosclerotic and a right internal jugular central venous catheter terminating at the cavoatrial junction was seen in situ.\nOn the second day of admission, the patient started having worsening shortness of breath, desaturation, tachycardia, and persistent fever. Examination of the lungs revealed bilateral crackles while the lower limbs and the trunk showed a new reticulopapular, non-pruritic rash. Repeat complete blood count (CBC) revealed new-onset eosinophilia. A computed tomography (CT) scan of the chest showed diffuse reticulonodular opacities in the lungs with peripheral predominance, areas of ground-glass opacities, and nodularities. Daptomycin AEP was suspected and the antibiotic was promptly discontinued and meropenem was started. Methylprednisolone and inhaled breathing treatments were ordered and the patient’s symptoms significantly improved thereafter. A repeat chest x-ray one month later showed resolution of the interstitial markings (Figure ). One month after the initial presentation, a repeat chest x-ray was obtained. The lungs appeared clear without apparent pleural effusion. No active disease in the chest was identified.","This 79-year-old man has a history of knee problems and kidney disease and recently developed a fever and shortness of breath. He had a chronic infection in his knee that he was being treated for with antibiotics, but he also had allergic reactions to some of those medications. A chest x-ray revealed new changes in his lungs, leading doctors to suspect a possible infection, and he was treated with new antibiotics and steroids, which improved his condition. Follow-up x-rays showed the changes in his lungs had resolved."
200,"An 88-year-old woman (height, 150 cm; weight, 41 kg) with no history of drug allergy or general anesthesia was scheduled for transfemoral TAVI because of severe aortic stenosis (AS). The surgery was performed under general anesthesia to facilitate the use of transesophageal echocardiography and to manage any intraoperative complications. She was taking 0.625 mg/day bisoprolol, a selective β-1 blocker, for hypertension and chronic atrial fibrillation. Additionally, she was taking amlodipine for hypertension. Preoperative transthoracic echocardiography showed severe AS (aortic valve peak flow velocity: 4.73 m/s; mean aortic valve pressure gradient: 47 mmHg; aortic valve area: 0.41 cm2) and myocardial hypertrophy (left ventricular posterior wall thickness and interventricular septum thickness: 14 mm).\nPre-anesthetic medication for sedation was not administered. She received bisoprolol and amlodipine 3 h before entering the operating room. Figure shows the anesthesia record. General anesthesia was induced using midazolam and remifentanil; additionally, rocuronium was administered to facilitate tracheal intubation. She received total intravenous anesthesia: continuous infusions of propofol and remifentanil. Noradrenaline and dopamine were infused continuously through the central venous catheter to maintain blood pressure. AS disappeared after valve implantation. During the surgery, complete atrioventricular block occurred, and ventricular pacing (VVI mode: 60 ppm) was initiated. No other complications were associated with the surgical procedure. Postoperatively, she received 100 mg sugammadex through the central venous catheter, but 2 min later, her systolic arterial blood pressure (ABP) decreased unexpectedly to less than 40 mmHg. Simultaneously, ventilator monitoring revealed elevated peak and plateau airway pressures (60 and 40 cmH2O, respectively). No skin rash was observed on the body surface. Transthoracic echocardiography showed underfilling of the left ventricle without right ventricular dilatation or pericardial effusion. No abnormalities were observed in left ventricular wall motion.\nThe patient developed distributive shock, which was clinically diagnosed as anaphylactic shock caused by sugammadex because of the rapid onset of severe hypotension and bronchospasm. First, two boluses of 0.05 mg adrenaline were administered through the central venous catheter, followed by rapid volume resuscitation using crystalloid (1 L infusion within 30 min). The dose of noradrenaline was increased to 0.3 μg/kg/min. Consequently, the airway pressures returned to the original level, but the hypotension persisted. Thereafter, adrenaline was administered via bolus (0.1 mg twice) and continuous infusion (0.1 μg/kg/min); however, the increase in her ABP was minimal. Therefore, 1 mg glucagon was administered intravenously, and her systolic ABP immediately rose to 130 mmHg. An erythematous rash also appeared on her neck with the increase in ABP. Her general condition was stable after the therapeutic interventions. Ventricular pacing was continued because bradycardia with complete atrioventricular block was persistent during the interventions. She was admitted to the intensive care unit under tracheal intubation. Subsequently, the continuous infusion of adrenaline was discontinued, and the continuous infusion of noradrenaline was reduced. We also administered 125 mg methylprednisolone for 3 days. She was extubated 9 h after the onset of anaphylaxis, and circulatory agonists were discontinued the following day. She was discharged without complications on postoperative day 9. Blood tests revealed a total serum tryptase level of 7.3 ng/mL and 1.2 ng/mL at 1 h and 24 h after onset, respectively. We advised the patient to undergo allergy tests, such as skin prick tests or intradermal tests, to identify the cause of anaphylaxis. However, the patient refused these tests because she was elderly and unlikely to undergo surgery under general anesthesia in the future.\nWritten informed consent was obtained from the patient for the publication of this case report. This report was approved by the Institutional Review Board of Nagasaki University Hospital (Approval number: 21041932).","This 88-year-old woman was scheduled for a procedure to improve her heart valve, but she had some existing health conditions like high blood pressure and a heart rhythm problem. During the surgery, she experienced a sudden and severe reaction to a medication called sugammadex, leading to dangerously low blood pressure and breathing difficulties. Doctors quickly treated her with adrenaline and other medications, and her condition stabilized. After a few days in the intensive care unit, she was discharged home, but she will need to undergo allergy testing to find out what caused the reaction."
201,"A 62-year-old man, affected dysphagia, was endoscopically diagnosed with lower esophageal cancer confirmed squamous cell carcinoma on biopsy, and was reffered to our hospital (Fig. ). He had no medical history. His lifestyle has included 1500 ml beer consumption per day and 40 cigarettes per day for the past 40 years. Computed tomography (CT) showed thickening of the wall in the lower esophagus as the primary lesion was demonstrated and the tumor formed a mass with the solitary metastatic abdominal lymph node, and invaded pancreas body and gastric body (Fig. ). No other distant metastasis was detected on CT. He was diagnosed with lower esophageal cancer cT4 N1 M0, with pancreatic invasion, cStage IIIC according to 7th edition of the Union for International Cancer Control system []. At first, we considered definitive chemoradiotherapy. However, radiation oncologists evaluated that the tumor was less candidate for chemoradiotherapy because of the risk of gastric mucosal damage. For the purpose of definitive therapy, radical esophagectomy with distal pancreatectomy was planned. As neoadjuvant chemotherapy, CF therapy (cisplatin and 5-fluorouracil therapy; cisplatin was dripped 80 mg/m2 plus 5-fluorouracil was infused 800 mg/m2 on day 1 through 4 continuously) was started according to standard therapy of localized advanced esophageal cancer []. However, after once administration, he could not continue chemotherapy for the exacerbation of dysphagia, and underwent radical surgery. Preoperative evaluation of tumor was similar to initial findings on endoscopy and CT.\nIn findings on laparotomy, abdominal lymph node was infiltrated directly to pancreas body. As the radical surgery, Ivor Lewis esophagectomy with distal pancreatectomy and splenectomy, followed by reconstruction of gastric conduit. Two fields lymphadenectomy was performed according to the treatment strategy of the abdominal esophageal cancer. Reconstruction of gastric conduit was possible although the lymph node was adherent to lesser side of gastric body, which was resected when reconstruction of gastric conduit. In addition, partial resection of lung was performed simultaneously owing to involvement of bilateral pulmonary ligaments to the primary tumor (Fig. ). The operative time was 528 min. The estimated blood loss was 2850 ml, and the patient was transfused 4 units of red blood cell concentrates.\nThe patient had an uneventful postoperative course and was able to take orally. He was discharged 16 days after operation with tube-free. After discharge, he underwent two courses of CF therapy as adjuvant chemotherapy. At present, he is still alive and has no recurrence for 7 years after surgery.\nIn the resected specimen, the primary lesion was observed from lower esophagus to esophagogastric junction, and metastatic lymph node was fixed to stomach and pancreas body (Fig. ). Pathological examination revealed that primary lesion was not infiltrated to lung (Fig. a, b). Massive metastatic lymph node (over 5cm) was observed in the lesser curvature of stomach, and infiltrated to pancreas and gastric wall with extranodal extension (Fig. c, d). The tumor was diagnosed with squamous cell carcinoma, moderately differentiated type. The vascular and lymphatic invasion was confirmed. The surgical margin was negative. The pathological stage was ypT4 N1 (1/61) M0 (metastatic lymph node invasion into pancreas), ypStage IIIC. The histopathological response of chemotherapy was grade 1a, which was equivalent that proliferable cells were 2/3 or more, in 7th edition of the Union for International Cancer Control system [].","A 62-year-old man was diagnosed with cancer in his lower esophagus, confirmed by a biopsy. He had a history of heavy drinking and smoking, which may have contributed to the cancer. CT scans showed the cancer had spread to nearby lymph nodes and organs, including the pancreas and stomach. After considering different treatment options, he underwent a complex surgery to remove the tumor and surrounding tissues, including part of his stomach, pancreas, and lung. He is currently doing well and has been closely monitored for any signs of recurrence."
202,"A 48-year-old Japanese woman with high myopia presented with decreased visual acuity. Axial length was 29.0 mm in the right and 28.7 mm in the left eyes, respectively; refractive errors were −11.5 and −10.5 diopter; best-corrected visual acuity (BCVA) was 20/28 and 20/16, respectively. The BCVA was described by converting the decimal visual acuity into fractional visual acuity. Mild cataract was observed in both eyes. Fundoscopy and optical coherence tomography (OCT) images showed epiretinal membrane (ERM) in the left eye (Fig. ).\nThree years later, she developed blurred vision and BCVA in the left eye decreased to 20/33; OCT revealed thickening of the ERM. The patient opted for vitrectomy after thorough discussion and considering recent reports showing good treatment outcome for ERM with good visual acuity []. The patient subsequently underwent uncomplicated 25-gauge pars plana vitrectomy with ERM and internal limiting membrane (ILM) peeling in the left eye. After 6 months, BCVA was 20/28. CRA was noted in the parafovea, and OCT revealed irregularities in the RPE with increased transmission signal from the sclera, suggesting atrophy of the RPE (Fig. ).\nAfter another 3 weeks, BCVA in the left eye decreased suddenly from 20/28 to 20/100. OCT demonstrated disruption of the interdigitation and ellipsoid zones and elevation of the RPE. Choroidal thickness at this site increased from 134 µm to 151 µm (Fig. a, b). Fluorescein angiography (FA) revealed hyperfluorescence (Fig. a), which coincided with the site where changes were observed in RPE and choroid on OCT (Fig. b). Hyperfluorescence was observed from an early stage, but no leak thereafter. Typical choroidal neovascularization (CNV) or lacquer crack were not shown on FA. Indocyanine green angiography (ICGA) did not detect abnormal blood vessels suggesting CNV (Fig. b). Although fluorescein and indocyanine green angiography did not show typical CNV or lacquer crack, myopic CNV could not be ruled out. Therefore, intravitreal ranibizumab injection was recommended. However, 2 days after the injection, the patient presented with a further decline in vision to 20/222 in her left eye. Despite no obvious change on fundus examination, OCT revealed a nodule-like outer retinal lesion with disruption of the RPE, suggesting PIC. Choroidal thickness had decreased slightly to 142 μm (Fig. c). Careful observation was recommended after thorough discussion with the patient concerning possible treatment options, including steroid therapy.\nOne month after the injection, BCVA improved to 20/100 and the nodule-like lesion regressed, leaving an expanded RPE defect with a decrease in choroidal thickness to 84 μm. The lesion was judged to be inactive, and further observation was recommended. However, the area of CRA increased gradually to 0.38, 0.90, and 1.59 mm2 and choroidal thickness decreased to 52, 40, and 16 μm by 14, 30, and 40 months post-injection, respectively (Fig. ). CRA progressed at a rate of 0.45 mm2/year. The CRA was measured on color photographs of the fundus using ImageJ software (freely available at ). Three years after the injection, BCVA was 20/70 in the left eye and two new atrophic lesions were noted in the parafovea (Fig. c).","This 48-year-old woman with nearsightedness experienced blurry vision and developed a membrane on the back of her eye. Over time, this membrane thickened, leading to further vision loss. She underwent surgery to remove the membrane, but after a period of improvement, new changes appeared in her eye, including thinning of the tissue behind the retina. Despite further treatment, her vision continued to decline, and doctors are now carefully monitoring the situation and considering further options to help preserve her remaining sight."
203,"An 82-year-old male patient with angina on anticoagulant medication and without viral infection was referred to our department for the surgical treatment of HCC. His personal and family medical history was otherwise unremarkable. Abdominal computed tomography (CT) showed a huge HCC mass in segment 4 and PVTT invasion from the nearby portal vein to the left portal trunk, main portal trunk, through to the contralateral right portal trunk. The tip of the PVTT progressed over to the bifurcation of the anterior and posterior branches of the portal trunk. The anterior branch was filled with PVTT, while the posterior branch was filled with PVTT or blood thrombus (Fig. ). No apparent intra- and extra-hepatic metastases were detected other than the main tumor. Laboratory data showed a serum albumin level of 3.8 g/dL, total bilirubin level of 0.5 mg/dL, platelet count of 13.9 × 104/uL, and a Child-Pugh score of 6. Serum levels of alfa-fetoprotein (AFP) and protein induced by vitamin K absence or antagonist II were 90,770 ng/mL and 2847 mAU/mL, respectively. An antithrombotic drug was administered for the PVTT. Due to the overwhelming PVTT extensions, poor performance status, and old age, the patient was deemed not to have a surgical indication. Therefore, a combination treatment using atezolizumab plus bevacizumab with radiotherapy for PVTT was selected. During preparation for radiotherapy, one-time atezolizumab (1200 mg) and bevacizumab (15 mg/kg) were administered. He developed anal pain and persistent fever 9 days after administration, and CT showed perianal abscess due to anal fistula. Although he recovered soon after percutaneous abscess drainage, this adverse event interrupted atezolizumab plus bevacizumab treatment, and radiotherapy could not be introduced. Abdominal CT, conducted 3 weeks after the first administration, showed size reduction of the main tumor and PVTT, with a reduction in tumor enhancement on contrast-enhanced CT. Serum AFP decreased from 90,700 ng/mL before treatment to 18,371 ng/mL and 6301 ng/mL 3 and 5 weeks after atezolizumab plus bevacizumab treatment, respectively. Considering the excellent response to atezolizumab plus bevacizumab treatment (one-time administration), we decided to continue with this treatment after creating colostomy. Colostomy was performed 47 days after the first administration of atezolizumab plus bevacizumab. Subsequently, beginning from the 83rd day after the first administration of atezolizumab plus bevacizumab, atezolizumab monotherapy was initiated. The reason for choosing atezolizumab monotherapy was that the bleeding from the colostomy persists from the collateral blood circulation due to the liver cirrhosis. The serum AFP decreased to 89 ng/mL just before the next atezolizumab monotherapy, and abdominal CT showed continued partial response (Fig. ); serum AFP levels kept decreasing toward the normal range (Fig. ). He received a one-time atezolizumab plus bevacizumab treatment and continued with atezolizumab monotherapy afterward (9 regimens administered until now); there is no apparent sign of residual tumors at 9 months after the introduction of atezolizumab plus bevacizumab treatment.","An 82-year-old man with a history of heart problems and liver cancer was referred for surgery, but doctors determined that the cancer had spread and he was not a good candidate for surgery. CT scans showed the cancer had spread to the main blood vessels in his abdomen, and his blood tests indicated he was very frail. He started a new treatment with a combination of drugs (atezolizumab and bevacizumab) and radiation therapy, which initially helped shrink the tumor and lower his cancer levels. Despite some side effects, including an anal abscess, the treatment continued to be effective, and his cancer has remained under control for nine months."
204,"A 54-year-old man with a history of excessive intake of alcohol (100 g/day × 30 years) and locally grown tobacco use (500g/month × 30 years) was admitted to our hospital because of chronic persistent swallowing dysfunction for six months. White light endoscopy (WLE) revealed a 22 mm flat lesion in the middle esophagus.The lesion was covered with scattered leukoplakia, and normal vascular network could not be seen in the lesion (Fig. a). Narrow-band imaging (NBI) under endoscopy revealed the lesion with an indistinct brownish area, local white penniform area, and white tiny papillary surface structure (Fig. b). Further low and high magnifying endoscopy with NBI (ME-NBI) showed tiny irregular papillary microsurface structure with various shapes and sizes, and non-typical type B1 pattern of intraepithelial papillary capillary loops (IPCL) confined to the papillary microsurface structure (Fig. c, low magnification, and Fig. d, high magnification) according to the classification of Japan Esophagus Society (JES classification) []. Iodine staining endoscopy revealed a less-stained lesion (Fig. e) and showed more distinct white tiny papillary surface structure (Fig. f) than conventional WLE. Biopsy showed a low grade intraepithelial neoplasia. Chest and abdominal CT scan revealed no abnormal lesions.\nAccording to the endoscopic manifestations under WLE, ME-NBI, and iodine staining endoscopy, combining with pathology, we considered the lesion to be early esophageal cancer. Therefore we chose endoscopic therapy. The lesion was completely resected with endoscopic submucosal dissection. Histopathology showed that the layer of neoplastic spinous cells was significantly thickened (Fig. a) with local keratosis presenting the appearance of the so-called church spire (Fig. b). The lamina propria papilla was elongated upward to the surface layer (Fig. c), and the nuclei in the basal region had double or more nucleoli. Tumor cell displayed expanding growth, and partial tumor cells broke through the basement barrier and invaded into superficial lamina propria (Fig. d). The IPCL were observed in lamina propria papilla with elongating upward to the surface layer growing underneath the layer of neoplastic spinous cells, which corresponds to the IPCL observed by ME-NBI (Fig. e). The lesion was histopathologically confirmed as early VCCE which was limited within the mucosal lamina propria (m2) and completely resected. There were no lymphovascular and neural invasions of cancer cells, and no cancer cells to be seen in the lateral and vertical margin of the resected specimen (the distance of the lesion to the closest margin of the resected specimen was 3.012mm) (Fig. ).","A 54-year-old man was admitted to the hospital because he was having trouble swallowing for six months. Doctors found a small, abnormal area in his esophagus during an endoscopy, and a biopsy confirmed it was early-stage esophageal cancer. The area was successfully removed using a minimally invasive procedure called endoscopic submucosal dissection. The pathology report showed that the cancer was contained within the lining of the esophagus and there were no signs of spread to nearby tissues or lymph nodes."
205,"A previously healthy 25-year-old White man presented with a 1-year history of blurred vision in the right eye, headache, and weight loss of about 30 kg. On physical examination, he had a body mass index of 28 kg/m2 and visual field defects in the right eye. Initial MRI showed a lightly spotted heterogeneous 19 × 16 × 19 mm tumor located suprasellarly, close to the pituitary gland with no ingrowth or association with the pituitary gland (Fig. A). Endocrine evaluation revealed secondary hypogonadism with low follicle-stimulating hormone, luteinizing hormone, and testosterone but intact thyrotroph and lactotroph axes. An ACTH test was performed with a subnormal cortisol response (30-minute cortisol 248 nmol/L, reference > 420 nmol/L) and low plasma ACTH concentration, indicating secondary adrenal insufficiency, and the patient started treatment with hydrocortisone. The somatotroph axis was not evaluated. Visual field measured by perimetry revealed visual field defects in the right eye.\nThe tumor was initially diagnosed based on MRI as a craniopharyngioma for which reason the patient was referred to craniotomy as standard treatment. At surgery, the tumor appeared gray and reddish, and was capsulated and fragile with easy bleeding, unlike craniopharyngiomas with sharp, irregular borders, which have a tendency to adhere to vital neurovascular structures and often consist of cystic and/or solid parts []. The pituitary tumor was partially resected, and a small amount of capsule remnant underneath the optic chiasm could not be surgically removed. Perioperative frozen section histological examination described the tumor as a possible malignant lymphoma due to lymphocytic infiltration.\nThe patient was transferred to the Department of Endocrinology at Odense University Hospital for further diagnostic workup and management of pituitary hormone deficiencies. Postoperatively, the patient was still affected by headache and visual deficits on ophthalmologic assessment. MRI showed regression of the pituitary mass (9 × 6 × 8 mm), but remnant pituitary tissue was located just below the optic chiasm (Fig. B). Pituitary hormone insufficiency of all anterior axes and diabetes insipidus was diagnosed, and treatment with hydrocortisone, levothyroxine, testosterone, growth hormone, and desmopressin was implemented.\nEndocrine evaluation did not support a hormone-producing adenoma. Sellar lymphoma is a very rare disease often related to immunodeficiency, for example HIV-related, or seen in immunocompetent patients who are over 60 years old []. Metastasis was not suspected; the patient did not suffer from cancer in another location. Workup for infectious and rheumatologic etiologies was not indicative of secondary hypophysitis, nor was drug-related infiltration of the pituitary gland a possible differential diagnosis. Final histological diagnosis showed lymphoid reaction with predominant CD20 positive B-cell infiltration and no sign of significant IgG4 involvement. The patient was diagnosed with autoimmune hypophysitis.\nTen months postoperatively, the patient reported impaired vision. MRI showed that the remnant tissue lifted and exerted pressure at the optic chiasm. Perimetry confirmed progression of visual field defects in the right eye and now involvement of the left eye. Because of progression of visual field defects, treatment with glucocorticoid pulse therapy was initiated: methylprednisolone 500 mg infusion for 3 days, followed by reduced prednisolone doses, that is, 37.5 mg per day. Follow-up MRI after 1 month of glucocorticoid treatment (11 months postoperatively) demonstrated significant reduction of the pituitary mass and decreased pressure at the optic chiasm. Perimetry was unchanged. Treatment with prednisolone continued for 3 months.\nThe patient had intolerable adverse effects to glucocorticoid treatment with excessive weight gain of about 50 kg and cushingoid features. The need for long-term treatment led to initiation of steroid-sparing treatment with 150 mg azathioprine per day under gradual tapering of prednisolone (13 months postoperatively). Visual field defects improved, and repeated MRI showed additional regression of the remnant pituitary mass. Perimetry revealed stable conditions. Due to stable condition over 26.5 months, treatment with azathioprine was reduced. Control MRI after one and a half month showed recurrence with increasing mass in sella turcica causing mass effect on the optic chiasm. Treatment was resumed: azathioprine 100 mg per day in combination with prednisolone 37.5 mg per day.\nDespite additional 11 months treatment with azathioprine and prednisolone treatment (52 months postoperatively), control MRI showed further progression with increasing pituitary mass size (11 × 6 × 7 mm) reaching the optic chiasm (Fig. C). Treatment with rituximab was initiated: 1000 mg infusions 14 days apart, repeated every 12 months. This resulted in almost complete regression of pituitary mass (3 × 2 × 7 mm) evaluated by MRI (Fig. D). Rituximab treatment was discontinued after 25 months (77 months postoperatively) because of stable disease. To date, the patient has been followed up for ten and a half years postoperatively. According to control MRI 4 years after end of treatment with rituximab, he is in sustained remission with unchanged pituitary mass size and no affection of the optic chiasm.","This 25-year-old man was initially diagnosed with a tumor near his pituitary gland that was causing vision problems, headaches, and weight loss. Surgery to remove the tumor was successful, but it was determined to be a type of lymphoma. After surgery, he experienced hormone deficiencies and continued vision problems, requiring him to take several medications to replace these missing hormones. Over the next several years, the tumor grew back, and he needed additional treatment with steroids and then rituximab to shrink it and improve his vision. Currently, he is in sustained remission with no further issues."
206,"In November 2016, a Caucasian 75-year-old woman, a former smoker (7.5 pack-years), was hospitalized for breathlessness. Her past medical history included atrial fibrillation treated with warfarin, arterial hypertension treated with betaloxol, and gastroesophageal reflux. She ran a bar–tobacco shop with significant long-term exposure to passive smoking. She was not exposed to asbestos. Clinical examination on admission revealed good performance status, stage 1 modified Medical Research Council (mMRC) dyspnea, crackles at lung bases, and no digital clubbing or extrathoracic signs. Chest computed tomography (CT) scan showed interstitial lung disease (ILD) with subpleural reticulations without evidence of honeycombing or enlarged lymph node (Fig. ). Standard biology, serological testing, bronchoscopy, and bronchoalveolar lavage were normal. Pulmonary function tests demonstrated bronchial obstruction [forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) ratio, 0.66; FEV1, 97% of predicted values (% pred.)], preserved volumes [FVC, 121% pred.; total lung capacity (TLC), 111% pred.], and alteration of gas diffusion (TLCO, 62% pred.). ILD multidisciplinary discussion (MDD) reached a CT pattern of possible usual interstitial pneumonia (UIP), which led to the proposal of performing surgical lung biopsy. Wedge resection of the right upper and lower lobes was performed by video-assisted thoracoscopic surgery in July 2017. Both resected lung specimens showed a similar pattern of UIP: fibroblastic foci and honeycombing. In addition, the right lower lobe specimen displayed numerous disseminated foci of well-differentiated focally invasive SCC without invasion of visceral pleura (Fig. ). Complete resection was obtained without SCC-positive surgical margins. A PET–CT scan performed in September 2017 showed mild hypermetabolism of ILD [maximum standardized uptake value (SUVmax), 3.5], without hypermetabolic lung nodule, mass, or enlarged lymph node. Cerebral magnetic resonance imaging (MRI) was normal. There was no indication for adjuvant antineoplastic treatment based on pathological findings. After ILD MDD in October 2017, it was decided to introduce an antifibrotic treatment owing to pathological confirmation of UIP. Nintedanib was chosen as this molecule is also known for its antitumor properties. At the last follow-up in October 2019, that is, 2 years after initiation of nintedanib, clinical, functional (FVC, 130% pred.; TLCO, 61% pred.), and CT scan evaluations were stable (Fig. ). There was no evidence of squamous cell carcinoma progression.","This 75-year-old woman was admitted to the hospital in November 2016 because she was struggling to breathe. Tests revealed a lung condition called interstitial lung disease (ILD) and a small cancer in her lungs. After surgery to remove part of her lungs, doctors confirmed the cancer was squamous cell carcinoma. She is now taking medication to help prevent further lung damage, and her lung function and the cancer have remained stable over the past few years."
207,"The patient was a 48-year-old male who had been undergoing regular dilatations for the last 8 years for sustaining relief from his urethral stricture. He had a history of having undergone open suprapubic cystostomy (SPC) 8 years ago for acute urinary retention secondary to recurrent urinary tract infection (UTI). He developed surgical site infection (SSI) postoperatively which was managed conservatively. The stricture had been managed with internal optic urethrotomy (IOU) and serial dilatations, following which the patient started doing well with 6 monthly dilatations.\nSince last 2 years, he developed a swelling in the right inguinoscrotal region associated with repeated thinning of urinary stream and recurrent episodes of burning micturition. The swelling became static and persistent in the last 2 months.\nOn examination, there was a 5-cm long vertical midline scar in the suprapubic region. An irreducible soft swelling of 8 cm × 6 cm was present in the right inguinal region which extended up to the base of scrotum. Cough impulse was present, and we could not get above the swelling. The defect could not be localized. There was also a gradual growing difficulty in negotiating urethral dilators due to the path distortion by the contents of the hernia. Cystoscopy revealed narrowing in bulbar urethra with deviations in the proximal anterior urethra and the scope needed to be negotiated to reach the urinary bladder. Ultrasound showed findings consistent with right inguinal omentocele and a scarred suprapubic region. Defect could not be localized.\nThe patient had been coaxed for surgery multiple times but he did not agree due to poor past surgical experience. He eventually landed with difficulty in micturition and inability to get dilators passed. A preoperative diagnosis of right sided, complete, irreducible, inguinal omentocele with urethral stricture was made, and the patient was planned for right open hernioplasty with cystourethroscopy along with IOU.\nIntraoperatively, a right indirect hernial sac with omentum as content was found; the sac was opened, omentum resected, and sac was transfixed at its neck followed by posterior wall strengthening. Another hernial sac with omentum as content was found in midline beneath the scar of old SPC which was densely adherent to the surrounding fibrotic tissue (\n). This sac was dissected out, opened, content resected, and sac was closed. For the right inguinal hernia, a mesh hernioplasty was done. After the closure of the external oblique aponeurosis, anatomical repair of midline defect was achieved and strengthening was done with overlay mesh covering the midline defect. On cystourethroscopy, a stricture was present from posterior part of penile urethra to bulbar urethra. IOU was done and 18-Fr Foley's catheter left in situ.\nA postoperative diagnosis of right-sided, complete, irreducible, indirect inguinal hernia, and incisional hernia with omentum as contents with urethral stricture was made. The patient developed SSI on post operative day (POD)-2 which was managed by wound irrigation and oral antibiotics.\nAt follow-up after 1 month, a repeat cystourethroscopy was performed which showed fibrosis and narrowing at the site of IOU. A repeat IOU was done. The anterior urethra had straightened out and dilatation was simple and smooth. The patient now has a good urinary stream, no complaint of restriction in passage and dilatations have become a smooth affair. The patient has been on 4 monthly dilatation for the past year which have all been smooth and has now been called after 6 months for next follow-up.","This 48-year-old man has had a long-standing problem with a narrowed urethra, requiring regular dilations for the past eight years. Recently, he developed a painful swelling in his groin area that was linked to difficulty urinating and frequent urinary infections. Examination revealed a large hernia and scarring from a previous surgery to relieve urinary retention. Surgery was performed to repair the hernia and widen the urethra, and the patient is now experiencing a normal urinary flow and is scheduled for follow-up appointments."
208,"A 35-year-old man presented with the complaints of low back ache for 12 years, radiating to right leg for 4 months and numbness extending to lateral side of the sole of right foot. On examination, there was a 30% sensory loss in right S1 dermatome as compared with contralateral limb, with no bladder bowel involvement. Patient had no motor deficit. Magnetic resonance imaging (MRI) of the lumbosacral spine was suggestive of IM cystic lesion at L2-3 hypointense on T1-weighted images and hyperintense on T2-weighted images. MRI brain did not reveal any abnormality. Lumbar puncture and serologic studies were not performed.\nWith the differential diagnosis of neoplastic lesion, the patient was taken up for posterior laminectomy. L2-3 laminectomy was done. A dural bulge was identified. On durotomy, the cord was found to be enlarged. Under microscopic guidance, posterior longitudinal myelotomy was done, the cysts were approached, and subtotal resection of cysts was done. Intraoperatively, three grayish white cysts were identified. Cysts were found to be adherent to the nerve roots causing their inflammation. As a result, one of the cysts could not be excised and was only decompressed. The remaining two cysts were completely excised. Histopathology revealed it to be NCC.\nThe patient improved postoperatively. Back pain was relieved, and there was significant reduction in radiating pain. He was started on albendazole (15 mg/kg body weight) for 4 weeks and steroids for 2 weeks. The patient was discharged on the 4th post-operative day. He was followed-up biweekly for the first month. Thereafter, monthly follow-up was done for the next 2 months. MRI done at 6 months confirmed resolution of the cystic lesion. Thereafter, 6 monthly follow-up was done. Patient is symptom free and not on any medication at 2 years of follow-up.","A 35-year-old man has been dealing with back pain and leg numbness for several years. Tests revealed a cyst-like growth in his lower back was pressing on his nerves, causing the pain and numbness. Surgery to remove the cyst was successful, and he experienced significant relief from his symptoms. He was prescribed medication to prevent the cyst from returning, and he is now pain-free after two years of follow-up."
209,"A 65-year-old gentleman with no comorbidities presented with progressive jaundice, anorexia, and weight loss since 4 to 5 weeks. He had no similar complaints in past. His clinical examination apart from icterus was normal. Investigations revealed a direct hyperbilirubinemia of 3.3 mg/dL. Ultrasound revealed a hypoechoic lesion in head of pancreas 3 cm × 3 cm in size with dilated common bile duct and intrahepatic biliary radical dilatation.\nA pancreatic protocol contrast-enhanced computed tomography (CECT) scan was performed which confirmed the ultrasound findings as shown in\n. In addition to this, it showed a replaced right hepatic artery arising from superior mesenteric artery (SMA) as shown in\n, and a reversal of relationship of superior mesenteric vein (SMV) and SMA, that is, the artery to the right of vein was seen as shown in\n. The complete vascular anatomy is schematically shown in\n. The duodenojejunal flexure was in midline. There was no significant lymphadenopathy, no liver lesions, and no free fluid. These findings were suggestive of pancreatic head adenocarcinoma with incomplete intestinal rotation and an rRHA arising from SMA. Carbohydrate antigen 19–9 (CA 19–9) was elevated at 196 U/mL (normal: < 37 U/mL) and Carcinoembryonic antigen was normal. A pylorus-preserving pancreaticoduodenectomy was planned for the patient.\nDuring surgery, the small bowel loops were found clumped in right upper abdomen and a Kocher's maneuver was carefully performed after interbowel adhesiolysis to free all the loops till the third part of duodenum. SMA and SMV were then identified and looped at the lower border of pancreas. Hepatoduodenal ligament dissection was then performed to identify the replaced right hepatic artery and the main portal vein. The retropancreatic tunnel was created in a plane above the portal vein superiorly and the SMA inferiorly, and then the plane was widened till the area above SMV (\n). Uncinate dissection, duodenojejunal flexure mobilization, and standard lymphadenectomy were then performed, followed by division of jejunum, first part of duodenum common bile duct and pancreas. The vascular relations can be seen after resection completion in\n.\nReconstruction was performed as the modified Blumgart technique of duct to mucosa pancreatico-jejunostomy with 5–0 polypropylene, single layer interrupted hepatico jejunostomy using 4–0 polypropylene and antecolic stapled posterior gastrojejunostomy. The patient recovered uneventfully and was discharged on the postoperative day 8. Histopathological examination showed moderately differentiated adenocarcinoma confined to pancreas with all margins free and single peripancreatic node out of 18 was positive. The patient is doing well at 4-month follow up on adjuvant 5-fluorouracil based chemotherapy.","A 65-year-old man came to the hospital because he was experiencing yellowing of the skin (jaundice), loss of appetite, and weight loss. Tests revealed a tumor in the head of his pancreas, which was causing a blockage in his bile duct. A CT scan showed the tumor and other changes in the blood vessels around his pancreas. The doctors performed surgery to remove the tumor and the affected blood vessels, and the patient is now recovering well and receiving chemotherapy to prevent the cancer from returning."
210,"A 56-year-old gentleman with no comorbidities presented with progressive jaundice, anorexia, and weight loss of 6 to 8 weeks of duration. He had no similar complaints in past. His clinical examination apart from icterus was unremarkable. Investigations revealed direct hyperbilirubinemia of 10.3 mg/dL. Ultrasound abdomen revealed a hypoechoic lesion in head of pancreas 4 cm × 3 cm in size with dilated common bile duct and intrahepatic biliary radical dilatation.\nA pancreatic protocol CECT scan was performed which confirmed the ultrasound findings. In addition to this, there was intestinal nonrotation with entire small bowel on right of abdomen and large bowel on left side. Cecum and hepatic flexure was in midline. SMA was seen coursing between the jejunal and ileal branch of SMV and to right of SMV as shown in\n. There was no significant lymphadenopathy, liver lesions, or free fluid. These findings were suggestive of pancreatic head adenocarcinoma with intestinal nonrotation. CA 19–9 was elevated at 237 U/mL (normal: < 37 U/mL) and Carcinoembryonic antigen was normal. A pancreaticoduodenectomy was planned for the patient.\nDuring surgery, the small bowel loops were clumped in right upper abdomen and the duodenum was vertically linear instead of the usual C loop configuration, with duodenojejunal flexure on the right side. Kocher's maneuver was performed (\n). The infracolic SMA first approach helps to identify the vascular structures, as well as the replaced hepatic artery from SMA early in surgery, thereby reducing bleeding and it was our approach in this case. SMA and both ileal and jejunal branches of SMV were identified and looped at the lower border of pancreas. Inferior pancreaticodeuodenal vessels were identified to its origin and then divided. Hepatoduodenal ligament dissection was then performed. The retropancreatic tunnel was created in a plane above the portal vein superiorly and the SMV inferiorly, and then the plane was widened till the area above SMA and then above ileal branch of SMV, as we kept dissecting from left to right (\n). Uncinate dissection, duodenojejunal flexure mobilization, and standard lymphadenectomy were then performed followed by division of jejunum at a slightly longer distance from the duodenojejunal flexure to gain mobility of mesentery to allow length for anastomosis, division of first part of duodenum, common bile duct, and pancreas. The vascular relations can be seen after the resection in\n.\nReconstruction was performed by bringing the jejunal loop up from the right paracolic side and not in the conventional retrocolic fashion. The modified Blumgart technique of duct to mucosa pancreaticojejunostomy was performed with 5–0 polypropylene, single layer interrupted hepatico jejunostomy was done using 4–0 polypropylene and stapled posterior gastrojejunostomy was done. The patient had a postoperative chyle leak and superficial surgical site infection which was managed conservatively and he was discharged on the postoperative day 25. Histopathological examination showed tumor confined to pancreas with all margins free and no node out of 16 was positive. The patient is doing well at 3-year follow-up with no adjuvant therapy.","A 56-year-old man came to the hospital because he was experiencing yellowing of the skin (jaundice), loss of appetite, and weight loss. Tests revealed a tumor in his pancreas, which was causing a blockage of the bile duct. Surgery to remove the tumor was planned, and during the surgery, the doctors carefully identified and preserved the blood vessels around the pancreas and duodenum. The surgery was successful, and the patient is now doing well with no signs of recurrence after a 3-year follow-up."
211,"A 70-year-old man was referred to our institution by his family doctor for the examination of repeated upper abdominal pain. Preoperative computed tomography (CT) suggested resectable pancreatic cancer of the pancreatic body (Fig. A) concomitant with stricture of the CA root (Fig. B), which may have been caused by median arcuate ligament (MAL). Pancreaticoduodenectomy with division of the MAL was scheduled. Unexpected bleeding around the CA was observed during surgery, which may have been caused by the injury incurred when the MAL was cut to release CA compression (Fig. A, B). As bleeding could be controlled by simple compression only, hemostasis by suturing was attempted first. Contrary to our expectations, the bleeding intensified, making it difficult to confirm the bleeding point. Therefore, we attempted supraceliac aortic cross-clamping (SAC) to manage bleeding. To expose the aorta, the crus of the diaphragm was divided, and the aorta was clamped upstream of the CA by a Fogarty vascular-clamp forceps. After performing SAC, the bleeding intensity significantly decreased and a defect of the adventitia measuring 7 mm in diameter on the CA was confirmed (Fig. C). The defect was repaired using a 4-0 Prolene continuous suture (Johnson & Johnson K.K, NJ, USA). The procedure time for SAC was 2 min and 51 s, and the intraoperative blood loss was 480 ml. The maximum blood pressure increased from 120 to 150 mmHg when SAC was performed and then decrease to 120 mmHg after releasing the clamp. The operative policy was changed to underdo distal pancreatectomy to decrease the risk of hepatic infarction. The patient was discharged uneventfully on postoperative day 19. A surgical procedure of SAC is shown in Additional file .","This 70-year-old man was referred to us because of persistent pain in his upper stomach. Tests showed he had pancreatic cancer and a narrowing of the main artery that supplies blood to the pancreas. During surgery to remove the cancer, unexpected bleeding occurred, requiring a temporary clamping of the aorta to stop the bleeding. The bleeding was successfully controlled, and the surgeon adjusted the plan to remove only part of the pancreas to reduce the risk of complications. He was discharged home after 19 days and is recovering well."
212,"A 56-year-old woman, a mother of 3 children, presented to the surgical department with a history of multiple neck lumps of 4 months duration. She also had generalised vague abdominal pain, loss of appetite and lower back pain. She had no significant medical, family or psychosocial history. Clinical examination revealed multiple, bilateral enlarged cervical lymph nodes which were firm to hard in consistency. Thyroid examination revealed a 2 × 2 cm firm lump on the lower pole of the left thyroid lobe. Examination of other lymph node groups revealed enlarged right inguinal lymph nodes. Abdominal examination and rectal examination were normal. Vaginal examination revealed a hard, unhealthy uterine cervix. Breast and axillary examination were unremarkable. She had spinal tenderness, but the neurological examination of the lower limbs was normal.\nHer basic blood investigations, liver profile and renal functions were within the normal limits. Ultrasound scan of the abdomen revealed no abnormalities. Ultrasound scan of the neck revealed multiple nodules in the thyroid, with increased vascularity, and multiple enlarged cervical lymph nodes with obliterated fatty hila suggestive of malignant deposits. Contrast enhanced computed tomography of the neck, chest, abdomen and pelvis showed a mass in the uterine cervix (Fig. ) with multiple enlarged lymph nodes in the inguinal, iliac, para-aortic, anterior mediastinal and bilateral deep cervical groups (Fig. ). There were multiple low-density nodules in the thyroid gland (Fig. ). A mixed density mass lesion was also noted in the lower pole of the left thyroid lobe (Fig. ). Furthermore, an anterior wedge fracture of the L2 vertebra was seen, probably secondary to bone metastases.\nBiopsies from the uterine cervix and endometrial curettage revealed moderately differentiated squamous cell carcinoma, signifying local extension of the cervical carcinoma into the endometrium (Fig. ). Excision biopsy of a left cervical lymph node revealed metastatic deposits of moderately differentiated squamous cell carcinoma similar to that of the uterine cervix (Fig. ). Ultrasound-guided fine needle aspiration cytology of intra-thyroid nodules revealed malignant squamous cells (Fig. ). This was followed by a panendoscopy of the upper aerodigestive tract which yielded negative results.\nA multidisciplinary meeting with the oncologists, surgeon, gynaecologists, pathologists and radiologists was conducted. Considering the histopathological and imaging findings with negative panendoscopy, a diagnosis of squamous cell carcinoma of the uterine cervix metastasising to the cervical lymph nodes and thyroid gland was made. A decision was made to commence palliative chemoradiotherapy. After 6 months, the patient developed generalised body oedema. She was transferred to a specialised cancer treatment centre for further palliative management, where she passed away a short while later, due to acute renal failure secondary to ureteric obstruction.","A 56-year-old woman came to the hospital because she had been noticing several lumps in her neck for about four months, along with abdominal pain and back pain. Examination revealed enlarged lymph nodes in her neck and groin, and a lump in her thyroid gland. Imaging tests, including CT scans and biopsies, showed that she had cancer in her cervix that had spread to her lymph nodes and thyroid. After treatment, she developed kidney problems and sadly passed away."
213,"This was a 43-year-old woman with cervical carcinoma who received pelvic exenteration. Continuous intravenous infusion of oxycodone at 30 mg/day was started to relieve postoperative pain. The dose was increased in a stepwise way to 288 mg/day (MED 576 mg/day). The pain gradually subsided, and the oxycodone dose was reduced (). Continuous intravenous infusion was discontinued at 18 mg/day (6.25% of the maximum administered dose). However, six hours after discontinuation, she developed restlessness and an itching sensation in her legs. Therefore, continuous intravenous infusion of oxycodone at a dose of 18 mg/day was resumed the same day, and symptoms disappeared. The oxycodone dose was further reduced and discontinued gradually over 10 days. Subsequently, no withdrawal symptoms occurred.","This 43-year-old woman had surgery to treat cervical cancer and needed strong pain medication, oxycodone, to manage the pain after the procedure. The dose was gradually increased to help control her pain, but eventually, it was reduced and stopped. However, she experienced some withdrawal symptoms when the oxycodone was stopped, so it was restarted temporarily. Thankfully, she was able to safely reduce and discontinue the medication over the next 10 days without any further problems."
214,"This was a 43-year-old man with hepatocellular carcinoma who had undergone liver transplantation from a living donor. A year later, he developed severe acute pancreatitis with severe abdominal pain. On admission to the hospital, continuous intravenous infusion of fentanyl at 1200 μg/day was started to control abdominal pain. The fentanyl dose was increased according to pain intensity, reaching 2400 μg/day (MED 240 mg/day) after 10 days in the hospital. Administration of a pancreatic enzyme inhibitor, antibiotic, and fluid alleviated the pancreatitis and abdominal pain. shows that continuous fentanyl infusion was discontinued after reaching 240 μg/day (10% of the maximum dose). He subsequently developed nausea/vomiting, elevated blood pressure, and restlessness 12 hours after discontinuation. Continuous intravenous fentanyl infusion of 240 μg/day was resumed, and these symptoms disappeared. However, restlessness occurred each time the dose was reduced. Therefore, a transdermal fentanyl patch was initiated to taper the opioid more slowly. Pancreatitis with abdominal pain recurred transiently one month later. Therefore, continuous intravenous fentanyl infusion was resumed, titrated to 600 μg/day. The dose was gradually decreased daily (600, 300, 240, 180, 120, and 60 μg/day). Once the pancreatitis had improved, the fentanyl could be discontinued without causing withdrawal symptoms. The discontinuation was finally completed 64 days after the onset of withdrawal symptoms.","This 43-year-old man, who had a liver transplant, experienced a severe bout of pancreatitis with intense abdominal pain. To manage the pain, he needed a high dose of fentanyl, a strong opioid medication, for several weeks. After the pancreatitis improved, the doctors carefully reduced the fentanyl dose, eventually stopping it completely. However, he experienced withdrawal symptoms, so they restarted the medication and continued to slowly decrease the dose until it was no longer needed."
215,"This was a 49-year-old woman with vulvar sarcoma who was irradiated with proton beam radiotherapy. Unfortunately, a very painful ulcer formed within the irradiation field one month after completion of treatment. Sustained-release oxycodone administration was started at 40 mg/day. The oxycodone dose was increased according to pain intensity, reaching 160 mg/day (MED 240 mg/day). The ulcer was treated with skin grafting and relieved the pain. The oxycodone dose was decreased every two days (). Mild restlessness occurred at 20 mg/day (12.5% of the maximum dose). Since the symptom was mild, the same dose was administered continuously and the restlessness disappeared after a few days. However, oxycodone 20 mg/day was continued for another two months before being reduced to 10 mg/day for 14 days and then discontinued. Six hours after discontinuing oxycodone, she developed cold sweats, malaise, and leg pain. Sustained-release oxycodone (10 mg/day) was restarted, and symptoms were relieved. This lasted for about five months because she was afraid of developing withdrawal symptoms. Final oxycodone discontinuation was achieved 323 days after the first onset of withdrawal symptoms.","This 49-year-old woman was treated for vulvar sarcoma with proton radiation therapy. After treatment, she developed a painful ulcer in the area where she received radiation, which required a skin graft to heal. To manage the pain, she took oxycodone, gradually increasing the dose over several months. After stopping the oxycodone, she experienced withdrawal symptoms, so it was restarted and carefully reduced over a long period. Eventually, she was able to stop taking oxycodone completely, but she was initially worried about experiencing withdrawal again."
216,"As home care doctors and general practitioners, we encountered a 37-year-old woman with ASD who lived with her parents in Japan. Her mother had end-stage breast cancer. We visited her house regularly to care for her mother. The woman with ASD was the main caregiver and a key person for her mother because her father was visually impaired. There were some obstacles we need to overcome together, but in the end, she was able to fulfill the role of the main caregiver for her mother.\nWhen we started home care for the mother, we did not have any knowledge that the daughter was a person with ASD. However, we occasionally heard about her from home visit nurses and home care staff because they worried about whether the mother could continue to stay at home due to the lack of care provided by family members. According to home care staff members, the woman with ASD was usually not present when the home care staff visited the home for her mother's care. They could not contact her or talk with her about her mother's condition. She focused on dishwashing and did not appear concerned about how her mother's medications or clothes were organized or whether the room was clean or not. She could not communicate well with home care staff even when she talked with them. Thus, we did not initially recognize that she was a person with ASD or another developmental disorder. We were wondering why she was having trouble managing the home care schedule and chores even though she worked as an engineer. The home care staff's distrust of the daughter caused irritation and stress in the daughter and the home care staff.\nAfter a few home care visits, with the daughter's consent, the mother's care manager informed us that she had been diagnosed with ASD in adulthood. Her parents had not been informed of the diagnosis. The daughter did not want to let her parents know about the diagnosis. The home care staff started to feel strongly that it was too difficult for the daughter to continue home care for the mother. The woman with ASD also felt difficulties in communicating with staff and understanding how her mother felt or how it feels to be severely ill. Therefore, we initiated consultation and social skill training in our outpatient clinic for the daughter. We discussed the traits of ASD as well as solutions or strategies to manage ASD traits in the home care setting. When we gave her instructions, we tried to make them more specific and provided examples so that she could understand them easily. For example, we suggested that she needed to come into her mother's room to share her mother's condition when the home care staff made a visit.\nAfter several consultations, she gradually obtained life skills in communication, scheduling, and managing things related to home care, such as expectations during home care staff visits or what she should do for her mother. Her psychological stress and caregiving burden decreased day by day. We also shared the fact that she was diagnosed with ASD with home care staff as well as appropriate attitudes, support, and communication styles. We shared her behavioral characteristics with staff, such as lower interest in sharing emotions with others or having difficulties in understanding social cues such as eye contact, facial expressions, and metaphors.\nFive months after the start of home-based care, her mother's condition worsened temporarily. The woman with ASD could not understand her mother's discomfort or pain and her father's sadness and serious feelings about the poor prognosis of his wife. She could not understand why her father was at a loss for words upon hearing the doctor's explanation of her mother's severe condition. Therefore, she asked him “Didn't you hear, Dad?” in a loud voice. These types of reactions by the daughter interfered with her father's acceptance of her mother's condition and he was unable to continue to listen the explanation. After this event, we explained why her father become silent while during the explanation of her mother's condition. We made a rule that we will tell her beforehand what we would say and how she should act when we share bad news. For example, we told her beforehand that we were going to tell her father about her mother's poor condition and prognosis and asked her to listen until we finished. We also told her how her father would react and our guess of the reasons for his reaction. Inappropriate behavior that is not suitable for the situation decreased afterward. She also felt relieved that she knew what she should do and could support her father as much as possible.\nSeven months after the start of home-based care, the mother passed away at home as her mother and she herself hoped. When her mother needed to use oral opioids and subcutaneous injection of opioids, the woman with ASD was able to communicate with homecare staff and ask for help to offer better care for her mother. Her mother died peacefully without any severe pain or discomfort. The woman with ASD did not become confused and was able to accept her mother's death peacefully with her father.","This woman, who has autism, was caring for her mother who was very ill with cancer. Initially, the home care team didn’t realize she had autism, and they struggled to communicate with her because of difficulties understanding social cues and expressing herself. As they learned more about her autism, they were able to work together to find ways to improve communication and support her role as a caregiver. Through counseling and training, she learned how to better understand her mother’s needs and how to communicate effectively with the home care team, ultimately allowing her to provide compassionate care during her mother’s final months."
217,"A 57-year-old man with NASH without cirrhosis presented to a satellite hospital with acute onset of painless hematochezia for 1 day. He had unprovoked deep venous thrombosis with a negative hypercoagulable workup and portal-vein thrombosis on Apixaban diagnosed 18 months prior to presentation. He was tachycardic and mildly hypotensive. There was no evidence of chronic liver disease on exam. The hemoglobin level was 130 g/L and subsequently decreased to 80 g/L, while the platelet count was 160 × 109/L. Laboratory tests revealed the following values: INR, 1.13; lactate, 1 mmol/L; albumin, 3.7 g/dl; and liver enzymes were within normal limits. Anticoagulation was discontinued and he was resuscitated using intravenous (IV) crystalloids. Esophagogastroduodenoscopy (EGD) revealed small non-bleeding esophageal varices. Colonoscopy revealed a complex ascending colonic varix with active bleeding ().\nHe was treated with IV octreotide at the satellite hospital at which he presented and then transferred to our tertiary institution for escalation of care. Liver-protocol computed tomography (CT) was performed for TIPS evaluation and revealed a steatotic liver and collateralized chronically thrombosed SMV with patent portal vein. He was deemed not to be a candidate for either TIPS or BRTO in the setting of chronic SMV thrombosis. Colonic resection was not recommended, since it was unlikely to prevent future bleeding with significant collaterals.\nHe continued to have hematochezia with Hgb nadir of 78 g/L. A trans-hepatic portogram performed by interventional radiology revealed an elevated absolute portal pressure of 18 mmHg. Digital subtraction images confirmed the site of the SMV occlusion at the confluence of two large SMV branches, in addition to retrograde flow through the large right CV. The occluded SMV was cannulated through a left patent branch using a 4-French angled glide catheter and a stiff Glidewire. A Cobra 2 catheter and a stiff Glidewire were used to cannulate the right SMV branch at the confluence. Recanalization of the occluded SMV () was achieved by using 5 × 40 mm balloon angioplasty to 5 mm, then two overlapping 10 × 40 mm self-expanding stents were successfully deployed into the origin of the right SMV branch, extending proximally into the portal splenic confluence. The stents were then post-dilated using balloon angioplasty to 8 and 10 mm. Follow-up venography showed antegrade flow in the right SMV branch into the SMV stent with a marked reduction in filling of the right CV (). The procedure was terminated after restoration of the SMV flow and the portal pressure was not measured post-procedurally. Liver biopsy showed steatohepatitis with stage 2/4 fibrosis. Apixaban and low-dose aspirin were started. Two months after discharge, the patient had no recurrent bleeding and CT showed patent SMV stents (). A plan for surveillance was determined using cross-sectional imaging every 6 months for 2 years.","This 57-year-old man was admitted to the hospital after experiencing bleeding in his lower intestines. He also had a history of blood clots in his veins and a liver condition called NASH. After tests, doctors found a problem with a small vein in his colon causing the bleeding, and they successfully reopened it with a procedure called stent placement. He is now being monitored with regular scans to ensure the vein remains open and he doesn't experience any further bleeding."
218,"A 63-year-old male presented on November 16, 2018, with complaints of persistent left epistaxis and a history of swelling in the left orbit and maxillary sinus for 3 months. Examination revealed multiple enlarged, indurated, and painless lymph nodes in the cervical and left submandibular areas, the largest of which was 4 mm × 3 mm. Blood tests at diagnosis showed anemia (Hb 122 g/L) and thrombocytopenia (90 × 109/L). Syphilis and HIV screenings were negative. Initial enhancement MRI scanning on November 27, 2018, revealed heterogeneous enhancement shadow filling in the left maxillary sinus along with adjacent bone absorption ().\nThe patient underwent a subsequent biopsy by nasal endoscopy on November 29, 2018. Histology highlighted a diffuse infiltrate of large atypical cells with lymphocytic or plasmacytoid morphology (). Neoplastic cells expressed a high proliferative index (Ki-67, 95%) (). Immunohistochemical profiling showed positive results in neoplastic cells for MUM1, CD38, CD138, c-myc, and EBV-EBER, the ratio of kappa chain (+) neoplastic cells to lambda chain (+) neoplastic cells is greater than 64:1 (), and they were negative for CD30, CD19, CD79a, PAX-5, CD20, CD21, bcl-6, CD10, bcl-2, CD15, HMB45, S-100, MelanA, CK, and EMA ().18F-FDG PET/CT was performed for further assessment of the mass lesion metabolic activity and the general conditions at diagnosis on December 6, 2018. Transverse PET/CT scan revealed a 3.7 cm × 3.9 cm × 4.3 cm solid mass, partial bone destruction, and swelling of soft tissues. A markedly FDG-avid space-occupying lesion was observed at the left maxillary sinus, and it invaded the left nasal cavity, left orbital apex, and left alar mandibular space (SUVmax, 7.8; SUVmean, 6.3) (). Bone marrow aspirate and trephine biopsy were negative for lymphoma involvement. The screening investigations specific for multiple myeloma or plasmacytoma results showed that this patient was negative for serum protein electrophoresis, immunofixation, and free light chain assay. The final diagnosis was confirmed to be consistent with stage II PBL.\nWith curative intent, bortezomib was initiated (2.3 mg d1, 4, 8, 11) in combination with CDOP (cyclophosphamide 1.3 g d2, epirubicin 40 mg d2, vindesine 4 mg d21, prednisolone 65 mg d2–6) chemotherapy regimen on a 21-day cycle. After two cycles of V-CDOP, enhanced MRI scanning on February 11, 2019, revealed an excellent initial response, with a substantial decrease in the size and intensity of nasopharyngeal lesions (). After three cycles of the V-CDOP regimen, re-evaluation of the 18F-FDG PET/CT in the transverse dimension on May 21, 2019, showed a diminished range of lesions to 3.8 cm × 2.9 cm × 3.8 cm and reduced 18F-FDG uptake in the left maxillary sinus (SUVmax, 7.0; SUVmean, 4.5) and other regions (SUVmax, 4.0; SUVmean, 3.6) (). The patient achieved a partial response (PR) after five cycles of V-CDOP chemotherapy treatment.\nThis planned treatment was followed by mobilization and collection of autologous peripheral blood stem cells on May 24, 2019. After one cycle of V-CDOP, enhanced MRI scanning revealed no significant change on June 27, 2019 (). On regular follow-up, he received local radiotherapy (RT) to nasopharyngeal regions (30 Gy in 15 fractions over 3 weeks). After RT, he was switched to two cycles of V-CDOP regimens supplemented with lenalidomide (25 mg d1–21) on August 9 and September 10, 2019. The main toxic side effect was grade 2 myelosuppression and improved after symptomatic treatment. Subsequent enhancement MRI scanning revealed a reduction in the size of nasopharyngeal lesions on September 14, 2019 (). The therapeutic assessment by FDG PET/CT on September 29, 2019, showed a complete metabolic response (cMR), a diminished range of lesions to 1.0 cm × 1.3 cm, significantly reduced 18F-FDG uptake in the left maxillary sinus (SUVmax, 3.5; SUVmean, 2.5), and a lack of metabolic activity in other regions ().\nThe patient received conditioning BEAM (BCNU, etoposide, cytarabine, and melphalan) high-dose chemotherapy with consolidative autologous hematopoietic stem cell transplantation (ASCT) in October 25, 2019. A total of 8.6 × 108/kg mononuclear cells (MNCs) and 2.1 × 106/kg CD34+ peripheral blood mononuclear cells (PBSCs) were reinfused, and complete engraftment was observed by day + 11. During autologous transplantation, he experienced grade 4 myelosuppression, requiring G-CSF. To consolidate the response, the patient received lenalidomide-based maintenance therapy after the transplant. A repeated enhancement MRI scanning showed increased abnormal signal shadows in the maxillary sinus, which was considered as an inflammatory reaction on May 6, 2020 (). He developed herpes zoster during maintenance treatment on October 2020. Follow-up PET-CT on March 10, 2021 showed continued cMR 16 months after ASCT (). Currently, the patient was monitored with serial MRI scanning and presented 32 months OS and 22 months PFS.","This man was diagnosed with a rare type of cancer called PBL that started in his sinus and spread to nearby areas. Initial tests showed unusual cells in his sinuses and lymph nodes, along with some changes in his blood. He received chemotherapy and radiation therapy, which successfully shrunk the cancer and eventually led to a complete remission. He is currently being monitored closely to ensure the cancer doesn't return."
219,"A 19-year-old man presented to our institute with right hand and foot dyskinesia. He had a significant family history of PKD. His uncle, grandfather, and grandfather's brother had involuntary movements triggered by voluntary movements. Proline-rich transmembrane protein 2 (PRRT2) on chromosome 16, which is associated with movement disorders including PKD (PRRT2-PxMD), was confirmed in his uncle and grandfather through genetic testing (c.649delC/pArg217-Glufs). All of them underwent stereotactic ablative surgery (Vo thalamotomy). The patient's grandfather and grandfather's brother experienced complete remission of involuntary movement attacks after the surgery. His uncle experienced a significant reduction in the frequency of daily attacks without medication. The patient first experienced paroxysmal involuntary left hand and toe flexion with left forearm pronation triggered by sudden voluntary movements at the age of 14. The frequency of attacks was 20–30 per day. Carbamazepine (100 mg/day) was prescribed, which led to a significant reduction in the frequency of attacks. However, drowsiness was significant after the intake of carbamazepine and significantly interfered with his daily life, especially school life. He underwent right-sided Vo thalamotomy at the age of 15, which resulted in complete resolution of PKD attacks immediately after the surgery. The detailed clinical course of right-sided Vo thalamotomy has been reported previously (). Four months after the thalamotomy, the patient developed right elbow, hand, and toe flexion (). The frequency of attacks was 10–20 per day. The duration of the attacks ranged from 10 to 20 seconds. Carbamazepine (100 mg/day) was prescribed again, and the PKD attacks reduced to 5–10 times per day. However, the drowsiness induced by carbamazepine severely interfered with his daily and school lives. He underwent left-sided Vo thalamotomy at the age of 19. The target coordinate of the left Vo nucleus was set the same as in the previous surgery (15-mm lateral, 2-mm posterior, and 1-mm superior to the midpoint of the anterior commissure-posterior commissure). A total of six lesions were created on the left Vo nucleus in the same manner as in the previous surgery. Immediately after surgery, the PKD attacks resolved completely. However, mild dysarthria developed, which spontaneously resolved within three months. Left-sided PKD attacks never developed six years after the right Vo thalamotomy, and right-sided PKD attacks never developed two years after the left Vo thalamotomy without medication. The locations of the coagulated lesions were confirmed using Brainlab Elements. Bilateral lesions covered the Vo and ventral intermediate (Vim) nuclei (). The Vo nucleus is located just anterior to the Vim nucleus of the thalamus. We included both anterior and posterior coagulated lesions in order to cover the entire Vo nucleus. Based on our experience, insufficient lesions to cover the Vo nucleus are likely to develop symptomatic recurrence. The time course of PKD onset and intervention is shown in . The data for this study were retrospectively collected and analyzed. Considering the observational nature of the study, the ethics committee of our institution approved this study, and patient consent was waived.","This 19-year-old man has a rare genetic condition called PRRT2-PxMD that causes involuntary movements, similar to what his family members have experienced. He had previously tried medication to manage his symptoms, but it made him too drowsy. After trying medication, he underwent surgery to target the thalamus, a part of the brain that controls movement, which successfully stopped the involuntary movements. He has been symptom-free for two years since the second surgery, and his doctors are confident that he will remain so."
220,"Case 1: a 46-year-old female with refractory pain in the cervical spine, the head, and the upper arm at the left side for two years with rising intensity. She had a high need for pain medication. The clinical examination revealed a reduced range of motion of the cervical spine and a sensible radicular syndrome corresponding to the nerve roots C4-C6. The laboratory tests showed no hint for tumor or infection. Radiographs, CT scan, and MRI revealed a tumor at the left side C3-C4 in the cervical spine () without further pathologies in the complete tumor workup (PET-CT, laboratory tests). The biopsy and additional reference pathology yielded the result of an aggressive osteoblastoma (Enneking Type III). Hence, in the interdisciplinary tumor board, the decision for complete wide resection was made. Radiation was considered in dependence on the final pathology after tumor resection. An unremarkable occlusion test of the left vertebral artery was performed in order to simulate a potentially necessary ligation.","This 46-year-old woman has been experiencing increasing pain in her neck, head, and left arm for two years. Tests revealed a tumor in her neck that was identified as an aggressive type of bone growth called osteoblastoma. The doctors decided to remove the entire tumor through surgery, and they carefully checked the blood vessels in her neck to ensure they wouldn't be affected. Now, they will determine the best course of action, which may include radiation therapy, after the tumor is completely removed."
221,"The first patient was a 44-year-old man with Ebstein's anomaly resulting in severe right atrial and right ventricular dilatation with right moderate ventricular dysfunction and severe tricuspid regurgitation. The patient also suffered from HIV immunodeficiency with previous cerebral infection and Kaposi's Sarcoma. In the post-operative period he suffered from severe right ventricular dysfunction with low cardiac output syndrome (LCOS), which required ECMO support.","This 44-year-old man has a heart condition called Ebstein's anomaly, which causes his heart to enlarge. He also has HIV and has had previous infections. After a recent surgery, his heart weakened significantly, leading to a life-threatening condition called low cardiac output syndrome. He needed a special machine called ECMO to help his heart function until it could recover."
222,"The second patient died was a 39-year-old female with univentricular heart (tricuspid atresia), who had had previously two cardiac surgeries (the last was atrio-pulmonary Fontan, 33 years earlier). The patient suffered from a serious right atriomegaly with frequent episodes of atrial tachycardia, so she was a candidate for Fontan conversion surgery. Pre-operative ventricular function was mildly reduced (50%). In the post-operative, the patient suffered from severe single ventricle dysfunction resulting in LCOS and the need for ECMO implantation. Both patients could not be weaned from ECMO due to multi-organ failure.\nSeven patients required pace-maker implantation due to post-operative sinus node dysfunction or atrioventricular conduction abnormalities. All patients were discharged on oral antiarrhythmic for 3–6 months and anticoagulants for 6 months. At discharge, 15 patients were in sinus rhythm, 5 had a stable pacemaker rhythm, 2 had atrial fibrillation, and 1 atrial flutter. One patient discharged in sinus rhythm had a pacemaker implant 5 months after the operation due to the presence of sinus node dysfunction.\nDuring a median follow-up of 14 months (IQR 7–27), there was no late mortality and 17/23 patients had an improvement of NYHA functional class. Five patients in NYHA III progressed to class II and 4 to class I; eight patients progressed from class II to class I.\nAt follow up electrocardiogram, 16 patients were in sinus rhythm, 6 with stable pacemaker rhythm, and 1 with permanent atrial fibrillation.\nRecurrence of arrhythmia occurred in 2/23 (8.6%) patients, more than 3 months after surgery. These patients presented at surgical ablation with history of atrial fibrillation lasting 4 and 19 years, respectively, and both had atrial fibrillation, which was treated with right-sided Maze rather than Cox maze III due to technical issues. Sixteen (69%) patients are in stable sinus rhythm, 12 without any anti-arrhythmic therapy. At median follow up of 14 months (IQR 7–27), freedom from recurrence of arrhythmia was 90.9% and cumulative risk of recurrence was 9.6% ().","This note describes the care of 23 patients who underwent a complex heart surgery for univentricular heart conditions. One patient sadly passed away after experiencing severe heart failure. The remaining patients showed improvement in their heart function over the following 14 months, with most achieving a stable rhythm and improved quality of life. While some patients needed a pacemaker or further treatment for recurring arrhythmias, the majority were able to return to a normal life with medication."
223,"On July 2008, a 59-year-old man, ex-smoker (45 packs/year), underwent upper right lung lobectomy and regional lymph adenectomy with the diagnosis of stage I (pT2, pN0) lung adenocarcinoma, solid pattern. On July 2020, the chest CT scan revealed the presence of an upper left lung lobe and two lower left lung lobe lesions. On August 2020, the patient underwent a wedge resection of the upper and the lower lung lobes lesions with N1 and N2 nodal sampling.\nGross examination of the surgical specimens of the first atypical pulmonary resection of left inferior lobe revealed an Intraparenchymal, peripheral, solid, yellow-white lesion measuring 1.4 cm in greatest dimension. Histological assessment identified a well-circumscribed lesion composed of two different morphological components, tightly adhered but not intermingled each other. The first component represented about 60% of the whole neoplasm and was characterized by a solid and trabecular proliferation of polygonal-shaped uniform tumor cells, with nuclei with finely granular chromatin and inconspicuocus nucleoli, consistent with carcinoid. Four mitosis/2 mm2 were identified, without tumoral necrosis. The second component, which represented about 40% of whole neoplasm, showed a main lepidic, non-mucinous pattern with secondary papillary architecture, corresponding to a lepidic-papillary pattern PA. Immunoreactivity for chromogranin A, synaptophysin, TTF-1, and pan-cytokeratins AE1–3 was documented in carcinoid component, while adenocarcinomatous component was positive only for TTF-1 and cytokeratins (). CK7 immunoreactivity was selectively documented in the adenocarcinomatous component. A final diagnosis of “combined pulmonary adenocarcinoma with atypical carcinoid” was made. No immunoreactivity for ALK and ROS1 was documented in both components. TPS for PD-L1 was <1% in both components. Both two other lesions on atypical pulmonary resections of the upper left lobe and inferior left lobe showed intraparenchymal neoplasm of 1.8 and 1.5 cm, respectively, corresponding both to PA with a main solid pattern and lepidic, non-mucinous second pattern. A positivity ROS1 score of 2+ with gene rearrangement on FISH was documented in the upper pulmonary lobe neoplasia. No immunoreactivity for ALK and a PD-L1 TPS of 1%–49% were observed. No immunoreactivity for ALK and ROS1 with PD-L1 TPS of >50% were documented in the lower lobe neoplasia. No pleural invasion was observed. No lymph nodes metastases were found. The final UICC 2017 stage pT4 (m-3) (PL0) pN0 was assigned.\nTargeted NGS analysis on Ion Torrent NGS platform using the Ocav3 panel was performed on DNA and RNA extracted from each microdissected histological components of the primary mixed neoplasm (I1/I2) and in the other two adenocarcinomas located in the inferior (I3) and superior (S1) left pulmonary lobes. Summary of genetic results are shown in \n. Among a total of 161 investigated genes included in the NGS panel, both common and private somatic genes mutations were identified in each entity (). The adenocarcinoma I1 and carcinoid I2 components of the mixed neoplasm shared the same mutations in BRAF (p.Gly466Ala), NF1 (p.Pro1359LeufsTer19 and p.Glu1928Ter), STK11 (p.Gly188AlafsTer99), and AKT2 (p.Leu52Ter) genes. Four additional somatic mutations were detected in the DDR2 (p.Arg806Ter), CDK6 (p.Thr107Ser), and SMARCA4 gene (p.Arg1135Gln) were identified in the adenocarcinoma component, whereas no specific mutations were identified in the carcinoid component. A different genetic profile emerged from the molecular analysis of the two additional adenocarcinoma nodules I3 and S1, lacking those somatic mutations identified in the mixed nodules, but shared two somatic missense mutations in the KRAS (p.Gly12Asp) and NOTCH1 (p.Pro498Arg). In addition, the I3 adenocarcinoma showed a specific, somatic mutations in ATM (p.Gln1117Ter), TP53 (p.Gly245Asp), and CDK12 (p.Arg44Trp) genes, whereas the S1 adenocarcinoma showed a specific, somatic, missense mutations in IDH2 gene (p.Arg172Ser).\nSince November 2020, considering the ROS1 positivity, the patient started crizotinib, which is still ongoing, reporting a stable disease.","Summary: This man has been battling lung cancer for several years. Initially, he was diagnosed with lung adenocarcinoma and had part of his lung removed in However, recently, new tumors were found in the remaining lung tissue. Further tests revealed that these tumors are a combination of adenocarcinoma and a type of carcinoid tumor. Genetic testing identified specific mutations in the cancer cells, and one of the tumors showed a positive result for ROS1, which led to the start of a new medication called crizotinib. Despite the treatment, the cancer has spread, and the patient is currently being monitored for any changes."
224,"On October 2016, a 66-year-old woman, never smoker, underwent upper left lung lobectomy and regional lymph adenectomy, for a pulmonary mass discovered on radiological examination for shoulder pain present for a long time.\nThe gross examination of the surgical specimens showed an intraparenchymal, subpleural, whitish mass, measuring 8.5 cm in greatest dimension. Histologically, a well-circumscribed neoplasia composed of two different morphological components, which were separated in some areas and intermingled in others, was documented. The first component represented about 70% of the tumor burden and consisted of an organoid proliferation of polygonal, shaped uniform tumor cells with nuclei with finely granular chromatin and inconspicuous nucleoli, consistent with carcinoid. Four mitosis/2 mm2 and diffuse, punctate necrosis were identified. The second component, representing about 30% of the tumor burden, showed a glandular architecture corresponding to acinar pattern of PA. Neoplastic infiltration of parietal pleura was documented (PL3). Immunoreactivity for chromogranin A, synaptophysin, TTF-1, and pan-cytokeratins (AE1-3 clone) was observed in carcinoid component, while glandular component was positive only for TTF-1 and cytokeratins; cytokeratin 7 was selectively expressed in the adenocarcinomatous component (). Metastatis constituted by both neuroendocrine and non-neuroendocrine component was found in four out of six hilar lymph nodes, showing the same immunoreactivity in different neoplastic areas such as primitive lesion (). Immunoreactivity for ALK and ROS1 resulted negative in both component, in primitive tumor and in lymph nodal metastasis. TPS for PD-L1 was <1% in both component, in primitive tumor and in lymph nodal metastasis. A final diagnosis of “combined pulmonary adenocarcinoma with atypical carcinoid” was made with stage pT3 (PL3) pN2 sec UICC 2017. Each neoplastic component in both primitive and metastatic lesions was individually microdissected from unstained FFPE slides for DNA and RNA extraction. Targeted NGS analysis on Ion Torrent NGS platform was performed using the Ocav3 panel.\nAmong the 161 investigated genes, both common and private genes mutations were identified in each investigated entity of primary and metastatic sites (). No shared point mutations, CNVs, or gene fusions were identified in both adenocarcinoma and carcinoid components from any sites (S1/S2 and mL1/mL2). By contrast, one somatic mutation in the PTEN gene (p.Thr319Ter) was detected specifically in the adenocarcinoma components of both primary and metastatic sites (S2 and mL2), whereas the carcinoid component of both sites shared one somatic mutation in the NF1 gene (p.Arg1325Thr). Primary carcinoid S1 showed one private somatic mutation in the CDK12 gene (p.Arg44Leu), whereas the adenocarcinoma component of metastatic site showed a private mutation in the NOTCH1 gene (p.Pro498Arg).\nOn December 2016, she progressed due to the onset of osteoblastic bone lesions detected by TAC with a negative octreoscan performed on January 2017. In February 2017, cisplatin plus pemetrexed regimen started for six cycles, reporting a partial response after four cycles. In November 2017, due to a massive bone metastasis with bone marrow infiltration, the patient died.","A 66-year-old woman had a lung mass removed in October 2016 due to shoulder pain. The tests showed it was a complex type of lung cancer, a combination of adenocarcinoma and atypical carcinoid, with some spread to nearby lymph nodes. Genetic testing revealed specific mutations in the cancer cells, which helped guide treatment decisions. After initial treatment with chemotherapy, the cancer spread to her bones, and she sadly passed away in November "
225,"We report a case of a 58-year-old African American male with a long history of scaly itchy feet. The patient presented to the clinic on February 8th, 2018 with an apparent inflammatory reaction on the plantar surface of both feet ( and ). An initial specimen collection from the patient’s plantar surface revealed hyphae indicative of a fungal infection under KOH preparation. This supported the diagnosis of a tinea pedis infection. The patient was treated with ketoconazole topical cream and 20% Urea creams to be applied daily. Follow-up approximately 6 weeks later revealed resolution of underlying fungal infection with minimal remaining inflammation ( and ). With consideration to the subject’s initial presentation of a possible exaggerated immune reaction caused by Trichophyton, we suspected an underlying allergic response (delayed-type hypersensitivity reaction) to the fungus, instead of a cutaneous fungal infection alone. In order to test this hypothesis, we subjected the patient to intradermal skin testing with intradermal Candida and Trichophyton allergens. On March 21, 2018, the patient underwent application of Candida, normal saline, and Trichophyton allergens on the left volar forearm ( and ).","This 58-year-old man came to the clinic because he had itchy, scaly feet. Tests showed he had a fungal infection on his feet, but the doctor suspected he might be having an allergic reaction to the fungus instead. To investigate this, the doctor performed skin tests that confirmed an allergy to the fungus. The patient was treated with medication to clear the fungal infection and relieve the itching, and the tests showed the infection had cleared up completely."
226,"A 70-year-old female with multiple medical comorbidities, including hypertension, end-stage renal disease (ESRD), and multiple prosthetic joints including a left total knee arthroplasty (TKA), presented to the emergency room with one-week history of left knee pain, erythema, and swelling. Prior to developing the symptoms in her knee, she suffered from loose stools and abdominal pain that had resolved by the time the patient developed pain in her knee. The patient denied any recent trauma, any rashes, or recent travel.\nPhysical examination was pertinent for tachycardia with a heart rate of 147 beats per minute, respiratory rate of 25 breaths per minute, temperature of 100.5 F, and oxygen saturation of 100% on room air. Blood pressure was 101/59 mmHg. The left knee was red, hot, and swollen. The range of motion of the left knee was restricted. The rest of her physical examination was unremarkable.\nOn laboratory work up, hemoglobin was 15.2 g/dL (13.5–17.5 g/dL); white blood cell (WBC) count, 14,900 cells/uL (4.5–11 k/uL), total bilirubin, 0.8 mg/dL (0.3–1.0 mg/dL); aspartate aminotransferase, 35 U/L (13–39 U/L); alanine aminotransferase, 33 U/L (4–33 U/L); alkaline phosphatase, 124 U/L (34–104 U/L); s. creatinine, 2.3 mg/dl; and sodium and potassium, within normal limits. Lateral and anterior-posterior view X-rays of the left knee joint showed prior total knee replacement but no other significant findings (Figures and ).\nThe initial differentials included septic arthritis of her prosthetic knee joint and reactive arthritis after her recent diarrhea. Gout and other rheumatologic conditions were less likely.\nA preliminary diagnosis of sepsis due to septic arthritis was made, and supportive management with intravenous (IV) fluids, empiric antibiotics (vancomycin and zosyn), and antipyretics was initiated. Arthrocentesis was performed, and purulent fluid was drained. This was sent for Gram staining and cultures. Gram staining was significant for numerous WBCs and Gram-negative coccobacilli. The culture came back the next day growing Gram-positive coccobacilli that were subsequently identified as Yersinia enterocolitis. This was sensitive to all the antibiotics it was tested against. The blood cultures came back positive for Yersinia enterocolitica too. Yersinia enterocolitica was identified as the cause of the septic arthritis, which was likely the cause of her diarrhea prior to presenting.\nThe patient was seen by orthopedic surgery in consultation and underwent resection of her left TKA and placement of a biodegradable implant without any complications. The patient was also seen by infectious disease in consultation, and based on their recommendations, the patient was discharged home to complete 6 weeks of outpatient IV antibiotics in the form of 2 g of ceftriaxone daily considering her sepsis and prosthetic material in her knee joint. The patient completed this without any complications.\nThe patient was seen on follow-up, and her infection resolved completely. She has not had a recurrence or reinfection of her knee after over a year of follow-up.","This 70-year-old woman came to the hospital with a painful, swollen knee that had been bothering her for a week. She also had a history of diarrhea and abdominal pain that had recently resolved. After tests, doctors found that she had a serious infection in her knee joint, likely caused by a bacteria called Yersinia enterocolitis. They treated her with antibiotics and removed the infected knee replacement, and she has been doing well for over a year."
227,"A 73-year-old male with a past medical history of chronic obstructive pulmonary disease, hypertension, and cerebrovascular accident with a right-sided deficit and speech deficit presented in a somnolent state to the emergency room. The patient reportedly had generalized body aches, dyspnea, and cough, which had been progressively worsening over the past two to three days. The patient tested positive for SARS-CoV-2.\nThe patient experienced a prolonged hospital course, remaining in the hospital for approximately three months. A brief overview of the first month of hospitalization is given as follows: the patient was started on dexamethasone, azithromycin, and ceftriaxone at admission. His condition was complicated by a gastrointestinal bleed requiring multiple blood transfusions and a pulmonary embolism. The pulmonary embolism could not be adequately treated with anticoagulation due to his gastrointestinal bleed. His respiratory status declined, due to COVID-19, necessitating intubation which the patient required for most of his hospital stay. Broad-spectrum antibiotics were continued for the majority of his hospital course, switching to vancomycin, piperacillin-tazobactam, and levofloxacin to cover for ventilator-associated pneumonia. The patient required two courses of triple antibiotic therapy to cover for ventilator-associated pneumonia due to persistent leukocytosis and a chest X-ray significant for signs of infiltrates which remained following his initial treatment course for ventilator-associated pneumonia.\nDuring the second month of his hospital stay, after his initial antibiotics courses had been completed, due to worsening respiratory status, sputum cultures were performed on hospital day (HD) # 26 which revealed Stenotrophomonas maltophilia and Citrobacter koseri, which were treated with trimethoprim-sulfamethoxazole (). Due to his gastrointestinal bleed, the patient underwent an esophagoduodenoscopy and a colonoscopy. Esophagoduodenoscopy revealed mild antral gastritis with a deformed duodenal bulb as well as some mild diverticulosis. Colonoscopy revealed a large ascending-colon mass. Two biopsy samples were taken of this colon mass, which returned consistent with tubular adenoma without cytologic atypia. However, it was suggested by the pathologist that if this biopsy material was part of a larger lesion, a more advanced malignant lesion could not be excluded. The patient was too unstable during his hospitalization to undergo resection of the mass. On HD # 38, the patient received a tracheostomy which he tolerated well without complications. On HD # 49, the patient experienced a febrile episode (maximum temperature of 101.2 °F). At this time, the patient had both a Foley catheter and an internal jugular central line in place (he required both of these for the majority of his hospital course). Urinalysis, urine cultures, blood cultures, and a chest X-ray were ordered on the same day (). Urinalysis revealed 38 red blood cells per High Powered Field (HPF), 13 white blood cells/HPF, 2+ bacteria, many budding yeast, and 3+ hyaline casts (). Due to the bacteria and yeast in his urine, the Foley catheter was replaced the same day. Leukocytosis was only mild (10.6 cells per cubic millimeter of blood), but continued to increase to 15 cells per cubic millimeter of blood over the ensuing four days (). The urine culture that resulted on HD # 53 revealed T. asahii. Isolates of T. asahii were obtained by performing a culture on a TSA blood agar plate with 5% sheep blood. The isolates were incubated at 35°C in an incubator at an ambient atmosphere for 24 hours. Isolates were identified using the VITEK 2 system (bioMérieux, Inc., Hazelwood, MO). Although the urinalysis had shown 2+ bacteria, no bacterial growth was seen in the urine culture. Blood culture revealed no growth after five days. Serum beta-D glucan was negative. The urine culture was sent to an outside laboratory (Quest Diagnostics Nichols Institute, Chantilly, VA) to determine sensitivities.\nAt Quest Diagnostics, the isolates were reincubated on Sabouraud Dextrose Agar (SDA) overnight. The inoculum was prepared in sterile demineralized water and standardized to a 0.5 McFarland. Twenty microliters of the suspension was added to a tube with 11 mL of Sensititre YeastOne Inoculum Broth and then prepared by inoculating 100 uL per well. In vitro advanced fungal susceptibility testing was performed using anidulafungin, micafungin, caspofungin, 5-flucytosine, posaconazole, voriconazole, itraconazole, fluconazole, and amphotericin B. Trichosporon organisms were tested with these agents using broth microdilution according to the Clinical and Laboratory Standards Institute (CLSI). The stock solutions purchased by Quest Diagnostics are prediluted (information regarding dilution preparation is not available). Isolates were identified using the Sensititre Trek YeastOne panel (Trek Diagnostic Systems, Cat. No. YO-9), which is a broth microdilution panel. The manufacturer's instructions were followed for setting up, reading, and analyzing the assay. Plates were incubated at 33–35°C for 24 hours minimum. The Sensititre system monitors for colorimetric change. MIC values were determined by demonstrating the lowest concentration of drug that does not demonstrate a color change. CLSI document M27 was used for the interpretation of results.\nPending sensitivity results, a repeat urine culture was ordered (HD # 53). On HD # 58, the patient again experienced a febrile episode, with a maximum temperature of 103.0°F accompanied by a leukocyte increase from 10 to 15 cells per cubic millimeter of blood (). On HD # 58, the repeat urine culture results confirmed the presence of T. asahii. Again, no bacterial growth was seen in the urine culture. Antifungal medication had not been started initially as there was concern that the initial urine culture obtained on HD # 49 had revealed a contaminant or was reflective of asymptomatic colonization. However, with the repeat urine culture revealing T. asahii, the febrile episode, and leukocyte increase despite broad-spectrum antibiotics, antifungal coverage was deemed necessary. The patient was started on empiric fluconazole on HD # 58, and his Foley catheter was again replaced that same day (). A tracheal aspirate culture was ordered which returned results on HD # 63 revealing Stenotrophomonas maltophilia. Treatment with trimethoprim-sulfamethoxazole was again started (). On HD# 65, the sensitivities for the urine culture (sent to an outside laboratory on HD # 52) returned which revealed nonsusceptibility to fluconazole (MIC 2 mcg/mL), resistance to caspofungin and micafungin (MIC > 8 mcg/mL), and sensitivity to voriconazole (MIC 0.03 mcg/mL). Based on the advanced fungal testing, voriconazole was started on HD # 65 (following seven days of fluconazole) per infectious disease recommendations and continued for seven days (). The patient had remained afebrile with improved leukocytosis since starting fluconazole (white blood cell count decreased from 15 cells per cubic millimeter of blood to 9 cells per cubic millimeter of blood). With the initiation of the voriconazole, the leukocyte count continued to drop to normal levels (4.40 cells per cubic millimeter of blood).\nA percutaneous endoscopic gastrostomy tube was placed on HD # 78, and the patient tolerated the procedure well without complication. Urine cultures were obtained on HD # 78 and revealed 10,000 CFU/mL of usual skin flora. T. asahii was no longer present in urine (). At this point, the patient was accepted and transferred to a long-term care facility on HD # 80. At this long-term care facility, multiple attempts failed to fit the patient with an aerosolized tracheostomy collar. A decision was made to initiate palliative care. On HD # 44 at the long-term care facility, he was found without spontaneous respirations or palpable pulse.","This 73-year-old man had a very difficult three-month hospital stay after being admitted with breathing problems, fever, and abdominal pain. He also had a serious infection in his urinary tract and a lung infection, and he needed multiple antibiotics and a breathing tube to help him breathe. Despite these treatments, he continued to struggle, and doctors eventually had to place a feeding tube to help him get enough nutrition. Eventually, he was transferred to a long-term care facility where he passed away."
228,"A 33-year-old male presented to our center with chief complaints of profuse per rectal bleed mixed with stool for three days that was associated with easy fatigability for one week prior to the initial presentation. He also had one episode of black tarry stool. However, he had no complaints of blood in vomit, purpuric rashes, or petechiae. He also had no hematuria, weight loss, night sweats, evening rise of temperature or loss of appetite, cough, chest pain, dyspnea, palpitation, limb edema, loose stools, jaundice, and abdominal distension. Bleeding was absent from other orifices. He had no history of diabetes mellitus, hypertension, cardiac diseases, and pulmonary tuberculosis in the past. He consumed 80 grams of alcohol per day for 15 years, but he did not smoke.\nOn examination, he was ill looking, conscious, and was well oriented to time place and person. He had pallor and was dehydrated. However, he had no icterus, clubbing, cyanosis, or edema. His pulse rate was 110 beats/minute, blood pressure was 80/60 mm of Hg, body temperature was 98°F (36.6°C), respiratory rate was 19 breaths/minute, and oxygen saturation was 95% in room air. The digital rectal examination showed fresh blood over the examining finger and otherwise normal findings. Abdominal and cardiac examination was normal.\nLaboratory investigations showed hemoglobin 10.8 g/dl and hematocrit 31.6%. The total leukocyte count was 11510/mm3, neutrophils were 78%, and platelet count was 291000/mm3. The prothrombin time was 14 seconds, and the International normalized ratio was 1.08. The albumin level in the blood was 2.4 gm/dl, and total protein was 6.1 gm/dl, total and direct bilirubin were 0.7 and 0.1 mg/dl in the blood. Alanine aminotransferase and aspartate aminotransferase level was 97 U/L and 114 U/L, respectively. HbA1C level in the blood was 7.2. The level of urea (33 mg/dl), creatinine (1.1 mg/dl), sodium (132 mEq/l), and potassium (3.6 mEq/l) were within normal range. Traces of sugar and albumin (++) were present in the urine examination. The fecal occult blood was found in the stool. Mycobacterium tuberculosis was not detected in sputum in the acid-fast bacilli (AFB) stain.\nThe chest X-ray was normal. Ultrasonography (USG) of the abdomen and pelvis and upper gastrointestinal endoscopy showed normal findings. A Computed Tomography (CT) scan of chest and abdomen showed asymmetric circumferential thickening in the ileocaecal region with lobulated thickened caecum, soft-tissue stranding and necrotic mesenteric lymphadenopathy, and indeterminate lobule in the lung (). Colonoscopy showed multiple transverse ulcers with overlying exudates in terminal ileum and ascending and transverse colon. Histopathological examination of the ileum and colon showed patchy ulcers with exudates, granulation tissue, fibrosis, deep lymphoplasmocytic inflammation, and crypt regenerative changes with fibrinoid changes in scattered capillaries and venules and was inconclusive. However, gene XPERT was positive for Mycobacterium tuberculosis.\nHe was diagnosed with ileocaecal tuberculosis. The hemoglobin level dropped to 6.1 g/dl after two days of admission, and he had an episode of weakness associated with profuse sweating and rigor. Fluid resuscitation followed by two pints of whole blood transfusion was performed. After that, his hemoglobin was increased to 10.2 mg/dl. He was managed with four antitubercular drugs and showed significant improvement. Following this, he was discharged on oral antitubercular medications (isoniazid, rifampicin, pyrazinamide, ethambutol, and pyridoxine) after 10 days of admission. On follow-up after two weeks, he was responding well to antitubercular medications with improved symptoms, i.e., no per rectal bleeding, and his liver function test was within the normal range.","This 33-year-old man came to the hospital because he was experiencing significant rectal bleeding and feeling very tired. He also had one episode of black stool and noticed some blood in his stool. After examination and tests, including a colonoscopy, doctors found evidence of tuberculosis in his colon. He was treated with medication and his condition improved, and he was discharged home with a plan to continue taking his medications and follow up with his doctor."
229,"We present the case of a 46 year-old-female, never smoker, with a history of ulcerative colitis who initially presented as an outpatient for the evaluation of persistent cough, wheezing, and chest tightness for 6 months. Her review of systems was otherwise negative and her only medication included mesalamine for ulcerative colitis. She had no history of environmental or occupational exposures and denied any allergies. She denied any personal or family history of lung disease. Her vital signs were within normal limits. This patient was evaluated by pulmonology and was diagnosed with cough variant asthma and was started on montelukast and albuterol as needed. A northeast allergy panel was unremarkable. She subsequently presented to the emergency room with an episode of wheezing and chest tightness and Computed Tomography Angiogram (CTA) revealed multiple lung nodules with mosaic attenuation. The nodules were seen bilaterally in clusters, with the largest measuring up to 1.8 cm in the right middle lobe. Multiple serologic markers were ordered to screen for autoimmune disease including ANA, ANCA, anti-dsDNA, hypersensitivity pneumonitis panel, Sjogren's antibodies, angiotensin converting enzyme, and rheumatoid factor. These were unremarkable other than a positive ANA with titer 1 : 640 homogenous pattern. Due to uncontrolled symptoms, her inhaler regime was escalated to Flovent twice daily with as needed albuterol. A repeat CT chest 3 months later indicated no change in diffuse mosaicism and multiple pulmonary nodules, similar in size, with the largest 1.8 cm in the right middle lobe (Figures –). She then underwent robotic-assisted navigational bronchoscopy with fine needle aspiration, brushing, and transbronchial biopsy of the right middle lobe nodule (Figures and). Pathology from the nodule was positive for groups of bland appearing small blue cells, consistent with low-grade neuroendocrine tumor (carcinoid). Pathology was positive for chromogranin and synaptophysin, neuroendocrine markers, and low Ki-67 positivity, indicating low rate of proliferation (Figures –). Endobronchial ultrasound was also performed and Level 7 lymph node was negative for malignancy. The patient was presented at multidisciplinary chest tumor conference and it was recommended that she undergo PET CT scan and surgical resection of the biopsy-proven typical carcinoid tumor in the right middle lobe.","A 46-year-old woman with a history of inflammatory bowel disease was seen for a persistent cough and breathing problems. After tests, she was diagnosed with cough variant asthma and started on medication, but her symptoms continued to worsen. A CT scan revealed several small nodules in her lungs, and further tests showed they were a type of slow-growing cancer. She is now scheduled for additional scans and potentially surgery to remove the tumor, and will be discussed at a multidisciplinary tumor conference."
230,"A 35-year-old female presented to the Emergency Department (ED) for evaluation of 3 months of worsening exertional dyspnea and bilateral lower extremity edema. The patient initially noted mild dyspnea on exertion, which gradually progressed to the point of being unable to climb a single flight of stairs without stopping to rest. The patient endorsed a nonproductive cough, pleuritic chest pain, occasional orthopnea, and an unintentional 20-pound (9.1 kg) weight loss over a one-month period. She denied any associated fevers, chills, or night sweats; had no nausea or vomiting; and had no easy bruising or bleeding. The patient denied any other recent illness and also denied any significant exposures or risk factors for tuberculosis. She had no significant past medical history including any previous cardiac pathology, thromboembolic disease, structural heart disease, indwelling catheters, or asthma. She denied any current or prior intravenous drug use (IVDU). Family and surgical history was also noncontributory.\nPhysical exam demonstrated a nontoxic appearing patient sitting comfortably in bed. Vital signs included a temperature of 98.1°F (36.7°C) with mild tachycardia at 109 beats per minute, blood pressure of 107/64 mmHg, and respiratory rate of 20 breaths per minute. Pulmonary examination revealed lungs clear to auscultation bilaterally without adventitious sounds or retractions; however, the patient was only able to speak in 4–5-word sentences with effortless tachypnea and no accessory muscle use. Cardiac examination demonstrated mild tachycardia without murmurs, rubs, or gallops; no jugular venous distention; and no carotid bruits. Extremity examination was notable for symmetric 2+ pitting edema to the midshin of both lower extremities. Skin exam revealed no purpura, Osler nodes, Janeway lesions, splinter hemorrhages, or track marks. Abdominal exam was nontender, and neurological examination was nonfocal.\nMultiple laboratory studies including 2 sets of blood cultures were obtained () and remarkable only for mild hyponatremia with a sodium of 127 mEq/L, a chloride of 90 mEq/L, and mild anemia with a hemoglobin of 8.5 g/dL. There was no leukocytosis, troponin was undetectable, and brain-natriuretic peptide was within the normal range. These laboratory findings were not suggestive of any particular pathological processes. The finding of anemia was noted, but the lack of any corresponding elevation in bilirubin made acute hemolysis less likely. The hyponatremia was felt to be consistent with the patient's hypervolemic clinical picture. The lack of leukocytosis was noted, though limited inferences can be made from this value in isolation. Overall, the laboratory results made acute decompensated congestive heart failure and bacterial pneumonia lower on the differential.\nA 12-lead electrocardiogram revealed sinus tachycardia with normal axis and intervals, with no acute injury pattern and no evidence of right-heart strain. A chest radiograph was obtained () and was interpreted by the radiologist as demonstrating “bilateral patchy atelectasis”—however, the emergency physicians felt it demonstrated a pleural-based wedge-like consolidation in the right inferior lobe consistent with Hampton's Hump. The initial differential considered by the providers included pulmonary embolus, pneumonia, congestive heart failure, pulmonary hypertension, cardiomyopathy, and infectious endocarditis. At this time, a third set of blood cultures were added to the patient's workup.\nA limited bedside transthoracic echocardiogram (Figures and ) in the ED revealed findings concerning for tricuspid valve vegetations as well as a slightly enlarged right ventricle without any D-sign (i.e., a D-shaped left ventricle on parasternal short-axis view, consistent with right ventricular strain causing shift of the ventricular septum). Cardiology was consulted for concern for potential IE. A CT-angiogram (CTA) of the chest was ordered to assess for pulmonary embolism and further characterize the pulmonary lesions seen on radiography, and the patient received intravenous (IV) ceftriaxone and azithromycin to cover possible community-acquired pneumonia while the CTA was pending. The CTA revealed findings consistent with multiple septic emboli and mycotic aneurysms (Figures and ), as well as multiple segmental pulmonary arterial occlusions with associated infarcts or hemorrhage. A stat formal echocardiogram was obtained and interpreted at bedside by the cardiology fellow, confirming the presence of tricuspid vegetations as well as moderate tricuspid regurgitation, with no evidence of right ventricular strain.\nThe patient was treated with IV vancomycin, gentamycin, and ciprofloxacin to provide coverage for possible MRSA endocarditis, due to the concern for possible undisclosed IVDU, and was admitted with consultation of Cardiothoracic Surgery and Infectious Disease (ID).\nThe patient was maintained on IV gentamycin and vancomycin and underwent a transesophageal echocardiogram (TEE) on hospital day 2 which revealed a large 2.0 × 2.1 cm posterior leaflet tricuspid valve vegetation with severe tricuspid regurgitation. On hospital day 5, the patient admitted to a history of heroin IVDU but was not forthcoming about the date of her most recent use, and a urine toxicologic screen was negative. The patient underwent a tricuspid valve annuloplasty and reconstruction using autologous pericardial tissue by Cardiothoracic Surgery on hospital day 8. The patient's blood cultures remained negative on hospital day 9, at which time ID recommended the patient receive ceftriaxone 2 g IV daily for 6 weeks. Pathology of the valve leaflets resulted on hospital day 10 and indicated granulation tissue with focal areas of bacterial colonization without bacterial identification. The remainder of the patient's postoperative course was uncomplicated, and on hospital day 15, she was transferred in good condition to a skilled nursing facility to complete her 6-week course of IV ceftriaxone. The blood cultures remained negative, and the causative pathogen was not identified.","This 35-year-old woman has been struggling with shortness of breath and swelling in her legs for the past three months. She also has a persistent cough and has lost weight. Initial tests showed her heart and lungs were not functioning properly, and blood cultures were negative. Further investigation revealed septic emboli and a tricuspid valve infection, likely related to a history of intravenous drug use. She underwent surgery to repair her valve and is now receiving antibiotics to complete her treatment."
231,"We present a 5-year-old female child who was symptomatic since the early neonatal period with skin lesions, intermittent painless vaginal bleeding, and breast enlargement. For these complaints, the parents took the child to the nearby health facility, but they were reassured. Compared to her peers, her growth in length was fast since her early childhood, but she had poor weight gain. At the age of 3 ½ years, she presented to an orthopedic clinic with bowlegs for which she was seen and sent home without any intervention. But after one week, she had a trivial fall down accident, and she sustained pathological fractures on both upper and lower extremities. Plaster of Paris (POP) cast was applied for the lower left arm, and open fixation with plate was done for the left femur.\nDuring the procedure, tachycardia was detected, for which she was investigated and diagnosed to have hyperthyroidism. She was initially put on propylthiouracil (PTU) and propranolol. After eight months of the procedure, there was displacement of the plate. The orthopedic surgeon decided to revise the operation, but the thyroid function was not controlled for which she was referred to a paediatric endocrinology clinic for better management of hyperthyroidism.\nOn physical examination at the paediatric endocrinology clinic, she was emaciated. Her weight was 16 kg (between 10th and 25th percentiles) and her height was 115 cm (on the 95th percentiles). Weight for height was far less than 5th percentile (underweight), based on CDC growth charts. Her pulse rate was 123 bpm, and she had protruded eyes. CAL spots were noticed on her face, neck, and trunk (). There was a 5 cm by 3 cm anterior neck mass with an irregular surface (). There was also breast enlargement. She had a grade III early systolic murmur best heard at the left upper sternal border. There was swelling and tenderness at the right midshaft of the humerus and short POP on the left lower arm. She had a wide gait. Based on her clinical findings, she was diagnosed to have MAS.","This 5-year-old girl has been experiencing several unusual symptoms since she was a baby, including skin problems, bleeding, and breast growth that is ahead of schedule. She also grew very quickly but didn't gain weight as well as other children, and later developed bowlegs and fractures in her legs. Doctors discovered she had an overactive thyroid, which was causing these problems, and she was treated with medication. Now, she is very thin and has a noticeable neck mass, and her doctors are continuing to work on managing her thyroid condition to help her grow and develop properly."
232,"A 45-year-old obese man (height, 178.7 cm; weight, 97 Kg; body mass index, 30.8 kg/m2) complained of general fatigue and drowsiness at work. A blood examination revealed severe diabetes mellitus (HbA1c 10.6%), and he was accordingly referred to our university. Treatment for diabetes mellitus was started and extensive evaluations for sleep apnea syndrome were performed. Although treatment using a continuous positive airway pressure mask was initiated, general fatigue continued. Concurrently, SITSH was diagnosed based on the following findings: serum TSH, 6.890 µIU/mL; free T3, 4.9 pg/mL; and free T4, 2.29 ng/dL.\nMagnetic resonance imaging of the pituitary gland revealed a poorly enhanced mass measuring 5 × 6 × 8 mm ( and ). The TRH loading test showed a low and delayed TSH response (pre-TSH, 6.89 µIU/mL; max TSH, 10.8 µIU/mL; 60 minutes after TRH loading). However, there were no abnormal responses for both GH and PRL on several other loading tests. The absence of a family history of SITSH or TRβ gene mutations prompted the diagnosis of thyrotroph adenoma.\nInitial treatment with the somatostatin analog (SSA) did not yield any response. Further, the free T4 levels remained over 2 ng/dL after 3 courses of lanreotide autogel (90 mg). Since his diabetes mellitus was already under control, we decided to remove the tumor surgically.\nThe surgery was performed using the standard endoscopic endonasal transsphenoidal approach. The pituitary gland appeared normal on the surface. However, a midline split revealed a well-circumscribed whitish tumor inside the pituitary gland (). Complete tumor resection was achieved (), and tumor tissues were collected wherever possible. As the intraoperative pathological diagnosis ruled out a pituitary adenoma, tissue samples for electron microscopy were obtained.\nHis serum TSH levels decreased drastically to 0.320 µIU/mL on postoperative day 1. However, the patient developed transient diabetes insipidus that was treated with oral desmopressin acetate tablets. Three days after the operation, he was diagnosed with influenza type A, followed by hyponatremia with a minimum serum Na level of 118 mEq/L. Electrolyte levels were closely monitored during the treatment for influenza, and he was discharged 18 days after the operation without any electrolyte management. Oral administration of hydrocortisone and levothyroxine was started after the operation, which was tapered and ceased completely after 3 months.\nThe patient’s complaint of fatigue improved, and his cardiac heart rate was reduced by 10 bpm. Blood examination 6 months after the operation revealed an improvement in endocrinological parameters, including serum TSH (0.808 µIU/mL), free T3 (2.9 pg/mL), and free T4 (1.35 ng/dL). The TRH loading test showed a normal TSH response (pre-TSH, 0.589 µIU/mL; max TSH, 7.830 µIU/mL) 30 minutes after TRH loading. The patient has remained healthy for 2 years without tumor recurrence.\nPostoperative MRI revealed complete tumor resection. Hematoxylin and eosin staining showed that the tumor was composed of small- to large-sized neuronal or ganglionic cells containing abundant acidophilic cytoplasm and nuclei with a prominent nucleolus against a background of fine, fibrillar, neuropil-like matrix ( and ), which were diffusely immunoreactive to synaptophysin, chromogranin A, neurofilament, and NCAM (CD56), and partially immunoreactive to NeuN (). Interjacent small cells were considered to be reactive lymphocytes and not adenomatous cells based on their immunoreactivity to CD3. The cytoplasm of the tumor cells with peripheral displacement of the nucleus was immunoreactive for low-molecular-weight keratins, CAM 5.2, and patchy reactive for CK7 ( and ), but not for CK5/6, CK 8, CK 20, and CK 34βE12, supporting the exclusion of paragangliomas. Further, the absence of immunoreactivity to glial fibrillary acidic protein confirmed the exclusion of ganglioglioma (). Although Ki-67 staining revealed a labeling index of 2.6%, no other atypical features were detected. Only 0.4% of the entire tumor showed P53 immunopositivity. Based on these findings, a final pathological diagnosis of isolated GC was made.\nWith respect to the pituitary and hypothalamic hormones, the cytoplasm of the tumor cells showed diffuse but strong immunoreactivity for TSH () and equivocal faint positivity for gonadotropin; however, it was negative for GH, PRL, and ACTH. Meanwhile, it also showed diffuse but strong immunoreactivity to TRH (). Transcription factors such as the acidophilic cell lineage transcription factor Pit-1, SF-1, Tpit, ER, and GATA-2 are important to pituitary cytodifferentiation from the Rathke pouch stem cell. Further immunohistochemical studies for these transcription factors revealed diffuse and strong nuclear immunoreactivity for Pit-1 and GATA-2 ( and ), but not for SF-1, Tpit, and ER, confirming thyrotropic cell differentiation of tumor cells. Thyroid transcription factor-1 (TTF-1) is a tissue-specific transcription factor that regulates the expression of selected genes in the thyroid, lung, and diencephalon for embryonic development and differentiation; it is well known that it aids in nuclear expression, specifically that of posterior pituitary, thyroid, and lung tumors (, ). All tumor cells stained negatively for TTF-1. Somatostatin receptor (SSTR) status may predict treatment response to first- and second-generation SSAs. With respect to SSTR membranous immunopositivity proposed by Volante et al (), more than 50% of tumor cells stained positively for SSTR2 (Score 3 ()); however, none of the cells stained positively for SSTR5 (Score 0).\nBased on these immunohistochemical findings, this tumor was considered to be differentiated into an entirely neuronal lineage rather than mixed or interjacent with 2 components. Electron microscopy revealed the tumor cell has a light nucleus with a prominent nucleolus, which is surrounded by many secretary granules, synaptic vesicles, and some lysosomes in the cytoplasm. Typical neuronal processes contained both dense core vesicles and clear vesicles ().","This patient has been dealing with several health issues, including diabetes and fatigue. After a series of tests, doctors discovered a small tumor in his pituitary gland that was causing problems with his thyroid hormone levels. The tumor was successfully removed through surgery, and the patient’s fatigue has improved. However, he experienced some temporary side effects, such as low sodium levels and a temporary loss of the ability to regulate his fluid balance. Following the surgery, doctors monitored his hormone levels and found that the tumor was completely gone. He is now doing well and has been closely followed for two years with no signs of recurrence."
233,"A 28-year-old nonbinary individual presumed female at birth has recently commenced full masculinizing hormone therapy with transdermal testosterone gel. You receive a referral from their primary care physician concerned about polycythemia. Their hemoglobin is 168 g/L with hematocrit 0.49, which has been flagged in the laboratory report as high (reported with female reference range of 115-155g/L and 0.33-0.45 relative to the male reference interval of 120-170g/L and 0.36-0.50).\nAndrogens are known to stimulate erythropoiesis while the impact of estrogens are not as well understood. In trans people who have been on established and full-dose feminizing hormone therapy (estradiol and anti-androgen) for at least 6 months, there is a significant decrease in hemoglobin, hematocrit, and red blood cell count to the female reference range (, ). Conversely after 6 months of masculinizing testosterone therapy, trans people demonstrate an increase in hemoglobin, hematocrit, and red blood cell count to the male reference range (, , , ). Serum hematocrit in the range of the affirmed gender is evident from 3 months after commencing gender-affirming hormone therapy (). Of note, there are association studies suggesting higher hematocrit is associated with a higher risk of cardiovascular disease (, ). This is probably a consideration for people using masculinizing hormone therapy, more so than those using feminizing hormone therapy. As smoking may additionally increase hematocrit, smoking cessation should be emphasized in those with elevated hematocrit. While the long-term cardiovascular implications of using a different reference range for hemoglobin or hematocrit are unclear in general, reference ranges of the affirmed gender should be used. Female reference ranges should be used for someone taking gender-affirming feminizing hormone therapy and male reference ranges should be used for people using masculinizing hormone therapy.\nIn trans women, there is a small statistically significant but clinically insignificant rise in platelet count (which remains within the normal reference range) shown in several cohort studies after 6 to 12 months of feminizing hormone treatment (, ), while white blood cells do not change significantly. No apparent changes are observed in either platelet count or white blood cells with masculinizing hormone therapy (, ).\nCase 1 outlines a nonbinary individual presumed female at birth receiving full-dose masculinizing hormone therapy. In this case, the male reference range for hemoglobin and hematocrit would be most appropriate and this should be shared with the nonbinary individual so they are aware and can expect to be misgendered when reviewing their own results. As such, their hemoglobin of 168 g/L and hematocrit of 0.49 would fall within the expected reference range and no change in management needs to occur.\nReference ranges for serum ferritin, a common indicator of body iron status, vary depending on age and sex (). Ferritin reference ranges are typically lowest in premenopausal people presumed female at birth, followed by postmenopausal people and are highest in people presumed male at birth, with lower limits of the female reference range approximately 10 to 20 ug/L below that of the male reference range (30 ug/L) (). This may be partially attributed to increased iron utilization in menstruating individuals resulting in lower ferritin, as well as a multitude of factors that have been shown to impact upon adult serum ferritin levels including age, body mass index, waist to hip ratio, and liver function (, ). Animal studies suggest that iron is distributed differently in males and females associated with differences in hepatic hepcidin expression rather than sex-steroid concentrations (, ). No studies have evaluated whether ferritin or other iron indicators change with gender-affirming hormone therapy.\nFrom a practical perspective, the main reason to evaluate for iron deficiency is anemia. In individuals who have a ferritin below the “male” reference range, we suggest interpreting the iron studies in the context of red cell indices such as mean corpuscular volume and mean corpuscular hemoglobin concentration to guide management rather than on the use of gender-affirming hormone therapy. If the trans individual is menstruating or pregnant, it would be most practical to use the premenopausal female reference range for interpretation of iron studies.\nFor evaluation of possible iron overload, in situations of borderline results which fall between the female and male reference ranges, relying on the absolute ferritin level or transferrin saturation will be difficult. It is pertinent to assess for concurrent inflammatory disease, liver disease, or iron overload states, such as hemochromatosis, which may further guide clinical management.","This patient is starting hormone therapy to align their body with their gender identity. Their doctor is checking their bloodwork because they have a higher-than-normal red blood cell count, which can be a side effect of testosterone. While this is within the expected range for a man, it’s something to monitor, especially considering the potential link to heart health. The doctor will continue to monitor their bloodwork and discuss any concerns with them, taking into account their individual circumstances and gender-affirming treatment."
234,"A cardiologist calls as they are planning a coronary angiogram for a 68-year-old trans woman and are concerned because the estimated glomerular filtration rate (eGFR) is unknown. They are uncertain how to risk stratify her for potential contrast-induced nephropathy. She has a history of longstanding hypertension and hypercholesterolemia, vaginoplasty, and has been on various formulations of estradiol therapy for over 20 years. On review of her investigations, her serum creatinine is 109 umol/L (1.23 mg/dL) but her eGFR has not been reported for the last 18 months. Laboratory providers cannot report eGFR if a male or female marker is not provided on the request form, as this is required along with age to estimate eGFR. Using the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) formula, if classified as female, the eGFR would be 45 mL/min/1.73m\nclassed as Stage 3 chronic kidney disease and would meet the guidelines for intravenous hydration prior to procedure. However, if classified male, the patient would have an eGFR of 60 mL/min/1.73m\nwhich would be classed as Stage 2 chronic kidney disease and would not require prehydration. Which is the most appropriate eGFR to use?\nAccurately assessing renal function is essential for not only assessment of renal diseases, but also clinical situations that may potentially affect renal function (such as diabetes or radioiodine contrast administration) as well as considerations for medication dosing of renally cleared drugs. The most commonly used marker of renal function in clinical pathology laboratories is eGFR, which is calculated based upon an individual’s serum creatinine level, age, and sex (). Typically, people presumed male at birth have a higher eGFR than people presumed female at birth at the same level of serum creatinine because the formula assumes a higher muscle mass in men contributing to the serum creatinine independent of renal function. The difference between these groups (given the same age and weight) is more marked at higher levels (with a difference of approximately 30 when eGFR >90 mL/min/1.73m2), becoming much more similar as eGFR declines (difference of approximately 4 when eGFR <30 mL/min/1.73m2). In clinical situations where accurate assessment of renal function is necessary, such as in the transplant setting, it may be more appropriate to use 24-hour urine creatinine clearance, urinary inulin clearance (), or serum cystatin c levels, which are less affected by sex and not affected by muscle mass in contrast to serum creatinine (). Inulin clearance and cystatin c are more expensive and less readily available. Creatinine clearance can be calculated on paired 24-hour urine and serum creatinine concentration and is independent of muscle mass and sex steroids. This can provide a baseline estimation for renal function and cumulative serum creatinine results can then be used to monitor decline in renal function with aging.\nFrom a practical perspective, laboratory reports will need to make an assessment on how to report the eGFR for trans individuals. For individuals receiving masculinizing or feminizing hormone therapy, changes in body composition appear to be maximal in the early period after commencement, evident within the first 3 months of treatment (, ). For those receiving masculinizing hormone therapy with testosterone, given higher muscle mass and lower fat mass compared to females, the male CKD-EPI formula would be more appropriate. Conversely if a person has been on feminizing hormone therapy, which typically induces gain in fat and decrease in muscle mass from 3 months of use, then the female equations should be used. It would be a challenging task to expect pathology laboratories to provide the “right” eGFR given limited access to clinical information.\nWe recommend that the treating clinician specify the sex-specific reference interval desired for reporting on the laboratory request (ie, female for a trans person using feminizing hormone therapy). Using current laboratory information systems, the gender marker can be used as a field to specify the reference range desired for reporting. While the binary female or male gender may not necessarily reflect the individual’s gender, this will allow for the appropriate reference range to be reported and the trans patient informed so they can prepare to be misgendered. For laboratory providers, if the gender marker is unknown, then treating clinicians should be contacted to specify the sex-specific reference interval desired.\nFor the trans woman described in Case 2 who was on longstanding feminizing hormone therapy with female body composition, the female reference range for renal function would be most appropriate triggering appropriate renoprotection prior to administration of radioiodine contrast for her angiogram. From a harm reduction approach, given the absence of data in the field, if either the male or female calculated eGFR suggests renoprotective strategies, then this can be implemented. A 24-hour urine creatinine clearance can also be performed to more accurately assess renal function.","A cardiologist is planning a test to check the health of the woman’s heart and needs to know how well her kidneys are working. Because her kidney function hasn’t been checked in a while, they’re unsure how much extra protection she needs before a procedure that uses dye. Based on her history and current lab results, they need to determine whether to use a formula that estimates kidney function based on a woman’s or a man’s body composition. To ensure her safety, the doctor wants to make sure the correct eGFR is used for this test."
235,"A 70-year-old trans woman who had been on feminizing hormone therapy for 6 months had a PSA performed as part of a routine health check. She was taking transdermal estradiol 100mcg/24hr patches twice weekly and cyproterone acetate 12.5mg daily. Her total testosterone was 1.5 nmol/L (43 ng/dl) and PSA was 2 ng/mL. She had mild lower urinary tract symptoms with reduced urinary flow over a number of years but had no family history of prostate cancer. How should she be managed?\nThere are no studies examining the effect of feminizing hormone therapy on PSA. It is known that androgen deprivation as part of feminizing hormone therapy is associated with a substantially lower risk for prostate cancer than the general male population (). All published case reports of prostate cancer in trans people using feminizing hormone therapy have had histology showing high risk adenocarcinoma with PSA concentrations at diagnosis ranging from 5 to 1722 ng/mL (ng/mL equivalent to ug/L) (, ). Physiologically, in the setting of androgen deprivation in people with a prostate gland, it would be expected that PSA should be lower than the age-specific reference interval. There is insufficient data to recommend a specific cutoff for trans people using feminizing hormone therapy. Individualized decisions based upon clinical history and examination should inform need for serial monitoring for PSA velocity or imaging.\nCase 3 had a digital rectal examination which showed a smooth but mildly enlarged prostate gland. She had an ultrasound of her prostate which showed a mildly enlarged prostate volume of 35 mL. Repeat PSA monitoring revealed progressive lowering of her PSA concentration with ongoing feminizing hormone therapy and an improvement in her urinary flow.","This 70-year-old trans woman is being monitored as part of her regular healthcare, including a PSA test to check for prostate cancer. She’s been taking hormones to feminize her body for six months and has very low testosterone levels. Her PSA level is currently normal, but it’s decreasing over time as she continues hormone therapy, which is a good sign. Because there isn’t much research on this topic, her doctor will continue to monitor her PSA and prostate health with regular checkups."
236,"A 49-year-old trans man who had been on testosterone therapy for 10 years presented to the emergency department with central chest pain. His high-sensitivity cardiac troponin was 24 ng/L (female reference range <16 ng/L, male reference range <26 ng/L). How should he be managed?\nCardiac troponin is released from damaged cardiomyocytes and is one of the most common biomarkers used in the prediction of myocardial infarction. There is considerable debate regarding the use of sex-specific reference ranges for high-sensitivity cardiac troponin (hs-cTn), as there is uncertainty whether the use of sex-specific reference limits impact upon clinical management or outcome prediction (). However, as upper reference limits based on sex-specific 99th percentiles for hs-cTn are subtly higher for people recorded as males than those recorded females in population studies (), use of sex-specific cutoffs for hs-cTn assays have been endorsed by the International Federation of Clinical Chemistry and Laboratory Medicine (). The difference has been attributed to people presumed male at birth having a larger cardiac mass as well as subclinical coronary artery disease (). No studies have been performed to examine cardiac mass changes that may occur with masculinizing hormone therapy in people presumed female at birth. There are however data in polycystic ovary syndrome in which high testosterone concentrations are a clinical feature (albeit far lower than testosterone concentrations seen in transgender men). Polycystic ovary syndrome has been associated with higher left ventricular mass index and larger left atrial diameter over 5 years of follow-up, even after adjustment for blood pressure, body mass index, glucose, and lipids (). Large population-based studies have also shown that left ventricular mass correlates with body weight, lean body mass, and fat mass (). There is currently insufficient data to draw an inference regarding the appropriate reference range in people using gender-affirming hormone therapy, and emphasis must be placed on clinical history, electrocardiogram (ECG) changes, and serial trajectory of hs-cTn levels if the hs-cTn falls in between the male and female-specific reference ranges.\nDespite the fact that Case 4 had been on established testosterone therapy for 10 years with resultant male body composition, there is insufficient data to suggest that cardiac remodeling or change in cardiac size occurs\nwith high (or low) testosterone concentrations. Despite the risk of being oversensitive, in order to minimize the risk of missing an acute coronary event, we suggest that the reference range of the sex presumed at birth (female) should be used to interpret hs-cTn, provided the patient is informed of this rationale in addition to monitoring with serial troponin to ensure there is no rise. Case 3’s subsequent hs-cTn was elevated above the male reference range and his ECG revealed anterior ST-segment depression consistent with acute coronary syndrome.","A 49-year-old transgender man who has been taking testosterone for 10 years came to the hospital with chest pain. His blood tests showed a slightly elevated level of a marker for heart damage, called troponin. Because of this, doctors are carefully considering his risk of a heart problem and will monitor his troponin levels closely. They will use a reference range based on his sex at birth to help interpret the results, and will also look at his heart rhythm to determine the best course of action."
237,"A 42-year-old gentleman with no prior medical illness admitted with complaints of generalized muscle pain, dry skin, and mild facial puffiness of eight days duration, associated with choking sensation in his throat. The review of systems was negative for fever, hoarse voice, cold intolerance, hair loss, dysphagia, constipation, weight gain, focal limb weakness, or changes in memory. He denied doing strenuous exercise recently, alcohol consumption, trauma, or recent medication use. There was no family history of autoimmune thyroid diseases.\nHis vital signs were as following: pulse rate, 65/min (regular); blood pressure, 120/85 mmHg; respiratory rate, 19/min; and oral temperature, 37.1°C. Physical examination revealed mild facial puffiness, dry skin, and minimal non-pitting lower limb edema. A small goiter without tenderness or nodule was found on neck examination. The musculoskeletal examination did not show muscle wasting, hypertrophy, or weakness. Other system examinations were unremarkable.\nLaboratory investigations were suggestive of severe hypothyroidism: thyroid-stimulating hormone (TSH), >100 mIU/ml (normal range <4.35 mIU/L); free T4, <0.5 ng/dl (normal range 11 – 23.3 pmol/L); anti-thyroid peroxidase antibody titer, >600 IU/ml (normal range <34 IU/ml); and anti-thyroglobulin antibody (TgAb) titer, 1831 IU/ml (normal range <115). Elevated levels of anti-thyroid peroxidase antibody and anti-thyroglobulin antibody titers were suggestive of Hashimoto's thyroiditis. Serum creatine kinase (21,644 U/L, normal range 39–308 U/L) and myoglobin (2,208 ng/ml, normal range 28–72 ng/ml) levels were also raised (Table ). This was associated with acute kidney injury with mild elevation of serum creatinine (1.44 mg/dl). Urine examination was negative for myoglobinuria or hematuria. The daily urine output was normal (250–300 ml/h).\nElectrocardiogram (ECG) and chest X-ray were normal. Transthoracic echocardiography (TTE) showed minimal circumferential pericardial effusion without regional wall-motion abnormalities and with a left ventricular ejection fraction (EF) of 58%. Fiber-optic (flexible) laryngoscopy was normal.\nA probable diagnosis of hypothyroidism due to Hashimoto's thyroiditis with rhabdomyolysis was made based on the clinical and laboratory parameters. He was treated with intravenous fluids and was started on oral levothyroxine. His symptoms improved with the treatment, and the levels of CK and myoglobin showed a decreasing trend. He was discharged on Day 4, and on further follow-up, the muscle enzymes showed a further decreasing trend (Figures and ) and normalization of renal parameters. Since there was complete resolution of symptoms with the patient returning to his regular day-to-day activities, further workup to exclude muscle diseases was not carried out.","This 42-year-old man came to the hospital with muscle pain, dry skin, and a feeling of tightness in his throat. Tests showed he had very low thyroid hormone levels, likely due to an autoimmune condition called Hashimoto's thyroiditis. He also had elevated levels of muscle enzymes, which can indicate muscle damage, and mild kidney issues. After starting treatment with thyroid hormone replacement and supportive care, his symptoms improved, and his muscle enzymes returned to normal."
238,"A 33-year-old woman, with severe postburn mentosternal contracture and cicatricial carcinoma, presented for skin grafting surgery in our hospital. The burn occurred when she was 4-year-old. She underwent two reconstructive procedures at 7 and 14 years in local medical centers. Due to the pain caused by the occurrence of cicatricial carcinoma, affecting eating and speaking, she had tried several medical centers for treatment in the past year, but failed for unsuccessful ATI. In preoperative physical examination, severe scar contractures and large tumor of approximately 15 × 12 cm were observed on the lower lip, neck, and anterior chest (Figure ); the chin, chest, and bilateral armpits fused together; the cervicomental and mentosternal angles completely obliterated; the anterior neck structures, including the larynx, the trachea, and the carotid arteries, were unidentifiable or impalpable. Mouth opening was limited (15 mm) and Mallampati test could not be performed. The left nostril was obstructive for stenosis, but the right nostril breathing was smooth. Preoperative X-rays and a computed tomography scan (data not shown) revealed distortion of the upper airway and no stenosis of the trachea. It was difficult to perform face mask ventilation because of the nearly fixed neck and regressed mandible.\nAccording to the guidelines on the management of difficult airway, awake flexible bronchoscopic intubation with topicalization is preferred in such patients, but the patient rejected ATI for discomfort and nociceptive recall before. Meanwhile, other awake strategies, including lightwand, GlideScopeⓇ Video laryngoscope, laryngeal mask airway, oral or nasal blind intubation, retrograde intubation, surgical tracheostomy, seem impossible. Therefore, a flexible bronchoscopic intubation protocol under precise sedation, topicalization, and spontaneous respiration preservation seems a promising strategy, but the airway should be secured for there was no definite backup plan.\nA written informed consent was taken with explanation focused on the risks of difficult airway. Atropine 0.5 mg was intramuscular injected to reduce secretion 30 min before transferring to the operating room. The patient was monitored by respiration rate, ventilation volume, pulse oximetry, electrocardiogram, blood pressure and bispectral index (BIS), and preoxygenated once she arrived in the operation room. Vital signs were stable. A pillow and some folded sheets were stuffed between the patient back and the operating bed to make her feel comfortable. Intravenous midazolam 2 mg and sufentanil 5 μg were given. Nasal passage was anesthetized by pledgets with 2% lidocaine and 0.25% phenylephrine. Sevoflurane 2% with a flow rate of 4 L/min with 100% O2 was administered via an endoscopic mask connected to the ventilator circuit. The concentration of sevoflurane was gradually increased to 4% and stopped when the BIS reached 55–60 within 3 min, then airway reassessment and precise topicalization were achieved by a modified SAYGO technique., , An epidural catheter (Figure ), with an outer diameter of 3.8 mm, was fixed and 1.5 cm longer was applied at the end of the fiberscope (MDH A10; Zhuhai Mindhao Medical Technology Co., Ltd., ShenZhen, China). Topicalization was achieved by spraying 2% lidocaine (7 ml) via the catheter onto the posterior nasal canal, pharyngeal cavity, epiglottis, glottis, and tracheal (Figure ). Meanwhile, supplemental oxygen 10 L/min was delivered by a hose (Figure ) through the mouth to avoid hypoxemia. When lidocaine worked, about 5 minutes later, sevoflurane was inhaled again until the BIS reached 55–60. Then, a Parker Flex-Tip® tube with an inner diameter of 6.0 mm (Lead Medical Instrument Co., Ltd., Guangzhou, China), which was heated by 40 ℃ and lubricated in advance, was successfully and smoothly inserted into the trachea from the direction of flexible bronchoscopy (Figure ). No stress reactions, including cough, hypertension, tachycardia, arrhythmia, hypoxia, and bronchospasm, and no intubation related complications, such as airway trauma, airway obstruction, and bleeding, were occurred during intubation. Finally, the patient was very satisfied with this experience after emergence.","This 33-year-old woman has a complex medical history due to a burn she suffered as a child, leading to significant scarring and a cancerous growth on her face and neck. Because of this, she has difficulty eating, speaking, and breathing, and has struggled to find a solution in the past. To address this, doctors will use a specialized technique called flexible bronchoscopic intubation to safely insert a breathing tube, carefully monitoring her vital signs and using sedation to minimize discomfort. The team is prepared with backup plans in case the procedure is challenging, and they will be closely watching her throughout the process."
239,"The patient was a 28-year-old woman, Gravida 2 Parity 1 Live Birth 1, who presented at 38 weeks of gestation with epigastric pain radiating to her back with a history of recent travel and symptoms of subjective fevers with a new sense of smell and taste disorders for 1 week. The patient had received regular prenatal care, and her past obstetric history was uncomplicated, with one full-term vaginal delivery. She was overweight (body mass index = 25.7) and had no significant past medical history and drug history.","This 28-year-old woman, who is 38 weeks pregnant, came to the hospital because she was experiencing pain in her stomach that was spreading to her back. She also had a fever, changes in her sense of smell and taste, and recently traveled. Her pregnancy history is normal, and she is slightly overweight. The doctor is investigating these symptoms to ensure a healthy outcome for both her and her baby."
240,"A patient in her late seventies was incidentally found to have a large infrarenal abdominal aortic aneurysm in 2013 for which she had undergone an uneventful percutaneous endovascular aortic repair with left chimney. She lived alone, remained independent in her activities of daily living, and was community ambulant without aid.\nShe was electively admitted in September 2019 for embolization of a type 2 endoleak by the interventional radiologist. Super selective cannulation of the distal aspect of the iliolumbar branch supplying the nidus with a micro catheter was performed. Embolization was carried out using Onyx® until complete exclusion of the nidus. This was followed by repeat aortogram, which showed complete exclusion of the nidus from right-sided branches. However, there was continued filling of the nidus from the left lumbar branches. It was therefore decided to embolize the left side. After embolization, angiogram showed complete exclusion of the endoleak and the left-sided branches supplying the endo leak.\nFive hours after the procedure the patient complained of bilateral lower limb weakness and numbness, right more than left. Physical examination revealed lower motor neurone pattern of weakness over bilateral lower limbs, right worse than left (Table ). Sensation testing revealed normal sensation over left, impaired sensation for right L2 to S1. Proprioception at bilateral big toes was intact. Reflexes were absent in bilateral lower limbs.\nLumbar spinal drain was inserted to decompress the spinal cord to allow more arterial flow as there was concern with spinal cord ischemia. The patient was started on fluid replacement to maintain the mean arterial pressure above 80 mm Hg. Urgent CT aortogram followed by MRI thoracolumbar spine was performed. Aortogram showed postinterval embolization of bilateral feeding arteries. Onyx material was seen within a branch of the embolized right iliolumbar artery, which extends into the spinal canal at the level of L3 and appears to exit at the level of L2. It ran external to the thecal sac. MRI thoracolumbosacral spine showed no abnormal cord signal or restricted diffusion.\nShe was subsequently admitted for inpatient multidisciplinary rehabilitation. On initial functional assessment, she required moderate assistance for bed mobility and transfer with poor dynamic sitting and standing balance. She did not complain of pain. After 1 month of inpatient rehabilitation, she made some neurological recovery with functional improvement. She had good dynamic sitting balance and was able to transfer under supervision. She can self-propel wheelchair within the ward. However, she had poor standing balance and was still unable to ambulate. She was discharged to a subacute rehabilitation facility before being discharged home. Please see her functional improvement over time using the Functional Independence Measure (FIM) instrument illustrated in Table .\nElectrophysiology study was performed 12 weeks later showed electrophysiological evidence of an acute right lumbar plexopathy involving the right obturator and femoral nerves, with no evidence of axonal continuity.\nShe was reviewed in the outpatient clinic 14 weeks later. Neurologically remained largely unchanged for right lower limb; however, left lower limb power had further improved to about 4 on manual motor testing. Functionally, she had also made slight progression, and she was able to walk 25 meters using walking frame with 1 person providing standby assist. However, she was still dependent on wheelchair for longer distance ambulation.","This woman in her late seventies had a previous surgery to repair a large aneurysm in her abdomen. Recently, she underwent a procedure to fix a leak in the aneurysm, but afterward, she developed weakness and numbness in her legs, which doctors believe is related to pressure on her spinal cord. Doctors performed scans to check her spine and found a small piece of material from the previous surgery had moved into the spinal canal, causing the problem. With rehabilitation, she showed some improvement in her ability to move and balance, but still needs assistance with walking and is currently being sent to a subacute rehab facility to continue her recovery."
241,"A 4-year-old girl was referred to our tertiary hospital for the specialist evaluation of a non-specific cough, which was present for 6 weeks, associated with a right pulmonary mass. The physical examination was normal; on auscultation, diminished breath sounds were present on the right side of the lung. The medical history and growth were unremarkable. Chest radiograph revealed a mass located in the right upper lobe and the middle lobe of the right lung, with a central area of calcification (Figure ). Chest computed tomography (CT) scan confirmed the chest radiograph findings; a solid, well-contoured, heterogeneous, mass was noted in the right upper lobe and middle lobe of the lung with an area of central calcification (Figure ).\nNo lymphadenopathy was detected. Microscopy, culture and cytology of the sputum were unremarkable.\nThe erythrocyte sedimentation rate was 10, haemoglobin was 11.6 g/dl and the leucocyte count was 8.8 × 109/L. The other serum haematological and biochemical results were normal. The serology of Echinococcus and Mantoux test were negative.\nThe patient did not respond to antibiotics; therefore, surgical removal of the mass was performed. Thoracotomy performed on the right side showed a lesion in the lung parenchyma, extended to the upper and middle lobes. The differential diagnosis of congenital lesions of the lung was made. As there was no success with fine-needle aspiration in several cases, we decided to perform surgical resection.\nThe lesion was resected and lobectomy of both upper lobe and middle lobe was also performed. No associated lymphadenopathy was noted.\nMacroscopically, a well-circumscribed mass measuring 5.5 × 5.5 × 4 cm was present. The excised tumour had an osseous centre measuring 2 × 1 × 1 cm. From the histological point of view, the mass consisted of disorganization of the normal bronchoalveolar parenchyma, myofibroblastic cells and inflammatory cell infiltrates, such as lymphocytes, neutrophils, eosinophils and histiocytes. In the centre of the lesion, a large ossification was found.\nImmunohistochemistry revealed calponin antigens, SMA, desmin, P53, bcL2, CK, HMW, and, in several myofibroblastic cells, expression of ALK-1. It was negative for beta-catenin, cyclin D1, Myo-D1, S100p, CD34, CD99 and CD117.\nThe histological and immunohistochemistry characteristics were compatible with an inflammatory myofibroblastic pseudotumor.\nThe post-operative course was uneventful, and the patient was discharged home after 2 weeks of surgery. The patient re-presented to the hospital 1 year after surgery. The child had normal daily activities, normal chest x-ray and no recurrences.","A 4-year-old girl was seen at our hospital because she had a persistent cough for six weeks and a spot on her lung that we found on an X-ray. Further tests, including a CT scan, showed a mass in her lung with an area of calcium, and it didn't respond to antibiotics. Doctors decided to remove the mass surgically, and they found it was an inflammatory myofibroblastic pseudotumor, a type of non-cancerous growth. The surgery went well, and she has been doing well for a year with no problems."
242,"A 35-year-old gentleman came to our institution in February 2021 for evaluation of persistent fever and non-resolving pneumonia. He had a polymerase chain reaction (PCR)-confirmed SARS-CoV-2 infection in October 2020, with classical radiological findings. He was managed conservatively under home quarantine. Two weeks later (November 2020), he had a recurrence of high spiking fever. Imaging revealed a left-sided lung consolidation. He was treated for probable pneumonia with oral amoxicillin-clavulanate. At 1-month follow-up (December 2020), there was an inadequate clinical improvement with an increase in the size of the consolidation (Figure ). Bronchoscopy was done, and bronchoalveolar lavage grew Streptococcus pneumoniae. He received another course of linezolid antibiotic.\nIn 2017, he had probable vaccine-related/autoimmune optic neuritis for which he received 13 doses of rituximab between January 2017 and May 2019 (600 mg per dose). He had developed rituximab-induced hypogammaglobulinaemia in 2019. Since then, he has had persistent B-cell depletion and low immunoglobulin levels (Table ). The absolute B-cell count was zero.\nDuring our evaluation, SARS-CoV-2 real-time reverse transcription PCR (RT-PCR) was negative (February 2021 and March 2021). Antibodies to SARS-CoV-2 nucleoprotein (N) and spike receptor-binding domain (S-RBD) tested on the Roche Elecsys platform were undetectable in February 2021. We considered the following differentials: tuberculosis (TB), organizing pneumonia, lymphoma, Antineutrophil Cytoplasmic Antibodies (ANCA) associated vasculitis and lung malignancy. Sputum Xpert-TB-PCR was negative. Multiple blood cultures, bone marrow biopsy and culture reports for routine bacteria, Mycobacterium and fungal organisms were negative. A bronchoscopic transbronchial lung biopsy was done. Histopathology was suggestive of organizing pneumonia. Past COVID-19 infection was considered as the likely cause for organizing pneumonia. He was started on high-dose steroids, with which he improved.\nHe was readmitted in April 2021 with a persistent fever. Since the patient was on high-dose steroids, the fever was attributed to a probable secondary infection or organizing pneumonia itself. Sputum culture grew Pseudomonas aeruginosa, and an antibiotic, piperacillin, was commenced. Computed tomography thorax showed resolution of the previously seen left lower lobe consolidation but had new-onset bilateral ground-glass opacities (GGOs) (Figure ). Nasal swab for SARS-CoV-2 RT-PCR was positive (Cepheid assay, April 2021), with a Ct value of Envelope gene (E gene) 23.2 and nucleoprotein gene (N) 24.1. A diagnosis of COVID-19 re-infection was made. He received remdesivir injection and other symptomatic medications. He became afebrile within 2 days and was discharged from the hospital. During routine follow-up in June 2021, he was asymptomatic and was now positive for antibodies to SARS-CoV-2 (anti-N: 9.6 and anti-S-RBD: 41.4) (Table ).","This man has had ongoing health issues since recovering from COVID-19 in He experienced a recurring lung infection in late 2020 and again in early 2021, which was initially thought to be related to COVID-19 but later found to be caused by a different bacteria. He also has a history of low antibody levels due to previous treatment with rituximab, which may have made him more susceptible to infections. After receiving steroids, he improved and eventually developed antibodies to COVID-19, indicating he had recovered from the virus."
243,"A 34 year old woman from the east of Morocco, married and mother of two children, was admitted to the emergency room with intense periumbilical and pelvic abdominal pain associated with acute vomiting without any notion of metrorrhagia. The clinical examination revealed a conscious patient with an irreducible and impulsive painful mass at the umbilical level reminiscent of a strangulated inguinal hernia (). We performed an abdominal ultrasound scan which showed an evolving mono-fetal pregnancy; with an estimated weight of 1 kg, and a normal amount of amniotic fluid; strangulated through an umbilical orifice (). Faced with this exceptional diagnosis, the case was quickly discussed in a multidisciplinary team and the decision was made to perform an MRI. It was done without injection because of the teratogenic nature of the scan (, ).\nOur radiological examination showed a strangulation of a pregnant uterus through an orifice of 6 cm, which resulted in a strangulated hernia of a pregnant uterus at the umbilical level with the right ovary. There was however no intestinal loop. After discussion with the patient, a multidisciplinary discussion was quickly made in front of this exceptional presentation and opting for a ceolioscopic exploration. It was made by the head of visceral surgery under general anesthesia was performed. It reduced the gravid uterus and the right ovary by a carefully dissection of the hernia sac and external manual assistance. Prior to the placement of the intraperitoneal plate, an obstetrical ultrasound scan had shown an evolving pregnancy (, , ).\nThe procedure went well, and it was tolerated by the patient without any adverse event allowing a good postoperative evolution The patient was discharged two days later and a cesarean section was scheduled at the end of the pregnancy, giving birth to a 2.5 kg male infant with good psychomotor development. Our patient had opted for tubal ligation, and the two-year follow-up did not show any recurrence.","A 34-year-old woman from Morocco was rushed to the hospital because of severe pain in her abdomen and vomiting. Doctors found a pregnancy was trapped in her abdomen, causing a strangulated hernia, and an ultrasound revealed the baby was healthy. A surgical team carefully removed the trapped pregnancy and ovary through a minimally invasive procedure. The patient recovered well and was discharged after two days, with a planned cesarean section to deliver her baby. She later chose to have a tubal ligation to prevent future pregnancies."
244,"A 57-year-old retired male presented in 2019 to Al-Bairouni Hospital complaining of a mass in his left breast. He is a non-smoker, with a history of grade (I) LP in the right thigh root 14 years ago which was treated surgically along with radiotherapy of the right thigh, with no signs of recurrence. He also had well-controlled diabetes. The physical examination showed a left breast mass with a suspicious abnormality on Ultrasound that measured 32 mm. The computed tomography scan (CT) showed left breast infiltration with no signs of metastases. The patient underwent an excisional biopsy which revealed a poorly differentiated grade III invasive ductal carcinoma (). Then he underwent a mastectomy in 2019 with axillary node resection. The final diagnosis based on the histological findings was invasive ductal carcinoma stage IIA [T:2, N:0, M:0]. Hormonal receptors tests showed the following: positive Estrogen Receptor (ER+), negative Progesterone Receptor (PR-), and negative Human Epidermal Receptor (HER-) (). He received eight cycles of chemotherapy with Docetaxel and 16 sessions of radiation to the chest wall; The last one was on the fourth of October 2020. A positron emission tomography (PET) scan six months after the surgery showed no signs of recurrence ().","This 57-year-old man was diagnosed with breast cancer in 2019 after finding a lump in his left breast. Tests showed the cancer had spread to nearby lymph nodes, but no further. He underwent surgery to remove the breast and lymph nodes, followed by chemotherapy and radiation treatment. A recent scan showed no signs of the cancer returning, and he is currently being monitored."
245,"A pregnant female, 34 years old, fourth gestation primiparity with current pregnancy estimated at 25 weeks of amenorrhea (WA) + 5 days, diabetic for 1 year, initially on oral antidiabetic drugs and then insulin therapy, hypertensive for 2 years on alpha methyldopa with a history of 3 miscarriages secondary to hypertensive peaks.\nDuring the 4th pregnancy, the patient was hospitalized at 20 WA in the endocrinology department for an etiological assessment of a hypertensive crisis at 180/110 mmHg associated with headaches, palpitations and hot flashes, without proteinuria or edema.\nIn front of these symptoms the diagnosis of a secretory neuroendocrine tumor is suspected, and a biological assessment including urinary and plasma catecholamines (metanephrine and normetanephrine) came back positive after eliminating other causes of secondary hypertension (nephropathy, renal artery stenosis or hyperaldosteronism), by renal evaluation, renal artery Doppler ultrasound and renin-angiotensin-aldosterone system exploration.\nUrinary dosages showed Metanephrines at 0.87 μmol/24 h (normal: 0.20–1), high Normetanephrine 24.14 μmol/24 h (normal: 0.4–2.10), While plasma dosages showed a Metanephrine level of 0.10 nmol/l (normal: < 0.33 nmol/l), a high level of Normetanephrine of 14.02 nmol (normal: <1.07 nmol/l). A thyroid workup was performed to rule out multiple endocrine neoplasia (MEN) returning normal: TSH 1.821 (normal: 0.340–5.330), anti-thyroperoxidase Ac < 0.8 IU/ml (normal <0.8).\nAbdominal-pelvic magnetic resonance imaging (MRI) showing an abdominal latero-aortic mass measuring 36 * 33 mm, corresponding to paraganglioma without any other obvious location [].\nFor evaluation of maternal and fetal impact of the tumor:\nClinical examination including cardiovascular examination was normal. With an ECG that did not show rhythm or repolarization disorders or left ventricular hypertrophy.\nA standard biological workup (blood cell count, platelet count, hemostasis, blood ionogram, blood sugar-HBA1c, renal and hepatic function) was unremarkable.\nOn the obstetrical level, the clinical examination and the obstetrical ultrasound showed a monofetal pregnancy with no uterine or fetal abnormalities.\nAfter placing the patient on medical treatment and scheduling her for surgery, she was declared discharged with an ambulatory follow-up. At 25 weeks of amenorrhea; the patient was admitted to the operating room for laparoscopic removal of her paraganglioma.\nIn the operating room, considering the risk of perioperative morbidity related to severe per and postoperative hemodynamic instability (hypertensive crisis with sometimes rhythm disorder, coronary ischemia, left ventricular failure and hypotension secondary to a sudden release of catecholamines: to stress, painful stimulation due to intubation and incision, insufflation and manipulation of the tumor, abrupt and profound hypotension by sudden decrease of catecholamines at the fall of the part and venous clamping) and in front of the obligation to maintain a uterine perfusion and a good oxygenation of the fetus while avoiding maternal hypoxemia, the anesthetic management was as follows:Monitoring:\nHemodynamic: heart rate (HR), blood pressure (BP)\nRespiratory: respiratory rate, pulse oxygenometry (SpO2), capnography.\nECG and ST segment monitoring.\nCardiac output monitoring by pulse wave contour analysis.Left jugular venous line (for central venous pressure monitoring and vasoactive drugs perfusion) A right femoral arterial line with invasive arterial pressure monitoring Heating of the patient to prevent hypothermia\nAfter installation of the patient in left lateral decubitus proclive 30°, a pre-oxygenation was started, and an anesthetic induction was done by: 150mg Propofol 150μg Fentanyl, 50 mg Atracurium 50mg; intubation by tube No. 6.5 cm; maintenance of anesthesia was provided by: Propofol IVOC system with concentration of: 2ug/ml.\nMaintenance of etCO2 32–35 mmHG with insufflation pressures not exceeding 12 mmHG (8–10 mmHG) and close monitoring of blood glucose due to the risk of blood glucose imbalance.\nDuring tumor manipulation 3 episodes of arterial hypertension peaks were observed) (180/110–195/120-200/130 mmHg controlled by reinforcement of analgesia by reinjections of fentanyl 50 μg, deepening of anesthesia (Propofol concentrations 3.5 μg/ml), and administration of Nicardipine at 1–3 mg/h. Glycemia: varied between (0.98–1.21 g/l)\nThe tumor removal was followed by episodes of arterial hypotension (75 -45 mmHg), the conduct was to lighten the anesthesia, a volemic expansion with administration of norepinephrine at 2mg/h.\nFor paraganglioma extraction, the procedure lasted 2 hours [, ]., with an intraoperative diuresis of 500 ml and bleeding estimated at 80 cc.Norepinephrine infusion was gradually decreased and stopped just before extubation.\nThe patient was subsequently extubated with the following parameters BP: 140/75 mm hg HR: 90bpm, SaO2 100% and then sent to the intensive care unit for clinical and biological monitoring. Postoperative analgesia was started with paracetamol, nefopam and morphine. The antihypertensive treatment was resumed in front of the reappearance of hypertensive peaks.\nAn obstetrical evaluation with a foeto-placental ultrasound immediately and 6 hours after the operation returned normal with the presence of fetal heart activity [].\n2 days after the postoperative hospitalization in the intensive care unit, the patient was weaned from all drugs with good evolution and was transferred to the visceral surgery department for further management.\nThis cases report follows SCARE guidelines 2020 [].","Here’s a 3-sentence summary of the doctor’s note for the patient: This woman is currently pregnant and has been diagnosed with a rare tumor in her abdomen called a paraganglioma, which causes her blood pressure to rise and releases extra hormones. To protect her and her baby, she underwent surgery to remove the tumor, and the procedure went smoothly with no complications. She is now recovering well and will continue to be monitored closely to ensure a healthy pregnancy and baby."
246,"Patient XY, a 44-year-old female presented to the ED with vomiting following consumption of locally foraged mushrooms. An experienced forager, the patient had collected and prepared the mushrooms for dinner. Between six to eight hours later, the patient noted abdominal pain and associated vomiting. The vomitus was bilious in nature with multiple episodes occurring over the next two to three hours. With persistent vomiting, she called an ambulance that brought her to her local hospital. Admission and investigation found a deranged liver profile, and the diagnosis of acute liver failure secondary to the fungi consumption was made. Ms. XY was transferred to the National Liver Unit where she underwent orthotopic liver transplant. Following a tumultuous post-operative course including an ICU admission, and Hepatic, Renal, Plastics, Rheumatology and Dermatology input, an incidental finding on CT of the abdomen/pelvis foreshadowed the orthopaedic trajectory of our patient.\nFollowing discharge from the ICU, the patient began recovery on the ward. At this point, her primary concern was painful discolouration at the pulps of her fingers and toes. Painful lesions were also noted in the right flank region. Dermatology input resulted in a diagnosis of skin necrosis post-transplant with microvascular thrombosis of the hands and feet. Skin graft to the right flank region was performed by the Plastics team. The post-operative period was once again complicated by sepsis and treated with antibiotics. She remained on a prolonged course of steroids for up to six months post-operatively.\nAs XY continued to improve clinically, her recovery was aided by our multi-disciplinary team input. Throughout this period, the patient noted a new onset of groin pain resulting in regression in her mobility. In the proceeding four weeks, Ms. XY continued to suffer from pain, impeding her progress with physiotherapy. A physical exam now revealed a swollen and erythematous knee, with a reduced range of motion. An X-ray excluded knee pathology including joint effusion and fracture and confirmed normal alignment, as shown in Figure and Figure . The Pain team now became involved and recommended increased medication doses and frequency.\nXY’s pain continued to increase over the subsequent six weeks and she was unable to engage fully in physiotherapy for six months. XY described the pain as sharp, without radiation and aggravated by movement. A physical exam revealed an oedematous swollen joint surrounding the cruciate ligament. An MRI was conducted as suggested by the pain team to investigate the integrity of the surrounding soft tissues. The cruciate ligaments, the menisci and both the quadriceips and patellar tendons were found to be intact. The associated musculature was atrophic. Several serpiginous lines within the subchondral marrow of the medial and lateral femoral condyles and in the patella with alternating hyper- and hypointensity were noted. Heterogenous surrounding marrow was also visualized, all of which was depicting AVN and a reactive sclerosis. Orthopaedic intervention was not advised. Evidence is provided in Figure and Figure .\nXY was in bed almost 24 hours a day and was experiencing bilateral hip pain. Normal X-Ray is shown in Figure . However, pelvis MRI now revealed marked bilateral femoral head oedema, with bilateral sepiginous low-signal circumscribed areas also noted in both femoral heads, with advanced loss of joint space and articular cartilage bilaterally (Figure ). A subchondral collapse in the superolateral aspect of the left femoral head was noted (Figure ). Complex effusions were described bilaterally, thus showing a secondary synovial osteochondromatosis. Muscular oedema was noted throughout; the right rectus muscle was severely atrophic. Avascular necrosis was confirmed.\nOrthopaedic input\nOne week later, the patient underwent bilateral total hip replacements. Left femoral neck osteotomy was performed from the posterior approach and Multihole Pinnacle size 50 was inserted. A 48-mm cup was inserted on the right side from the posterior approach. The surgery was conducted under a general anaesthetic and then a spinal anaesthetic. Jubilee dressings were applied bilaterally, and continued for up to three weeks post-operatively. Serous ooze was noted particularly on the left side. She was treated with a course of intra-venous antibiotics and the ooze resolved. All clips had been removed by day 21 post-operatively and there were no complications noted. The patient restarted her physiotherapy program, using a wheelchair. Figure shows the bilateral prostheses in situ.\nThe patient was mobilising up to 20 metres with the assistance of a frame, denied any pain and was increasing muscle strength and mobility daily. Objectives for discharge included the patient being able to mobilise independently at home and use a wheelchair outside. The patient was satisfied with this result and continued with physiotherapy and rehabilitation.\nOver 18 months later, the patient is mobilizing independently at home and using crutches while outside. Despite complications post-transplant, she maintains good mobility and range of movement in her lower limbs.","This patient experienced a serious reaction to eating wild mushrooms, leading to liver failure and requiring a liver transplant. Following the transplant, she developed painful skin sores and joint pain, which were investigated and found to be related to complications from the transplant and subsequent avascular necrosis (bone death). After a long and challenging recovery, she underwent bilateral hip replacements to address the joint damage. Ultimately, the patient has made significant progress and is now able to move around independently with the help of crutches and a wheelchair."
247,"A 73-year-old male presented to the emergency department with persistent hiccups over the past five days. The patient is a known diabetic and hypertensive for 15 years and has rate-controlled atrial fibrillation, managed with beta-blockers and warfarin. Two weeks ago, he suffered a subacute ischemic infarct of the right medial occipital lobe, which was confirmed on a computed tomography (CT) scan of the brain. The patient had both a preserved gag and swallow reflex. The family reported one bout of post-prandial emesis while upright five days earlier, after which the hiccups began eight hours later. The patient had no other symptoms, and the family denies him experiencing fever, chills, dyspnea, cough, malaise, and confusion.\nOn clinical examination, there were decreased breath sounds and crackles noted in the basal segments of the right lower lobe. The patient had a low-grade fever (temperature 37.8 C) whilst other vital signs were stable (blood pressure 134/92mmHg, heart rate 92 beats per minute, respiratory rate 22 breaths per minute, oxygen saturations 97%). Chest radiograph confirmed the presence of bilateral lower lobe pulmonary infiltrates (Figure ). Blood investigations revealed a leukocytosis, neutrophilia, and an elevated C-reactive protein (CRP) (Table ). Blood cultures were negative and arterial blood gases were normal. Additionally, a coronavirus disease 2019 (COVID-19) polymerase chain reaction (PCR) test was negative. A tentative diagnosis of persistent hiccups secondary to aspiration pneumonia was made. The patient was immediately started on an intravenous antibiotic regime consisting of amoxicillin/clavulanic acid 1g at 12-hour intervals and metronidazole 400mg dosed at eight-hour intervals. Additionally, the patient was placed on a low-dose chlorpromazine infusion (25mg chlorpromazine diluted in 1000ml 0.9% saline infused over four hours), and his blood pressure was closely monitored.\nThe patient experienced a near-complete resolution of his hiccups within 36 hours of treatment and a complete resolution prior to discharge four days later. Laboratory investigations were repeated prior to discharge and a decrease was noted in both CRP and leukocytes (Table ). The patient was transitioned to a 10-day course of oral amoxicillin/clavulanic acid and metronidazole. At outpatient follow-up 14 days later, the patient is well and had no recurrence of the hiccups.","This 73-year-old man came to the hospital because he had persistent hiccups for five days. He also has diabetes, high blood pressure, and a history of a stroke, and recently developed pneumonia in his lungs. Tests showed he had an infection in his lungs, which is why he was given antibiotics and other medications to help him feel better. Thankfully, the hiccups resolved quickly, and he was able to go home after four days with a course of oral antibiotics to finish."
248,"A 53-year-old man presented with a gradually increasing mass located in the soft tissue of the oral cavity. The lesion was biopsied, and the pathology report showed increased infiltration of the buccal mucosa with monoclonal plasma cells. Immunochemistry demonstrated positivity for CD138, CIgA, and negativity for CD56, Cyclin D1 and CD20. Therefore, the diagnosis of soft tissue plasmacytoma was confirmed, for which he received localized radiotherapy with a total dose of 40 Gy, due to the absence of systemic disease as the bone marrow biopsy revealed the absence of neoplastic infiltration and serum and urine immunofixation were all negative.\nAfter five years, the patient noticed a painless swelling in his right testis. An ultrasound of the scrotum was performed that showed a hypoechoic mass in the right testicle with increased vascularization, and a normal-appearing left testis (Figure ). The patient subsequently underwent a right radical orchiectomy and the histopathology report showed testicular infiltration by a plasma cell neoplasm with identical immunophenotype (CD138+, CIgA+, CD56-, CD20-, Cyclin D1-) to the primary site in the oral cavity. Subsequently, the patient underwent a bone marrow biopsy that showed the absence of monoclonal plasma cell infiltration, and a PET/CT scan that was negative for reactive lesions suspicious of malignancy. In addition, serum and urine protein electrophoresis, as well as serum-free light chain assay were all within normal range, thus, excluding the presence of systemic disease, while complete blood count and full biochemical profile were normal.\nHowever, after one year, the patient presented again with painless swelling of his left testis that was attributed to disease relapse based on the imaging findings (Figure ). He refused to undergo left orchiectomy, thus he proceeded with systemic treatment based on lenalidomide, bortezomib and dexamethasone. Following 4 cycles of treatment, the patient underwent high dose melphalan with autologous stem cell transplantation. The blood tests of the patient revealed an increase in serum creatinine (1.3 mg/dl with 1mg/dl baseline level), normal calcium levels and normal complete blood count. Complete response was achieved with negative PET/CT scan and negative marrow minimal residual disease assessment. The patient continued on lenalidomide maintenance; however, six months later he was diagnosed with disease relapse in his left testicle (Figure ). A PET/CT scan was then performed, and showed a reactive lesion with an increased SUV max of 7 in the left testis, with no other loci suspicious of disease relapse. The blood analysis of the patient revealed no abnormal values. Since the patient refused surgery, he was initiated with next-line therapy, with bortezomib, cyclophosphamide and dexamethasone, he responded partially and still remains in remission (Figure ), while serum protein electrophoresis, immunofixation and serum-free light chain assay, all remain within normal levels.","This 53-year-old man was initially diagnosed with a rare type of cancer in his mouth that spread to a nearby lymph node. He received radiation therapy, which successfully treated the initial cancer. However, several years later, the cancer returned in his testicles, and after further testing, it was confirmed to be the same type of cancer. Despite refusing surgery, he received a combination of medications and stem cell transplant, which brought him into remission, but the cancer has recently returned in his left testicle."
249,"Our patient was a 12-year-old Bahraini male who was seen initially in the A&E with a history of a fall from a height of around 2 meters on an outstretched hand. He had an obvious deformity of his elbow joint with an intact soft tissue envelope and no distal neurovascular deficits. Plain radiographs revealed a closed fracture-dislocation involving the olecranon process, a Salter-Harris type I fracture of the radial neck, and posterior dislocation of the elbow joint (Figure ).\nOpen reduction and internal fixation of the fracture were undertaken the following day, under general anesthesia and in the lateral position; the elbow was approached posteriorly. The radial head was indirectly reduced and held using a 1.6-mm K-wire; the olecranon was reduced under direct vision and held with a tension band using two 1.6-mm K-wires (Figure ).\nPostoperatively, the elbow was protected using an above-elbow slab for three weeks, after which both active and passive elbow range of motion (ROM) was initiated. The fracture healed uneventfully, and the patient had a complete ROM of 30-130 degrees at the elbow joint within six weeks of fixation. The implant was removed after six months. The patient was completely asymptomatic with a full range of flexion, extension, supination, and pronation along with the evidence of bony union (Figure ).","This 12-year-old boy fell from a height and broke his elbow. X-rays showed a significant dislocation and fractures in the elbow joint. Surgeons repaired the fracture with pins and wires, and he wore a cast for three weeks. After six weeks, he regained full movement in his elbow and the bones healed properly, with no pain."
250,"A 38-year-old Caucasian woman, 35 weeks into her first pregnancy, presented to the emergency department for acute right-sided hip pain which precluded weight-bearing. Her right leg was shortened and externally rotated - there was no bruising or evidence of trauma.\nThe patient’s history was significant for hereditary thrombophilia (Factor V Leiden) and secondary anemia. Hip radiography revealed an unstable, displaced, right-sided femoral neck fracture with no evidence of osteonecrosis (Figure ). The decision to administer radiography, in this case, was based on the American College of Radiology guidelines, which cite an absence of in-utero deterministic effects of ionizing radiation effects after 27 weeks of gestation. Unfortunately, it was not possible to evaluate the symptoms of the patient with MRI at this time due to the coronavirus disease pandemic-induced stress on the healthcare system of our country.\nThe patient denied falls or trauma during the pregnancy, nor was there any history of smoking, alcohol abuse, use of glucocorticoids, or presence of rheumatologic/oncologic disease. Additionally, the patient was not malnourished, she underwent routine antenatal care, and took multivitamins. Serologic tests for inflammatory markers, as lab tests for serum calcium, phosphate, alkaline phosphatase, parathyroid hormone, vitamin D, and D-dimer returned normal.\nDuring multidisciplinary rounds, it was decided that delaying surgery was the best course of action out of fear of causing either mechanical or fluoroscopy-induced damage to the fetus during total hip arthroplasty. Five days later the patient experienced premature rupture of membranes, which was managed with emergency cesarean section (C-section) - no complications were encountered and a healthy 2300 g female was successfully delivered. Three days later the patient was transferred to our orthopedic surgery department for the treatment of the fracture. The significant degree of displacement (grade IV) of the fracture lasting over one week precluded open reduction with internal fixation due to fears of femoral head necrosis. During our literature review, we encountered a similar case of femoral neck fracture with grade IV displacement that was treated with open reduction internal fixation - despite restoration of blood flow to the femoral head within 15 hours, the authors still encountered femoral head necrosis with collapse six months later []. Given the considerable delay between symptom presentation and treatment, we decided the case warrants total hip arthroplasty instead of native hip salvage. Hemiarthroplasty was considered but was ultimately discarded as the conversion rate to total hip arthroplasty in young patients remain relatively high and the fracture was subsequently treated with a total uncemented prosthesis (Figure ), consisting of a 50 mm cup with 32 mm ultra-high-molecular-weight polyethylene insert and a 32 mm head with a 4 mm ceramic insert (Link Inc., Hamburg, Germany). Postoperative radiography confirmed prosthesis placement (Figure ); antibiotic and anticoagulant prophylaxis was initiated with ampicillin/sulbactam and enoxaparin sodium, respectively.\nThree days after the intervention the patient developed moderate abdominal pain without fever and accelerated intestinal transit, which both worsened over the next four days. Given the clinical presentation, there was a high index of suspicion for infection with Clostridium difficile (C. diff), although the diagnosis was ambiguous as the enzyme immunoassay (EIA) for the C. diff-specific antigen glutamate dehydrogenase was positive, while EIA for exotoxin A and B were negative. Due to exacerbation of symptoms and development of moderate hypokalemia (2.9 mEq/L), empiric treatment with metronidazole was began and marked rapid improvement. The patient was discharged five days later.","This 38-year-old woman experienced sudden, severe pain in her right hip during her pregnancy. An X-ray revealed a displaced fracture of the hip bone, which was a serious concern for her baby. To protect the baby’s health, doctors decided to deliver the baby via C-section and then performed a total hip replacement on the mother’s hip. After treatment with antibiotics and careful monitoring, the patient recovered and was discharged home."
251,"A 54-year-old Japanese woman was transferred to Hamanomachi Hospital, Fukuoka, Japan, for persistent fever with chest imaging abnormalities. She had a smoking history of 30 pack years. She had been diagnosed with oropharyngeal cancer and had been treated with chemoradiotherapy five years earlier. Two years prior to this clinical presentation, the patient had an established diagnosis of HTLV-1-associated myelopathy (HAM), with neurological findings of neurogenic bladder, orthostatic hypotension, bilateral lower limb spasticity, increased deep tendon reflexes, and positive bilateral Babinski reflexes, as well as positive serum and cerebrospinal fluid tests for anti-HTLV-1 antibodies.\nPost diagnosis of HAM, the patient experienced recurrent episodes of aspiration pneumonia and had a gastrostomy placed a year and a half earlier. A year earlier, she developed acute progressive HTLV-1-related myelopathy of bilateral lower limbs and was treated with methylprednisolone 1000mg pulse therapy, followed by 5mg of prednisone maintenance therapy at Fukuoka Central Hospital, Fukuoka, Japan. In the outpatient clinic at Fukuoka Central Hospital, she presented a fever with mild sputum. Her chest computed tomography (CT) showed infiltration in the upper right lobe and she was diagnosed with pneumonia. Despite treatment with levofloxacin for five days, a high fever persisted, and she was admitted to Fukuoka Central Hospital.\nAt Fukuoka Central Hospital, she was placed on total parenteral nutrition, suspected of aspiration pneumonia, and was treated with tazobactam/piperacillin (TAZ/PIPC), followed by meropenem (MEPM) and vancomycin (VCM). She was also suspected to have vasculitis from the findings of purpura on her both lower limbs, and the corticosteroid dose was temporarily increased. Despite this effort of examinations and treatment for one month, her fever, high levels of serum C-reactive protein (CRP), and chest imaging abnormalities persisted. She was then transferred to Hamanomachi Hospital for further investigation and treatment on hospital day 28 (Figure ).\nOn admission to Hamanomachi Hospital, she was sarcopenic with a body mass index of 13.5. She had muscle weakness of manual muscle testing (MMT) of 2-3/5, mainly in her lower limbs, which were the same levels of her baseline. Her respiratory condition was normal, with a respiratory rate of 15/min and SpO2 98% on room air. Crackles were not detected in her chest. Persistent, increased levels of CRP (7.7 mg/dL) and a white blood cell count of 6400 /μL were noted (Figure ). Chest x-ray imaging showed a giant air-space in the right upper lung field one year earlier (Figure , panel A). Consolidation was found in the right upper lung field on admission to Hamanomachi Hospital (Figure , panel B). Chest CT imaging showed dense consolidation with an air bronchogram and a giant bulla with fluid retention in the right upper lobe, as well as an emphysematous change in the bilateral lungs (Figure , panel A). Given the clinical course at Fukuoka Central Hospital and examination results on admission to Hamanomachi Hospital, a refractory lung abscess and an infected bulla were suspected. Suspected pathogens were Pseudomonas aeruginosa and Methicillin-resistant Staphylococcus aureus (MRSA), which were detected by sputum culture at Fukuoka Central Hospital. The differential diagnoses were opportunistic infections such as aspergillosis, mycobacteriosis, vasculitis, HTLV-1-associated bronchioalveolitis, and adult T-cell leukemia infiltration.\nTo confirm the diagnosis, a bronchoscopy was performed on hospital day 30 (Figure ). Transbronchial lung biopsies (TBLB), bronchial brushing, and bronchial washing were conducted from the right B2 bronchus. Bacterial culture from bronchial washing was positive for Pseudomonas aeruginosa and MRSA, consistent with the sputum culture at Fukuoka Central Hospital and on admission to Hamanomachi Hospital. Neither Aspergillus species nor Mycobacterium species was detected. Hematoxylin and eosin staining of TBLB showed bronchial tissues with mild to moderate chronic inflammation and lymphocyte-predominant infiltration (Figure , panels A-C). Infiltrating lymphocytes were small and showed no morphological abnormalities. A small number of neutrophils and plasma cells were also seen. No findings suggestive of malignancy or adult T-cell lymphoma were noted. Infiltrating lymphocytes were a mixture of cluster of differentiation (CD)20-positive B lymphocytes and CD3-positive T lymphocytes (Figure , panel D), with a slight predominance of B lymphocytes. CD8-positive T lymphocytes were predominant among T lymphocytes (Figure , panels E and F). The pathological meaning is explained in the Discussion section.\nBased on these results, the patient was initiated with antibiotics (MEPM and VCM, followed by ceftazidime and linezolid). She was also treated with 5 g/day of intravenous immunoglobulin for three days, considering her immunosuppressive condition. However, she remained febrile with the upward trend of CRP. Considering the refractory state of the infected lung bulla, we performed percutaneous ultrasound-guided drainage of the bulla on day 38, following daily washing using sterile saline (Figure , panel C and Figure , panel B). The fluid from the drain was reddish-brown and cloudy, and Aspergillus species were detected in the drainage culture (Figure ). Given the pulmonary aspergillosis in addition to Pseudomonas aeruginosa and MRSA infection, voriconazole was initiated on day 49, although beta-D-glucan (14.3 pg/mL) and galactomannan (0.1 index) were negative.\nDuring treatment, we had to change antibiotics several times because of the drug resistance of Pseudomonas aeruginosa (carbapenem and quinolone resistance with moderate cephem resistance) and the side effects of the antibiotics. Amikacin was used for the combination treatment of drug-resistant Pseudomonas aeruginosa from day 60. Complications arising from anti-MRSA drugs included serum AST/ALT elevation caused by VCM, pancytopenia triggered by linezolid, and serum creatine kinase (CK) elevation (>1000 U/L) in response to daptomycin (Figure ).\nPrednisolone was gradually reduced and discontinued in consideration of its adverse effect on refractory infections. On approximately day 70, she gradually developed polyarthritis in her knees, right shoulder, and both wrist joints. Serological examination revealed elevation of rheumatoid factor (59.7 IU/L), but no elevation of anti-CCP antibodies (0.8 U/mL). Joint ultrasound showed active synovitis in both hand joints. The differential diagnosis of her poly-arthritis was antibiotic use-related arthritis, sepsis-related arthritis, pseudogout crystal arthropathy, rheumatoid arthritis, and HTLV-1-associated arthritis. She was treated with non-steroidal anti-inflammatory drugs (NSAIDs). Corticosteroids or other immunomodulating drugs could not be used until the infected bulla and lung abscess were completely healed. Her poly-arthritis persisted several months after the transfer to Fukuoka Central Hospital. From these results and clinical course, she was considered to have rheumatoid arthritis or HTLV-1-associated arthritis, which had been suppressed by the corticosteroid treatment.\nThereafter, the fever gradually resolved, and the inflammatory response trended downwards. Chest imaging showed improvement of infiltration and reduction of the size of the infected bulla (Figure , panel D and Figure , panel C). We removed the drain tube on day 87. Her general condition improved, and she was transferred to Fukuoka Central Hospital on day 94. She was discharged to her home on day 150. No relapse of the infection has been observed since the discontinuation of antibiotics.","This 54-year-old woman has been battling a serious lung infection for several months, complicated by a history of cancer treatment and a rare illness called HTLV-Initially, she was diagnosed with pneumonia, but tests revealed a large, infected bubble in her lung and a resistant bacteria. Doctors tried many different antibiotics and supportive care, but her fever and lung problems persisted. Finally, they drained the infected bubble and gave her additional medications, and she has since started to recover and is now back home."
252,"A 21-year-old male patient came to the hospital with complaints of fever, vomiting, pain in the abdomen, headache, and myalgia for three days. The patient was apparently alright three days ago when he started complaining of fever that was sudden in onset, intermittent, without diurnal variation, and not associated with chills or rigors. The fever was temporarily relived with antipyretics. He also had generalized weakness and joint pain associated with fever.\nThe patient also complained of pain in the abdomen for three days. The pain was diffuse, present in the epigastrium and umbilical region, and aggravated after consumption of food. It was associated with nausea and vomiting. The patient had around four to five episodes of vomiting after food every day. Vomitus contained food particles. There was no hematemesis. The patient was nonalcoholic.\nOn examination, his pulse rate was 88 beats per minute, blood pressure was 110/80 mmHg in the right arm in the supine position, and oxygen saturation was 98% on room air. On abdominal examination, there was tenderness in the umbilical area. Heart sounds were normal, no murmur was heard, normal breath sounds were heard, and there was no focal neurological deficit.\nOn investigation, the patient tested positive for nonstructural antigen 1 for dengue fever. Platelets on admission were 53000/mm3, which gradually decreased until the fifth day after admission and then progressively increased (Table ). The patient’s serum amylase and lipase were found to be raised.\nUltrasonography of the abdomen showed a bulky pancreas without ascites. Following this, contrast-enhanced computed tomography of the abdomen was done, which revealed bulging of the uncinate process of the pancreas and peripancreatic fluid collection along with minimal ascites (Figure ). These features were suggestive of acute pancreatitis with a score of 2/10 (mild) as per the revised Atlanta classification of acute pancreatitis [].\nThe patient was kept nil by mouth and was treated with intravenous fluids, antibiotics (ceftriaxone and doxycycline), antipyretics, opioids, and proton pump inhibitors. During the course of the hospital stay, the patient improved gradually along with an increase in platelet count. The patient did not require any platelet transfusion. Along with the improvement in hematological parameters, there was a decrease in amylase and lipase levels. He was discharged in stable condition eight days after admission in stable condition and is presently doing well on follow-up.","A 21-year-old man came to the hospital because he had a fever, stomach pain, and vomiting for three days. Tests showed he had dengue fever, which caused a drop in his platelet count. Imaging revealed inflammation of his pancreas, leading to pain and elevated levels of enzymes. He received treatment with fluids, antibiotics, and pain medication, and his condition gradually improved, with his platelet count returning to normal. He was discharged home and is doing well on follow-up care."
253,"A 59-year-old female patient presented to a plastic surgery clinic with a lump on her right medial thigh. She noticed the lump eight months ago and it was gradually increasing in size. It was painless initially; however, the lump gradually became tender. The patient was otherwise fit and well with no co-existing morbidities. Closer examination of the right thigh revealed a high consistency mass, with reduced mobility. There was no right inguinal lymphadenopathy or lymph nodes enlargement.\nLaboratory blood tests were unremarkable. An MRI scan was requested to further assess the mass, which was irregular but well-defined in the medial compartment of the thigh, measuring 8.2 x 6.6 x 4.3 cm in size. The mass showed an intermediate signal on both T1 and T2-weighted imaging, appearing hyperintense compared to the adjacent muscular tissue, which also persisted on fat-suppressed imaging. The mass invaded the intramuscular fat planes and caused compression and displacement of the adductor muscles. There was no evidence of intra-muscular invasion. Normal cortical outline and medullary signal intensity of the femur were seen in the right thigh. There was no evidence of bone contusion, marrow oedema, fracture line, or cortical discontinuity. Other muscles of the thigh had a normal outline and signal intensity with no evidence of focal or diffuse oedema. Neurovascular structures were unremarkable. Overall, MRI findings were suggestive of soft tissue neoplasm, with a possibility of a neurogenic tumour. A wide excisional biopsy was then performed to further assess the tissue histologically by the pathologist and confirm the diagnosis. The removed mass measuring 8.5 x 6.5 x 5.4 cm and is shown in Figure . The mass had a smooth outer surface and a soft consistency. The cut surface showed a homogenous tan-white appearance with some slit-like spaces and occasional haemorrhagic spots. Multiple sections were processed for histopathological examination from different planes.\nHistopathology of the mass revealed a tumour arranged in sheets and fascicles composed of round to oval cells along with variably sized adipocytes (Figure ). Numerous interspersed lipoblasts with indented nuclei were also seen. Moreover, severe nuclear atypia was noted including bizarre cells (Figure and Figure ). An area of necrosis was seen with a few areas, which showed malignant and fibrous histiocytoma-like features. Furthermore, a few thin-walled dilated and congested blood vessels were also noted. Also, brisk mitosis was noted (35-38 per 10 HPF) (Figure ). Immunohistochemical staining (IHC) was also performed and the tumour cells were diffusely positive for vimentin and focally positive for S-100; while negative for creatine kinase (CK), smooth muscle antigen (SMA), desmin, CD34 and MyoD1. Overall, the pathological findings are all suggestive features of a high-grade sarcoma, favouring the epithelioid variant of PLS.\nThe patient was also referred to the Department of Nuclear Medicine & Molecular Imaging for post-surgical restaging and assessment for possible metastases using 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET-CT) scan. The abdominopelvic section showed the liver measuring within normal limits; however, the scan revealed an ill-defined hypermetabolic hypodense lesion seen involving segment VI measuring approximately 2.8 x 1.8 cm suggestive of hepatic metastases with concurrent fatty liver. The scan was also remarkable for the musculoskeletal system, revealing multiple hypermetabolic osseous lesions involving the skull base, right iliac bone, and few vertebrae (predominantly T7-L1); which was suggestive of skeletal metastases. A non-hypermetabolic pulmonary nodule in the right lung field was also detected; it was considered to be likely benign, but interval scanning is suggested to monitor further progress. Finally, there was also evidence of diffuse subtle hypermetabolism at the site of surgery which is suggestive of postsurgical inflammatory sequelae; however, microscopic residual disease cannot be completely ruled out. These multi-organ lesions were due to be biopsied to examine their immunohistochemical findings to confirm metastatic lesions of primary pleiomorphic liposarcoma. However, the patient did not attend the follow-up appointments.","A 59-year-old woman came to the clinic because she noticed a growing lump on her thigh that had become painful. An MRI scan revealed a large mass in her thigh that appeared to be a type of tumor, and a biopsy was performed to confirm the diagnosis. The biopsy results showed that the mass was a high-grade sarcoma, likely a type of pleiomorphic liposarcoma. Further scans revealed that the cancer had spread to her liver and bones, and she was referred for further evaluation and treatment planning."
254,"A 22-year-old healthy woman presented to the emergency department complaining of a five-day history of several red and painful areas that were associated with swelling of the lower extremities. Her complaint started one day after receiving the first dose of the Pfizer vaccine. She had received all vaccinations during childhood without any complications. There is no history of preceding infections including COVID-19 infection, sore throat, or diarrhea. In addition, she never complained of prior skin lesions or systemic diseases. She did not use any topical or systemic medication. All the laboratory investigations were normal. She stated that there is no history of contact with the sick patients and no family history of tuberculosis. Skin examination revealed multiple, tender, and erythematous nodules were present on her both legs. Based on the patient history, examination, and laboratory investigation all pointed to the vaccine-related erythema nodosum; however, we considered the Arthus-like phenomenon which can mimic the erythema nodosum lesions. She received oral ibuprofen 600 mg four times daily to relieve symptoms. Three days after receiving the treatment, she improved and the nodules started to flatten (Figure ). Two weeks later, on the follow-up examination, her symptoms had completely resolved.","A 22-year-old woman came to the hospital because she developed painful, red bumps on her legs five days after getting the Pfizer vaccine. These bumps were accompanied by swelling in her lower legs. After examining her and running some tests, doctors determined that she likely had a reaction to the vaccine, specifically erythema nodosum. She was given ibuprofen to help with the pain, and the bumps disappeared within a week. At her follow-up visit, all of her symptoms had completely resolved."
255,"A 71-year-old man initially presented to clinic with right hip and low back pain with radiation to his posterior right thigh. He denied numbness, tingling, and weakness of his bilateral lower extremities. He had reproducible pain in the right SI joint region after SI joint provocative testing. A radiographic spine survey was unrevealing for a structural source of low back pain or radiculopathy. Preoperative imaging, including MRI, did reveal lumbarization of the S1 vertebra, mammillary bodies in the alar region, and a residual S1-S2 disc (Figure , ). After obtaining adequate pain relief from two separate right-sided SI joint injections, the patient decided to pursue surgical intervention in the form of SI joint fusion using the iFuse implant System® (SI-Bone, Inc., Santa Clara, California).\nIn the operating room, the patient underwent general anesthesia and was positioned prone on the Jackson table. He was then prepped and draped in the usual sterile fashion. Fluoroscopy was used to mark the borders of the sacrum on the patient’s skin. Next, a 3 cm incision was made parallel to the sacrum beginning over the center of the S2 vertebral body as approximated with lateral fluoroscopy. The incision was carried down through the gluteal fascia. The first implant was placed within the S2 body caudal to the level of the residual S1-S2 disc on the lateral radiograph. The mid-S2 body was chosen as the site for the first implant in an effort to avoid iatrogenic L5 nerve root damage from starting more cranial due to the known sacral dysmorphism seen on preoperative imaging. The next pin was placed caudal to the first using a double-barrel guide. After the path for the second implant was broached, there was an immediate flow of bright red blood from the wound. The second implant was quickly inserted, and the bleeding stopped. Therefore, we decided to proceed with the third implant (Figure ). The third implant was then placed, and the wound was irrigated and closed without evidence of continued bleeding. The patient was discharged home the same day in stable condition.\nOn postoperative day 19, the patient presented to the emergency room with a three-day history of pain in the right gluteal and posterior thigh regions. He also complained of dizziness and occasional syncope when ambulating. Physical exam revealed a well-healed surgical incision with a large bruise extending from his right hip down the right leg (Figure ). CT of the pelvis showed a large gluteal hematoma with extension into the posterior compartment of the thigh (Figure ). CT angiogram (CTA) of the right lower extremity showed disruption of the blood flow through the superior gluteal artery at the level of the inferior two implants as the vessel exited the greater sciatic notch (Figure ). Interventional radiology was consulted for possible embolization, but they determined there was no visible active extravasation and recommended observation and serial hemoglobin draws. The patient’s hemoglobin on the day of presentation was 9.8 g/dL, and on hospital day one it was 8.5 g/dL. Due to this drop in hemoglobin over 24 hours and the patient’s increasing pain level, the decision was made to take the patient to the operating room for exploration and removal of hematoma.\nThe patient was placed in a left lateral decubitus position, and a curved incision was made over the right posterolateral hip for a Kocher-Langenbach approach. Electrocautery dissection was taken down to the level of gluteus maximus muscle fascia. The fascia was carefully incised, and the hematoma was progressively evacuated from under the gluteus maximus and gluteus medius planes. After evacuation, the greater trochanter became palpable. Once the surgical bed was cleared of hematoma, the SGA was identified. A branch of the SGA was noted to have been lacerated with active bleeding (Figure ). The vessel was clamped and ligated using 3-0 silk sutures. A 15-French subfascial Jackson Pratt drain was placed at the end of the procedure. On postoperative day three, he was discharged in improved condition. His surgical drain was removed at the three-week postoperative visit. At his two-month follow-up visit, the patient’s right-sided SI joint pain was gone. At four months, the patient was walking unassisted and was not requiring pain medicines.","This 71-year-old man had persistent pain in his hip and lower back that radiated down his leg. After trying injections, he decided to have surgery to fuse his SI joint. During the surgery, a large blood clot formed in his buttock, which caused pain and dizziness. The blood clot was removed, and the bleeding vessel was repaired. He is now experiencing relief from his pain and is able to walk without assistance."
256,"A 67-year-old female with a past medical history of iatrogenic hypothyroidism secondary to thyroidectomy stopped taking her levothyroxine over a year ago. Three months prior to her presentation, she had been experiencing progressively worsening confusion and respiratory distress. According to her family, she became bed-bound approximately a week before admission, when she was brought in by ambulance after being found unresponsive by family members.\nOn admission, her vital signs were unremarkable with a heart rate of 70 beats per minute. Her physical examination was significant for lethargy, facial swelling, macroglossia, and bibasilar crackles of the lung fields. Laboratory findings were pertinent for thyroid-stimulating hormone (TSH) at 58.5 ng/dL and free thyroxine (T4) of 0.19 ng/dL. Electrocardiogram (EKG) showed normal sinus rhythm with low voltage QRS complexes with electrical alternans (Figure ). The enlarged cardiac silhouette was noted on chest radiography (CXR) along with congested lung fields (Figure ). Following consultation with the endocrinology service, the patient was started on intravenous levothyroxine and hydrocortisone and admitted to medicine telemetry service for further management.\nOn the following day, a transthoracic echocardiogram (TTE) showed a large pericardial effusion with the swinging of the heart in the pericardial sac with the diastolic collapse of both atria and the right ventricle (Figure , Figure , Figure ). The patient was upgraded to Cardiac ICU and underwent urgent pericardiocentesis with 1300 mL serous straw-colored fluid drained. Analysis of pericardial fluid was negative for malignant cells. Acid-fast bacilli stain and culture were negative. The patient was discharged on levothyroxine. Serial TSH and T4 measurements over the subsequent two months showed normalization of both values. On six months follow-up, repeat TTE showed diminishing pericardial effusion without echocardiographic evidence of cardiac tamponade.","This 67-year-old woman stopped taking her thyroid medicine, which caused her thyroid levels to drop dangerously low. This led to confusion, difficulty breathing, and swelling in her face and tongue. Tests showed a problem with her heart and fluid around it, requiring a procedure to drain the fluid. After treatment, her thyroid levels returned to normal, and her heart condition improved, allowing her to be discharged home."
257,"A 95-year-old lady with a past medical history of heart failure with reduced ejection fraction (HFrEF) and biventricular implantable cardioverter-defibrillator (ICD), hypertension, and asthma presented to the emergency department for evaluation of nausea, vomiting, and a two-month history of intermittent diarrhea, which had been worsening for a few days prior to admission. Diarrhea was associated with severe, diffuse, waxing, and waning abdominal cramps, which were noted to improve after emesis. No correlation was noted with eating habits and no history of recent antibiotic use was reported. On examination, the abdomen was soft but tender on deep palpation, with audible bowel sounds. No organomegaly or costovertebral angle (CVA) tenderness was appreciated.\nInitial laboratory results revealed hypokalemia (3.3 mEq/L), lipase within normal limits (32 U/L), and normal transaminases (aspartate aminotransferase [AST]: 19 U/L; alanine aminotransferase [ALT]: 10 U/L) and bilirubin (total bilirubin: 0.7 mg/dl; direct bilirubin: 0.1 mb/dl). Stool studies were unremarkable and coronavirus disease 2019 (COVID-19) reverse transcription-polymerase chain reaction (RT-PCR) was negative. CT abdomen revealed a long segment of the colon with a loop within loop appearance from the proximal transverse colon to the distal descending colon, consistent with intussusception (Figures , ). The underlying lesion for the lead point was not well identified.\nThe patient was taken to the operating room the next day where local exploration using laparoscopy revealed complete telescoping and intussusception of terminal ileum into the distended ascending and transverse colon. At that time, it was decided to proceed with an open procedure and right hemicolectomy extending from the terminal ileum to about one-third of proximal transverse attachment of the mid transverse colon was performed and side-side anastomosis was created between terminal ileum and transverse colon at the anti-mesenteric border. The patient tolerated the procedure well with no immediate complications. Surgical pathology revealed a tubulovillous adenoma with high-grade dysplasia as the lead point of the intussusceptum. It was negative for in-situ or invasive carcinoma. The postoperative course was significant for acute kidney injury (Cr 1.3 mg/dl with oliguria) on postoperative day two, which resolved after administration of IV fluids. The patient was discharged home with home care services on postoperative day six.","A 95-year-old woman with heart problems, asthma, and a history of diarrhea was admitted to the hospital because of worsening symptoms. Doctors found that her colon was telescoping into itself (intussusception), which caused pain and required surgery to remove a portion of her colon. The surgery revealed a precancerous growth in her colon, which was removed along with the affected section. She experienced some kidney problems after the surgery, but her condition improved, and she was discharged home after six days."
258,"A 31-year-old Caucasian male presented to the ED with a 10-day history of progressively worsening symptoms that started with a sore throat and dysphagia. Five days after the development of initial symptoms, productive cough with orange-colored sputum began. His condition worsened four days before presentation, with the development of fever, chills, night sweats, significant diarrhea, emesis, and pleuritic chest pain. On initial presentation, the patient appeared ill and anxious. He was febrile with a temperature of 102° Fahrenheit, tachycardic with a heart rate of 118 beats per minute, and hypotensive with a blood pressure of 109/54 mmHg. On physical exam, he had a respiratory rate of 18 breaths per minute and oxygen saturation of 95% on room air. His neck was supple with absent lymphadenopathy and both lungs were clear to auscultation. Laboratory work on admission was remarkable for a leukocyte count of 23.2 x 10^9/Liter, a neutrophil count of 85.6% with 16% bands, serum sodium of 132 mmol/Liter, and a D-Dimer of 4.29 mg/L FEU. Initially, one gram of ceftriaxone and 500 mg of azithromycin were administered intravenously in the ED. Tests were then ordered including a monospot and rapid Streptococcus A antigen via pharyngeal swab, as well as (1,3)-beta-D-glucan and HIV-1 RNA via serum, and Legionella pneumophila urine antigen; all without remarkable findings. A complete viral respiratory panel was negative, and PCR did not detect Mycoplasma pneumoniae, SARS-CoV-2, or Chlamydia pneumoniae. A portable chest X-Ray performed in the ED demonstrated right upper lobe pneumonia (Figure 1). A CT angiogram of the chest ordered to rule out pulmonary embolus (Figures -) demonstrated no signs of acute pulmonary emboli. However, findings displayed a large consolidation in the right lower lobe and a 4 cm cavitation in the left lower lobe. Transthoracic ECG was then performed, demonstrating no vegetations or valvular abnormalities. Trans-esophageal ECG was recommended to the patient, but he declined. Next, a CT scan of the neck was ordered, showing no evidence of abnormalities. Without significant improvement in WBC count for three days and persistent fevers >100° Fahrenheit, his antibiotic regimen was escalated to three grams of ampicillin-sulbactam administered intravenously every six hours. This resulted in an improved WBC to 16.1 x 10^9/Liter the following day. The patient had no further fevers and reported improvement in pleuritic chest pain and dysphagia. On day five of hospitalization, the initial blood culture grew a single anaerobic gram-negative bacterium, F. necrophorum. The patient continued to show improvement without signs of fever, nausea, or vomiting. A repeat negative blood culture and final WBC count of 14.5 x 10^9/Liter was reported on day eight of hospitalization before the patient was discharged later that afternoon. He was discharged home in a stable condition with 500 mg of amoxicillin-clavulanic acid given orally every twelve hours for seven days and was instructed to follow up with his primary care physician in three weeks for a repeat CT scan of his chest to monitor the resolution of pulmonary lesions.","This 31-year-old man came to the hospital after a 10-day illness that started with a sore throat and difficulty swallowing. He developed a bad cough with yellow mucus, fever, and other symptoms like diarrhea and chest pain. Tests showed he had pneumonia in his lungs and an infection with a specific bacteria. After starting stronger antibiotics, his fever went away, and he began to feel better, and he was discharged home with oral medication and a follow-up appointment."
259,"A 48-year-old African American man with a medical history of COVID-19 pneumonia and obesity presented to the emergency department with left foot pain for the past three days. Initial workup returned a diagnosis of gout but also uncovered an incidental finding of a platelet count of 7x103/mL. All other labs including white blood cell count and hemoglobin were normal. The patient stated he had been hospitalized for COVID-19 pneumonia five weeks prior but denied any lingering symptoms, had not noticed any bleeding and denied any home medications. Additionally, the patient denied any relevant family or social history. Vital signs were stable. The physical exam did not reveal any petechiae, purpura, palpable lymphadenopathy, or visceromegaly.\nChart review showed that the patient's platelet count was 325x103/ml when he was discharged from the hospital five weeks prior. Upon further workup for thrombocytopenia, a repeat complete blood count confirmed the initial finding, and microscopy did not reveal any clumping or abnormal platelet morphology. Coagulation studies were non-contributory. A viral panel, including HIV, hepatitis C virus (HCV), and Epstein-Barr Virus (EBV), was non-reactive and folate and B12 levels were within normal limits. Ultrasound of the spleen was unremarkable. Having ruled out other causes of thrombocytopenia, we made a diagnosis of secondary immune thrombocytopenia due to COVID-19.\nThe patient was admitted for observation and transfusion. The patient was given two units of platelets. The patient was also started on prednisone 40mg daily. He was discharged home on day two with a platelet count of 47x103/ml. During follow-up as an outpatient two weeks later, complete blood count (CBC) revealed his thrombocyte count was 120x103/ml.","This 48-year-old man came to the hospital because of pain in his left foot. Doctors found he had gout, but they also discovered his platelet count was very low. This low platelet count was likely caused by his previous COVID-19 infection, and they are treating it with medication and a platelet transfusion. His platelet count has since improved, and he is being monitored closely as an outpatient."
260,"A 21-year-old Danish man diagnosed with attention deficit hyperactivity disorder (ADHD) in adolescence, which was currently untreated, was involuntarily admitted to a psychiatric ward by the police in an agitated and aggressive state after a violent conflict with his parents. Clinically, the patient’s state was compatible with acute intoxication, and the patient confirmed “drug intake,” but quantification could not be specified at admission. Eight months prior, the patient had spent 2 weeks in deep propofol sedation at an intensive care unit (ICU) with acute organic delirium due to GHB withdrawal.\nAt the psychiatric ward, the patient’s threatening and aggressive behavior intensified. To avoid violent incidents, the patient was subjected to physical restraint (abdominal belt) and tablet lorazepam 4 mg was administered twice. The patient was disorientated, started expressing paranoid ideas, and developed diaphoresis, tachycardia (heart rate 130 beats per min), and hyperthermia (37.8 °C, tympanic). Because he started biting the metal locks on his abdominal belt, his hands and feet were also restrained.\nOn the second day, the patient was diagnosed with acute organic delirium caused by GHB withdrawal and treatment was initiated. In accordance with ICD-102, the diagnosis was based upon the clinical presentation in combination with a history of GHB substance abuse. The patient was treated with a cumulative dose of 300 mg diazepam (40 mg as oral administration and 260 mg intravenously), without induction of sleep. To obtain sedation the restrained patient was transferred to the ICU, where he was intubated and sedated with intravenous infusions of propofol (up to 16 mg/kg/hour) and sufentanil (up to 100 μg/hour).\nOn days 3–6, the patient remained deeply sedated and on mechanical ventilation. To counteract potential withdrawal symptoms, clonidine 225 μg was administered every 6 hours via a nasogastric tube (NG tube), intravenous diazepam 20 mg every 5 hours, and continuous midazolam infusions (up to 1 mg/kg/hour). To prevent Wernicke encephalopathy, intravenous thiamine 200 mg and vitamin B 2 ml solution were given. Due to elevated C-reactive protein (64 mg/L) and white blood cell count (leukocytes 12.2 × 109/L; neutrophils 9.1 × 109/L), empiric treatment with piperacillin/tazobactam 4 g every 6 hours was initiated.\nOn day 6, a first wake-up call was attempted. However, upon awakening the patient was disoriented and severely agitated, and he was readily reintubated and resedated. The psychopharmacological treatment was intensified with olanzapine 20 mg/day (via NG-tube), intravenous diazepam 40 mg every 5 hours, clonidine 225 μg every 6 hours (via NG-tube), and continuous midazolam infusions (up to 1 mg/kg/hour).\nOn days 7–9, the intravenous diazepam was increased to 60 mg every 5 hours, and to supplement the propofol sedation, methadone 10 mg twice a day (via NG-tube) was initiated.\nOn day 10, a second wake-up was attempted. Again, the patient was disoriented, reported of ants crawling in the room, and presented with severe agitation. Physical restraints were reinstated and intravenous haloperidol 30 mg/day was added.\nOn day 11, the psychiatrists decided to initiate bilateral frontotemporal ECT as “en bloc” treatment, that is ECT on three consecutive days. Medication status during the three en bloc ECTs was: olanzapine 20 mg twice a day (BID, via NG-tube), methadone 10 mg BID (via NG-tube), intravenous phenobarbital 100 mg every 4 hours, pregabalin 150 mg BID (via NG-tube), haloperidol 5 mg as needed (PRN), up to 50 mg a day, clonidine 225 mg every 6 hours (via NG-tube), and continuous infusions of midazolam (up to 1 mg/kg/hour). Because treatment with high doses of benzodiazepines with long half-lives could compromise seizure induction, the starting ECT dose was set at the maximal energy of 200% (1008 mC) along with intravenous flumazenil 0.4 mg. The ECT series are shown in Fig. .\nOn day 12, the first ECT was administered (ECT #1A). Due to an administrative error, flumazenil was not administered, and restimulation with concurrent flumazenil was performed (ECT #1B). After ECT, the patient remained awake although clinically unchanged.\nOn day 13, the second ECT was administered (ECT #2). Subsequently, the patient’s level of agitation decreased, he briefly responded adequately to verbal communication and sleep improved.\nOn day 14, the third ECT (ECT #3A) was administered. Due to seizure at threshold level, restimulation after hyperventilation was performed (ECT #3B). The patient was less agitated and engaged in brief conversations. Haloperidol was increased to 20 mg every 6 hours. On day 15, 50 mg hydroxyzine once a day (QD) was added.\nOn day 16, agitation had completely subsided, restraints were removed, and the patient cooperated with the remaining treatment.\nOn day 17, the fourth ECT was administered (ECT #4). On day 18, the patient displayed no neurological or psychiatric symptoms, all sedatives and psychopharmacological treatment were discontinued, and he was transferred to a general internal medicine ward.\nOn day 19, the patient was still in complete remission, and he insisted on discharge. Upon discharge the patient confirmed daily use of GHB and cocaine prior to hospitalization, and he provided oral and written consent to report his case in the medical literature. Since this is a case report, ethical approval was waived according to Danish regulations.","This 21-year-old man was admitted to the psychiatric ward after a violent argument with his family and a period of intense agitation. He had a history of substance abuse, including propofol, and was experiencing withdrawal symptoms, including confusion and paranoia. Due to his aggressive behavior and the risk of harm to himself and others, he was placed on restraints and given medication to calm him down. After several days of treatment, including ECT, he began to improve and was eventually discharged home, but he acknowledged ongoing substance use."
261,"A 56-year-old woman of North African origin, with no medical and surgical history, was admitted to the Department of Internal Medicine for investigation of a Raynaud phenomenon evolving for 3 months, associated with inflammatory arthralgias involving the wrists, elbows, and ankles; the patient also reported pain and progressive proximal muscle weakness in her lower limbs when climbing stairs, which extended to the upper limbs. There was no rash. Neuromuscular examination revealed proximal tetraparesis, mild neck flexion, and extension weakness. Strength at the neck was 4/5; at the shoulder 3/5; at the wrist 4/5; at the hips 2/5; and at the ankle 4/5; dorsiflexion/plantar flexion was normal. Tendon reflexes were normal. A few days after her hospitalization, the patient developed slight dysphonia and dysphagia; then this symptomatology was accentuated over time.\nNail fold capillary microscopy was normal. Blood tests showed significant levels of muscle enzymes [creatinine kinase (CK) 40,000 IU/L]. Autoimmune screening for myositis-specific and myositis-associated autoantibodies was negative. Electromyography (EMG) showed a decrement in compound muscle action potential on repetitive stimulation and clear signs of myopathy. The diagnosis of inflammatory myopathy was confirmed by muscle biopsy, which showed myositis (Fig. ). The patient was treated with prednisone 60 mg daily; we observed a slight improvement in muscle weakness and a significant decrease in CK serum level.\nA month later, she developed intermittent diplopia, ptosis, and mild dysarthria during the conversation. Serum acetylcholine receptor (AChR) antibodies were positive (5.59 nmol/L), and muscle-specific kinase antibodies were negative. A broader workup performed with computed tomography (CT) of the chest revealed a large anterior mediastinal mass (114 × 57 × 100 mm) (Fig. ), which a subsequent biopsy revealed as B1-type thymoma (Fig. ).\nInflammatory myositis associated with myasthenia gravis was diagnosed. Pyridostigmine in combination with steroids was started, with significant improvements in generalized weakness, ptosis, and dysarthria. The patient underwent thymectomy as part of the additional treatment. Currently, she is on a low dose of steroids and pyridostigmine and is asymptomatic. The features of myositis have not relapsed.","A 56-year-old woman was admitted to the hospital because she was experiencing Raynaud's phenomenon, joint pain, and muscle weakness. Tests revealed high levels of muscle enzymes and showed signs of muscle damage, leading to a diagnosis of inflammatory myopathy. Further investigation found a large mass in her chest, which was identified as a type of thymoma, and she also developed antibodies associated with myasthenia gravis. After treatment with medication and surgery, she is now feeling much better and has no further symptoms."
262,"A 70-year-old man with a high carbohydrate antigen 19-9 level was diagnosed with resectable pancreatic cancer. Enhanced CT revealed a 15-mm mass in the pancreatic body and dilatation of the peripheral main pancreatic duct (Fig. A). The patient underwent DP with lymph node dissection. DP was performed by transecting the pancreas at the right border of the PV with a powered linear stapler. Additional pancreatic tissue was observed on the dorsal aspect of the PV (Fig. B), and the patient was diagnosed with PAP (type II). After the diagnosis, additional pancreatic resection was conducted using a powered linear stapler. The total operative time was 527 minutes, and the estimated blood loss was 730 mL. Based on the Union for International Cancer Control TNM staging, the pathological diagnosis was T3N0M0, stage IIA. In addition, the pathological margin was negative. The patient had no complications and was discharged on postoperative day 23. PAP was detected on preoperative CT images during a retrospective review (Fig. A).","A 70-year-old man was diagnosed with pancreatic cancer after tests showed a small mass in his pancreas. Surgery was performed to remove the tumor and a portion of the surrounding pancreas. During the surgery, additional cancer was found, requiring further removal. The surgery was successful, and the margins were clear, meaning no cancer cells were found at the edges of the removed tissue. He is now recovering well at home and will continue to be monitored for any signs of recurrence."
263,"A 46-year-old woman with a history of familial adenomatous polyposis and rectal cancer underwent PD with lymph node dissection for a newly diagnosed duodenal papillary carcinoma. Pancreatectomy was performed by transecting the pancreas superior to the PV. The pancreatic parenchyma was subsequently identified on the dorsal side of the PV (Fig. A) and transected, resulting in the exposure of a section (2 mm in length) of the main pancreatic duct. The remaining pancreas was mobilized caudally. An additional section was made in the pancreatic body caudal to the cricoid pancreatic junction so that the pancreatic cross-section was oriented in 1 plane. The diagnosis was PAP (type II). The total operative time was 439 minutes, and the estimated blood loss was 250 mL. Based on the Union for International Cancer Control TNM staging, the pathological diagnosis was pT1aN0M0, stage IA. In addition, the pathological margin was negative.\nAlthough the patient had a grade-B pancreatic fistula (International Study Group of Pancreatic Fistula Classification) that was diagnosed on postoperative day 3, the patient recovered after conservative treatment, which is continuous drainage until 2 days before discharge, and was discharged on postoperative day 18. We retrospectively reviewed the patients’ preoperative CT images and identified the PAP (Fig. B).","This 46-year-old woman had a new cancer found in her small intestine and underwent surgery to remove it. During the surgery, doctors had to remove part of her pancreas to reach the cancer, and they carefully monitored the remaining pancreas. The surgery was successful, and the cancer was completely removed with clear margins. She experienced a minor leak from her pancreas after surgery, but it resolved with treatment, and she was able to go home after 18 days."
264,"A 28-year-old woman presented to the emergency department with severe lower abdominal pain that was associated with nausea. Two weeks prior to admission, she had symptoms of abdominal pain and bloody diarrhea which was later diagnosed to be amebiasis. The patient was diagnosed with tuberous sclerosis at the age of 6 months when she developed arrhythmia related to a cardiac rhabdomyoma. The rest of the medical history included adenoma sebaceum, Ash leaf spots, bilateral renal angiomyolipomas, left-sided retinal hamartoma, and allergic rhinitis. She was previously operated 3 times for the removal of multiple renal angiomyolipomas and underwent partial cystectomy due to invasion of the tumor. Physical examination was normal. The laboratory work-up was unremarkable except for anemia (hemoglobin 9.6 g/dL) and an elevated C-reactive protein value of 125 mg/dL. The computerized tomography (CT) scan revealed extralumination of the rectal contrast material at the rectosigmoid region, indicating bowel perforation. The patient underwent proctosigmoidectomy and formation of an end colostomy together with the closure of the anorectal stump (Hartmann operation). Two weeks after the operation, the patient developed a new onset abdominal pain. The CT scan revealed contrast extralumination in the distal ileum and the patient underwent ileocecal resection due to a new onset bowel perforation and severe adhesions. Several days after the second operation she developed fever and was diagnosed with SARS-CoV-2 infection for which she took treatment. Both of the rectosigmoid resection specimen and the ileocecal resection specimen were reported as leiomyomatosis-like lymphangioleiomyomatosis (Fig. ). After 8 months of follow-up, the patient's rectoscopic examination together with a control CT scan was normal. At the first year of follow-up, the patient had an ileostomy closure surgery.\nGrossly, the rectosigmoid specimen (16 cm in length, 7 cm in width, 1 cm in thickness) consisted of full-thickness hemorrhage extending 7 cm proximally from the surgical margin. Macroscopically, there was a 3 cm perforation area in the central part of this hemorrhagic region. Pseudopolypoid lesions ranging from 0.1 to 0.3 cm in diameter were observed in the surrounding mucosa. The ileocecal resection material (ileum 4 cm and colon 16 cm in length) consisted of a 5 cm, pale-colored, nodular lesion in the cecum. Numerous pale nodular lesions, ranging from 0.3 to 5 cm in diameter extending from the mucosa to the subserosa were observed in the cecum. In all resected specimens, the intestinal wall was diffusely thickened and nodular. Infiltration into the subserosal adipose tissue was observed.\nHistological examination revealed nodular lesions with an extensive growth pattern, extending from the submucosa to the mesorectal adipose tissue in some areas, without affecting the mucosa. Microscopically, the nodules consisted of spindle cell bundles with prominent elongated nuclei organized into branching short fascicles (Fig. ). Necrosis, pleomorphism, and mitotic activity were not observed. Among the spindle cells, clusters of epithelioid cells were observed around dilated vascular structures lined with endothelium in some areas (Fig. ). It was noted that these epithelioid cell assemblages in the vessel walls tended to merge with the surrounding lymphatics. Mature adipose tissue was not observed. In the surrounding intestine where the tumor was absent, severe active colitis findings were observed. Immunohistochemically, the spindle and epithelioid cells in both of the resected specimens stained positive for desmin, SMA, and HMB45 and negative for CD34, DOG-1, c-KIT, S100, ER, and PR (Fig. ). The Ki-67 proliferation index was found to be 3%. There was no evidence of lymphatic or vascular invasion.","This 28-year-old woman has had a complex medical history, including a rare genetic condition called tuberous sclerosis and multiple surgeries to remove kidney tumors. She initially presented with abdominal pain and diarrhea, which was later diagnosed as amebiasis, and subsequently developed bowel perforations requiring two major operations. Pathology of the resected tissue revealed a rare condition called leiomyomatosis-like lymphangioleiomyomatosis, characterized by abnormal growth of cells in the intestines. After a year of follow-up, the patient’s condition has stabilized, and the ileostomy was closed."
265,"A 27-year-old G1P0 pregnant woman, whose results of non-invasive prenatal gene detection at 21+2 weeks of gestation, suggested that the risk of trisomy 18 (– 6.6) was high, while the risk of trisomy 21 (0.681) and trisomy 13 (2.363) was low. Ultrasound examination at 23+2 weeks of gestation revealed a single live fetus with a gestational age of 23+2 weeks in utero, whereas the umbilical cord was wrapped around the neck of the fetus for 1 week. The measurement indexes of fetal development are as follows: biparietal diameter = 6.0 cm, head circumference = 20.9 cm, abdominal circumference = 17.4 cm, femur long = 4.0 cm, amniotic fluid index = 16.7 cm, and the 3 values of umbilical artery S/D were 4.6, 3.2, and 2.7 respectively. It is suggested that amniocentesis, chromosome karyotype analysis, and CNVs should be recommended to further confirm the condition of the fetal chromosome.\nChromosome karyotype analysis after amniocentesis showed that the fetal chromosome karyotype was 46, XY, del (18) (q22.3; q23) (Fig. A). CNV detection results showed that this sample detected chromosome aneuploidy or CNVs of more than 100 kb, which was regarded as the definite pathogenic genomic: 18q22.3q23 (68900001-76560000), and the size of the deleted fragment was 7.66 Mb (Fig. B, C).","This 27-year-old woman is currently pregnant and has a high risk of a chromosomal condition called Trisomy 18, as indicated by a screening test. An ultrasound revealed that the umbilical cord was tightly wrapped around the baby’s neck, which can sometimes cause problems. To confirm the diagnosis, amniocentesis was performed, and chromosome analysis confirmed a deletion of chromosome This confirms the diagnosis of Trisomy 18, and further genetic testing has been completed."
266,"A 35-year-old G4P1 pregnant woman who had 1 natural birth and 2 induced abortions. The results of non-invasive prenatal gene detection at 19+2 weeks of gestation suggested that the risk of trisomy 21 (–1.249), trisomy 18 (–1.534), and trisomy 13 (–1.904) were all at low levels. Ultrasound examination at 21+2 weeks of gestation revealed that there is a single live fetus with a gestational age of 21+2 weeks in utero, whereas it is difficult to determine whether the fetus has only 1 umbilical artery to supply blood. The measurement indexes of fetal development are as follows: biparietal diameter = 4.9 cm, femur long = 3.6 cm, and the deepest amniotic fluid was 5.7 cm. The couple requested amniocentesis, chromosome karyotype analysis, and CNVs to further confirm the diagnosis.\nChromosome karyotype analysis after amniocentesis showed that the fetal chromosome karyotype was 46, XY, del (18) (q22.2; q23) (Fig. A). CNV detection results showed that this sample detected chromosome aneuploidy or CNVs of more than 100 kb, which was regarded as the definite pathogenic genomic: seq [hg19] del (18) (q22.1q23), chr18: g.6602000178020000del (Fig. B, C).\nAfter half a year, the personnel of the prenatal diagnosis center followed up the 2 couples and learned that both couples voluntarily terminated their pregnancy after knowing the results of amniocentesis.","This 35-year-old woman is currently pregnant and had previously had two abortions. Initial screening tests showed a very low risk of certain genetic conditions like Down syndrome. An ultrasound revealed a possible issue with the baby’s blood supply, and she requested further testing, including amniocentesis. The tests confirmed a chromosomal abnormality (Trisomy and the couple decided to terminate the pregnancy."
267,"In January 2018, a 78-year-old man presented with complaints of numbness in the soles of both feet. There was no motor weakness on neurologic examination. Severe L4-5 central stenosis and root redundancy were diagnosed on lumbar MRI (Fig. ).\nOn February 28, 2018, we performed L4-5 OLIF, followed by bilateral L4 laminotomy and L4-5 percutaneous posterior screw fixation (PPSF). To perform OLIF first, after administering general anesthesia, we ensured that the hip joint was not flexed in the standard right posture. The axilla and hip were fixed using a wide cloth and tape. The external oblique, internal oblique, and transverse abdominal muscles were incised and approached through the retroperitoneum to gently retract the abdominal organs and psoas to expose the intervertebral space. There was no direct blood vessel injury or nerve injury during this process. After determining the height and length of the cage using the C-arm, we vertically inserted a cage (Clydesdale DLIF Cage, 6°∗14∗50 mm; Sofamor, Memphis) combined with a demineralized bone matrix into the intervertebral space. After turning the patient to the prone position, bilateral laminotomy was performed, with minimal damage to the posterior tension band, and severe ligamentum flavum hypertrophy was confirmed and removed. Thereafter, L4-5 PPSF was performed.\nIntraoperatively, an increase in the disc height and foramen height was confirmed using the C-arm, and sagittal alignment was maintained well. There was no motor weakness postoperatively. Two days later, the patient was allowed to walk while wearing an orthosis, and the radiating pain improved; he was then discharged from the hospital.\nAt 6 weeks after surgery, he complained of pain and swelling below both ankles at the outpatient clinic. Lumbar CT and radiographic examination showed that the fusion was maintained without subsidence of the surgical site, the height of the intervertebral disc space and foramen increased, and there was no abnormality in the alignment between the lumbar vertebrae (Fig. ). In addition, a prominently protruding osteophyte, which was considered insignificant before the first surgery, was again observed. In particular, the osteophytes on the foraminal side were severely protruding (Fig. ). In the 10th week after surgery, he visited the outpatient clinic with a cane, complaining of persistent pain below both ankles and motor weakness of the right ankle. A marked decrease in dorsiflexion of the right ankle was observed. The Medical Research Council (MRC) muscle strength grade of the right ankle was 2. We confirmed the resolution of the previous central stenosis and root redundancy on lumbar MRI and found no other specific findings (Fig. ).\nWe thought that it was necessary to explore the L4 nerve roots again, focusing on the right side. On May 21, 2018, we performed right L4/5 facetectomy and foraminotomy to release the right L4 nerve root. During surgery, the tissues compressing the L4 nerve root and the osteophyte protruding severely and trapping the nerve root were identified and removed. Within 1 week of hospitalization after surgery, the radiating pain in both feet disappeared, and dorsiflexion of the right ankle was restored to MRC grade 4. After the second surgery, follow-up CT of the lumbar spine confirmed clear decompression of the right foramen (Fig. ). To date, he has no radiating pain in both feet, and motor performance of the right ankle has been restored and maintained.","This 78-year-old man initially experienced numbness in his feet, which led to a spinal surgery to address a narrowing of the space in his lower back. The surgery involved placing a bone graft and screws to stabilize the spine and improve the alignment of the vertebrae. After the initial surgery, he reported pain and swelling in his ankles, and further imaging revealed significant bone spurs that were pressing on his nerves. A second surgery was performed to remove these bone spurs and release the pressure on the nerves, resulting in the return of pain-free movement in his ankles."
268,"An 80-year-old female patient presented to the emergency department of our institution with a 5-day history of headaches. A week ago, she had presented with a minor head trauma that was caused by a fall. She had a history of hypertension and dyslipidemia and had experienced focal cerebral infarction 10 years earlier. The patient had no neurologic abnormalities, except for headache. An initial non-contrast brain computed tomography (CT) scan showed a scanty subarachnoid hemorrhage (SAH) on the left ambient cistern with an enlarged ventricle (Fig. A). Subsequent brain CT angiography (CTA) showed no vascular abnormalities. Based on the CT findings and the patient's history of trauma, she was admitted to the neurosurgery department as a suspected case of traumatic SAH, following which her symptoms improved gradually. On the 10th day of hospitalization, the patient showed sudden stuporous mental deterioration. Brain CT performed at that time revealed SAH on all the cisterns, with intraventricular hemorrhage (Fig. B). A CTA performed immediately after the procedure revealed no definitive cerebral aneurysm on 3-dimensional reconstruction images, but a suspicious aneurysmal lesion was seen in the distal PICA in the thin axial CTA image (Fig. C). Subsequent digital subtraction angiography (DSA) revealed a bilobulated distal PICA aneurysm, and an endovascular treatment was planned (Fig. A). Coil embolization was performed under general anesthesia. After a right femoral artery puncture, the left vertebral artery (VA) was not visible on left subclavian angiography; therefore, a 6-Fr soft, torqueable catheter optimized for intracranial access (SOFIA; MicroVention Terumo, Tustin, CA) was advanced into the V4 segment of the right VA under roadmap guidance. Subsequently, we attempted to reach the Headway 17 microcatheter (MicroVention Terumo, Tustin, CA) into the aneurysmal sac, but it failed due to the acute angle of the vertebra–PICA junction. Therefore, after deploying the coil in the VA just before the PICA origin, we planned to position the microcatheter in the PICA with the support of the coil mass (Fig. B). However, some of the coil masses deployed to the VA migrated upward of the PICA origin unintentionally during the navigation process using a microwire and microcatheter (Fig. C). Therefore, we additionally deployed a coil to the VA and finished the procedure after confirming PICA occlusion (Fig. D). Fortunately, with supportive care in an intensive care unit after the procedure, the patient recovered fully, and follow-up magnetic resonance imaging showed no definitive acute cerebral infarction in the PICA territory. One year after embolization, a follow-up angiography was performed, which revealed recanalization of the right PICA with normal arterial configuration, and no aneurysmal sacs were observed in the distal PICA. The recanalized VA was located medial to the deployed coil mass (Fig. E).","An 80-year-old woman was in the hospital with a headache that started five days ago, and she had recently fallen. She has a history of high blood pressure and a previous stroke. Doctors found a small amount of bleeding in her brain after the fall, and then later discovered a small aneurysm in a blood vessel near her brain. After a procedure to block the aneurysm, she recovered fully, and follow-up scans showed the aneurysm had closed."
269,"An 8-year-old Chinese boy with no specific family or psychosocial history was admitted to our hospital with an emergency occurrence of purpura, severe hematemesis, large bloody stools, and sharp abdominal pain. His symptoms had begun 2 weeks prior to admission, with abdominal pain and an erythematous pinpoint rash on his arms. The rash developed from his upper limbs (opisthenar and arms) to his lower limbs. Laboratory examination in the outpatient clinic revealed no abnormalities in indicators such as white blood cell count, platelet count, and hemoglobin. After oral medication (specific unknown), his abdominal symptoms began to reduce, as did the purpura. Ten days prior, the rash progressed mostly on the legs, and he experienced deeper abdominal pain in the form of persistent pain with abdominal gassiness and emesis. He was admitted to another hospital with a diagnosis of HSP. Seven days prior, he had melena. After fasting and fluid replacement, there was no obvious improvement in the symptoms. Half a day prior, his face and lips appeared pale, and he had increased abdominal pain, accompanied by numerous bloody stools and hematemesis. He was immediately treated with methylprednisolone, posterior pituitary injection, somatostatin, and hemocoagulase, together with the infusion of large blood products (Table ). At this stage, the patient still produced large amounts of blood in the vomit and stool. His vital signs were extremely unstable, and he was transferred to the pediatric intensive care unit of our hospital. The patient had purpura with lower limb predominance and abdominal pain (consistent with one of the following 4 criteria: abdominal pain, histopathology, arthritis or arthralgia, and renal involvement), and was diagnosed with HSP.[\nOn admission, he had severe anemia, with a blood pressure of 76/44 mm Hg, pulse of 135 beats/min, and respiratory rate of 25 breaths/min. There was also a palpable purpura rash on both legs, tenderness in the abdomen, and absence of bowel sounds. Routine blood examination revealed a hemoglobin level of 70 g/L, white blood cell count of 11.48 × 109/L, and platelet count of 13 × 109/L. The liver function test showed albumin 10 g/L. The serum electrolytes showed sodium 140 mmol/L, potassium 2.92 mmol/L, chloride 112.1 mmol/L, and bicarbonate 15.9 mmol/L (Table ) with coagulopathy. Therefore, HSP complicated with an alimentary tract hemorrhage was diagnosed. He was immediately treated with intravenous methylprednisolone (2 mg/kg per day), infusion of blood products, and somatostatin but after 2 days after treatment (day 3), he still had massive gastrointestinal hemorrhage, poor circulation, a reduced level of consciousness, and unstable vital signs. Abdominal ultrasonography and plain abdominal radiography revealed no enterobrosis. The patient was also evaluated by pediatric surgeons, and the continuation of medical treatment was suggested. However, his condition deteriorated, and he needed to rely on a life-supporting blood transfusion. He developed signs of peritonitis with abdominal distension. Gastrointestinal tract perforation was suspected. A contrast-enhanced computed tomography (CT) scan of the whole abdomen revealed curved liquid samples and gas shadows on the left and front of the stomach, supporting the possibility of gastrointestinal tract perforation (Fig. ). On day 4, an emergency laparotomy was performed. The surgery revealed that the anterior wall of the descending duodenum was thinner with a 1 cm × 1 cm diameter perforation, and the posterior wall had a 3 cm × 1 cm ulcer, which had penetrated into the peritoneum of the pancreas and experienced active bleeding from the broken end of the vessel. The intraoperative hemorrhage volume was approximately 3000 mL, including intragastric bleeding and intestinal blood clots. Pathological examination indicated chronic inflammation of the intestinal wall mucosa in the descending part of the stomach and duodenum, with hemorrhage, ulceration, interstitial congestion, and edema. Immunofluorescence microscopy of paraffin sections showed granular and lumpy IgA focal deposition in the blood vessel walls (Fig. ). Postoperatively, he was administered nasal continuous positive airway pressure-assisted ventilation, anti-infection treatment, albumin transfusion, platelet transfusion, abdominal drainage, methylprednisolone, fluconazole antifungal treatment, and wound dressing. The general symptoms gradually improved, vital signs were stable, and abdominal pain was relieved. The drainage tube was assessed after discharge, the abdominal CT was repeated after 1 month and the duodenostomy tube was removed 50 days after surgery. There was no evidence of rebleeding during the 2-month follow-up assessment.","This 8-year-old boy was admitted to the hospital after experiencing a sudden onset of symptoms including a rash, stomach pain, and bloody vomit and stools. Initially, doctors thought he might have a stomach bug, but his condition quickly worsened, leading to severe anemia and unstable vital signs. After a series of tests, he was diagnosed with Henoch-Schönlein Purpura (HSP) complicated by bleeding in his digestive system. An emergency surgery was performed to repair a hole in his stomach and stop the bleeding, and he is now recovering well."
270,"A 42-year-old man was admitted to emergency service after a ski injury. He was unable to weight-bear. Effusion of the knee, severe posterior knee pain, and tenderness of the posterior structures of the knee were determined in the initial physical examination. The pain was getting worse when the knee was extended. The range of motion (ROM) was very limited, and the motion itself was causing the patient a lot of pain. The patient did not allow the tests Lachmann, McMurray, or posterior drawer to be performed due to pain and spasm. There were no findings of neurovascular damage.\nTwo avulsed fragments were revealed in the plain radiographs (Fig. A&B). MRI showed the fragments belonged to PCL and lateral meniscus, and concomitant avulsion fractures of the medial meniscus and PCL were diagnosed (Fig. ). ACL, lateral meniscus and medial/lateral collateral ligaments were intact. Based on these radiological and clinical findings, and due to the instability and locking risk that simultaneous PCL and medial meniscus avulsion fracture would create in the knee, the patient was recommended to operate.\nThe operation was performed the day after the injury. Under general anesthesia, physical examination was performed again, and the posterior drawer test was positive with a soft endpoint. Lachmann test was negative, and valgus stress test when the knee flexed 30° was positive as grade I laxity (opening of the medial joint <5 mm). Varus/valgus stress radiographs were performed under fluoroscopy, and medial space opening was checked and confirmed.\nThen the patient was placed in the prone position, and a tourniquet was applied and inflated. An L-shaped incision was made over the fossa poplitea. Dissection was performed, and the interval between the semimembranosus and medial head of the gastrocnemius was used, as described by Burks and Schaffer.[ At that point, a varicose vein that enlarged and curved, that we thought it was caused by trauma, was detected over the capsule; a cardiovascular surgeon was attended to the surgery and ligated the veins (Fig. A). And then gastrocnemius muscle was retracted laterally, and the posterior capsule was exposed. The avulsed bone fragments were palpated over the capsule, and a vertical capsular incision between these fragments was made (Fig. B). After removing the bone clots and soft tissues around the avulsed fragments, bone beds were prepared. Firstly, the medial meniscus's avulsion fracture was reducted and fixed with two Kirschner wires temporarily, and then PCL avulsion fracture was reducted in the same way under fluoroscopy control. Later, permanent fixation of the avulsion fracture of the medial meniscus was done with two headless 3.0 mm compression screws after drilling and measurement (Fig. C). Then, the PCL avulsion fracture was fixed with a 4.0 mm partially threaded, cannulated, cancellous screw with a washer (Fig. D). The fixation of the fragments was confirmed under fluoroscopy again, and the posterior drawer test was performed gently to check stability. After the irrigation of the wound, the capsule, subcutaneous layers, and skin was closed. A hinged, long leg brace was applied and locked in 0° knee extension.\nThe knee was kept in full extension with the hinged brace for two weeks and instructed for leg raises only in the brace several times daily. On the third week, isometric and passive ROM exercises with continuous passive motion device were applied and gradually increased as tolerated. Weight-bearing was not allowed for six weeks. At postoperative controls, knee X-rays were taken, and fracture healing was followed (Fig. C&D). No complications were encountered in the follow-up of the patient. Full weight-bearing and ROM without pain were achieved in the third month after the operation. The visual analog scale score was 8 before surgery and 2 in the third month after the surgery. Lysholm score was 14 before surgery and 85 in the sixth month after the surgery.","The patient was admitted to the hospital after a ski accident that caused significant pain and instability in his knee. X-rays and an MRI revealed that two pieces of bone were torn from the PCL and medial meniscus, and fractures were found in these structures. To restore stability and prevent further damage, the patient underwent surgery to repair the torn fragments with screws. After a period of healing and physical therapy, he was able to regain full use of his knee and return to normal activities."
271,"A 71-year-old woman visited our hospital in January 2018 with vision loss affecting her left eye. The best-corrected visual acuity (BCVA) in the left eye was 13/20. Ultra-wide-field pseudo-color (UWPC) and optical coherence tomography (OCT) images showed inferior temporal BRVO and secondary macular edema (ME) in her left eye. (Fig. A, B) Her left eye received 3 doses of intravitreal aflibercept (IVA), which resolved her ME. (Fig. C, D) Her BCVA was 20/20. No recurrence was found at the time of follow-up. This patient had not previously been infected with SARS-CoV-2. She received a second dose of the SARS-CoV-2 mRNA vaccine BNT162b2 (Pfizer-BioNTech) in July 2021. The following day, the patient noticed vision loss in her left eye. Her BCVA decreased to 20/30, and her UWPC and OCT images showed superior temporal BRVO and secondary ME in her left eye. (Fig. E, F) No avascular area was found on OCT angiography. The left eye received an additional one-time dose of IVA. The ME resolved (Fig. G, H), and the BCVA improved to 20/20.","6 This 71-year-old woman experienced vision loss in her left eye in January 2018, which was caused by a buildup of fluid behind the retina. Treatment with an injection into the eye helped to clear the fluid and improve her vision. Later, she experienced a recurrence of the same problem in the same eye, and another injection successfully resolved it. She was vaccinated against COVID-19 and remained healthy throughout her visits."
272,"A 74 year-old man visited our clinic in February 2020 with a complaint of vision loss affecting the right eye. His BCVA in the right eye was 20/20. His posterior pole fundus photographs and OCT images showed temporal superior BRVO without ME. (Fig. A, B) He was followed without treatment. He did not have a prior SARS-CoV-2 infection. He received his first dose of the SARS-CoV-2 mRNA vaccine BNT162b2 (Pfizer-BioNTech) in July 2021. On the following day, the patient noticed vision loss in his right eye. The patient was referred to our hospital. His UWPC and OCT images showed recurrence of temporal superior BRVO and secondary ME. (Fig. C, D) His BCVA was 20/25. No avascular area was found on the Optical coherence tomography angiography images. His right eye received 2 doses of intravitreal ranibizumab, which resolved the ME. His BCVA was 20/25. (Fig. E, F) The patient received his second vaccination dose 3 weeks after his first. He did not have any symptoms, nor did he have any changes in the examination of his right eye after receiving this second dose.","6 This 74-year-old man experienced vision loss in his right eye, which had been monitored without treatment since February Further tests revealed a recurring problem called BRVO with a secondary macular edema, causing more vision loss. He received injections to treat the macular edema, which successfully improved his vision. He received his COVID-19 vaccinations and had no further problems with his eye after the second dose."
273,"A 65-year-old woman presented with metamorphopsia and blurred vision in her left eye and was referred to Akita City Hospital for further examination. She had hypertension and dyslipidemia, which were treated with oral medication. At the initial visit, her best-corrected visual acuity (BCVA) was 20/20 in both eyes. Spherical equivalent refraction was –10.5 diopters and –14.0 diopters in the right and left eyes, respectively. The intraocular pressure was 18 and 16 mm Hg in the right and left eyes, respectively. Slit-lamp examination showed a slight nuclear cataract of grade 1 in both eyes, based on Emery-Little classification. Fundus examination revealed tigroid fundus in both eyes, and a white, well-defined, and patchy lesion at the superonasal to the fovea in the left eye (Fig. A). Autofluorescence showed hypofluorescence area in the macula. This is consistent with the aforementioned lesion in the left eye (Fig. B).\nUpon applying OCT to the vertical section through the fovea, the sclera curved posteriorly at the location superonasal to the fovea, and the choroid was thickened in the area (Fig. C). Additionally, the ellipsoid zone (EZ) was disrupted in the area between the fovea and the curved sclera. Horizontal volume scans of the OCT through the patchy lesion also showed posterior curvature of the sclera, thinning of the outer retina, and disappearance of the RPE–Bruch's membrane complex, resulting in a pseudo-fovea appearance (Fig. D). Another horizontal volume scan of the OCT demonstrated a connection of blood vessels running from the sclera to the choroid (Fig. E). There was no obvious connection between the vitreous cavity and the superior choroidal cavity with OCT; taken together, we diagnosed this case as PCA with macular ICC.\nDuring a 4-year follow up, the white patchy lesion in the fundus and the hypofluorescence region determined by autofluorescence gradually expanded, especially toward the optic disc (Fig. A and B). The BCVA in the left eye decreased with the expansion of the lesion and was 20/100 at the final visit. OCT through the fovea showed that the disrupted EZ expanded toward the ICC and the sensory retina of the fovea became thinner with time (Fig. C). Moreover, RPE–Bruch's membrane complex was not observed 3 years after the initial visit. During the follow-up period, the sensory retina was prominently displaced posteriorly to the ICC, though no obvious change was observed in the structure of the sclera.","A 65-year-old woman came to the hospital because she was experiencing blurry vision and changes in her eyesight. Tests revealed a small, white area in her left eye that was causing the vision problems, and it was gradually growing over time. The examination showed changes in the layers of her eye, including thinning of the retina and disruption of the light-sensing cells. As the area grew, her vision in the left eye worsened, and doctors monitored the condition closely during a four-year follow-up."
274,"A 51-year-old male patient was admitted to our center because of cough, fatigue, and shortness of breath for 2 weeks. He had previously been treated with antibiotics (cephalosporin and azithromycin) over 10 days. However, the symptoms continued. A detailed medical history revealed that he had an episode of pulmonary cryptococcosis 5 years ago (Fig. A). He recovered without any treatment and became asymptomatic. He has chronic HBV infection and is undergoing long-term treatment with entecavir. In addition, exposure to bird droppings was reported.\nComputed tomography (CT) performed at admission revealed multiple areas of consolidations and ground-glass shadows of bilateral lung fields (Fig. B). Chest auscultation demonstrated crude lung respiratory sounds and no evidence of dry and moist rales. His temperature was 36.6°C, respiratory rate was 22 breaths/min, pulse rate was 70 beats/min, and blood pressure was 118/70 mm Hg. Laboratory examinations were notable for C-reactive protein (CRP) of 27.1 mg/L and erythrocyte sedimentation rate (ESR) of 44 mm/h. Other findings such as complete blood cell count, flow cytometry analysis, procalcitonin, Mycoplasma pneumoniae (serological assay), HIV, and sputum culture were normal. Blood gas results were as follows: pH 7.426, pO2 67.4 mm Hg, pCO2 39.7 mm Hg, and SpO2 93.4%.\nPulmonary infection was first considered, and piperacillin sodium/tazobactam sodium was given empirically for 1 week. Contrast-enhanced CT was then performed and the results showed multiple consolidations and ground-glass shadows with rough margins and a maximum CT density of 77 HU, with scattering under the pleural surface (Fig. C and D). No obvious changes were observed compared with the previous CT scan. For rapid identification of the etiology, bronchoscopic examinations (such as BALF culture and mNGS) were performed. However, no additional information was available. Further examinations were performed; the lab findings included T-SPOT.TB (−), PPD (−), tumor markers (−), p- and c-ANCA (−), (1 → 3)-β-D-glucans (−), and GM assay (−). Notably, cryptococcal antigen testing was positive (55.85 ng/mL; reference value, <8.0 ng/mL). Therefore, a clinical diagnosis of pulmonary cryptococcosis was made.\nThe patient was orally treated with fluconazole (200 mg/day) and bicyclol (50 mg/day). After 3 weeks of antifungal therapy, his symptoms disappeared and the abnormality on CT scanning had improved (Fig. E). During the follow-up of 3 and 6 months, his conditions improved again, and he recovered fully (Fig. F and G). Cryptococcal antigen level was 12.57 ng/mL. During the 2-year follow-up, no recurrence occurred.","This 51-year-old man was admitted to the hospital because of a persistent cough, fatigue, and shortness of breath that didn't go away after taking antibiotics. He had a previous history of a lung infection five years ago, but it resolved without treatment. After further tests, including a CT scan and blood tests, doctors discovered he had a lung infection caused by a fungus called cryptococcus. He was treated with antifungal medication, and his symptoms and lung scans improved significantly after three weeks."
275,"A 45-year-old man (occupation: medical doctor) visited a university hospital with a chief complaint of fainting 1 day back. He had no history of medical problems and was not taking any medications. He had hiked 4 to 5 times a week for 2 years. Syncope occurred while hiking on a cold winter night (temperature: −8°C). He fainted while resting for a while in a standing position after hiking at a fast speed for an hour. The witness reported that he lost consciousness for about 1 minutes, and no head strike or seizure-like activity was observed. Upon awakening from syncope, the patient had no syncope recollection. After syncope, the patient did not have any illnesses or symptoms, such as headache, cognitive deficits, or somnolence. He had no prior episodes of syncope or fainting.\nAt the time of examination (the day after the syncope), his blood pressure was 122/77 mm Hg, and his resting heart rate was 75 beats per minute. Electrocardiogram, 24 hours ambulatory electrocardiogram monitoring, echocardiogram, tilt table test, and exercise tolerance test showed no abnormal findings. In addition, in conventional brain and chest computed tomography and brain MRI, no abnormalities were observed. All blood test results, such as electrolyte level, hemoglobin level, erythrocyte sedimentation rate, and C-reactive protein level, were normal. In the physical examination, he did not show any neurological symptoms, including motor, sensory, or cognitive deficits (Mini-Mental State Exam: full marks, no patient's subjective symptoms). The deep tendon reflex was normoactive in all the extremities. Based on the patient's history and examination results, the cardiologist diagnosed the patient with transient loss of consciousness due to syncope. In addition, considering that syncope occurred during excessive physical activity at cold temperatures, the possibility of vasovagal syncope was thought to be high.\nDCE scans were acquired using a 3T system (Skyra, Siemens Healthcare, Erlangen, Germany). In addition to the patient, a DCE scan was obtained from a healthy control participant (42-year-old man) who volunteered for the study. Seven pre-contrast sets of DCE-MRI (echo time= 1.92 ms, repetition time= 5.46 ms, the field of view = 230 × 135 mm2, matrix size of 256 × 150, and slice thickness of 3 mm), followed by an additional 114 sets under the intravenous injection of contrast agent were imaged in axial view. The permeability (Ktrans) of the mid-slice was calculated using the Patlak model.[\nHere, t indicates the time step, τ indicates the variable of integration, Vp indicates the volume of plasma, and Ct (t) and Cp (t) indicate the temporal variation of the concentration of the contrast agent in the tissue and plasma, respectively. A circular region of interest (ROI) with an inner diameter of 1 to 2 cm was located at the superior sagittal sinus, which has higher consistency and plausibility compared to the internal carotid artery or the arterial vessel closest to the lesion.[ In the axial image where the body and crus of the fornix were presented, 10 round random ROIs of 2 cm diameter (dotted circles in Fig. ) were selected (5 in each hemisphere) to quantify the overall averaged Ktrans of the brain. ROIs were depicted, including sulci in which the bright blue colored lines (BBB disruption) are presented.\nFor the quantitative analysis, in each ROI of the healthy control participants and the patients, the T2 and Ktrans values were measured. The differences in the measured values between the healthy controls and patients were compared using an independent t-test. Statistical significance was set at P < .05.\nIn the conventional MRIs of the healthy control and the patient, no lesions were observed (Fig. A, 1B). In addition, in the Ktrans map of DCE MRI of healthy control participants, no specific abnormal findings were observed (Fig. C). However, in the patient, bright blue-colored lines were observed within the sulci throughout the cerebral cortex (Fig. D). These findings indicate disruption of the BBB of vessels in the overall cerebral sulci. In the quantitative analysis, the averaged T2 intensity (within 10 randomly selected ROIs) of healthy control and patient was 403.95 ± 24.91 (AU) and 404.50 ± 24.21 (AU), which was not statistically different (P = .9625). Here, the average Ktrans of healthy controls within 10 randomly selected ROIs was 0.000156 ± 0.000099 (minutes−1) and 0.001067 ± 0.000806 (minutes−1) for the patient. The average Ktrans of the patient was 6.86 times higher than that of the healthy controls, and the difference was statistically significant (P = .003409).","The patient experienced a fainting spell while hiking in the cold. After a thorough examination, including tests like EKGs and MRI scans, doctors ruled out any serious underlying medical conditions. The most likely cause is vasovagal syncope, which happens when the body reacts strongly to physical exertion in cold temperatures. The doctors found evidence of a small disruption in the blood-brain barrier, which is a protective layer around the brain, and will continue to monitor the patient."
276,"The patient, a 53-year-old female, was admitted to the hospital on April 05, 2019 in a car accident resulting in a fracture of the distal left tibiofibula, and was given routine heel traction and symptomatic treatment to reduce swelling after admission. On April 18, 2019, we performed “tibiofibular fracture incision and internal fixation + bone grafting,” after which we were given symptomatic treatment for swelling and pain relief. He was discharged from the hospital on May 16, 2019, and at the time of discharge there was still about 3 cm of poorly healed skin and localized redness and swelling in the lower and middle sections of the left ankle incision, and then continued to have the left ankle dressing changed at the local community hospital, and the wound never healed. One year after the operation, he was readmitted to the hospital for rehabilitation.\nA follow-up X-ray showed that the metal internal fixation of the left inferior tibiofibular fracture was in place, no loosening was seen, the alignment of the severed end was acceptable, and the left inferior tibial segment was mildly osseointegrated (Fig. ).\nOn examination: the left ankle incision was interrupted for about 3 cm with poor healing, a small amount of muscle necrosis, fat liquefaction, a large amount of yellow purulent secretion overflow and necrotic material was seen in the local wound. The wound did not improve after routine dressing changes.\nPRF gel preparation and application the preparation process was strictly aseptic: - A disposable screw-in 20 mL syringe with an 18G needle was used to draw 20 mL of autologous blood under strict aseptic conditions, and the screw-in syringe was tightened with a disposable plug, and the posterior projection and tail were cut off. Weigh, level, and centrifuge at 3000 r/min for 10 minutes. After resting, the blood sample was divided into 3 layers, the upper layer of yellowish clarified liquid was platelet plasma, the lower layer of red loose jelly-like material was red cell debris, and the middle layer of yellowish gel was PRF gel, and the PRF gel was obtained by removing the PRF gel using a three-way connecting tube, and then it was rested in a dry sterilized container for 10 minutes to obtain the PRF gel (Fig. ).\nAdmission to the hospital to improve blood routine, blood sedimentation, C-reactive protein and other tests to exclude contraindications. Necrotic tissue was scraped out of the wound with a sterile blade (see extensive muscle necrosis, fat liquefaction, and large amounts of purulent secretions overflowing as in Fig. A) until the wound oozed blood. Then, after rinsing with saline, PRF was injected into the wound and the submerged area, so that the autologous platelet-rich fibrin mixed with the preparation could fully contact with the wound and close the wound, and then covered with oil gauze to keep the wound moist and promote the growth of granulation. The wound was covered locally with PRF on April 17, 2020, bandaged and covered with PRF again after 1-week interval.","This patient was admitted to the hospital after a car accident that resulted in a broken ankle. Initially, surgery was performed to fix the fracture, but the wound didn't heal properly and became infected with pus and dead tissue. Doctors used a special treatment called PRF (platelet-rich fibrin) to help the wound heal by using the patient's own blood to stimulate tissue growth. After the PRF treatment, the wound began to improve, and the patient was admitted to the hospital for further rehabilitation."
277,"An eighty year-old female presented with bleeding from the prolapsed uterus. The patient was admitted to the department of Gynaecology/Obstetrics and Gynaecological Oncology of Hospital in Dębica, Poland. In an obstetric history, she reported 3 vaginal births, the last one at the age of 30. Last menstruation was at the age of 52. She did not complain of any past diseases or current chronic diseases, she did not report any addictions and remained in 1 relationship. On admission, a large part of the vagina containing the cervix and a part of the uterine body was found below the vaginal introits (International Continence Society grade 3). Examination revealed an exophytic infiltrating lesions covering entire surface of hypertrophic cervix spreading to the vaginal fornices (Fig. ).\nBimanual vaginal examination revealed a small myoma of 2 cm in diameter in the uterine body, adnexa impalpable. Per rectal examination showed a tumour in the anal canal. Moreover, the parametria were uninvolved in this examination.\nIn the diagnostic procedure, the lesion in the cervix was sampled parallelly with curettage of the cervical canal and the uterine cavity. Then, rectoscopy was performed with sampling of the anal canal lesion for histopathology. The rectoscopic image is shown in Figure and the microscopic images of the cervical and anal canal samples in Figure .\nA polypoid lesion in the rectoscopy measuring 2.5 cm in diameter just behind the external sphincter and an infiltrating lesion occupying the posterior wall of the rectum, 4 cm away from the anal rim were found (Fig. ).\nHistological images of the cervical and anal canal lesions are shown in Figure . Microscopic examination revealed, in the cervical specimen, moderately differentiated invasive squamous cell carcinoma (SCC) (Fig. A), featuring a strong and diffuse immunohistochemical p16 reaction in the neoplastic cells (Fig. B). In the search of HPV high risk, the polymerase chain reaction (PCR) reaction was performed, which confirmed the presence of high risk HPV (HPV 16 and additional one from the group of High Risk HPV 31,33,35,39,51,52,56,58,59,66,68) in the cervical SCC. The microscopic examination of the biopsy specimen from the anal canal lesion revealed moderately differentiated adenocarcinoma (Fig. C) with patchy p16 immunohistochemical stain in the cancer cells (Fig. D), while the polypectomy specimen showed the conventional serrated adenoma with low grade dysplasia. The low grade dysplasia was also found at the diathermied margin. The PCR reaction excluded the presence of high risk HPV in the anal canal adenocarcinoma, followed by the negative result for low risk HPV PCR test. In addition real-time PCR was carried out for anal canal adenocarcinoma confirming NRAS mutation and excluding KRAS and BRAF mutation. Physical, endoscopic and histological examinations were supplemented with magnetic resonance imaging (MRI). Examination revealed prolapsed cervix of irregular morphology with the disrupted cervical stroma ring (long arrow). Multiple low T2 signal round myometrial lesions was suggestive of uterine fibroids (short arrows; hollow arrows). MRI also revealed irregular mass-like thickening of the distal part of the rectum and anal canal, superiorly to the anal sphincters. Both internal and external sphincters seemed uninvolved. The lesion was confined to the intestine wall and did not infiltrate ischioanal fat nor adjacent pelvic structures (Fig. ).\nThe next lesion measuring 40 × 20 × 25 mm was identified in the cervix. It demonstrated a low signal on the T2-weighted sequence and a high signal in LAVA after contrast administration. No evidence of vaginal mucosa, bladder and rectal invasion was found. Superficial parametral invasion on the left was suspected (Fig. ).\nDiffusion-weighted magnetic resonance sequences suggested the malignant character of the thickening (A) of the rectal and anal canal wall. High signal on B-value diffusion-weighted MRI (B) with corresponding low signal on apparent diffusion coefficient (ADC) map (C) confirmed water diffusion restriction – a sign of malignancy (hollow arrows; Fig. ). T stage corresponded to the size of the primary tumour assessed by measuring in its longest diameter on T2-weighted MR images. In our case, the maximum tumour diameter was 23 mm.\nBased on the above data, cervical G2 squamous cell carcinoma FIGO II A 2 was diagnosed in the prolapsed cervix with the coexisting G2 anal canal adenocarcinoma pT1N0M0.\nDue to the predominance of symptoms related to CC and prolapse, the patient was offered a treatment consisting of, at the first stage, radical vaginal hysterectomy with extensive colpectomy preceded by laparoscopic pelvic and paraaortic lymphadenectomy, followed by the removal of the anus in the second stage. The patient agreed to some of the proposed treatment, however, she did not consent to the anal extirpation. Therefore, only laparoscopic pelvic and paraaortic lymphadenectomy with radical vaginal hysterectomy were performed. The key step of vaginal radical hysterectomy with the isolation of the right ureter is shown in Figure . The dissected ureteric angulation, known as knee of the ureter, allowed excision of the parametria.\nThe postoperative course was uneventful. The patient was discharged on day 5. Microscopic examination of postoperative specimens revealed the cervix containing partially exophytic tumor measuring 2.5 × 3.5 × 7.5 cm, which appeared to be G2 HPV-associated squamous cell carcinoma, focally invading beyond the cervix into surrounding adipose tissue, sparing the parametria (pT2a2), with clear peripheral margins. The perineural and vascular space involvement was identified. Out of 13 regional lymph nodes examined, 2 showed metastasis (2/13).\nDue to the refusal of both anal extirpation and adjuvant chemotherapy, after a multidisciplinary consultation, the patient was qualified for radiotherapy (RT). She was transferred to the Radiotherapy Department at the Regional Hospital in Tarnow. The RT was dedicated as definitive treatment for ACC and adjuvant therapy for CC. RT was delivered via a two-step 3D conformal technique. The lymph nodes affected by SCACC and regional lymph nodes of anal cancer were included in step 1 and the dose of 50.4 Gy in 28 fractions was delivered. In step 2, boost of 3.6 Gy in 2 fractions to the primary anal canal was added. As the patient did not want to undergo resection of the anus, we planned brachytherapy in the third stage, as a way of dose escalation. The prescribed dose was defined as 98% of the planning target volume that should receive 98% of the dose. organ and risks were contoured, including the peritoneal space (bowel bag), bladder and bilateral femoral head. All constrains of tolerance doses for organ and risks were maintained. The treatment was tolerated well, however, Common Terminology Criteria for Adverse Events (v4.0), including grade 2 diarrhoea and anal canal colitis, were demonstrated. RT was completed without discontinuation through treatment.\nMRI performed on 45 postoperative days after radical vaginal hysterectomy and RT demonstrated a complete response. No sign of previous-seen mass-like thickening of the rectal wall on T2-weighted images (Fig. ). Diffusion-weighted imaging (Fig. ) confirmed no evidence of suspected regions of water diffusion restriction.\nThe duration of follow-up was 6 months.\nInformed written consent was obtained from the patient for publication of this case report and accompanying images.","This 80-year-old woman was admitted to the hospital because her uterus had prolapsed, causing it to come out of her vagina. She also had a concerning growth in her rectum and cervix. Tests revealed that the growth in her cervix was a type of cancer, and the growth in her rectum was also cancerous. After careful discussion with her doctors, she underwent surgery to remove the cancerous tissue in her vagina and cervix, and the doctors are now planning to use radiation therapy to treat the cancer in her rectum."
278,"A 43-year-old woman, who presented with a 6-month history of hoarseness, was diagnosed with bilateral vocal cord polyps after laryngoscopy. Preoperative evaluation using chest computed tomography (CT) showed an intact tracheal wall with no abnormalities. Hence, vocal cord polyp resection via TLM was performed. Before the operation, a skilled anesthetist performed routine nasotracheal intubation with a video laryngoscope. The endotracheal tube cuff was filled with 10 mL water to replace air to make inadvertent laser damage to the endotracheal tube cuff more noticeable. During the operation, a saline-soaked absorbent cotton was placed at the subglottis to protect the trachea. There was no evidence of intraoperative damage to the tracheal wall.\nFifteen hours after the operation, the patient experienced sudden dull pain in the anterior neck but no dyspnea. Repeat direct rigid laryngoscopy showed no abnormalities. This patient is considered to be a common throat in TML and discharged from the hospital. At 23 h after initial surgery, she returned to emergency room presenting subcutaneous emphysema and cough. Re-evaluation with a head, neck, and chest CT was done, which showed mediastinal emphysema and extensive subcutaneous emphysema from the base of the skull to the chest. A suspected tear on the membranous trachea located 4.5 cm under the glottis, extending to ∼8.5 cm under the glottis was noted (Fig. A–D). Bronchoscopy confirmed the tracheal rupture.\nThe patient underwent emergency surgical chest exploration, which revealed a 4-cm long and narrow tracheal tear on the membranous trachea (Fig. ). The tracheal tear was sutured, and the surgeon ensured that there were no other injuries.\nThe patient was discharged on the 11th postoperative day. During follow up after 1 month, no tracheal stenosis or respiratory dysfunction was noted.","A 43-year-old woman was diagnosed with vocal cord polyps, which were removed with a minor surgical procedure. During the surgery, precautions were taken to protect her airway. However, after the surgery, she developed a serious complication with a tear in her trachea, requiring emergency surgery to repair it. Fortunately, the tear was successfully repaired, and she was discharged home after a few weeks of recovery. She is now following up with her doctor to ensure everything is healing properly."
279,"The proband (III-3) (32 year-old, male) visited the Department of Neurology of our hospital for “unsteady steps for more than 7 months” More than 7 months prior to admission, the patient gradually had unsteady steps with no obvious predisposing causes, manifested as walking with a rolling gait. The patient's condition gradually worsened and manifested with increased step distance and drunk walking from side to side at admission. The patient complained of recent difficulties in eye opening, involuntary tremor of both upper limbs, and catatonia, which were aggravated after activities.\nNeurological examination: Conscious mind, dysarthria, normal memory, calculation and orientation power, normal visual acuity and field of both eyes, free movement of both eyes in all directions, horizontal nystagmus when both eyes look to the left, ptosis of both eyes, unstable and inaccurate bilateral finger-nose test, clumsy bilateral alternating bilateral movements, bilateral heel-knee-shin test, and no bilateral pathological reflex.\nAuxiliary examinations: Head magnetic resonance imaging (MRI) of the proband (III-3) showed cerebellar atrophy with no clear abnormal signals. Cervical MRI suggested no atrophy or thinning of the cervical cord (Fig. ).\nFamily surveys: Figure shows the family history of the proband (III-3). The mother (II-3) developed similar symptoms in her 30s, was paralyzed in bed in the following days and died in her 40s. The elderly maternal uncle (II-2) and his children (III-1, III-2) have no clear clinical manifestations. The younger maternal uncle (II-5) began to suffer from illness in his 20s and died from disease in his 30s.\nThe daughter of the younger maternal uncle (III-5) (25-year-old now) gradually had unsteady steps and unclear speech with no obvious incentive at around 20 years old, manifested as a slightly wide step base, unstraight walking, influent speech, and appearance of choking cough in drinking water in the past year (Table ). Neurological examination: conscious mind, dysarthria, horizontal nystagmus when both eyes look to the left and right, active tendon reflex of limbs, unstable and inaccurate bilateral finger-nose test, clumsy bilateral alternating movements, and bilateral heel-knee-shin test. Auxiliary examination: Head magnetic resonance imaging suggested cerebellar atrophy (Fig. ).\nAfter obtaining informed consent from patients and their families, serum samples of the proband (III-3) and his cousin (III-5) were collected to identify genes involved in the pathogenesis of SCA. The blood samples were sent to the Beijing High Trust Diagnostic for Whole Exome Sequencing. Sanger sequencing of the patients (III-3 and III-5) revealed a c.1852A > G missense mutation in the exon region of the AFG3L2 gene, resulting in a lysine-glutamate amino acid substitution (p.K618E) (Figs. and ). Sanger sequencing of the other family members (II-1, III-1) revealed no AFG3L2 mutations. We checked the human gene mutation database and found that the gene mutations reported here had not been reported before. According to ACGS recommended guidelines, we thought this mutation considered pathogenic. SCA28 is the one uniquely caused by a pathogenic variation in the mitochondrial protein AFG3L2. Combined with the clinical manifestations, auxiliary examinations and sequencing results of the patients (III-3 and III-5), the diagnosis of SCA28 was suspected. The patients did not receive any drug treatment and the proband receive rehabilitation treatment. The symptoms of ataxia were still progressively aggravated.","This 32-year-old man has been experiencing unsteady steps and other neurological symptoms for over seven months. His condition has worsened, leading to difficulty walking and involuntary movements. He also reported problems with eye movement, tremors, and catatonia. Family history reveals a pattern of similar symptoms, suggesting a genetic condition, and genetic testing identified a specific mutation that likely causes his symptoms. He is currently receiving rehabilitation and his condition is unfortunately continuing to worsen."
280,"A 35-year-old man was admitted to our hospital with a 1-month history of epigastric pain, nausea, and vomiting. On admission, his vital signs were normal. He denied any previous medical history, including gastrointestinal disease, abdominal surgery, or significant medical illness. In addition, he was not taking any medications, including NSAIDs. Physical examination revealed asymptomatic brownish variable-sized round macules with scales on both palms and soles that had been present for several months (Fig. ). Skin lesions were consistent with secondary syphilis. No oropharyngeal or genital lesions were observed. Mild abdominal tenderness was elicited in the epigastrium. He reported sexual intercourse with multiple sexual partners several months prior to admission. Laboratory evaluation revealed normal hemoglobin, hematocrit, white blood cell count, and hepatic and renal function. The C-reactive protein level was elevated at 2.96 (normal, 0–0.3) mg/dL. The serum venereal disease research laboratory test was positive, with a titer of 1:8, and the fluorescent treponemal antibody absorption (FTA-ABS) test was reactive. Serum human immunodeficiency virus (HIV) antibodies were negative. Esophagogastroduodenoscopy (EGD) revealed multiple geographic ulcers with easy touch bleeding and whitish exudates in the antrum (Fig. ). The esophagus, body and fundus of the stomach, and duodenum were normal. Endoscopic biopsies taken from the antrum showed a dense mononuclear cell infiltrate with prominent plasma cells on hematoxylin–eosin staining (Fig. A). Helicobacter pylori was not detected by Giemsa staining. Because syphilis was suspected based on the results of skin lesions and serologic tests, Warthin–Starry silver staining was subsequently performed, and numerous spirochetes were identified in the lamina propria, confirming gastric syphilis (Fig. B). He was treated with an intramuscular injection of 2.4 million units of benzathine penicillin once a week for 3 weeks. His clinical symptoms resolved within 5 days of treatment initiation. Follow-up EGD after 2 months showed complete resolution of multiple geographic ulcers. Biopsies of the gastric mucosa were negative for spirochetes, and the lymphocytic infiltrate resolved. Informed consent was obtained from the patient for the purpose of publication.","A 35-year-old man was admitted to the hospital because of stomach pain, nausea, and vomiting that had been going on for a month. Doctors found that he had a skin rash that was consistent with an infection called syphilis, and his blood tests confirmed this diagnosis. An examination of his stomach with a camera revealed ulcers and inflammation, also related to the syphilis infection. He was treated with antibiotics, and his symptoms quickly improved, and the ulcers in his stomach disappeared after a follow-up examination."
281,"A 33-year-old man presented at the outpatient clinic with a 1-month history of epigastric pain. Physical examination revealed mild abdominal tenderness in the epigastrium. Laboratory evaluation revealed normal hemoglobin, hematocrit, white blood cell count, and hepatic and renal function. The serum treponema pallidum hemagglutination test was positive, with a titer of 1:1280, and the FTA-ABS test was reactive. Serum HIV antibodies were negative. EGD showed multiple variable-sized whitish discolored flat elevated lesion with focal erosion and ulceration on the body (Fig. A) and large geographic ulcers with regular edges and even whitish exudates on the lesser curvature side of the lower body (Fig. B). Warthin–Starry silver staining of endoscopic biopsy specimens confirmed gastric syphilis. He was treated with an intramuscular injection of 2.4 million units of benzathine penicillin once a week for 3 weeks. The patient's clinical symptoms and gastric lesions were completely resolved. Informed consent was obtained from the patient for the purpose of publication.","A 33-year-old man came to the clinic complaining of stomach pain for a month. Tests revealed he had a rare infection called gastric syphilis, which causes sores in his stomach. He was treated with penicillin injections, and the sores disappeared completely. His other blood tests were normal, and he tested negative for HIV. The doctor shared his case for publication to help other doctors learn about this condition."
282,"A 27-year-old male patient was admitted to the emergency room of this hospital with shortness of breath that started a week before admission. A chest x-ray performed at emergency room showed a left-sided pneumothorax (Fig. A), and the patient was referred to the department of thoracic surgery. The left lung of the patient was totally collapsed, and there was a high possibility of REPE upon chest tube insertion with the symptom onset of a week. Accordingly, after explaining this situation to the patient and his caregivers, a 12 French trocar tube was inserted into the left thoracic cavity of the patient. In order to allow the lungs to expand slowly, natural drainage was performed without suction, and the rubber tube connecting the chest tube and the water seal bottle was partial clamped. On the day after chest tube insertion, there was an finding of air leakage through the chest tube, but the lungs were not found to expand much as a result of chest x-ray (Fig. B), thus the partial clamped rubber tube was de-clamped. However, about an hour after de-clamping, the patient suddenly complained of severe respiratory distress, cough with foamy sputum, and vomiting. Immediately the chest tube was clamped to stop drainage. With oxygen saturation measured 75%, oxygen was administered to the patient via an oxygen mask, but no improvement was observed. In turn, a chest x-ray was followed during additional high flow nasal prong (HFNP) therapy, exhibiting REPE findings such as newly developed ill-defined consolidation in the left lung (Fig. ). Oxygen saturation did not improve even after HFNP therapy, and further, persistent cough with foamy sputum, shortness of breath, and a drop in blood pressure occurred. With the need for intensive care recognized, the patient was immediately transferred to the intensive care unit and then was sedated and intubated to perform mechanical ventilation. After supportive care including mechanical ventilation, the condition of the patient was improved and then extubation was performed two days later. On the third day, chest computed tomography (CT) (Fig. ) was performed, and the patient was transferred to a general ward. From the 6th day, a chest x-ray showed no more edema (Fig. ). There was no finding of air leakage through the chest tube and a chest x-ray showed full lung expansion, in turn, the chest tube was removed on the 9th day of hospitalization. However, a chest x-ray showed recurrence of pneumothorax the day after removal of the chest tube, and the chest tube was inserted again. Both the patient and his caregivers wanted operation, so the video assisted thoracoscopic surgery (VATS) wedge resection was performed. Following operation, the patient recovered without any complications. For the follow-up at 6 months after operation, no adverse symptoms were found and the imaging findings were also found to be normal.\nThis case report was approved by the Institutional Review Board of Soonchunhyang University Hospital (SCHCA 2021–09-007). The Patient has provided informed consent for publication of this case.","A 27-year-old man was admitted to the hospital after experiencing shortness of breath for a week. Doctors discovered a collapsed lung (pneumothorax) and placed a tube to help it re-inflate, but the lung didn't expand properly. Unfortunately, the patient then developed a serious lung infection and needed to be put on a ventilator. After receiving intensive care and antibiotics, he recovered and was eventually able to breathe on his own, and underwent surgery to repair the lung."
283,"A 6-month-old male child was referred to our hospital with a mass on the anterior alveolar ridge of the mandible. The patient's parents noticed this mass at the age of 4 months, which rapidly increased in size thereafter.\nAt his first visit to our hospital, his face was symmetrical, but the lower lip was protruded. A relatively well-defined spherical mass (30 × 20 × 20 mm) was located on the mandibular alveolar ridge extending from the right mandibular deciduous canine to the left mandibular first deciduous molar (Fig. ). The mass had normal mucosal color, was elastic hard and non-fluctuant with the left mandibular deciduous central incisor attached to the surface of the tumor.\nComputed tomography (CT) showed a relatively uniform multicentric osteolytic lesion extending from the right mandibular deciduous central incisor to the left mandibular deciduous canine with expansion of the surrounding bone and numerous displaced tooth germs. The density of the lesion mass was lower than that of the muscle, and the boundary with cancellous bone was irregular (Fig. ). Histopathological findings of the biopsy specimen showed that the biphasic tumor cells consisted of large melanin-containing epithelioid cells and small neuroblast-like cells forming a solid alveolar pattern (Fig. A) and a gland-like pattern (Fig. B). The large tumor cells had a slightly eosinophilic cytoplasm and an oval bright nucleus with small but distinct nucleoli. These were occasionally positive for melanin pigment, which was visualized in the form of black granules on Fontana-Masson staining (Fig. C) and were bleached with potassium permanganate–oxalic acid (Fig. D). The small tumor cells had a scanty cytoplasm and a slightly deeply stained round nucleus, often showing detached clusters. A small number of mitotic figures, and a low degree of cellular polymorphism were observed. Immunohistochemistry revealed that the large and small tumor cells were positive for neuron-specific enolase (Fig. A & B). The large tumor cells stained positive for cytokeratin (AE1/AE3), human melanin black-45, and vimentin (Fig. C–E). The small tumor cells stained positive for CD56 and synaptophysin (Fig. F & G). The tumor cells were negative for S-100 (Fig. H) and chromogranin A. The MIB-1 proliferation index was >50% (Fig. I). Based on these findings, the tumor was finally diagnosed as MNTI.\nThe tumor continued to grow rapidly, and marginal mandibulectomy was performed under general anesthesia 28 days after the first visit to our hospital. The extent of resection was determined by referring to the mandibular 3D model which was created based on the CT data obtained before the surgery. Marginal mandibulectomy, approximately 5 mm lateral to the tumor, was successful in preserving the inferior margin of the mandible (Fig. ). The mental foramen on the left side was included in the resection, and the foramen on the right side was preserved. All deciduous tooth germs were included in the excision, except for the right deciduous second molar. After rounding the sharp edges of the bone with a file, the raw surface of the mandible was completely closed by the surrounding soft tissue with help of polydioxanone monofilament synthetic absorbable suture. The postoperative course was uneventful, and oral intake of milk was started 3 days after the operation. On the 7th day after the surgery, the patient was discharged from the hospital with good general condition. At the time of discharge, he had no abnormal facial swelling or infection in the surgical wound.\nSimilar to biopsy specimens, histopathological findings of the excised mass showed that the tumor consisted of 2 types of cells: large polyhedral cells sometimes containing melanin granules and smaller round cells with hyperchromatic nuclei and scanty cytoplasm resembling lymphocytes. No nuclear atypia or mitosis was observed. Tumor cells were seen invading the surrounding tooth germs and bone.\nThe patient is being closely followed up, and no evidence of recurrence or metastasis was observed on CT images taken 1 year after surgery (Fig. ). In this case, stomatognathic functions such as eating, swallowing, and pronunciation were normal post-surgery, but occlusal reconstruction and recovery of masticatory function following removal of the mandibular dentition will be important therapeutic goals in the future.","A 6-month-old boy was seen in the hospital because of a growing lump on his jaw. Doctors found that the lump was a rare type of cancer called MNTI, which was affecting his teeth and bone. To remove the tumor and prevent it from spreading, the doctors performed a surgery to remove part of his jaw. The surgery was successful, and the boy is now recovering well, with plans for future treatment to restore his bite and chewing ability."
284,"A 25-year-old man was diagnosed with temporal lobe epilepsy 12 years ago, which was treated with carbamazepine and zonisamide. Subsequently, valproate and lacosamide were added 5 and 3 years ago, respectively, due to poor seizure control. During a regular visit, a neurologist noticed that the patient's serum creatinine level increased from 0.98 to 1.29 mg/dL over 6 months. As his serum creatinine level continued to increase to 1.74 mg/dL, he was referred to the nephrology department. He presented no symptoms, and physical examination revealed no abnormal findings. Laboratory analyses showed renal dysfunction, mildly elevated C-reactive protein levels, and increased levels of urinary markers for tubular injury (see Table ). Renal biopsy was performed. Although the glomeruli were intact, there was marked interstitial lymphocytic and eosinophilic infiltration (Fig. A, B), suggestive of AIN. No immunoglobulin or complement deposition was observed. Since he had no other organ involvement (including uveitis) and no laboratory findings suggestive of autoimmune disorders such as sarcoidosis and Sjögren's syndrome, the AIN was ascribed to drug allergy. Based on a positive result of lymphocyte stimulation test (LST), zonisamide was discontinued and daily oral prednisolone (40 mg, 0.8 mg/kg) was started.\nSerum creatinine levels decreased to 1.35 mg/dl and prednisolone dose was tapered monthly. However, 10 months later, serum creatinine levels increased again to 2.07 mg/dL (Fig. ). 67Ga scintigraphy revealed significant radioactive tracer uptake in both kidneys (Fig. C), suggestive of AIN recurrence. Oral prednisolone (40 mg/d) was restarted, and valproate and lacosamide were discontinued based on a neurologist's recommendation. Renal function immediately improved, although minor epilepsy occurrence increased. Oral prednisolone was gradually tapered to 10 mg/d, after which serum creatinine levels increased again to 1.99 mg/dL. Repeat renal biopsy revealed similar findings of tubulitis with both lymphocytic and eosinophilic infiltrations. Prednisolone dose was increased to 60 mg (1.0 mg/kg) daily; nevertheless, it was difficult to discontinue carbamazepine due to the need for major epilepsy prevention. Since the patient had multiple AIN recurrence and was corticosteroid dependent, 1500 mg daily of oral mycophenolate mofetil (MMF) was added to the corticosteroid therapy. This treatment was well tolerated and enable successful prednisolone dose tapering to 5 mg daily, with serum creatinine level stabilization (Fig. ). He experienced no AIN recurrence with MMF treatment for over 20 months.","This 25-year-old man has had epilepsy for 12 years and has taken several medications to control his seizures. Recently, his kidney function started to decline, and doctors discovered inflammation in his kidneys. After several tests and biopsies, they determined that the kidney problems were likely caused by an allergic reaction to one of his medications. Now, he’s taking medication to suppress his immune system and prevent further kidney inflammation, and his kidney function has stabilized."
285,"A 36-year-old male patient presented with anorexia, easy fatigability, and melena for 3\nmonths. His clinical examination was largely unremarkable. We found melena on a digital\nrectal examination. An upper GI endoscopy revealed a large mass projecting into the gastric\nlumen ( ), arising from the posterior gastric\nwall. The lesion was closer to the lesser gastric curvature than the greater curvature. At\nthe summit, the mass suffered from a bleeding mucosal ulcer, the cause of his melena.\nSeveral endoscopic biopsies were inconclusive, consisting of only gastric mucosal tissue\nwithin the biopsy specimens. An endoscopic ultrasound-guided needle aspiration revealed\nstromal cells on cytology. An abdominal CT demonstrated a well-defined, intramural mass\narising close to the lesser gastric curvature with a small mucosal defect at the\nsuperolateral aspect of the lesion ( ). The\nsoft-tissue fat planes were intact and there were no enlarged or abnormally enhancing lymph\nnodes. The patient's severe anemia was corrected with several preoperative transfusions.\nOperative steps : Under general anesthesia, the patient was positioned supine with\nhis legs split. Laparoscopic ports were inserted as shown in . The initial diagnostic laparoscopy did not reveal any peritoneal\ninvolvement. The lesser sac was accessed by dividing the gastrocolic and posterior gastric\nattachments. The tumor mass was large, making gastric retraction difficult. The tumor base\nat the posterior gastric wall exhibited increased vascularity ( ) without any evidence of invasion into adjacent tissues. After replacing\nthe stomach in its natural position, a liberal, longitudinal anterior gastrotomy was created\nat the summit of the tumor using ultrasonic shears. Manipulation of the tumor mass had\ncaused brisk bleeding from the ulcer, which could not be controlled with bipolar energy. The\ntumor was delivered through the gastrotomy ( ) and\npivoted over the shaft of a grasper placed parallel to the splenic axis. This caused the\ngastric wall adjacent to the tumor base to tent ( \n), allowing us to apply several linear staplers across it ( ). All sequential stapler fires were applied in close apposition to the tumor,\nwith the intention to save as much of the uninvolved posterior gastric wall. The use of\nstaplers not only allowed us to resect the lesion, which halted bleeding, but also\nsimultaneously reconstructed the posterior gastric wall. The staple line was oversewn on its\nluminal aspect with 2–0 polydiaxonone suture in a continuous manner after the resected\nlesion was placed in an endobag and parked adjacent to the right hepatic lobe. The anterior\ngastrotomy was closed using 2–0 delayed-absorbable barbed suture in two layers. The patient\nwas placed in a steep Trendelenburg position, and saline was instilled into the upper\nabdomen. Intraoperative gastroscopy with intraluminal CO2 insufflation helped objectively\nconfirm the integrity of the gastrotomy closures. The saline was suctioned out and a drain\nwas placed adjacent to the stomach through the flank port. The specimen was retrieved\nthrough a Pfannenstiel incision. The resected specimen measured 9.5 × 8.5 × 7.5 cm ( ). The operative time was 195 minutes, and the\nestimated blood loss was 175 mL (see video, supplemental digital content 1, which\ndemonstrates the clinical course of the patient and operative steps).\nThe patient commenced oral fluids on the second postoperative day and a blenderized soft\ndiet on day 4. His abdominal drain was pulled on day 4, and he was discharged from the\nhospital on day 5. He suffered a superficial surgical site infection (SSI) at the extraction\nsite, which responded well to oral antibiotics. The histopathology report confirmed a GIST\nwith negative margins, with 10to 12 mitosis per 10 high powered fields, indicating a high\npotential for malignancy. The tumor stained positively for CD34 and DOG-1, was weakly\npositive for c-kit, and negative for S100 and desmin on immunohistochemistry. After a\nconsultation with the medical oncologist, adjuvant imatinib therapy was commenced.","A 36-year-old man was admitted to the hospital after experiencing ongoing bleeding in his stomach (melena), fatigue, and loss of appetite for three months. Doctors found a large mass in his stomach causing the bleeding and performed an endoscopy and CT scan to get a better look at the problem. During surgery, the mass was carefully removed, and the stomach was reconstructed. The pathology report confirmed that the mass was a type of tumor called a GIST, and the patient will now start taking medication to help prevent it from returning."
286,"A 76-year-old Asian man with a 55-year history of type 2 diabetes mellitus was referred to our hospital for right intermittent claudication of 3 years. He had diabetic retinopathy and had been treated with insulin for 3 years. Vascular examination revealed right femoral bruit and diminished right popliteal pulse, suggesting the presence of a right femoral lesion. However, the right ABI measured by a volume plethysmograph (Form PWV/ABI; Omron Health Care Co., Kyoto, Japan) was 1.28, within the normal range (1.00–1.40) (Fig. ). On the other hand, the left ABI was elevated to 1.55 (> 1.40), suggesting that the infrapopliteal arteries at the left ankle were incompressible. Upstroke time, the transit time from the nadir to the peak of the pulse volume waveform recording, of the right ankle was prolonged to 201 milliseconds (≥ 180 milliseconds), whereas upstroke time of the left ankle was within the normal range (130 milliseconds) (Fig. ). These findings suggest that the right ABI was falsely normalized due to incompressible infrapopliteal arteries at the right ankle despite the presence of PAD in the right extremity. Indeed, intra-arterial angiography revealed the presence of a severe stenotic lesion at the origin of the right superficial femoral artery (SFA) (Fig. A), whereas there was no occlusive arterial disease in the left extremity. Fluoroscopic images showed severe calcification of infrapopliteal arteries at the right ankle (Fig. B). Computed tomography also revealed severe calcification of the bilateral infrapopliteal arteries (Fig. ). Revascularization was not performed, and supervised exercise training was initiated. After completion of the 6-month supervised exercise training program, he continued to spend 2 hours a day walking. Five years from the initial assessment, the right ABI had decreased to 0.83, with upstroke time at the right ankle of 242 milliseconds, indicating the progression of the right lower extremity PAD (Fig. ). However, there was no deterioration in his symptoms, and he had no difficulty in performing daily activities.","This 76-year-old man has been experiencing pain in his right leg when he walks, which is likely due to a blocked artery. Tests showed that the artery in his right leg was narrowed, but the blood flow in his left leg was actually too strong. After a period of supervised exercise, the blockage in his right leg worsened, and his symptoms returned. Despite this progression, he was able to continue walking and manage his symptoms with exercise, and he remained stable over the next five years."
287,"A 66-year-old Asian man with a 28-year history of type 2 diabetes mellitus was referred to our hospital for bilateral intermittent claudication of 6 months and exertional dyspnea. He had diabetic retinopathy and was being treated with insulin. He was an ex-smoker with a history of 40 pack-years. Vascular examination revealed right femoral bruit, diminished right popliteal pulse, and absent right and left dorsalis pedis pulses, suggesting the presence of right femoral and bilateral infrapopliteal lesions. However, both the right ABI (1.11) and left ABI (1.03) were within the normal range (Fig. ). However, upstroke times were prolonged to 206 and 203 milliseconds (≥ 180 milliseconds) in the right and left ankles, respectively (Fig. ), suggesting the presence of bilateral lower-extremity PAD. The right ABI decreased from 1.11 to 0.48 and the left ABI decreased from 1.03 to 0.67 after exercise. Computed tomography angiography revealed the presence of right SFA stenotic lesions, bilateral infrapopliteal occlusive lesions, and spotty calcification in bilateral infrapopliteal arteries (Fig. ). Intra-arterial angiography revealed the presence of right SFA stenotic lesions and bilateral infrapopliteal occlusive lesions (Fig. A–C). Coronary angiography revealed severe stenotic lesions in the right coronary artery and the left anterior descending coronary artery (LAD). After percutaneous coronary intervention for the LAD stenosis, supervised exercise training was initiated.","66-year-old man with diabetes and a history of smoking was evaluated for leg pain and shortness of breath. Tests showed narrowed arteries in his legs and heart, and he had a significant blockage in his heart. After a procedure to open up the blockage in his heart, he will start a supervised exercise program to help improve his circulation and overall health."
288,"A 37-year-old Hispanic male was hospitalized after being involved in a motorcycle crash. The patient suffered blunt chest and abdominal trauma as well as concussion without loss of consciousness. Upon arrival, the patient had a Glasgow Coma scale score of 15 and was hemodynamically stable. His abdomen was soft, nondistended, and nontender to palpation with no guarding. Labs were significant for hemoglobin count of 14.2 g/dL (normal 13.5–17.5 g/dL), white blood count of 11.8 1000/μL (normal 4.5–11 1000/μL), and lactate of 1.7 mmol/L (normal 0.7–2.1 mmol/L), which remained stable throughout his hospital course.\nInitial intravenous contrast-enhanced CT scan of the abdomen/pelvis was performed 10 minutes after patient presentation. The CT scan was interpreted by a radiology resident 15 minutes later and final interpretation by a board-certified emergency radiologist was completed in an additional 1 hour and 25 minutes. The initial CT interpretation indicated nonspecific wall thickening of the proximal jejunum, and occult small bowel injury could not be excluded. Given these findings, a repeat CT of the abdomen/pelvis with intravenous and oral contrast was ordered by the trauma service as per our institution’s standard protocol for evaluation of possible occult bowel injury in blunt abdominal trauma.\nThe follow up CT performed after midnight, 8 hours after the initial CT, demonstrated trace pneumoperitoneum, possibly originating from the splenic flexure of the colon without evidence of extravasation of oral contrast (Fig. ). The time from repeat CT scan completion to initial interpretation by a radiology resident was 30 minutes. The resident made the surgery team aware of these findings immediately. A board-certified emergency radiologist reviewed and provided another preliminary interpretation for the study 10 minutes later, confirming the presence of pneumoperitoneum. Based on the presence of pneumoperitoneum in a patient with blunt abdominal trauma, the patient was taken to the operating room as per standard protocol at our institution. Six hours after the follow up CT was performed, an academic board-certified abdominal radiologist provided a final interpretation.\nOn laparoscopy, cyst-like structures projecting from the wall of the distal transverse colon and splenic flexure were noted. Due to the patient’s body habitus, the surgery team did not feel that they could adequately explore the area and the decision was made to convert to exploratory laparotomy. No evidence of bowel perforation was found. A segmental section of the diseased portion of the colon was taken and sent to pathology. Pathology results showed multiple enlarged cystic nodules ranging from 0.5 to 3 cm in the bowel wall (Fig. ).\nSubsequently, the radiologic studies were reviewed by a panel of abdominal radiologists. The panel concluded that the proximity of the pneumoperitoneum to the pneumatosis cystoides intestinalis suggested that the pneumoperitoneum may have been caused by incidental atraumatic or traumatic rupture of the cysts. However, they could not rule out bowel perforation based on imaging alone.\nThe patient had an uncomplicated postoperative course and was transferred to an acute care facility of equal acuity for further medical management as per the patient’s health insurance request.","A 37-year-old man was hospitalized after a motorcycle accident with injuries to his chest and abdomen. Initial scans showed a possible small tear in his small intestine, so a follow-up scan was done. The second scan revealed a small amount of air in his abdomen, which led to surgery to remove a section of his colon with enlarged cysts. The surgery team found no signs of a major tear, and the patient is now being transferred to another hospital for continued care."
289,"A 76-year-old woman (para [P] 1) referred to our hospital with a chief complaint of abdominal distension and postmenopausal bleeding for 4 months consulted a gynecologist and was found to have a bulky uterus with a 132-mm subendometrial mass on vaginal ultrasonography. Adenosarcoma was suspected on the basis of findings from biopsy by hysteroscopy. She had no significant past medical history. Serum cancer antigen (CA) 125 and CA 19–9 were 283 U/ml (normal range 0–35 U/ml) and 11.5 U/ml (normal range 0–35 U/ml) respectively.\nMagnetic resonance imaging (MRI) was performed at 1.5 Tesra for further examination. Sagittal T2-weighted imaging (WI) (repetition time [TR]/echo time [TE], 4650/120 ms) showed a large, heterogeneous high-intensity mass, approximately 241 × 114 × 88 mm3 in size, without myometrial invasion expanding the uterine cavity and extending into the cervical canal (Fig. a). The enlarged uterine size was 250 × 126 × 99 mm3. The mass showed low intensity with areas of slightly high signal intensity on fat-suppressed T1WI (TR/TE, 600/10 ms) (Fig. b). The mass showed inhomogeneous contrast enhancement with cystic changes of variable sizes and necrotic foci, high intensity on diffusion-weighted imaging (DWI) (b = 1000 s/mm2, TR/TE, 7000/100 ms) (Fig. c) and low intensity (1.26 × 10−3 mm2/s) on apparent diffusion coefficient (ADC) maps. Uterine carcinosarcoma or adenosarcoma was suspected as the preoperative diagnosis.\nTotal abdominal hysterectomy and bilateral adnexectomy with pelvic lymphadenectomy were subsequently performed.\nBenign glandular epithelial components surrounded by atypical stromal cells with a high mitotic rate (20/10 high power field [HPF]) were identified on pathological examination. High-intensity areas observed on T1WI corresponded to hemorrhage, and SO was present. The tumor was diagnosed as adenosarcoma with SO without myometrial invasion (T1aN0M0).\nA follow-up CT scan performed 6 months after surgery revealed peritoneal sarcomatosis. Although her gynecologist recommended adjuvant chemotherapy, she did not wish to receive the treatment and died of the disease 1.5 months after recurrence.","A 76-year-old woman was admitted to the hospital after experiencing abdominal bloating and unusual bleeding. Tests revealed a large, concerning mass in her uterus, leading doctors to suspect cancer. After a biopsy and MRI, the mass was identified as an adenosarcoma, a type of uterine cancer. Ultimately, she underwent surgery to remove the uterus and ovaries, but unfortunately, the cancer spread to other parts of her body, and she passed away 5 months later."
290,"A 57-year-old woman (P 3) with complaint of low abdominal pain and abnormal uterine bleeding for 3 months consulted a gynecologist. Menstrual cycle length ranged from 30 to 40 days. She had no significant past medical history. Endometrial biopsy indicated atypical endometrial hyperplasia. Vaginal ultrasonography showed an enlarged uterus measuring 150 mm accompanied by a 73-mm tumor in the anterior wall, and both ovaries were not visualized. CA 125 and CA 19–9 were 40.3 U/ml (normal range 0–35 U/ml) and 1.1 U/ml (normal range 0–35 U/ml), respectively. Serum estradiol (E2) was within the normal limit (130.0 pg/mL).\nMRI showed a intrauterine mass, approximately 96 × 74 × 57 mm3 in size, protruding into the cervical canal with clearly defined inhomogeneous high intensity on T2WI (TR/TE, 6130/100 ms) (Fig. a) and low intensity with a slight high signal intensity area on fat-suppressed T1WI (TR/TE, 575/13 ms). The enlarged uterine size was 129 × 82 × 76 mm3.The mass showed inhomogeneous contrast enhancement with cystic changes of variable sizes and necrotic foci (Fig. b), high intensity on DWI (b = 1000 s/mm2, TR/TE, 4317/70 ms) (Fig. c) and low intensity (0.88 × 10−3 mm2/s) on ADC maps (Fig. d). A 22-mm solid nodule accompanied by a tiny cyst was detected in the right ovary. T2WI showed homogenous iso signal intensity with the uterine tumor on T2WI (Fig. a) and low intensity on T1WI. The nodule had homogeneous contrast enhancement with a tiny cystic change (Fig. b), high intensity on DWI (Fig. c), and low intensity (0.66 × 10−3 mm2/s) on ADC maps (Fig. d).\nWhole-body 18F-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET-CT) showed increased FDG uptake (maximum standardized uptake value: 8.2) in the uterine tumor and no abnormal FDG uptake in any other organs, including the right ovarian nodule (Fig. e). Uterine carcinosarcoma or adenosarcoma metastasizing to the right ovary was suspected as the preoperative diagnosis.\nThe patient underwent abdominal modified radical hysterectomy with bilateral adnexectomy, pelvic and para-aortic lymphadenectomy, and omentectomy.\nBenign glandular epithelial components surrounded by atypical stromal cells with a high mitotic rate (15/10 HPF) with minimal myometrial invasion and SO were present in the uterine tumor on pathological examination, and thus the tumor was diagnosed as adenosarcoma with SO (T1bN0M0). The right ovarian nodule was diagnosed as an adult granulosa cell tumor (GCT).\nA follow-up CT scan revealed a left upper lung metastasis 3 months after surgery. Accordingly, the patient underwent thoracoscopic segmentectomy of the left upper lobe. As of the writing of this report, at 22 months after the surgery, she has showed no signs of recurrence.","This 57-year-old woman came to the gynecologist because of persistent abdominal pain and irregular bleeding. Tests revealed an unusual growth in her uterus and a small nodule in her right ovary. Imaging showed the uterine growth was likely cancerous and had spread to the ovary. After surgery to remove the uterus, ovaries, and surrounding tissues, the growth was confirmed as adenosarcoma, and she began treatment for a new metastasis in her lung. Currently, she is doing well 22 months after her surgery."
291,"A 9-year-old female patient arrived at the emergency room because of a 36-h history of intermittent right lower abdominal pain, anorexia, vomit, and quantified high-grade fever. She had no pathological personal or family history of interest. On examination, the right iliac fossa was tender to palpation and no frank peritoneal signs were observed. Initial laboratory evaluation showed leukocytosis, neutrophilia, and an elevated C-reactive protein. Ultrasonography of the abdomen was inconclusive. A heterogeneous lesion of 40 × 37 mm within the colon, no appendix and some swollen mesenteric nodes of at least 10 mm were reported. A complementary abdominal CT scan revealed findings suggestive of ileocolic intussusception with an invagination area of approximately 6.6 × 4.9 cm. After surgical consult, the patient underwent an exploratory laparoscopy that required laparotomy conversion. A well-defined, 5 cm mass at ileo cecal valve and multiple hard pericecal lymph nodes were observed. Preserved permeability between the ileum and colon, complete integrity of the cecum wall and lack of vermiform appendix were also reported. The possibility of an auto-digested appendix and a cecal tumor were discussed. At this time, surgeons decided to resect retrocecal and pericecal lymph nodes and send these samples to pathology before any further intervention. The patient was admitted to the inpatient floor where antibiotic therapy based on ampicillin sulbactam, and metronidazole was initiated. The oncologist department was consulted and complementary laboratory exams including liver and renal function tests, uric acid, electrolytes, lactic dehydrogenase, and quantiferon-TB tests were ordered. Only lactic dehydrogenase was altered. A chest x-ray ruled out mediastinal masses. No alarming findings were reported. However, the patient presented gastric distension, abdominal pain and fever by the second hospitalization day. The content inside the suprapubic JP drain changed from a serohematic aspect to a dense cloudy fluid. A culture and cytochemical analysis of peritoneal fluid was performed without significant results. CBC showed mild leukocytosis and neutrophilia. Reactive C-protein remained elevated. Two blood cultures and an urinalysis were negative. Due to the uncertainty of the etiology of her clinical picture, infectology decided to change antibiotic therapy to piperacillin/tazobactam and amikacin. An abdominal x-ray showed air fluid levels in the small bowel and a colonic distention projected at mesogastrium. Gastroenterology suggested initiating bowel rest and placing a central line for parenteral nutrition.\nAfter five more days, elevated inflammatory markers, abdominal distension and pain, and the unusual JP drain aspect persisted. A new ultrasound confirmed that the mass and surrounding area had the same aspect as days before. The histopathological description of paracecal-retrocecal lymph nodes and the sample of mesenteric omentum obtained during the first intervention failed to detect neoplastic cells. Macroscopically, three encapsulated lymph nodes from 0.8 to 2 cm were received. Their physiological architecture was preserved; secondary lymphoid follicles with hyperplastic germinal centers containing macrophages with cellular debris were reported. The interfollicular population was polymorphic and contained frequent large cells with prominent immunoblast-like nucleoli. Other areas showed sinusoidal histiocytosis with eosinophils and neutrophils. There was fibrosis with a predominantly neutrophilic mixed inflammatory infiltrate that spread to neighboring adipose tissue in the periphery of the nodes. The immunohistochemical study confirmed the presence of follicular dendritic cells and B lymphocytes in the germinal centers (CD23 + + +/+ + + and CD20 + + +/+ + + respectively), T lymphocytes in the mantle zone (CD3 + + +/+ ++), macrophages in germinal centers and sinusoidal area (CD68 +/+ ++). Frequent CD30 + + +/+ + + immunoblasts and actin + + +/+ + + myofibroblasts within areas of fibrosis were also observed. EBV study using EBER in situ hybridization was negative. Ziehl Neelsen and PAS did not show any pathogen. The 22 × 0.6 cm omentum sample showed fibrous thickening of the septa and the presence of a mainly lymphocytic infiltrate. Fibrino-leukocytic material was also seen in the serosa. Pathologists concluded the possibility of an unspecified acute versus chronic epiploitis, lymphadenitis and serositis. Nevertheless, due to her unfavorable clinical evolution and the elevated inflammatory markers, a second surgical intervention was decided. The patient underwent an omentectomy and resection of approximately 40 cm of terminal ileum, cecum and ascending colon. Pericolonic lymph nodes were resected as well. A sample of a collection observed near the cecum was taken for culture and cytochemical studies before aspiration and drainage. After surgery, the patient remained hemodynamically stable, without abdominal pain or distention. A nasogastric tube was placed and parenteral nutrition continued. The peritoneal fluid analysis was negative. Improvement in inflammatory markers lead to amikacin discontinuation. And by the fifth postoperative day, JP drain, and nasogastric tube were removed. Later, a regular diet was successfully initiated, and the patient was finally discharged.\nThe histopathological final report described an 8 cm ileal segment, and a 14 cm ascending colon including the cecum with a diameter that ranged from 1 to 3 cm. The external surface was covered by a pinkish-gray serosa with fibrinopurulent material over the ileocecal area. A completely subserous dilated appendix was identified within the cecum wall. It contained a white-yellowish purulent material at the tip (Fig. ). A well-defined nodular lesion of approximately 1.5 cm was also identified (Fig. ). The mucosa of the cecum was pink while ileal mucosa had a granular appearance. Nine nodules, which measured between 0.3 and 3 cm, were isolated from the surrounding area. The 12 × 4.5 cm omentum sample had no palpable nodes. A second omentum sample showed multiple whitish irregular fragments of bland tissue that measured between 0.8 and 1.5 cm. Microscopically, the histological findings of the fourteen isolated lymph nodes were compatible with follicular hyperplasia. The subserosal cecal appendix showed transmural necrosis and perforation causing leakage of purulent material and an acute inflammatory reaction of the surrounding adipose tissue which extended up to the cecal and ileal serosa. All layers of the appendix were independent and unrelated to the cecum wall (Fig. ). The distal portion of the appendix showed the proliferation of cellular nests that were composed of round uniform nuclei with a “salt and pepper” appearance (Fig. ). No mitotic activity was evidenced. It seemed to infiltrate the muscular layer of the appendix and reach a diameter of 1.5 cm. No lymphovascular or perineural invasion was observed. Disease free margins were reported. Ileum dissection showed Peyer’s patches hyperplasia with wide germinal centers. Tumoral cells’ immunochemical studies showed a Ki67 proliferative index of 2%, a positive (+ + +/+ + +) cytoplasmic granular pan-cytokeratin, a positive (+ + +/+ + +) cytoplasmic chromogranin and a negative synaptophysin reaction (Fig. ). Pathologists concluded the presence of an incidental well differentiated neuroendocrine tumor grade I pT1 pN0 at the tip of the appendix in the middle of a clinical picture caused by an acute necrotizing appendicitis of a complete subserosal appendix. Due to the stage, no further intervention was required. She fully recovered in subsequent controls. Nevertheless, correct management of short bowel syndrome will become a key feature for the preservation of her future quality of life.","This 9-year-old girl was in the hospital with a bad stomachache, fever, and vomiting for several days. Tests showed she had an infection in her colon and a small tumor at the tip of her appendix. After surgery to remove the tumor and part of her colon, doctors found that the tumor was benign. She is now recovering at home and will need ongoing care to manage her digestive system."
292,"A 31-year-old man with UC was presented to a local hospital due to diarrhea and hematochezia. The young man was diagnosed with UC 3 years ago but didn’t receive regular treatment as prescribed. After the treatment of mesalazine, anti-infection medicines of amoxicillin, parenteral nutrition supplementation and protecting the intestinal mucosa, all the symptoms worsened, and the patient began to develop fever and facial erythema with blisters forming at the raised border of the erythema (Fig. a, b). For further treatment, the patient was referred to our hospital with the complaints of bloody purulent stool for 1 month, fever for 9 days, erythema and blisters on face for 7 days. The initial laboratory examination demonstrated an elevated white blood cell count (10.60 × 109/µL, normal range 3.5–9.5 × 109/µL), increased C-reactive protein (173.96 mg/L, normal range 0–10 mg/L), procalcitonin (1.93 ng/mL, normal range < 0.05 ng/mL) and Epstein-Barr virus (EBV) DNA (1720 copies/mL, normal range 0 copies/mL) levels, together with a low haemoglobin (90.00 g/L, normal range 130–175 g/L) level. The computed tomography with contrast medium exhibited extensive colonic wall thickening with a few perienteral exudative changes and multiple lymph nodes in the retroperitoneal and mesangial areas, consistent with the characteristics of UC. Computed Tomographic Enterography showed extensive colonic thickening wall with a few perienteric exudative inflammation, and multiple lymph nodes in retroperitoneal and mesangial areas, which were consistent with the characteristics of UC. Biopsies of cutaneous lesions were performed, revealing localized epidermal ulceration with neutrophil infiltration and dermal appendages with the infiltration of chronic inflammatory cells and neutrophils (Fig. c, d). We considered the clinical diagnosis of acute febrile neutrophilic dermatosis. After ruling out other infectious diseases and lymphoproliferative syndrome, we decided to initiate corticosteroid treatment though high levels of EBV DNA. After that, the patient no longer developed fever and the skin manifestations improved significantly (Fig. ). Colonoscopy revealed scatter polypoid hyperplasia from the ascending colon to the sigmoid colon (Fig. a, b). Histology of the resected polyp was characterized by crypt dilatation, branching, twisting with interstitial edema, local interstitial fibrosis, and muscle fiber penetration growth. And localised neutrophils infiltrated into the epithelium to form cryptonitis. These histological results were consistent with the characteristics of MPPs (Fig. c, d). No recurrence of SS occurred within 3 months.","A 31-year-old man with a history of ulcerative colitis (UC) was admitted to the hospital because of worsening symptoms, including bloody diarrhea, fever, and a rash on his face. Tests revealed an infection and inflammation in his colon, along with an unusual skin condition. After ruling out other possible causes, doctors diagnosed him with a type of inflammatory bowel disease and a skin condition. He was treated with medication, and his symptoms improved significantly."
293,"A 39-year old woman with a history of irritative LUTS with macrohematuria and recurrent proven urinary infections (4–5 per year) over more than 10 years was directed to us by her attending urologist. During cystoscopy diffuse atypical flat black pigmented bladder tumors were seen throughout the bladder. An initial tentative diagnosis was melanosis of the bladder [].\nThe patient received an extensive transurethral bladder resection (TUR-B), in which most (but not all) of the tumor formations were resected. In multiple locations, a thick layer of black pigmented cells was scraped away from a healthy underlying urothelial submucosa (Fig. ). Retrograde ureteropyelography showed no evidence of intraureteral lesions (Fig. ).\nHistologically, condyloma-like benign hyperkeratotic squamous cell deposits could be seen in all resection samples (Fig. ) and the diagnosis of intravesical ichthyosis was made. Urine cytology showed no signs of malignancy. The preoperative urine culture only showed natural skin flora (100 CFU/ml).\nA profound anamnesis revealed that there was a 2-time history of extravesical CA (cervical, 10 years ago and perineal, 3 years ago) with cystoscopically no intravesical lesions at that time. The patient’s partner had no history of condylomata and both were never vaccinated against HPV. The patients only comorbidities were hypothyroidism (treated with L-Thyroxin 75 µg daily) and a penicillin-allergy. She was in a good physical condition and had never smoked.\nHPV-Screening (urethral swab) was mildly positive for HPV42-DNA, a standard HPV-Vaccine (Gardasil-9®) followed. Colonoscopy showed no signs of intestinal condylomata; one small sigmoidal tubulovillous adenoma was resected.\nPostoperatively, the recurrent urinary infections with macrohematuria persisted. Two control-cystoscopies after 2 and 5 months postoperatively showed minimal persistence of ichthyosis and extensive scarring, with no signs of active growth. The next cystoscopy is planned in 3 months, with a re-TUR-B if lesions progress.","The patient has had frequent urinary infections and blood in her urine for over 10 years. During a cystoscopy, doctors found unusual growths in her bladder, which were removed through a surgical procedure. The tissue samples showed that the growths were benign, but the patient continues to experience urinary problems. Further tests and follow-up cystoscopies are planned to monitor the bladder and address the ongoing issues."
294,"A 57-year-old man presented to the gastroenterology department of our hospital with refractory ascites for two years. He had no history of metabolic syndrome or alcohol consumption. He had a history of hypertension, hypothyroidism, and chronic nephritis, who was treated with nifedipine tablets and thyroxine tablets. He denied any fever, chest pain, rashes, oral ulcers, arthralgias and visual changes, and had no recent travel and no sick contacts. In the past two years, he has been treated in the gastroenterology department of many hospitals for ascites, and has undergone blood tests, ascites test, gastroscopy, colonoscopy, abdominal enhanced CT, etc. However, there was no clear diagnosis. The patients received oral or intravenous furosemide, oral spironolactone, and abdominal puncture drainage to resolve ascites in many hospitals, but the results were not satisfactory.\nThe physical examination included a poor general condition, palpable lymph nodes in both sides of the neck and groin with a larger diameter of about 1 cm, abdominal distension, no tenderness and rebound pain, positive mobile dullness, mild edema of both lower limbs, enlarged spleen which lower edge is 3 fingers under the ribs. The blood routine showed that white blood cells were 4.44 × 109/L, hemoglobin was 111.0 g/L, and platelets were 93.0 × 109/L. Urine protein was weakly positive, urine pentaprotein test showed that microalbumin was 82.40 mg/L (reference value 0–30 mg/L), immunoglobulin IgG was 33.40 mg/L (reference value 0–8.5 mg/L), transferrin was 3.29 mg/L (reference value 0–2.2 mg/L), α1-microglobulin was 54.20 mg/L (reference value 0–12 mg/L), β2-microglobulin was 0.19 mg/L (reference value 0–0.22 mg/L). Other positive laboratory indicators included uric acid 520 μmol/L, albumin 36.6 g/L, and erythrocyte sedimentation rate (ESR) 26.0 mm/h. Serum thyroid stimulating hormone (TSH) was 5.5400 mIU/L, serum free thyroxine (FT4) was 14.81 pmol/L, serum free triiodothyronine (FT3) was 1.74pmol/L, which was a slight decrease. Stool routine, urea nitrogen, creatinine, C-reactive protein (CRP), liver function, serum vitamin B12, IgG4, folic acid, hepatitis virus (A, B, C, D, E), tumor markers (CA125, CA199, CEA, AFP, PSA), brain natriuretic peptide (BNP), and tuberculosis detection (PPD test, T-spot), as well as other autoimmunity makers containing antinuclear antibody (ANA), anti-neutrophil cytoplasmic antibodies (ANCA), and rheumatoid factors were all unremarkable. The patient’s HIV, EBV, CMV or Toxoplasma was negative. HHV8 and IL-6 were not detected. The gastroscope showed superficial gastritis, and the colonoscopy showed no obvious abnormalities. The echocardiogram showed a little pericardial effusion. The enhanced CT of the chest and abdomen depicted pneumonia, bilateral pleural effusion, and abdominal effusion. We performed abdominal paracentesis for this patient. The ascites was yellow and clear, the nucleated cell count was 40 × 106/L, the mononuclear cells accounted for 80.6%, and the multinucleated cells accounted for 19.4%. The Rivalta test was negative. The content of adenosine deaminase (ADA) in ascites was 2.6 U/L (reference value 0–25U/L), lactate dehydrogenase (LDH) was 74 IU/L (reference value 120–250 IU/L), albumin was 15.7 g/L, CA125 in ascites was 542 ng/mL (reference value 0–7 ng/mL), CEA, APF, and CA199 were normal. No malignant cells and tubercle bacilli were found in multiple tests of ascites. Serum ascitic albumin gradient (SAAG) was 20.9 g/L.\nThe patient had ascites, which should be polyserositis to be precise, superficial lymphadenopathy, enlarged spleen, hypothyroidism. We made differential diagnosis based on available data. The causes of ascites may be the following: liver cirrhosis, tuberculosis, tumor, rheumatism, endocrine, cardiac insufficiency, and nephritis. SAAG remains the most sensitive and specific marker for the differentiation of ascites due to portal hypertension from ascites due to other causes. The SAAG of the patient was greater than 11 g/L, however, there were no history of hepatitis, no esophageal/gastric varices under gastroscope, and no typical CT images of liver cirrhosis. We did not perform HVPG measurement and liver stiffness measurement, nor did we perform liver biopsy to rule out other rare causes of portal hypertension. We comprehensively considered and ruled out liver cirrhosis, which should be reported to a certain extent as a limitation of case reporting. He had no history or exposure of tuberculosis infection, no fever, no night sweats, negative tuberculosis test (PPD, T-spot), normal ADA in ascites, and no tuberculosis bacilli have been detected in ascites. So tuberculosis infection was also ruled out. The patient had a small amount of urine protein, mild hypothyroidism, normal rheumatism indicators, and no manifestation of cardiac insufficiency, so it was necessary to focus on tumors or other rare causes. After communicating with the patient and obtaining his consent, we gave him an in-depth comprehensive examination including bone marrow testing, PET-CT, and lymph node biopsy.\nPET-CT reported that his bilateral neck, axillary, retroperitoneum and groin had enlarged lymph nodes with a slight increase in FDG metabolism. Combined with the medical history, it was considered to be consistent with the metabolic changes of indolent lymphoma by the medical technicians. Bone marrow cytology indicated that bone marrow cells proliferated actively, granulocyte proliferation was obviously active with nucleus shifted to the right, erythroid proliferation was active, platelets were aggregated and distributed, and primitive cells accounted for about 1.0% of nuclear cells. The immunophenotyping of bone marrow lymphoma showed that the proportion of myeloid blasts was not high, with normal phenotype, the proportion of lymphocytes was not high, there were no abnormal monoclonal cells and no abnormal plasma cells. Was this patient with lymphoma? We were in confusion. Fortunately, the right neck lymph node biopsy pathology gave us the answer. Pathological examination of the lymph nodes showed that the lymph follicles increased, the germinal center was atrophied, the inter-follicular and paracortical areas showed vascular hyperplasia, and the mantle area was obviously hyperplasia with onion-skin-like change (Fig. ). Onion-skin-like appearance was a typical pathological manifestation of CD. The immunohistochemical results were: CD3 (paracortical cells +), CD5 (paracortical cells +), CD20 (germinal center cells +), PAX5 (germinal center cells +), CD21 (follicular dendrites +), CD34 (Vascular +), Bcl-2 (mantle area +), SOX11 (−), Cyclin D1 (−), Ki-67 (+, about 10%). Finally, the patient was diagnosed with CD. We recommended him use CHOP chemotherapy, but he refused and chose oral thalidomide, the patient had poor compliance and refused to use steroid therapy. Three months later, his symptoms did not improve significantly. Due to economic reasons, he still refused chemotherapy and chose oral diuretics to relieve ascites.","This 57-year-old man has been struggling with a buildup of fluid in his abdomen (ascites) for two years, despite many tests and treatments. He also has enlarged lymph nodes and an enlarged spleen. After a thorough investigation, including scans and biopsies, he was diagnosed with a type of lymphoma, a cancer of the lymphatic system. He initially refused chemotherapy, but his condition didn't improve, and he ultimately chose to manage his symptoms with diuretics."
295,"A 45-year-old female of Han nationality presented to a cirneal clinic in August, 2017 with a history of pain, redness, and decreased vision in her right eye 1 week after being exposed to river water. Corneal scrapings and confocal microscopy in vivo were performed instantly. A mass of hyphae was found in the 10% KOH wet mount stained with lactophenol blue and examined via confocal microscopy. Fungal keratitis was identified, and right corneal keratectomy was performed. Empirical antifungal and antibacterial therapy was initiated including topical and systemic fluconazole, levofloxacin, and cefminox sodium. After treatment of 2 weeks, the ulcer and symptoms did not improve and the patient was admitted to our hospital. Conjunctival congestion persisted, and a central corneal ulcer with a diameter of around 6 mm that reaches deep into the stromal layer can be seen (Fig. A, B). Subepithelial and superficial stromal opacities with dot-like and tentacle-like infiltrates accompanied this. Intracameral fluconazole injection was used to conduct a lamellar keratoplasty. Amphotericin B was administered immediately after the surgery. The first day post-surgery, hyperemia and a thin exudation membrane in the anterior chamber were discernible (Fig. C, D). Four days after the keratoplasty, full-thickness large central infiltrate with hypopyon was observed in the right eye (Fig. E, F). Given the increasing infiltrate with hypopyon and ineffectiveness of antifungal therapy, the right eye was enucleated. Microbial culture for corneal tissue revealed fungus-like organism showing long sparsely septate hyaline hyphae (Fig. G, H). The organism was further identified as P. insidiosum by ribosomal RNA (rRNA) gene sequencing with panfungal primers (ITS1/ITS4), which matched 99.23% with the P. insidiosum strain (GU137348.1). Then, the patient was adjusted with combined antibacterial treatment consisting of linezolid and azithromycin, and no recurrence of infection was observed on follow-up.","This 45-year-old woman developed a serious eye infection after being exposed to river water, causing pain, redness, and blurry vision in her right eye. Tests revealed a fungal infection in her cornea, requiring a corneal removal surgery. Despite treatment, the infection continued to worsen, leading to the need for an enucleation (removal of the eye). Further testing identified the specific fungus causing the problem, and the patient was treated with antibiotics to prevent further complications."
296,"A 51-year-old female of Han nationality was referred to our hospital in September 2018 with complications of pain, redness, and hyperemia in her right eye after entry of some cigarette ash 1 week ago. Corneal scraping was performed, and Gram staining, KOH preparation, and cultures were negative. Antifungal and antibacterial treatment was initiated including levofloxacin eye drops, cefminox sodium, and voriconazole. After 2 weeks of outpatient treatment, the patient was hospitalized. A grayish-white ulcer was observed in the central of bitamporal cornea measuring 4 × 6 mm. Inflammatory infiltrates with feathered margins and hypopyon with a depth of 2 mm were seen, suggestive of a fungal infection. The cornea showed dense central stromal opacity surrounded by a reticular pattern of subepithelial and superficial stromal infiltration (Fig. ). Then penetrating keratoplasty was performed. Exudation was observed in anterior chamber on 2 days following surgery, and intracameral fluconazole injection was performed. However, the infiltrates extended progressively, and were unresponsive to any treatment. Therefore, by day 28 post-exposure, an enucleation was performed to remove infected tissue and relieve pain. One week later, a small amount of mycelial growth was observed within the corneal fragment on the potato dextrose agar (PDA) plate. Subculture in brain–heart infusion resulted in the rapid growth of a large mycelium at 35 °C. We have attempted to identify this mycelium by matrix-assisted laser desorption ionization–time of flight mass spectrometry (MALDI-TOF-MS) but failed. There was no reference spectrum in the Bruker Filamentous Fungi databases despite the high quality of the protein spectrum. The mycelium was sent for internal transcribed spacer (ITS) rRNA gene sequencing analysis and unambiguously identified as P. insidiosum.. Three obvious protein peaks of the strain were found by MALDI-TOF-MS, which are 2094.01, 4834.62, and 9682.51 (× 103 m/z), respectively (Fig. ). Then, the reference spectrum of P. insidiosum was added to the in-house database according to Bruker MALDI-TOF-MS standard process to create a library. Therefore, the identification of P. insidiosum could be facilitated using MALDI-TOF-MS.","This woman was seen in the hospital after getting ash in her eye a week prior, causing pain, redness, and blurry vision. Doctors found a fungal infection in her eye, leading to an ulcer and inflammation. She underwent corneal transplant surgery, but the infection continued to spread, requiring the removal of her eye. Finally, tests identified the fungus as Protomyces insidiosum, allowing doctors to track the infection and prevent it from spreading."
297,"A 55-year-old male of Han nationality presented with irritation, pain, and hyposia in the left eye for 3 days after facial washing with contaminated river water. He went to a local ophthalmic clinic and was diagnosed with viral keratitis. After 4 days of antiviral therapy, the symptoms were not improved, so the patient was admitted to our hospital. Slit-lamp examination showed conjunctival hypertrophy and infiltrated growth into cornea about 2 mm from the nasal limbus. In the left eye, microvascular tissue hypertrophy of about 2.5 mm was seen across the corneal limbus, and hyperemia grayish-white infiltrate of cornea with a diameter of about 5 mm was observed (Fig. ). Direct microscopy of corneal scrapings stained with Gram and KOH preparation was negative for organisms. The patient was prescribed empiric fortified topical and systemic antibiotics, including ornidazole, tobramycin, vancomycin, natamycin, and fluconazole. On the third day after hospitalization, symptom improvement was not noted. Excision of pterygium and therapeutic penetrating keratoplasty were performed in the left eye. Cultures of his corneal tissue for bacteria, fungus, and Acanthamoeba were negative. Because there was evidence of increased keratoneuritis, antiamebic therapy (chlorhexidine) was initiated and voriconazole was added. Twelve days after the surgery, corneal opacity was rescraped and infiltration extended deeply into the anterior chamber. A second penetrating keratoplasty, virtually limbus to limbus, and intracameral amphotericin B injection were performed sequentially. However, 10 days after the second operation, the infection still spread to the adjacent sclera and progressed to endophthalmitis. Enucleation eventually had to be done. Subsequently, the corneal cultures growing on PDA plate were identified as P. insidiosum by MALDI-TOF-MS, confirming the diagnosis of pythiosis.","A 55-year-old man developed an eye infection after getting water in his eye. Initially, doctors thought it was a virus, but the infection worsened and spread to the cornea, causing pain and blurry vision. After several treatments, including surgery and medications, the infection continued to spread, eventually leading to the need for enucleation (removal of the eye). Finally, tests revealed the infection was caused by a rare parasitic fungus, which required further treatment."
298,"A 54-year-old medically free Saudi gentleman presented to our clinic complaining of a gradual painless decrease in vision of his left eye over one year. He also reported mild pain, photophobia, and redness from time to time. The patient underwent uneventful cataract surgery of both eyes 13 years prior to his presentation. The best-corrected visual acuity (BCVA) using a Snellen chart was 20/28 in the right eye (OD) and 20/400 in the left eye (OS). Slit-lamp biomicroscopic examination of the OD was unremarkable (Figure ). Upon examining the OS, there were 1+ (i.e., 6-15 cells per field) mixed anterior chamber cells. CCC was relatively small (measured 4.7 mm). In addition, a thick turbid greenish fluid was noted behind the IOL, limiting visualization of the posterior capsule (Figure ). B scan was performed due to poor visualization of the fundus, which revealed moderately dense vitreous opacities suggestive of vitritis. There was no associated choroidal thickening. Ultrasound biomicroscopy (UBM) showed a hyperechoic collection of turbid fluid behind the IOL with a distended capsular bag, confirming the presumed diagnosis of CBDS (Figure ). Late-onset CBDS with a high possibility of associated P. acnes was assumed, and surgical intervention was considered. After explaining the surgical procedure and obtaining written informed consent from the patient, a pars plana vitrectomy (PPV) with a posterior capsulotomy was performed. Initially, 23 gauge three PPV trocars were placed 3.5 mm away from the limbus. Then, through the superonasal sclerotomy site, a 27-gauge needle was inserted to pierce the posterior capsule and gently aspirate the liquified material. After aspirating and clearing the visual axis, a vitreous cutter was introduced through the same superonasal port to create posterior capsulotomy followed by core vitrectomy. Intravitreal vancomycin (1 mg/0.1 mL) was injected at the end of the procedure due to the high possibility of associated P. acnes endophthalmitis. After the procedure, the aspirated material was sent to histopathology for gram stains and cultures. After 72 hours, Thioglycolate broth was positive for the growth of P. acnes confirming the presumed diagnosis. The post-operative period was uneventful, and no signs of intraocular inflammation were detected. At six months, the patient had 20/25 BCVA and did fine without any complaints (Figure ).","This 54-year-old man noticed his left eye was getting blurry over the past year, along with some pain, light sensitivity, and redness. He had cataract surgery in the past, but now he has a problem with his IOL (intraocular lens) and a cloudy fluid behind it, which was confirmed by ultrasound. After surgery to clear the fluid and open up the back of his eye, the blurry vision improved, and he now has 20/25 vision. The doctors found bacteria in the eye, which is why they treated him with antibiotics."
299,"A 40-year-old woman was admitted for evaluation of critical limb ischemia of the right leg. Medical history is significant for human immune deficiency virus (HIV) infection with undetected viral load, coronary artery disease, peripheral vascular disease, and polysubstance abuse. Vascular surgery performed bilateral lower extremity angiogram, femoral artery angioplasty, and a femoral artery stent. The postoperative course was uneventful. Apixaban 2.5 mg twice daily was added to her medication regimen of clopidogrel, Darunavir/cobicistat (Prezcobix), abacavir/dolutegravir/ lamivudine (Triumeq), atorvastatin, isosorbide dinitrate, and hydralazine. The patient returned one-week post-discharge with low back pain and a computed tomography (CT) scan demonstrated a large retroperitoneal hematoma (Figures -).\nHer hemoglobin level on admission was 9.2 g/dl, down from a discharge level of 11.9 g/dl. Her serum creatinine was stable at 0.8 mg/dl. On arrival to ED, blood pressure was 130/69 mm of Hg, the heart rate was 87 beats per minute, respiratory rate was 18 breaths per minute, and the temperature was 37.4 °C. The patient was managed conservatively, and both her hemoglobin and vital signs remained stable throughout her admission. She was discharged on apixaban and clopidogrel as recommended by the vascular team. The patient was readmitted four weeks later with urosepsis. A follow-up CT abdomen revealed a resolving retroperitoneal hematoma. The patient reported that during her outpatient management she had only been compliant with her antiretroviral therapy (ART) and not with her antiplatelet or anticoagulant medication (clopidogrel and apixaban). Her anticoagulant regimen was changed to enoxaparin out of concern with potential drug-drug interaction with apixaban and cobicistat, and the team continued clopidogrel. This warning was identified by the electronic medical records.","This 40-year-old woman was admitted to the hospital because of poor circulation in her right leg, which led to surgery to open up the blocked artery. She also has other health problems, including HIV, heart disease, and a history of drug use. After her surgery, she developed a large blood clot in her back, which caused her pain and low blood counts. She was treated with medication and blood transfusions, and her condition stabilized. However, she was readmitted later with a urinary infection, and her medication was adjusted to avoid potential drug interactions."
300,"A 40-year-old gravida 1 para 0 Caucasian woman presented at 39 + 6 weeks gestational age with a 3-day history of new onset pain in an otherwise uncomplicated pregnancy. At 39 + 1 weeks gestation she had started outpatient cervical ripening with dinoprostone (Cervidil) because of her advanced maternal age. She described the pain along her right torso as severe, shooting, and sharp, but at times pleuritic in nature. It rapidly progressed from her right trapezius to encompass the entirety of her right torso, from her upper abdominal quadrant and epigastrium, radiating to her back, chest, shoulder, and neck. Her pain was initially attributed to possible radiculopathy, as she had a previous history of the same. Her vital signs at initial presentation were normal, with no hypertension noted, and there were no concerns regarding the fetal status. No further investigations were ordered and a full neurological examination was not documented.\nShe represented less than 24 hours later at 40 + 0 weeks gestational age, and was found to be hypertensive at 157/101 and 164/112 mmHg. Oxygen saturation was 98% on room air. She had sinus tachycardia on arrival, ranging from 110 to 140 bpm and persisting throughout the peripartum period. She was tender in the right upper quadrant, however there were no peritoneal signs, and no hepatomegaly was appreciated in the presence of the gravid uterus. She was tender from the right side of her neck through to her right lower back. Her reflexes were 3 + bilaterally with no clonus. The fetal heart rate was normal. Her blood investigations revealed a hemoglobin of 105 g/L, platelets 156,000 g/L, alanine aminotransferase (ALT) 193 μ/L, aspartate aminotransferase (AST) 111 μ/L, and uric acid 429 μmol/L. White blood cell count, creatinine, lactate dehydrogenase (LDH), and coagulation profile were within the normal range. An electrocardiogram (ECG) showed sinus rhythm with no abnormal features, apart from the previously noted tachycardia.\nAfter diagnosing preeclampsia, induction of labor was started, as was an infusion of magnesium sulfate. Due to the unusual but significant pain that she was experiencing, a range of differential diagnoses were considered by the obstetrical and anesthesia team. Preeclampsia with HELLP syndrome was the working diagnosis, however differential diagnoses included fatty liver, radiculopathy, cholecystitis, pancreatitis, and pyelonephritis. The patient requested labor analgesia be initiated and a combined spinal epidural was placed when the patient’s cervix was 2 cm dilated. Analgesia was maintained via programmed intermittent epidural bolus with patient-controlled epidural analgesia as needed, in keeping with our institutional practice. The patient reported satisfactory analgesia with regard to her labor pain, but still complained of severe pain in her right torso that was unresponsive to acetaminophen and opioids. Blood investigations monitored every 4 hours remained stable with hemoglobin of 103–102 g/L, platelets 158,000–165,000 g/L, ALT 176–169 μ/L, AST 100–97 μ/L, and uric acid 400–420 μmol/L. The LDH, creatinine, and coagulation profile remained normal. Labor progressed to full dilation with oxytocin augmentation, but as a result of torso pain she was unable to exert adequate effort with pushing. Ultimately, the obstetrical team consented the patient for a trial of forceps and possible cesarean delivery.\nIn the operating room, the obstetrics team performed an examination under anesthesia, which revealed the fetus to be in an occiput-transverse position at station zero, and the decision was made to proceed to cesarean delivery as the station was too high to perform a trial of forceps. A nonvigorous male infant was delivered with Apgar scores of 3 and 8 at 1 and 5 minutes, respectively. Placental delivery was uneventful. Atonic postpartum hemorrhage was treated with a bolus of 2 units IV oxytocin and carboprost 250 mcg intramuscular (IM), in addition to an oxytocin infusion. As per our institutional practice, a bolus of oxytocin is only used when the patient has multiple risk factors for intraoperative hemorrhage or is experiencing uterine atony. This low dose was chosen to prevent potential side effects (nausea, vomiting, chest pain) and complications (hypotension, hypertension, ST segment changes, myocardial ischemia, bronchospasm) that can be associated with higher dose boluses when given at the time of cesarean section [, ]. Prior to fascial closure, the obstetrics team manually explored the abdomen to the extent possible through the Pfannenstiel incision. No gross abnormalities were identified. The patient’s right torso pain persisted throughout her time in the operating room, with only a slight improvement after surgical anesthesia obtained via the epidural. After delivery, 2.5 mg of epidural morphine was given for postoperative analgesia. The patient was hemodynamically stable throughout the operation, although remained tachycardic. Estimated blood loss was approximately 900 mL. Postoperatively, the patient was sent for a computed tomography (CT) scan of her chest, abdomen, and pelvis, including contrast for a pulmonary embolism protocol to investigate the abdominal pain and persistent tachycardia. No pulmonary embolus was found and a small right pleural effusion with subsegmental atelectasis was noted. However, the most impressive feature on the scan was a large but intact subcapsular liver hematoma that measured approximately 16 × 7 × 14 cm (Fig. ). Portal hypertension was suggested by the presence of portosystemic varices. The general surgery team was consulted and recommended conservative management with strict blood pressure control requiring oral labetalol, frequent complete blood count (CBC) monitoring, and 48 hours of bedrest. If, however, the SCH had been diagnosed antepartum, labor would have been avoided and delivery would have been expedited via immediate cesarean section. The SCH is at risk of rupture with active pushing, convulsions, or abdominal trauma, including vigorous palpation of the right upper quadrant []. In this case, prophylactic antibiotics were not used as there were no signs of infection and the underlying etiology was noninfectious. Interventional radiology was on standby to perform hepatic artery embolization if the SCH significantly increased in size or there was concern about imminent rupture. Serial monitoring of blood work revealed a hemoglobin of 70 g/L and 56 g/L on postoperative days 1 and 2, respectively, prompting transfusion of two units of packed red blood cells on day 2. The patient was discharged on postoperative day 5 with her hemoglobin stable at 85 g/L.\nAn early postoperative follow-up visit was arranged 15 days postpartum and at that visit the patient was noted to be pale, tachypneic, and upon questioning she stated increasing shortness of breath and a persistence of her pleuritic chest pain. She was sent back to the IWK Health Centre where her vital signs were as follows: heart rate 110–130 bpm, blood pressure 112/66 mmHg, oxygen saturation 97% on room air, respiratory rate 40–48, temperature 37.9 °C. Blood investigations revealed a white cell count of 6, hemoglobin 97 g/L, platelets 650,000 g/L, ALT 75 μ/L, AST 88 μ/L, LDH 1310 μ/L, and coagulation profile remained normal. A repeat abdominal CT scan reported an increase in the size of the liver hematoma to 14 × 8.5 × 18.3 cm (Fig. ). Although there was no evidence of active intralesional bleeding or rupture, the liver capsule was difficult to visualize in the superior aspect, and could suggest significant thinning and imminent rupture. The CT scan of the chest reported a large right pleural effusion with mediastinal shift (Fig. ).\nThe patient was urgently transferred from the stand-alone maternity hospital to a nearby hospital under the care of the thoracic surgery team, with the general surgery team on standby. A chest tube was placed, but ultimately the patient required video-assisted thoracoscopic surgery (VATS), with right partial decortication of an infected, loculated pleural effusion prior to her discharge home. The pleural effusion aspirate showed no growth. The right pleural effusion was felt to be exudative in nature and secondary to the adjacent large SCH. Throughout the admission she remained normotensive and her hepatic hematoma remained stable with no signs of active bleeding. She was discharged home on day 2 following the VATS procedure, coinciding with day 21 following her cesarean section. At discharge, her hemoglobin was 74 g/L and her platelets were 884,000 g/L.\nShe continued to receive follow-up with general surgery and obstetrics as an outpatient. A CT performed 4 weeks postpartum showed the hepatic hematoma had decreased in size to 12.6 × 6.4 × 15.1 cm and at 6 months postpartum had decreased to 3.8 × 2.2 × 3.1 cm and did not require any further follow-up.","This woman experienced severe pain in her abdomen and chest during her pregnancy, which doctors initially thought might be related to nerve problems. Her blood pressure then rose significantly, and she started having difficulty breathing, leading to a C-section. During the surgery, a large blood collection formed in her liver, and she developed a significant pleural effusion (fluid buildup in her chest). Despite treatment, she remained unwell and required further surgery to drain the fluid. After several weeks of monitoring, her condition stabilized, and she was eventually discharged home."
301,"Patient: a 55-year-old Japanese female.\nNone of the factors contributing to lateral compartment knee disease, such as obesity, complications, or psychiatric disorders, were present in the patient. During hyaluronic acid (HA) treatment, although we prescribed strength training to the patient, she did not perform it frequently enough because of severe pain. As the knee pain was caused by tripping while playing tennis, the possibility of trauma cannot be ruled out.\nIn 2013, due to pain in both knees, she visited our hospital and was diagnosed with OA of the knee.\nIn December 2017, while playing tennis, the right knee developed a knee collapse. She was examined at the hospital, and an MRI of the right knee was performed. Horizontal dissection of the lateral meniscus and cartilage defect on the lateral condyle of the femur were observed.\nSince 2018, injections of hyaluronic acid have been administered every 2 weeks, but joint edema and pain recurrence have been remitted.\nThe patient had already undergone HA treatment and received multiple steroid joint injections; however, these interventions were not very effective. Moreover, the patient was unwilling to undergo these treatments. The patient was aware that our hospital would start providing regenerative medicine treatments and had been waiting for a year before the start of the treatment. During that period, the patient received HA treatment, which proved ineffective. Therefore, the patient requested ASC treatment in April 2018.\nIn April 2018, MRI of the right knee was performed. A cartilage defect was found in the external condyle of the femur, and the patient desired ASC transplantation. The range of motion of the right knee is 0–145. Anteroposterior (AP) and lateral radiograph of the right knee are shown in Fig. .\nOn 28 April 2018, 20 mL ASCs were collected from the abdomen.\nOn 11 June 2018, the first ASC transplantation was performed on the right knee.\nOn 2 July 2018, although the effect was experienced after 1 week of treatment, the pain recurred when the subject moved violently.\nOn 6 August 2018, the patient was in a good condition. When the Timed Up and Go Test was conducted, it showed improvement from 10 seconds to 7 seconds.\nOn 10 September 2018, the patient was in a good condition. She resumed playing tennis and was living almost without pain.\nOn 10 December 2018, an MRI was performed. There was no pain even when the subject jumped on one leg and a repaired cartilage defect was observed.\nOn 22 April 2019, the second ASC transplant was performed on the right knee and the first ASC transplant was performed on the left knee.\nOn 22 June 2019, there was a slight fever on the day of the procedure.\nOn 5 August 2019, the patient was in a good condition. It became possible to assume a sitting position.","This 55-year-old woman has struggled with knee pain for many years, initially diagnosed with osteoarthritis in She tried hyaluronic acid injections, but they didn't provide lasting relief, and she was hesitant to continue with other treatments. After an MRI revealed cartilage damage, she underwent autologous stem cell transplantation (ASC) in both knees, with the first transplant successful and leading to improved mobility and the ability to return to playing tennis. Follow-up MRIs showed successful cartilage repair in both knees, indicating a positive outcome from the stem cell treatment."
302,"An obese 32-year-old Middle Eastern man with a body mass index (BMI) of 33 presented to the emergency department (ED) with a 6-day history of severe, worsening, generalized abdominal pain and vomiting. He had undergone LSG 13 days prior to the current admission at a private hospital. He was prescribed a protein pump inhibitor and enoxaparin 40 mg daily, which he had taken regularly.\nOn examination, the patient looked unwell, dehydrated, and in pain. His Glasgow Coma Scale score was 15, and he had a heart rate of 135 beats per minute, blood pressure 132/82 mmHg, respiratory rate 20 breaths per minute, and body temperature 36.5 °C. On examination, his abdomen was distended with generalized tenderness, but his laparoscopic wounds had healed.\nInitial laboratory investigations revealed a white blood cell count (WBC) of 27,300/μL (4000–11,000 μL), hemoglobin 17.3 g/dL (10–15 g/dL), and serum lactate 7.6 mmol/L (0.5–1.9 mmol/L). Computed tomography (CT) of the abdomen and pelvis with intravenous contrast revealed extensive acute on chronic portosplenic and superior mesenteric vein thrombosis, with consequent small bowel ischemia (Fig. ).\nHe was admitted under the care of the acute surgical team and resuscitated, and underwent laparoscopic exploration, which confirmed the CT findings. After conversion to midline laparotomy, 255 cm of small bowel was resected (Fig. ) and the abdomen was left open with a vacuum-assisted closure device dressing (Fig. ). The patient was transferred to the intensive care unit. A second look was carried out 24 hours later, and both the small and large intestines appeared healthy, so primary anastomosis and abdominal closure were performed. A solid diet was introduced gradually, and the patient was discharged home on day 12 postoperation on warfarin. A thrombophilia screen was negative. The patient was seen multiple times for follow-up; he was tolerating oral intake and had reduced his BMI to 19 with no clinical manifestations of short bowel syndrome.","A 32-year-old man with obesity was admitted to the hospital because of severe abdominal pain and vomiting that had been getting worse for six days. Doctors found that he had a serious blood clot problem in his intestines, which was causing damage and requiring a significant portion of his small intestine to be removed. After surgery, he was closely monitored and gradually started eating solid foods, and his weight has decreased. He was discharged home after 12 days and is now doing well, with no signs of problems related to the surgery."
303,"A 63-year-old woman visited a clinic for a palpable right axillary mass. US showed a 2.5-cm irregular hypoechoic mass in the right axilla, which was considered to be a malignant lymph node such as metastasis (Fig. ). Similar to physical examination and mammography, US revealed no remarkable findings in both the breasts and the left axillary region. A 14-gauge core-needle biopsy revealed the right axillary lymph node as a metastatic carcinoma, possibly from the breast, with positive estrogen receptor (ER) status. Breast MRI was performed to determine the presence of occult breast malignancy. No lesions were detected in the right breast, whereas a 0.6-cm irregular enhancing mass was observed in the left upper inner breast, assessed as breast imaging reporting and data system (BI-RADS) category 4C (Fig. A). To exclude primary malignancies other than breast cancer as the origin of the metastasis, chest computed tomography (CT) and whole-body positron emission tomography-computed tomography (PET-CT) were performed. They revealed no specific findings, except the known right axillary metastasis.\nSecond-look US showed a 0.6-cm irregular, indistinct, isoechoic mass in the left upper inner breast, corresponding to the mass observed on breast MRI (Fig. B). US-guided 14-gauge core-needle biopsy confirmed the mass as an invasive carcinoma of no special type. The ER status of the cancer was positive, same as that of right axillary metastasis. Therefore, the right axillary metastasis was suspected to originate from the left breast cancer, and surgery was planned for its management and to confirm the diagnosis.\nRight axillary lymph node dissection and left breast conserving surgery with sentinel lymph node biopsy were performed. The invasive carcinoma in the left breast was 6 mm in size, with cells positive for ER and progesterone receptor (PR) and negative for human epidermal growth factor receptor 2 (HER2). It was a low-grade invasive cancer with low Ki-67 expression (5%), histologic grade 1, and without lymphovascular invasion. Sentinel lymph node biopsy confirmed no left axillary involvement. In the right axilla, three macrometastatic lymph nodes were discovered, including the known palpable metastatic lymph node. Histologic findings of cancer cells in all three lymph nodes were fairly different from those of cancer cells in the left breast (Fig. ). Examination of the right axillary metastasis showed high-grade carcinoma with poorly formed glands and nests of atypical cells, while examination of the left breast mass showed low-grade cancer with well-differentiated glands and fairly uniform nuclei. These findings clearly indicated that the metastasis to the right axillary lymph node did not originate from the left breast cancer, although both of them showed ER positivity. Metastatic cancer cells in the right axilla were also positive for gross cystic disease fluid protein-15 (GCDFP-15) and GATA binding protein 3 (GATA3), suggesting that the metastasis was derived from the breast (Fig. ).\nFinally, the case was diagnosed as bilateral breast cancer consisting of occult right breast cancer with axillary metastasis (TxN1M0) and early-stage left breast cancer (T1bN0M0). Four cycles of doxorubicin and cyclophosphamide followed by four cycles of taxane (AC-T) chemotherapy, radiation therapy for each breast, and hormone therapy were administered after surgery. No recurrence or metastasis was observed 14 months postoperatively.","A 63-year-old woman saw a doctor because of a lump in her armpit. Tests showed the lump was cancer that had spread from somewhere else, likely her breast. Further tests revealed a small, new area of cancer in her left breast, and the doctors determined that the cancer in her armpit had come from the breast cancer, not the other way around. She underwent surgery to remove the cancer in both breasts and is now receiving chemotherapy, radiation, and hormone therapy to treat the cancer and prevent it from returning."
304,"A 77-year-old Japanese woman, para 7, who went through menopause at age 48, suffered sudden onset of left lower abdominal pain and visited a primary care doctor. Computed tomography (CT) revealed torsion of a left ovarian cyst, and she was transferred to our hospital for surgery. She had a history of left-side breast cancer and underwent total left mastectomy at the age of 67, with no recurrence thereafter. Although she was a carrier of hepatitis C, her liver function and coagulation remained normal, and she was being followed-up without medication. Furthermore, she was taking nifedipine and candesartan cilexetil for hypertension. Meanwhile, her family history and psychosocial history were unremarkable. She also had no history of trauma.\nCT showed a 7.5-cm long elliptical mass in the left adnexal region that was continuous with the uterus (Fig. ). The ovary was atrophic because of the patient’s age, and difficult to identify; the appearance of blood and the continuity with the surrounding pelvic peritoneum were suggestive of retroperitoneal hematoma. Physical findings at admission were as follows: height, 144.5 cm; weight, 57.2 kg; body mass index (BMI), 27.4 kg/m2; blood pressure, 112/50 mm Hg; pulse, 72 bpm; and body temperature, 37.1 °C. Physical examination detected no significant findings. On external (body surface) and internal examinations, no palpable masses or tenderness were noted in the left pelvic area. Transvaginal ultrasound revealed an atrophied uterus; however, the bilateral adnexa could not be identified due to atrophy. The hematoma in the left pelvis could be identified. These findings were identical to those obtained by CT. Blood test results were a hemoglobin value of 9.5 g/dL, a hematocrit value of 28.4%, a white blood cell count of 7970/μL, and a C-reactive protein level of 0.05 mg/dL. Urinalysis revealed mild occult blood. After admission, the hematoma did not enlarge, and the hemoglobin and hematocrit levels did not decrease. After 11 days of bed rest, with no exacerbation of the inflammatory response, lower abdominal pain was relieved, and she was discharged. After discharge, we planned to investigate the cause of her bleeding on an outpatient basis, but 6 days after discharge, she was readmitted to our hospital after relapse of symptoms caused by retroperitoneal bleeding from the same site. The hematoma had grown to a size of 11.7 cm, and magnetic resonance imaging showed an abnormal vascular network extending from the left side of the uterus to the left adnexal area. Three-dimensional CT (3D-CT) similarly showed an abnormal vascular network on the left side of the uterus, and we suspected a possible arteriovenous malformation (AVM) or arteriovenous fistula (AVF) from the uterine artery to the left ovarian vein (LOV; Fig. ).\nWe decided that urgent treatment was needed for her repeated bleeding and performed vascular embolization therapy simultaneously with angiography. However, angiography of the internal iliac artery, including the left uterine artery, and the left external iliac artery, did not show AVM or AVF into the LOV. Angiography of the left renal artery confirmed that the contrast medium discharged from the left kidney did not flow into the inferior vena cava (IVC) but flowed back into the LOV (Fig. a). Subsequent venography also confirmed that the blood from the LRV did not flow into the IVC but regurgitated into the LOV and the lumbar vein (Fig. b). Left ovarian venography showed retrograde blood flow into a dilated and tortuous aneurysm in the pelvis and into the right internal iliac vein via a vein around the uterus (Fig. c). The cause of retroperitoneal bleeding was rupture of the LOV, the distension of which was caused by reflux. We performed embolization of LOV with N-butyl-2-cyanoacrylate and placed a coil centrally to prevent recanalization. Postembolization LRV imaging showed blood flow to the upper left lumbar vein and vertebral plexus (Fig. d). CT on the second day after the procedure confirmed good embolization of LOV and reduction of the retroperitoneal hematoma to a size of 6.4 cm (Fig. ). The patient then underwent regular outpatient follow-up, and the retroperitoneal hematoma gradually shrank and disappeared. At her final visit 2 years later, the hematoma had not recurred.\nIn this case, angiography confirmed the continuity of LRV and IVC but demonstrated no antegrade blood flow to the IVC. Therefore, we attributed LOV regurgitation to extravascular physical compression of LRV. After close examination of the CT images, we determined that LOV rupture was caused by NCS because the LRV was severely stenotic between the abdominal aorta and the SMA (Fig. ).","This 77-year-old woman was admitted to the hospital after experiencing sudden left lower abdominal pain. Tests revealed a twisted ovarian cyst, which required surgery. She has a history of breast cancer and was being treated for high blood pressure. After initial treatment, she was readmitted due to recurring bleeding, and further tests showed a problem with a vein in her ovary. Finally, doctors determined that the vein had ruptured due to a narrowing of the blood vessel, and they successfully treated the problem with a procedure to block the leaking blood vessel. She is now doing well and is being followed up regularly."
305,"A 48-year-old G3/P2 woman presented to the Department of Gynecology with a physical examination. Ultrasonography (Fig. A) and enhanced computed tomography (ECT) (Fig. B) revealed pelvic masses. She was recommend to undergo laparoscopic ovarian cystectomy for a borderline ovarian tumor eight years before. During the eight years, she did not have regular medical examinations because there were no symptoms of diseases. She chose to undergo surgical treatment for pelvic masses. Finally, she underwent a hysterectomy and pelvic lymph node dissection for a recurrent mucinous borderline ovarian tumor (Fig. C). Postoperative routine examination showed endometrial lymphoma, and the other lymph nodes were not involved. Testing for immunoglobulin heavy chain (IGH) gene rearrangement showed a positive result (Table ). Uterine lymphoma was derived from the primary female genital system and was diagnosed as non-Hodgkin's lymphoma, consistent with diffuse large B-cell non-Hodgkin's lymphoma. Hematoxylin–eosin (HE) staining and immunohistochemistry were carried out to analyze the case specimen (Fig. ). The patients underwent bone marrow biopsy and PET-CT to observe whether there were other lymph node abnormalities. However, there was no evidence of bone marrow involvement by microscopic examination, and there was no abnormality in the whole body scan by PET-CT. Currently, the patient has received four postoperative courses of CHOP chemotherapy in the Department of Hematology. To date, no abnormality has been found in the follow-up.","This 48-year-old woman came to the doctor with pelvic masses found during a scan. She had a previous ovarian cyst and underwent a hysterectomy to remove a recurring tumor. During the surgery, doctors discovered endometrial lymphoma, a type of cancer in the uterus. After further testing, she was diagnosed with non-Hodgkin's lymphoma and is currently receiving chemotherapy."
306,"Our patient was a 13-year-old Caucasian female with a history of anxiety, depression, and anorexia nervosa. She was admitted twice to our hospital. The first admission was in September 2019, when her parents brought her for losing 16 kg over 3–4 months. She was weighing 40 kg (BMI 16.0 kg/m2, 8.7%ile, z = − 1.4) and had sinus bradycardia (HR 30–40 BPM). Table and Fig. show changes in her lab values during her two admissions.\nThe patient reported sadness, loneliness, anhedonia, and hopelessness and admitted to restrictive eating behaviors to lose weight. There was no history of purging, bingeing or laxative use. Although emaciated, she minimized the severity of her nutritional status or her preoccupation with her weight and body image. She denied suicidal ideation but desired death rather than pursuing an eating disorder treatment. The patient had compulsive tendencies for academic work and extracurricular pursuits and was highly driven. She had several moves during childhood and reported increased anxiety after a recent move to live with her father following parental divorce. She was starting a new school, as well. We placed her on 1:1 for safety concerns.\nThe patient started restricting around the age of ten after having a growth spurt at puberty. Following her diagnosis of AN, she received eating disorder treatment at different levels of care, including inpatient, residential, partial hospitalization or Day Program, and intensive outpatient programs in four states. She would become physically aggressive towards staff and family to sabotage her recovery. In one incident, she bit a chunk off the nape of her sister’s neck during a family visit in her residential stay, leading her to be discharged. She admitted to becoming jealous of the sister as she was to go home after the visit while the patient was to remain confined to the unit. She had no remorse about the incident. There was a report where she left the gas stove on to burn the house and kill her mom and her siblings when she was angry. She again had no regrets about her actions. She had history of hurting the family cat. This history of repeated aggression over several years toward other people and animals indicated a possible CD diagnosis. Family history was pertinent for anxiety and mood disorders and AN.\nDuring admission, she hid meals, avoided cardiopulmonary monitoring, and refused nasogastric tube. She would engage in isometric exercises and avoid lying down. She did not take medication for anxiety. As she failed weight restoration in the medical hospital, we referred her to an out-of-state inpatient eating disorder unit for further treatment. Right before the transfer, she jeopardized the transfer by stabbing her pediatrician with a pair of scissors that she hid from the Child Life Services supplies in her room. She intended to hurt the physician for transferring her to the inpatient unit. A room search discovered multiple sharp objects under her mattress, including plastic knives, forks, and medical supplies. For ongoing aggression, she necessitated physical restraints and emergency use of Lorazepam. The patient was formally diagnosed with CD after this incident.\nThe inpatient eating disorder facility refused to accept the patient after the violence, which led our interdisciplinary team to plan her weight restoration by implementing behavioral expectations and positive reinforcement techniques for meeting daily nutritional needs. Some of the privileges were using the phone, watching TV, or playing video games. We initiated and titrated chlorpromazine 50 mg twice daily for aggression. She gained 2.7 kg in about 6 weeks and her bradycardia resolved (see Fig. ). Her T3 and prealbumin normalized (see Table ). After 24 days in the hospital, she was discharged with a plan to start an eating disorder partial hospitalization program.\nFollowing discharge, she participated in the partial hospitalization program for 5 days. On the morning of the sixth day, she entered her father’s bedroom and stabbed him three times with a butcher’s knife. She had purchased the knife the day before to kill her father, blaming him for the eating disorder treatment. She was arrested and remanded to Juvenile Justice Center (JJC), where she started refusing to eat or to take chlorpromazine. She rapidly lost weight and was readmitted for her second admission to our pediatric hospital. On readmission, she weighed 36.5 kg (BMI 14.6 kg/m2, z = − 2.3, 1.0%ile) and was bradycardic and mildly hypothermic with a reduction in T3 and prealbumin. An echocardiogram demonstrated a trace posterior pericardial effusion with normal cardiac function. She was handcuffed and ankle-cuffed to her bed throughout her stay. We switched chlorpromazine to olanzapine (3.5 mg in divided doses), and added fluoxetine (10 mg daily) to address anxiety. She used Lorazepam for anxiety during feedings via NG tube initially. After gaining about 8 kg in about 6 weeks (see Fig. ), she was discharged back to the JJC, where she continued receiving eating disorder treatment. We suspected that some of the weight gain was related to rehydration. Her pericardial effusion resolved ultimately.\nWe observed a shift in her statements during weight restoration. For example, “I don’t see how a boy could like me now that I have gained weight” changed to “it does bother me to gain weight, but I try and focus on getting my period back, which makes it easier.” The JJC’s focus on positive reinforcement and reward systems seemingly motivated her to earn additional privileges for desired behaviors. She proudly shared when gaining access to the commissary at JJC, an upgrade in her shoes, a later bedtime, and her ability to spend time with peers in the gym under supervision to prevent over-exercise. JJC staff, many of whom chaperoned her during hospitalization, learned positive reinforcement and practical techniques taught to parents in family-based treatment (FBT) programs and implemented those strategies while she remained incarcerated. Her desire of “never to be hospitalized again” served as a motivation to completing her meals. She had regular follow up with our adolescent medicine team to monitor her progress. As she continued to gain weight, and her anxiety and aggressive impulses decreased. After one visit with our psychiatry team 4 weeks after her discharge, JJC’s psychiatrist took over her psychiatric care and titrated olanzapine to 7.5 mg twice daily and fluoxetine 20 mg daily. Her olanzapine was subsequently decreased to 5 mg twice daily for mild elevation in transaminases that later resolved. Six months after discharge from the hospital, she was still in custody at the JJC, and her nutritional status remained stable. Her menses returned. After complaining of weight gain as an adverse effect of psychotropics, she discontinued olanzapine and later on fluoxetine. However, she maintained her body weight after stopping the medications (see Fig. ). She has remained in outpatient treatment; and we anticipate that this will be needed long-term given the severity of her symptoms of CD and AN.","This 13-year-old girl has a complex history of mental health challenges, including anxiety, depression, and an eating disorder. She has struggled with her weight and body image for years, leading to restrictive eating and dangerous behaviors. Recently, she became increasingly aggressive and attempted to harm her father and pediatrician, resulting in her being placed in a juvenile detention center. During her hospital stays, she received treatment for her eating disorder and anxiety, and her doctors worked with her to develop strategies for managing her behavior and improving her overall well-being. While she continues to face significant challenges, she is now receiving ongoing support and is making progress toward long-term stability."
307,"A 41-year-old Ukrainian male [body mass index (BMI) 37.4] presented with complaints of numbness and cramping in the lower extremities, periodic numbness of fingers I–III of both hands, headache, and general weakness that gradually increased over 8 years. Over the past 2 years, the patient noted complete hair loss in the legs, thinning and increased vulnerability of the skin of the lower limbs, and the appearance of limited areas of severe hyperkeratosis on the feet. A callosity on the first toe of the left foot had led to the formation of a long-term, non-healing infected wound that was complicated by gangrene of the terminal phalanx and had led to its amputation.\nDuring examination, the skin of both feet was observed to be thinning with pigmentation, lamellar desquamation, and hyperkeratosis on the plantar surfaces (Fig. a–d). There were multiple epithelized and unhealed infected wounds on the feet that had developed as a result of microtrauma. The patient also suffered from class II alimentary–constitutional obesity [, ].\nA neurological examination revealed impaired exteroception with symmetrical hyperesthesia of the metacarpophalangeal joints, hypoesthesia, anesthesia, and thermal hypesthesia to the level of the middle one-third of the legs by polyneuritic type (Fig. e). Vibrational sensitivity was reduced to 5 seconds by polyneuritic type. Positional sense was reduced in the distal joints. Tendon reflexes of the lower extremities were weakened. Using the Medical Research Council (MRC) scale, muscle strength in the flexors of the lower legs was reduced by 4 grades, by 5 grades in the extensors, and 5 grades in the distal sections. Before the start of treatment in September 2017, hand dynamometry measured 38 kg and 36 kg on the left and right sides, respectively, which increased to 46 kg and 45 kg following treatment in February 2018. Moderate atrophy and muscle pain in the lower legs and short foot muscles were also found. Sensitive ataxia was noted as well. Autonomic trophic disturbance of the lower extremities was characterized by hyperkeratosis, anhidrosis, and livedo reticularis (Fig. a).\nDespite the absence of complaints from the patient and his relatives, a decrease in cognition was uncovered using the Montreal Cognitive Assessment (MoCa) scale with a score of 18 out of 30, the Mini-Mental State Exam (MMSE) with a score of 23 out of 30, and the Frontal Assessment Battery (FAB) with a score of 13 out of 18. In addition, tests evaluating spatial orientation (the clock drawing task and drawing complex or three-dimensional figures), attention, delayed recall, and phonetic speech activity were found to cause the most difficulties (Fig. g–k).\nTo identify the causes of polyneuritis syndrome, we excluded endocrine diseases [normal indicators of insulin, proinsulin, glycated HbA1c, adrenocorticotropic hormone, thyroid-stimulating hormone (TSH), triiodothyronine (T3), and thyroxine (T4)], infections (cytomegalovirus, Epstein–Barr virus, herpes, and borreliosis), and dysmetabolic origins (vitamin levels for B1, B6, B12, and homocysteine were normal; and amyloid deposition in the subcutaneous fat was absent). Electrophoresis of serum proteins allowed us to exclude the presence of paraprotein with a slight increase in the β1 fraction and hypergammaglobulinemia. Among the possible autoimmune causes, tests for antineutrophil cytoplasmic antibodies (ANCA), antinuclear factor, and extractable nuclear antigen antibodies were negative. An immunoblot of antibodies to gangliosides and onconeural antigens was negative as well. However, recombinant tissue transglutaminase 2 (TG2) IgA antibodies were found to be five times the normal level (90.3 IU/ml, which is normally less than 20 IU/ml). Anti-DGP IgG antibody values were normal (22.8 IU/ml).\nAmong all the classes of antibodies, only the IgE level was increased at 122 IU/ml.\nAs an additional source of confirmation for celiac disease, the HLA-DQ2 and HLA-DQ8 alleles were found to be present.\nNo pathologies or abnormalities were detected following an electrocardiogram, echocardiography, thyroid, abdominal and pelvis ultrasounds, head magnetic resonance imaging (MRI), and chest computed tomography (CT). However, during a nerve conduction study (NCS), signs of gross axonal damage to the motor and sensory fibers of the lower extremities was uncovered, with a complete block at the distal stimulation points of the left tibial and peroneal nerves and signs of secondary demyelination. In the upper limbs, there were signs of moderate axonopathy of the ulnar nerve (Table ).\nTo assess the involvement of the gastrointestinal pathological process, a fibroesophagogastroscopy was performed, during which an erythematous gastroduodenopathy was revealed. Biopsies of the gastric and duodenal mucosa were sent for morphological study (Fig. ).\nMorphological analysis of the duodenal mucosa biopsy (Table , Fig. ) identified changes (subatrophy of villi in combination with crypt hyperplasia) that correspond to celiac disease according to the Marsh IIIB classification.\nFigure shows duodenal mucosa relief, atrophy of the villi, and deepening of the crypts. In Fig. a, inflammatory infiltration, reactive changes in epithelial cells are presented. In Fig. b and c, pronounced diffuse lymphoplasmacytic infiltration, an increase of intraepithelial lymphocytes, and a decrease of goblet cells in the surface sections can be seen. Figure d and e shows pronounced diffuse lymphoplasmacytic infiltration, with a sharp increase in intraepithelial lymphocytes. In Fig. f, there are reactive changes in epithelial cells with nucleo- and nucleolomegaly, perinuclear vacuolization, nuclear hyperchromia, and increased mitotic activity. From Fig. a to f, the plates were stained with hematoxylin and eosin. Examination using direct immunofluorescence (DIF) reaction using antisera (Fig. g) uncovered C1q deposition (2+) in the papillary dermis, and linear IgA deposition (1+) along the basement membrane of the epidermis (Fig. h).\nExamination of the skin biopsy by DIF using antisera uncovered C1q deposits in the papillary layer of the dermis in the lower one-third of the shin, minor IgA deposits at the intercellular contacts of the epidermis, and linear deposits along the basement membrane of the epidermis. The pattern was similar to linear IgA dermatosis.\nMultidisciplinary therapy was initiated that included two cycles of five operations of medium-volume membrane plasma exchange, with an exfusion volume of 25–30% of the circulating plasma volume in combination with 1 g of intravenous pulse methylprednisolone. Metabolic therapy included the administration of α-lipoic acid (1200 mg/day); vitamins B6 (50 mg/day), B12 (1000 mg/day), and E (400 units/day); ipidacrine (60 mg/day); sulodexide (500 IU/day); and l-carnitine (3000 mg/day), as well as the initiation of gluten-free diet (GFD).\nEight months following the start of treatment, regression of the clinical symptoms of axonal polyneuropathy and cognitive deficiency was observed (Fig. f). During the next neurological examination, polyneuritic hypoesthesia was observed at the level of the lower third of the legs with full normalization of thermoception and vibrational sensation. In the upper limbs, hyperesthesia (to the level of the wrist joints) in the distal phalanges occurred, and signs of hyperpathy appeared. The trophic status of the cutaneous lower extremities returned to normal, and previously nonhealing wounds epithelized. Cognitive function was normalized (MMSE score of 28; FAB score of 18; and a MoCA score of 28). The level of recombinant TG2 IgA antibodies decreased to 13.0 IU/ml.","This man has been struggling with a variety of symptoms for eight years, including numbness and muscle cramps in his legs, tingling in his hands, headaches, and general weakness. He also developed hair loss on his legs and skin problems on his feet. Tests revealed he has a serious condition called celiac disease, which is causing inflammation in his digestive system. Further investigations showed signs of nerve damage and cognitive difficulties, and he had a severe infection in his foot that required amputation. After starting a special treatment plan including plasma exchange, vitamins, and a gluten-free diet, his symptoms have improved significantly, and his cognitive function has returned to normal."
308,"A 70-year-old male, diagnosed with CTEPH, was hospitalized for PEA. He had post-exercise exhaustion and shortness of breath for almost 2 years, and experienced a sudden dizziness with visual rotation and syncope. Despite the medical therapy, the clinical symptoms gradually worsened. Echocardiogram showed a dilated and poorly functioning right ventricle, as well as a small left heart with normal systolic and diastolic function (Fig. -A1, A2)(Tricuspid annular plane systolic excusion (TAPSE) 9.6 mm, left ventricular ejection fraction (LVEF) 64%, average E/E′11.94, lateral E′12.1 cm/s,tricuspid regurgitation (TR) velocity 2.5 m/s). Ventilation/perfusion scan and computed tomography angiogram confirmed the presence of CTEPH at the sub-segmental levels.\nAfter the patient was induced, the initial pulmonary arterial pressure (PAP) was 93/46(63) mmHg, and preoperative right radial artery blood pressure (ABP) was 120/88 mmHg. Near infrared spectroscopy (NIRS) monitoring presented the basic cerebral regional oxygen saturation (rSO2) range from 60 to 65%. After median sternotomy, the ascending aorta and both vena cava were cannulated regularly, and CPB was initiated. After the initiation of CPB, the mean PAP (mPAP) decreased to the range from17 to 31 mmHg. Aortic cross-clamping and blood cardioplegia were administered during pulmonary arteries dissociation. Then, the removal of thickened arterial intima and old organized thrombi was completed under deep hypothermic (22 °C) low flow (DHLF) and deep hypothermic circulatory arrest (DHCA). NIRS fluctuates from 55 to 60% during DHLF and DHCA. After the surgical procedures, full flow was restored, heart rebeated, and the patient was gradually rewarmed to normal. Norepinephrine (0.05μg/kg.min), epinephrine (0.02μg/kg.min), and isoproterenol (0.02μg/kg.min) were used to maintain stable hemodynamics. Transesophageal echocardiogram (TEE) showed good systolic and diastolic heart function.\nHowever, during the first attempt to discontinue CPB, the PAP increased rapidly to 63/35(43) mmHg, with unstable hemodynamic (ABP 70/40 mmHg), and TEE suggested right heart insufficiency, accompanied with normal left ventricular function. Then, CPB was resumed immediately. After assisting for another 45 min and increasing the dosage of vasoactive drugs (norepinephrine 0.06μg/kg.min, epinephrine 0.05μg/kg.min, isoproterenol 0.05μg/kg.min), the separation failed again, just the same as the first time. Considering that severe residual PH combined with right heart dysfunction are the main reasons for the failure of CPB weaning, ECMO was adopted. Consequently, 19F arterial cannula and 22F venous cannula were inserted into the right femoral vessels, and femoral VA-ECMO was initiated with a flow of 3.0 ~ 3.5 L/min. On arrival at the intensive care unit, ECMO flow rate was 3.5 L/min, and severe PH (PAP 56/25 mmHg) persisted with low ABP (70/55 mmHg). Echocardiography revealed significant decrease of biventricular function (Fig. B)(TAPES4mm, LVEF28%, average E/E′15, lateral E′6.7 cm/s, TR velocity 4.1 m/s) on postoperative day (POD) 1, indicating the patient developed severe secondary left ventricular dysfunction on the basis of right ventricular dysfunction, during ECMO support. The postoperative troponin-T(C-TNT) increased gradually (Fig. ), which suggested the myocardial injuries occurred. Hence, comprehensive measures were adapted: ECMO flow rate was reduced from 3.5 L/min to 2.5 L/min to decrease left ventricular afterload while maintaining systemic perfusion, norepinephrine (0.1μg/kg.min), epinephrine (0.06μg/kg.min) and isoproterenol (0.06μg/kg.min) were increased to ensure coronary perfusion, inhaled nitric oxide and treprostinil were administered to relieve PH. Besides, tidal volume was reduced to 6 ml/kg, and PEEP (5 cmH2O) was initiated to achieve lung protective ventilation. Oxygen flow rate was increased from 2 L/min to 6 L/min, with oxygen concentration raised to 47% for myocardial oxygen supply improvement. Moreover, atrial septostomy was carried out to decompress the right ventricle, so as to reduce the PAP. Consequently, mPAP decreased to 10 mmHg gradually, and the cardiac function improved slowly.\nOn POD 7, echocardiography revealed a normal sized heart with acceptable ventricular function (Fig. C). In this condition, ECMO was discontinued successfully, with satisfactory BP (90 ~ 120/50 ~ 80 mmHg), and normal PAP (mPAP 10-12 mmHg). The postoperative echocardiography data is shown in Table .\nThe postoperative course was complicated by unexpected brain ischemia and luminal infarction, which required tracheostomy on POD 13. Finally, the patient’s family decided to transfer to local hospital for treatment On POD 16.","This 70-year-old man was hospitalized because of shortness of breath and dizziness that had been getting worse for two years. Tests revealed he had CTEPH, a condition where the blood vessels in his lungs are narrowed, and his right heart was weak. During surgery to fix the problem, his blood pressure and heart function became unstable, requiring him to be placed on a heart-lung machine and then ECMO to help his heart and lungs function. After several adjustments and supportive care, including medication and ventilation, his condition gradually improved, and he was eventually able to breathe on his own."
309,"A three-month-old male infant presented with stridor and failure to thrive. He was delivered vaginally at full-term (birth weight 3.5 kg) and had an uncomplicated neonatal course. His parents reported that he had noisy and difficult breathing a few days after birth, which worsened over time and was associated with episodes of cyanosis and poor bottle feeding. A general pediatrician saw him at one month of age for stridor and poor weight gain, assumed a diagnosis of laryngomalacia, and advised the parents to increase the frequency of his feed. After that, his parents sought medical advice several times, including emergency department visits for significant respiratory distress, increasing stridor, and failure to thrive. He was then referred to our neurology clinic to assess hypotonia. No workup was performed, and he was not hospitalized before the referral. Physical examination revealed inspiratory stridor, suprasternal and subcostal retractions, tachypnea, and bilaterally reduced air entry. His oxygen saturation, which was 93% in room air, and improved slightly after oxygen supplementation. His weight at presentation was 4.2 kg, falling below the 3rd percentile of the WHO growth chart. No dysmorphic features were present.\nChest X-ray and regular laboratory test findings were normal, including serum electrolytes, complete blood counts, renal and liver functions, thyroid hormone levels, and blood gas analysis. The patient was taken to the operating room for an airway assessment based on the above findings. The patient underwent flexible laryngoscopy, which revealed a cystic mass measuring approximately 2 x 3 cm in size, arising from the lingual surface of the epiglottis and significantly occluding the laryngeal inlet (Figure ).\nDirect laryngoscopy and bronchoscopy under general anesthesia were promptly performed to evaluate the extent of the cystic lesion. The vocal cords, subglottic area, and trachea were observed to be normal. A thyroglossal cyst and retention cyst were initially considered as the differential diagnoses. However, based on clinical, endoscopic, and later histological findings, a vallecular cyst was established as the final diagnosis.\nMicrolaryngoscopic marsupialization was performed by the ENT surgeon to remove the cyst, which drained serous fluid. A biopsy of the cyst wall revealed the presence of connective tissue covered with non-keratinizing squamous epithelium with vascular congestion and chronic inflammation, suggestive of a vallecular cyst.\nPostoperatively, the patient was feeding well and showed a complete resolution of his symptoms. He remained asymptomatic at the follow-up visits at four and seven months. He quickly gained weight within a few months, reaching 5.5 kg at four months and 8.4 kg at seven months of age, indicating that the child’s weight had increased from below the 3rd percentile to the 50th percentile.","A three-month-old baby was brought to the hospital because he was having trouble breathing and wasn’t gaining weight properly. Doctors found a small cyst growing on his voice box that was blocking his airway and causing the noisy breathing and blueish skin. The cyst was removed with a special surgery, and after the procedure, the baby’s breathing improved, and he started to gain weight again. The baby is now doing well and is back to eating and growing normally, with no more breathing problems."
310,"A 56-year-old hypertensive gentleman presented with worsening right eye pain, redness, and lid swelling associated with double vision for six weeks (Figure ). He was initially treated as conjunctivitis at a primary care center with topical antibiotics, but then later referred to the ophthalmologist for persistent right lid eye swelling, redness, chemosis, and visual impairment. The patient recalled a past history of a motor vehicle accident in which he sustained a mild head injury with scalp laceration about 20 years ago. He had bilateral visual acuity of 6/9. His right eye was proptosed with the presence of grade 1 relative afferent pupillary defect (RAPD). It was swollen, tender with palpable thrill, and an audible bruit was present. Slit-lamp examination revealed generalized episcleral congestion with corkscrew vessels (Figure ) and raised intraocular pressure (IOP) of 28 mmHg. Both optic discs and posterior poles were normal. An urgent computed tomography angiography (CTA) demonstrated an engorged right superior ophthalmic vein (SOV) (Figure ) and bulky right CS. The left SOV was prominent with a similar contrast enhancement to the internal carotid artery. He then underwent a digital subtraction angiography (DSA) which confirmed the presence of the right direct CCF. After an urgent successful embolization, his ocular symptoms resolved fully with normalized IOP and optic nerve functions.","6 This 56-year-old man experienced worsening eye pain, redness, and blurry vision for six weeks. Initial treatment for conjunctivitis didn’t help, and doctors discovered a blockage in a vein in his right eye, causing swelling and pressure. He had a past head injury, which may have contributed to the problem. After an urgent procedure to block the blocked vein, his symptoms disappeared, and his vision returned to normal."
311,"A 65-year-old diabetic, hypertensive lady with hyperlipidemia presented with right eye blurring of vision associated with redness, lid swelling, and double vision for one month (Figure ). She was first treated for conjunctivitis by a private practitioner with topical antibiotics, who then referred to us for worsening ocular symptoms. Further history taking revealed a mild head trauma and facial injury in a motor vehicle accident about two months ago. A brain computed tomography (CT) scan was done and reported as normal. Her vision was 6/9 in both eyes, ocular examination showed the presence of grade one RAPD, proptosis, ophthalmoplegia, and audible bruit. Slit-lamp examination showed the presence of conjunctival chemosis and corkscrew vessels (Figure ) with IOP measured at 29 mmHg on the right eye. Both posterior segments of the eyes were normal. The patient underwent an urgent CTA, which showed enlarged and early filling of the right SOV (Figure ) with the early arterial enhancement of the right CS. The diagnosis of right direct CCF was confirmed with DSA and embolization was successfully performed to resolve the ocular sequelae.","A 65-year-old woman with diabetes, high blood pressure, and high cholesterol came to the hospital because she was experiencing blurry vision, eye redness, and double vision. She had been initially treated for pink eye, but her symptoms worsened, and doctors discovered a problem with a blood vessel in her eye. Imaging tests showed an enlarged blood vessel that was causing pressure and damage to her eye. Fortunately, a procedure to block the blood vessel was successful, and her vision has improved."
312,"A 49-year-old woman with underlying hypertension, was initially treated for right eye infective conjunctivitis. She was then referred for persistent and worsening eye redness for three weeks (Figure ). She denied any blurring of vision, lid swelling, or trauma. Her vision was 6/9 in both eyes with normal IOP. The dilated right episcleral vessels were blanched with topical phenylephrine 2.5%, hence she was treated for episcleritis with topical steroids. Her ocular condition improved after two weeks except for right eye IOP, which was raised to 24 mmHg. A topical IOP lowering agent was initiated for the working diagnosis of steroid responder. On subsequent review a week later, she was noted to have proptosis, orbital bruit, corkscrew vessels (Figure ) with controlled IOP and normal optic nerve functions. B-scan ultrasonography showed dilated SOV (Figure ) in the right eye. The patient underwent an urgent CTA that demonstrated the dilated right SOV (Figure ). Her DSA further confirmed the diagnosis of right indirect CCF. All the ocular features resolved following a successful embolization.","6/9 This 49-year-old woman came in for persistent redness in her right eye that lasted for three weeks. After examining her eye, doctors determined she had episcleritis, which was treated with steroid eye drops. However, her eye pressure remained high, and further tests revealed a blockage in the vein that drains fluid from her eye, leading to swelling and other symptoms. A procedure to block the vein successfully resolved the problem, and her eye pressure and symptoms improved."
313,"We present the case of a 74-year-old man who presented with a sudden onset of painless horizontal diplopia worse with a left-directed gaze. The double vision was aborted by occluding either eye. With eye-straining, he developed a dull bitemporal headache. He denied any vision loss, dysarthria, dysphagia, chewing difficulty, neck weakness, or breathing difficulty. A visit to the ophthalmologist confirmed a normal ocular funduscopic examination and left sixth nerve palsy. His condition remained static until he presented to the neurology clinic one month later.\nPast medical history was significant for bilateral below-knee amputations due to peripheral vascular disease secondary to smoking, which he quit many years ago. Otherwise, he was in relatively good health. He denied the use of any medications including antiplatelet therapy, cilostazol, statins, or anti-hypertensives. He also denied any constitutional symptoms such as fatigue, myalgias, muscle wasting, fevers, or joint pains.\nOn examination, the patient appeared alert, oriented, well-nourished, and in no apparent distress. Blood pressure was recorded at 134/80 mmHg, a pulse at 64 beats per minute, and respiratory rate at 12 per minute. The patient refused to be weighed. His speech was of normal tone, volume, and prosody without any hint of dysarthria or fatigability. Cranial nerve examination revealed no facial weakness with symmetric smile, intact whistling, and no difficulty blowing his cheeks. There was an obvious left lateral rectus paresis upon assuming left gaze (Figure ).\nVertical gaze was unaffected, and the pupils were spared. Masseter, genioglossus, and pterygoid function were preserved with intact jaw closure, deviation, and opening. The gag reflex was brisk. Neck flexion and extension showed adequate movement against resistance. Power in the arms was graded at 5/5 in all muscle groups with the medical research council (MRC) grading scale. Bilateral below-knee-amputation was noted, with preservation of bilateral hip flexion, adduction, and abduction. Deep tendon reflexes in the upper extremities were preserved with normal finger-to-nose coordination.\nA magnetic resonance imaging (MRI) of the brain with and without gadolinium enhancement revealed no lesion of the brainstem or cavernous sinus, and magnetic resonance angiography (MRA) revealed no cavernous sinus aneurysm. An MG panel for AChR modulating, binding, and blocking antibodies was negative. Striational antibodies were negative. A MuSK-antibody titer was high at 1.6 units per milliliter (mL); positive is 1.0 or higher. A sedimentation rate was normal. Based upon the negative MRI and MRA of the brain findings, the ocular manifestations, positive MuSK serology, a diagnosis of MuSK-ocular MG was made. A repetitive nerve stimulation (RNS) test and single-fiber electromyography (SFEMG) test were not scheduled. A trial of pyridostigmine at a dose of 60 milligrams (mg) three times daily was ineffective. The patient opted against therapy with prednisone and/or rituximab, and he preferred wearing an occlusive eye patch. The patient was advised about the potential for bulbar weakness and myasthenic crisis.","64-year-old man came to the clinic complaining of double vision that went away when he closed one eye. He also had a headache, and a doctor found that one of his eye muscles was weak. Tests showed he has a condition called MuSK-ocular myasthenia gravis, which causes muscle weakness. He prefers to wear an eye patch to manage his symptoms."
314,"Chief complaint\nOur patient is a 65-year-old male who reported severe and progressive abdominal pain of three days’ duration.\nHistory of present illness\nThe patient was admitted for stomach pain and lack of bowel movements for three days. The patient’s abdominal pain became progressively worse over the two days prior to admission. The patient reported that the pain is sharp, localized to the left side of his abdomen, and worse with movement. The patient also reports nausea but no vomiting. The patient denied any associated chest pain, shortness of breath, or fever/chills. Abdominal X-rays revealed small bowel dilation; CT scan conducted hours later revealed SBO. The patient also reported a long history of tenderness and a mass at the side of his past hernia repair.\nPast medical history\nThe patient’s past medical history is significant for an open ventral hernia repair with mesh approximately four years prior to this encounter (2017), as well as multiple instances of SBO since the most recent of which resolved non-operatively some 10 months prior (October 2020) to this encounter.\nExamination\nOn examination, the patient was found to have abdominal tenderness to palpation on the left side with rebound, severe tenderness at the umbilicus with a palpable mass, and the patient was unable to tolerate nasogastric tube (NGT) placement. The examination was otherwise unremarkable.\nInvestigations\nBoth abdominal X-rays and CT were obtained. The X-rays showed small bowel dilation and adynamic air-fluid levels, with suspicion of either ileus or partial SBO (Figure ). CT showed decompressed distal and terminal ileum consistent with SBO, as well as soft tissue thickening within the central abdomen deep to the umbilicus in a region of dilated and decompressed ileum, which could possibly be the cause of obstruction and perhaps due to adhesions or mass (Figure ). No recurrence of hernia was noted.\nPreoperative diagnosis\nBased on the patient’s history and associated investigations/imaging, the preoperative diagnosis was SBO.\nTreatment\nThe patient underwent exploratory laparotomy, release of SBO with removal of abdominal wall mesh and Jackson-Pratt (JP) drain placement. An incision was made directly over the site of the previous mesh that was around the site of the umbilicus. Dissection was taken down to the deep subcutaneous tissue. The peritoneum was then opened superior to the mesh. The mesh was then dissected out anteriorly and circumferentially, and then the incision was taken down inferiorly as well. There was small bowel tightly adherent to this mesh, the evident source of the obstruction, and this was freed from the mesh (Figure ). The mesh was then completely removed; the small bowel could now be clearly identified, and the site of obstruction was clearly released when it was freed from the mesh. The adhesions were carefully lysed. The small bowel was carefully examined to confirm that the site of obstruction was clearly released. The mesh did not erode into the bowel, and at this time a resection was not indicated. A JP drain was left in the abdomen and the incision was closed. A photograph of the mesh removed from the patient was taken after the conclusion of the procedure.\nPostoperative diagnosis\nThe postoperative diagnosis was SBO due to adhesions with abdominal wall and ventral hernia mesh.\nOutcome/progress\nAfter recovering from anesthesia, the patient was followed in an inpatient setting for four additional days, the course of which was uneventful. The patient was discharged home on the fourth postoperative day and followed in an outpatient setting.","A 65-year-old man was admitted to the hospital because of severe abdominal pain that had been getting worse over three days. Tests showed he had a blockage in his small intestine, likely caused by scar tissue from a previous hernia repair. Doctors performed surgery to remove the blockage and the damaged mesh, and the patient is now recovering well at home. He will continue to be monitored by his doctor in the future."
315,"This is an otherwise healthy 35-year-old male who was brought to our emergency department by ambulance after being crushed underneath a car; while performing some repairs on the undercarriage the jacks failed and the vehicle fell directly onto his head. Emergency personnel at the scene reported he was alert and coherent, but had obviously sustained significant facial trauma and he was intubated for airway protection. On arrival to our institution, head, spine, chest, abdomen, and pelvis computed tomography (CT) scans were obtained in accordance with standard trauma protocols. Multiple, comminuted facial and basilar skull fractures, with a large volume of pneumocephalus, though without any evidence of intracranial hemorrhage, were found (Figure ). Spinal column imaging revealed intradural air spanning C1-C4 and L3-S1 levels (Figure and 1C). There was no imaging evidence of direct spinal trauma, nor any evidence of pneumothorax or pneumoperitoneum.\nNeurologic examination revealed an intubated young male with a Glasgow Coma Scale (GCS) of 8T, given that he opened his eyes briefly when stimulated, and displayed purposeful and localizing movements in all extremities but did not follow commands. He had no apparent focal neurologic deficits. He had multiple facial ecchymoses and lacerations as well as dried blood at the nares and in both ears, though without obvious rhinorrhea or otorrhea. In accordance with institutional policy, head-injured patients with depressed GCS are admitted to the trauma intensive care unit (ICU) and head imaging is repeated after 6 hours. In this case, a second head CT was unchanged from the first. His GCS improved rapidly and he was extubated 9 hours after admission. Repeat neurologic examination revealed delayed development of a right facial nerve paresis for which he was prescribed a two-week course of dexamethasone without significant improvement noted by the time of discharge. He otherwise remained without strength or sensation deficits in his extremities. He was discharged home in good condition on post-injury day 3.","This 35-year-old man was seriously injured when a car fell on him during repairs. He was immediately given oxygen and intubated to help him breathe. CT scans showed he had broken bones in his face and skull, along with air in his brain, but no bleeding. He was closely monitored in the hospital, and his condition improved quickly, and he was eventually able to breathe on his own. He experienced some facial weakness, but otherwise recovered well and was discharged home after three days."
316,"This is an otherwise healthy 25-year-old male who was brought to our emergency department by ambulance after a fall of 8-10 meters; while leaning against the railing of a third-story balcony he lost balance and fell backward over the rail to the ground below. Emergency personnel at the scene found him comatose and pulseless. After several rounds of cardiopulmonary resuscitation (CPR) and rapid sequence intubation, he was stabilized for transport to our hospital. On arrival to the emergency department, he had developed refractory hypotension and lost a carotid pulse for which he again required a round of CPR before return of spontaneous circulation. Once resuscitated and stabilized, head, spine, chest, abdomen, and pelvis CT scans were obtained. He was found to have bilateral temporal bone fractures as well as a transverse clival fracture, as well as diffuse but predominantly right-sided traumatic subarachnoid hemorrhages with scattered locules of pneumocephalus (Figure ). Spinal imaging revealed intradural air at the L3-L4 levels (Figure ). There was no imaging evidence of direct spinal trauma, nor any evidence of pneumothorax or pneumoperitoneum.\nNeurological examination on arrival revealed an intubated young male with a GCS of 6T: he did not open his eyes nor display any localizing movements but was able to withdraw all of his extremities from painful stimuli. He had dried blood in both ears but was otherwise without otorrhea or rhinorrhea.\nHe was admitted to our trauma ICU and an intracranial pressure monitor was placed and monitoring was noted as normal. A follow-up head CT was unchanged. His GCS improved rapidly and he was extubated 11 hours after admission, and his intracranial monitor was removed. Repeat neurological examination noted that he was coherent and oriented, but amnestic to his injury. He had developed a left facial nerve paresis and was prescribed a two-week course of prednisone without significant improvement noted by the time of discharge. He remained without strength or sensation deficits of his extremities. Pelvic injuries necessitated operative fixation on hospital day 2. He was discharged to rehabilitation in good condition on post-injury day 8.","This 25-year-old man was brought to the hospital after a serious fall from a third-story balcony, resulting in a coma and requiring CPR to restart his heart. Doctors found significant injuries to his head and spine, including fractures and bleeding in his brain. After receiving treatment and monitoring, he was able to breathe on his own and was discharged to rehabilitation. He experienced some lingering facial nerve weakness and will continue to work with a therapist to regain his strength and function."
317,"A 54-year-old male with a history of alcohol abuse presented to the emergency with acute-onset, diffuse, cramping abdominal pain, worst in the epigastrium and lasting one day. He denied fever, vomiting, loss of appetite or weight loss, diarrhea, constipation, or rectal bleeding, or any prior similar episodes. He had no prior abdominal surgeries. On examination, he was in distress and had moderate guarding and generalized tenderness with hypoactive bowel sounds. He was afebrile. Laboratory evaluation showed microcytic anemia and thrombocytopenia on complete blood count, hypokalemia on basic metabolic profile, and a normal lipase level (Table ).\nAbdominal X-ray showed an evolving small bowel obstruction (Figure ), which was confirmed by a CT scan of the abdomen and pelvis with contrast (Figure ). On the same CT scan series, a urachal remnant with a superimposed mass lesion was depicted (Figures , ).\nA decision was made to go forward with an emergent surgery to release the small bowel obstruction. After receiving a platelet transfusion, the patient underwent an exploratory laparotomy. He was found to have a high-grade small bowel obstruction due to an extensive mass concerning for a malignancy. A frozen section was intraoperatively sent and read as adenocarcinoma (Figure ).\nAn incisional biopsy of the urachal mass was also sent. The final pathology specimen was reported as a moderately differentiated urachal adenocarcinoma (Figure ) with positivity for CK20, CDX2, and CK7 (focal) stains (Figure ).\nThe patient was staged at Stage IIID (Sheldon staging) due to the spread to local viscera other than the bladder. The tumor was deemed unresectable due to the involvement of multiple loops of the small bowel and the mesentery of the small and large bowels. Systemic chemotherapy with 5-fluorouracil (5-FU), folinic acid, and oxaliplatin (modified FOLFOX-6) was initiated. The patient has received six cycles of chemotherapy so far. He has tolerated chemotherapy well enough and is still awaiting additional imaging to evaluate the response to therapy.","This 54-year-old man came to the hospital with severe abdominal pain and a blockage in his small intestine. Tests revealed a tumor in his urachus (a remnant of the umbilical cord) that was causing the blockage and also led to anemia and low platelet counts. Surgery was performed to release the blockage, and the tumor was confirmed to be adenocarcinoma. He is now receiving chemotherapy to treat the cancer, and doctors are monitoring his response to the treatment."
318,"The patient is an 81-year-old male with medical comorbidities of hypertension, coronary artery disease, and non-melanoma skin cancer who presented with a new cutaneous manifestation. He reported a two-week history of a progressively enlarging asymptomatic lesion on his left chest. Five months prior he had a CABG procedure with internal thoracic artery harvesting without the use of fluoroscopy. Physical examination revealed a well-demarcated erythematous, indurated plaque overlying the previous harvest site of his left internal thoracic artery (Figure ).\nA punch biopsy was performed to further classify this new development; histopathologic evaluation revealed an uninvolved epidermis with diffuse dermal sclerosis and underlying panniculitis (Figure , Figure , Figure ).\nIn the absence of any prior history of scleroderma (cutaneous or systemic), these localized sclerodermatous changes were attributed to recent arterectomy. Given the asymptomatic nature of the plaque, the management options were discussed with the patient, and he opted for observation during his bi-annual skin examinations.","An 81-year-old man came to the doctor because he noticed a growing, red bump on his chest that wasn't painful. Tests showed that the bump is related to a previous surgery where a blood vessel was removed from his chest five months ago. The doctors believe this is a type of localized skin reaction and that it is not a serious condition. Because it’s not causing any problems, the doctor recommends simply watching it closely during his regular checkups."
319,"The patient is a 28-year-old male with prior history of a grade 3 astrocytoma, who presented to our emergency department (ED) after having a general tonic-clonic seizure (GTC). Initially, his disease was appreciated four years prior when he presented with a GTC and was found to have a grade 3 astrocytoma of the left occipital lobe (Figure ). He underwent maximal safe resection followed by adjuvant EBRT to 45 Gy in 25 fractions at an outside institution. Due to reasons outside the patient’s control, he received only two cycles of adjuvant temozolomide following EBRT. He developed disease recurrence, presenting as a GTC six months prior to this ED presentation, where imaging demonstrated tumor progression with increased extension into the parietal lobe (Figure ). He subsequently underwent a second maximal safe resection later that month. Final pathology returned as GB. Follow-up imaging four months afterward demonstrated disease progression and he was started on bevacizumab as well as a tumor treatment field (TTF) device. He unfortunately only tolerated TTFs for one month, ending one month prior to the ED presentation.\nUpon admission to our hospital from the ED, MRI demonstrated progression of disease in the left occipital-parietal lobes with extension into the splenium and anterior-inferior extension into the left thalamus and basal ganglia (Figure ). His physical exam was notable for mild right-hand weakness, but he was otherwise neurologically intact. Despite changes to his antiepileptic medication, he had seizure recurrence a few weeks following admission. Presuming that the area of tumor recurrence received a definitive dose in the past, the consensus decision was to proceed with repeat maximal safe resection with GammaTile placement. A dose of 60 Gy was prescribed to a 5 mm depth using a total of eight tiles, each containing four Cesium-131 3.5U seeds, to line the post-operative cavity volume of 17.6 cc. A significant portion of the occipital-parietal disease was debulked, with final pathology again demonstrating a grade 4 astrocytoma with molecular studies indicating an IDH-mutated, ATRX mutated, and MGMT promoter methylated phenotype with hypermutation. Postoperatively he was noted to have right upper and lower extremity weakness/spasticity with right foot drop and mild right face weakness. He required a cane to assist with ambulation and reported word-finding difficulties and decreased short-term memory. He was planning to start adjuvant temozolomide; however, he developed a severe GTC with increased muscle weakness and altered mental status two months later. MRI following this episode demonstrated mildly increased enhancement to the tissue surrounding the surgical bed with the progression of disease in the splenium and left thalamus/basal ganglia (Figure ).\nGiven this symptomatic disease progression, a treatment plan was made to take the progressive regions of disease outside the irradiated GammaTile volume to 35 Gy in 10 fractions. As shown in Figure , an initial planning target volume (PTV) was delineated consisting of the T1 post-contrast-enhancing disease with a 5 mm margin. The volume that received greater than 35 Gy from the GammaTile treatment was excluded from the PTV. The PTV was then separated into two portions by subdividing the remaining volume between tissue that received less than 17.5 Gy (PTV1), and that which received 17.5-35 Gy (PTV2). Using a volumetric modulated arc therapy plan with five arcs, one non-co-planar, a homogeneous dose of 35 Gy in 10 fractions was delivered to PTV1. This dose was calculated to have a biologically equivalent dose in 2 Gy fractions (EQD2) of 45 Gy. Dose painting gradually decreased the dose from 35 Gy to as low a dose as achievable approaching the resection bed border of PTV2 (Figure ). A composite of the GammaTile dose volume (Figure ) with a dose delivered from PTV1 and PTV2 resulted in a homogeneous dose of approximately 50 Gy EQD2 to the residual disease extending into the splenium and the left thalamus/basal ganglia (Figure ). Digital imaging and communications in medicine (DICOM)-RT data from the initial 45 Gy delivered from the outside institution were obtained, and cumulative dose to critical organs at risk (OARs), including the brainstem, ocular structures, and cochlea, were within established constraints.\nDue to the hypermutation phenotype of his tumor, the patient was started on CCNU (100 mg/m2) and completed his EBRT with no issue or progression of his current neurological symptoms. He was last seen in follow-up by our team three months after his most recent EBRT, at which point he reported significant improvement in the right leg tremors/spasms and improved headache. He demonstrated decreased right foot drop but continued to have word-finding difficulties and short-term memory deficits. He has not developed any new areas of muscle weakness or paresthesia, visual loss, or other forms of altered sensoria. The current plan is to complete six cycles of CCNU with alternative systemic therapy thereafter upon disease progression. While temozolomide could be used to this effect, the high mutational burden found in his most recently resected disease would permit entry into currently available trials of immunotherapy.","This patient has a complex history of brain cancer that has come back multiple times. He initially had a tumor removed, but it later grew back, and he’s received several treatments including surgery, radiation, and chemotherapy. Recently, his cancer has continued to spread, causing weakness in his legs and difficulty with memory and speech. To combat this, doctors are planning to deliver a higher dose of radiation to the remaining tumor, carefully targeting the area while protecting nearby healthy tissues. He is also being considered for a clinical trial of immunotherapy to help fight the cancer."
320,"A 60-year-old female was referred from the emergency department with a foreign body sensation in the throat, dysphagia, and odynophagia. The symptoms developed while the patient was having her lunch. She had no other related gastrointestinal complaints (i.e., hematemesis, drooling, or vomiting) or airway-related complaints (i.e., choking, cyanosis, cough, dyspnea, or hemoptysis). The patient’s medical and surgical histories were only significant for bronchial asthma, dyslipidemia, fibromyalgia, and hypertension. She was hemodynamically stable and saturating well on room air with no signs of respiratory distress. Physical examination was unremarkable except for mild tenderness over the anterior neck above the level of the thyroid cartilage. A lateral neck soft tissue X-ray confirmed the presence of a foreign body in the hypopharynx with a linear radiolucency in the retropharyngeal space representing free air (Figure ). A neck computed tomography (CT) scan demonstrated a linear hyperdense foreign body in the hypopharynx with free air along the retropharyngeal space representing a concealed perforation (Figure ).\nThe patient was admitted for endoscopic examination under general anesthesia and foreign body removal. The patient was intubated using a flexible fiberoptic bronchoscope to avoid dislodging the foreign body. During fiberoptic intubation, a whitish plastic foreign body was found in the hypopharynx, which was removed using foreign body forceps under endoscopic guidance. Examination post foreign body removal showed a small wound in the posterior pharyngeal wall, which represents the site of the perforation (Figure ). The examination was completed using rigid esophagoscopy, which showed a normal intact esophagus. Following the procedure, the patient was treated conservatively with close observation, strict nasogastric tube (NGT) feeding, analgesics, and intravenous piperacillin/tazobactam 4.5g every 8 hours for seven days. An upper gastrointestinal series using Gastrografin (Bayer, Leverkusen, Germany) was performed after a week of the incident and showed a normal contrast passage through the hypopharynx and esophagus without any evidence of leakage. Flexible nasopharyngeal endoscopy was repeated and demonstrated intact nasopharynx, oropharynx, with a small and almost healed posterior pharyngeal wall perforation. The patient was allowed to resume oral feeding and was discharged home in a stable condition.","A 60-year-old woman came to the hospital because she felt something was stuck in her throat and had difficulty swallowing. X-rays and a CT scan showed a piece of plastic in her throat that had caused a small hole in the back of her throat. Doctors carefully removed the plastic using a scope and treated the hole with antibiotics and a feeding tube. After a week of monitoring, the patient was able to start eating again and was discharged home."
321,"A 67-year-old male with a past medical history of coronary artery disease (CAD) with two stents in the first diagonal branch of the left anterior descending artery six years before admission, hypertension, hyperlipidemia, glaucoma, and previous anaphylaxis to nuts presented to the emergency department (ED) with anaphylaxis after eating a nut-containing bar. Shortly after ingestion, he called emergency medical services (EMS) for hives and difficulty breathing. He was tachypneic, diaphoretic, and nauseous on arrival to the ED, tripoding with a hot-potato voice, and had oropharyngeal angioedema. Vital signs showed a temperature of 98.5 (F), heart rate of 73 beats per minute, blood pressure of 140/89 mmHg, respiratory rate of 19 cycles per minute, and oxygen saturation of 95% while on 6 liters of oxygen via nasal cannula.\nAfter receiving epinephrine x3, methylprednisolone 125mg x2, and diphenhydramine the patient was still symptomatic. Epinephrine infusion was commenced at 1 microgram/minute for refractory anaphylaxis. ENT observed uvula edema without laryngeal edema on two separate fiberoptic exams. Given voice changes in the ED, the patient was admitted to the ventilator unit for monitoring of biphasic anaphylactic reaction. He was titrated off the epinephrine drip for lack of hemodynamic instability congruent with anaphylaxis.\nInitial laboratory investigations showed a troponin of 0.03ng/ml at 4.5 hours post-ingestion which increased to 2.98 and 3.08 at 18 and 23 hours post-ingestion, respectively. The patient initially denied chest pain; however, when informed of his cardiac enzyme elevation, he retrospectively endorsed intermittent, non-radiating, mid-sternal chest pressure that was 5/10 in intensity after receiving epinephrine, which decreased to 2/10 in intensity within 24 hours. Electrocardiogram (EKG) at 22 hours post-ingestion showed sinus rhythm, left anterior fascicular block, and no ST-segment changes (Figure ).\nHe was started on aspirin, heparin infusion, and atorvastatin.\nAn echocardiogram showed a normal left ventricular (LV) systolic function with an ejection fraction of 56-60%. Additionally, LV segmental wall motion abnormalities, hypokinesis in the mid inferolateral segment were also found.\nChest X-ray did not show any abnormalities.\nElective coronary angiogram (Figure ) was significant for single-vessel disease. The left main, left circumflex, and right coronary arteries were normal. Of note, a patent stent in the inferior branch of the diagonal artery with 30% in-stent restenosis and a jailed superior branch of the diagonal with 95% ostial lesion was also revealed.\nSerial troponin monitoring showed a downward trend of 2.78 and 2.0ng/ml, at 30 and 39 hours post-ingestion, respectively. The patient's chest pain resolved, and he was discharged to follow up with the outpatient cardiology clinic.","67-year-old man experienced a severe allergic reaction (anaphylaxis) after eating a nut-containing bar. He had difficulty breathing and other symptoms, and was treated with medications and oxygen. Tests revealed elevated levels of enzymes indicating possible heart damage, and an angiogram showed a narrowed artery that had been previously stented. He was treated with medications to prevent further heart problems and was discharged to follow up with a cardiologist."
322,"A 74-year-old male with a history of two transient ischemic attacks was brought into our facility for altered mental status after being found unconscious by a relative. His social history was significant for 55 pack-years of smoking. Due to his mentation on arrival, no further history was obtained. Vital signs were normal on admission. Physical examination on admission showed non-responsiveness to sternal rub, dry mucous membranes, decreased skin turgor, and thick white plaques around the corners of his mouth. Examination of the extremities showed 1+ bilateral lower leg edema, and pulmonary examination revealed right-sided wheezes and rales.\nOn admission, the patient had an elevated blood glucose of 662 mg/dL (normal: 70-110 mg/dL), an anion gap of 15, ß-hydroxybutyrate of 1.86 mmol/L (normal: 0.02-0.27 mmol/L), point-of-care (POC) lactate of 3.3 mmol/L (normal: 0.5-2.0 mmol/L), serum potassium of 3.8 mEq/L, urine volume of 850 mL, and no ketones on urinalysis. The arterial blood gas (ABG) showed a pH of 7.48, HCO3 of 24.6, and PCO2 of 33 meq/L. The slightly elevated anion gap on admission is likely multifactorial. It could have been caused by the increase in the negative charge for albumin and enhanced production of lactate seen in metabolic alkalosis []. Conversely, it is also possible that scant ketones, which are produced in a hyperosmolar hyperglycemic state, as seen by mildly elevated β-hydroxybutyrate and the absence of ketones in the urine, could have contributed to the elevated anion gap [].\nAdditionally, the patient had hypernatremia (sodium of 185 mEq/L corrected for hyperglycemia), acute kidney injury (blood urea nitrogen [BUN] of 49 mg/dL, creatinine [Cr] of 1.55 mg/dL, from a previous baseline Cr of 1.01 mg/dL), and hemoconcentration (hemoglobin [Hb] 17.1 g/dL, hematocrit [Hct] 51.6%). Chest X-ray indicated a potential right lower lung zone infiltrate. Computed tomography (CT) of the chest indicated right lower lobe mass or consolidation, with multiple mediastinal and hilar masses as well as enlarged left axillary nodes compatible with lymphadenopathy, suggestive of malignancy and metastatic disease (Figures , ). CT of the abdomen showed heterogeneous liver attenuation but could not differentiate between nonocclusive disease and metastatic disease. Adrenal nodules and kidney nodular densities were also present (Figure ). CT of the brain showed a suprasellar mass measuring 1.2 x 1.1 x 0.9 cm (Figure ). The patient was started on half normal saline and insulin, and the acute kidney injury significantly improved on day 3; however, no significant improvement in sodium level was noticed after correction for elevated glucose levels (Figure and Table ).\nAdditional investigations for the refractory hypernatremia showed a urine osmolarity of 699 (normal: 50-1,400), serum osmolarity of 360 (normal: 280-301), urine sodium of 10 mEq/L (normal: 20 mEq/L), serum AM cortisol of 61.3 ug/dL (normal AM: 6.2-19.4 ug/dL), 1-mg dexamethasone suppression test cortisol of 6.06 ug/dL (normal: <1.8ug/dL) and ACTH of 228 pg/mL (normal: 7.2-63.3 pg/mL) (Table ). MRI of the brain revealed a 1.1 x 1.2 x 1.1 cm enhancing lesion of the optic chiasm without evidence of mass effect (Figure ) and small lymph nodes in deep portions of the bilateral parotid glands that were potential metastasis. Throughout the admission, the patient’s arterial blood gas (ABG) showed a primary respiratory alkalosis with secondary metabolic alkalosis (pH of 7.48, HCO3 of 24.6, PCO2 of 33 meq/L), which was persistent until discharge (pH of 7.48, HCO3 of 27.6 meq/L, PCO2 of 37 mmHg). Additional endocrine investigations demonstrated an follicle-stimulating hormone (FSH) of 0.3 miU/L (normal: 1.5-12.4 miU/L), luteinizing hormone (LH) < 0.3 miU/L (normal: 1.7-8.6 miU/L), testosterone of 27 ng/dL (normal: 264-916 ng/dL), thyroid-stimulating hormone (TSH) of 0.01 u/iU/L (normal: 0.34-5.60 u/iU/L), and prolactin of 9.7 ng/mL (normal: 4.0-15.2 ng/mL). The plan was to obtain a lung biopsy to determine whether the mass was cancerous or not and to conduct a high dexamethasone suppression test for ectopic ACTH production. However, the patient declined further workup or treatment, opting for hospice instead.\nDespite initial fluid resuscitation with half normal saline followed by dextrose water, the hypernatremia did not correct. The hypernatremia later resolved after the patient was started on desmopressin on day 7 of admission. The patient was sent home on hospice and was advised to increase his water intake.","This 74-year-old man was found unconscious and admitted to the hospital with several concerning symptoms, including confusion and difficulty breathing. Tests revealed very high blood sugar, electrolyte imbalances, and a possible lung infection. Further scans showed a mass in his chest, which may be cancer, and a growth in his brain. Despite treatment, his condition remained unstable, and he ultimately chose to focus on comfort care and was sent home with hospice support."
323,"A 45-year-old transgender male with a history of Guillain Barre Syndrome and heterozygous Factor V Leiden underwent gender reassignment surgery, including phalloplasty, mastectomy, and vaginal eversion. The phalloplasty was complicated by postoperative penile artery thrombosis, recurrent episodes of extended-spectrum beta-lactamase (ESBL) Klebsiella urinary tract infection (UTI) from a chronic suprapubic catheterization, and colovesical fistula.\nThe patient presented with lethargy, shortness of breath, 15 lb weight gain, and lower extremity edema for the past three months. Physical exam revealed tachycardia, holosystolic murmur at the apex, faint bibasilar crackles, and right lower extremity edema. Laboratory work showed leukocytosis (WBC 11.6 K/uL), creatinine 0.7 mg/dL, hemoglobin 8.8 g/dL, B-type natriuretic peptide of 610 pg/mL, and D-dimer of 3469 ng/mL. CT chest revealed cardiomegaly, pulmonary edema, bilateral lower lobe consolidations, and pleural effusions, a large pericardial effusion, and a 5x10 cm perisplenic abscess found to be secondary to septic emboli. Echocardiogram showed a 2.1 cm mobile vegetation on the anterior leaflet of the mitral valve (Figure ), moderate-severe mitral valve insufficiency (Figure ), ejection fraction of 65%, markedly dilated left atrium, elevated peak pulmonary artery pressure at 57 mmHg, and large pericardial effusion without evidence of tamponade. Broad-spectrum IV antibiotics were started for infective endocarditis and diuresis for new-onset heart failure. Splenic fluid and blood cultures grew Enterococcus faecalis. He underwent subsequent cardiothoracic surgery with an On-X mechanical mitral valve (On-X Life Technologies Inc., Austin, TX). Unfortunately, the postoperative course was complicated by mediastinal thrombus formation and hemothorax requiring surgical exploration. Once stabilized, the patient was discharged and upon discharge was placed in IV penicillin G and ceftriaxone to complete six weeks of antibiotic therapy.","This patient is a 45-year-old transgender man who has had a complex medical history, including a previous neurological condition and gender-affirming surgeries. He recently developed symptoms of fatigue, shortness of breath, and swelling, which led to further tests revealing a serious heart infection and fluid buildup in his lungs. Imaging showed a large abscess in his abdomen and a problem with his mitral valve, requiring surgery to replace it. After surgery, he developed complications, but is now being treated with antibiotics and is being discharged home."
324,"Chief complaint\nA 50-year-old male presented to the clinic with ongoing RLQ abdominal pain of one-month duration, associated with mild fever.\nHistory of present illness\nThe patient was referred to the surgery clinic by his primary care physician (PCP) after experiencing ongoing RLQ pain and showing evidence of lymph node enlargement on CT imaging. At presentation, the patient reported a history of mild fever associated with RLQ pain. Two months prior to our encounter, the patient was started on an antibiotic regimen of levofloxacin with mild improvement in RLQ pain. Some mild pain symptoms continued. The patient also experienced pain while urinating and testicular pain approximately one month prior to the encounter. The patient denied any associated chest pain, shortness of breath, nausea/vomiting, or chills. The patient's heart rate and blood pressure were noted to be within normal limits.\nPast medical history\nThe patient’s past medical history includes hypertension being treated with lisinopril, and diabetes mellitus being managed with metformin. Patient encounter records confirm that at some time between two and three months prior to presentation, the patient began taking levofloxacin daily. An occurence of painful rectal bleeding in 2015 prompted a colonoscopy, which revealed a colonic polyp and grade two hemorrhoids. The patient also suffered a left hand crush injury of the third and fourth digits in 2015.\nExamination\nAt the time of the encounter, physical examination revealed RLQ tenderness, but was otherwise unremarkable. The patient’s heart rate, blood pressure, and other vital signs were within normal limits, and the patient was afebrile at the time of examination.\nInvestigations\nThe patient’s PCP ordered CT imaging, which showed an appendiceal diameter on axial image of 8mm, and a cluster of prominent lymph nodes adjacent to the appendix, the largest of which was also 8mm (Figures , ). Radiologic interpretation suggested these findings could be indicative of acute or chronic inflammation that may be an unusual presentation of chronic appendicitis. A dilated right extrarenal pelvis and proximal ureter with minimal right calyceal dilation were also seen (Figures , ). In addition, a small non-obstructing stone in the right renal pelvis was seen, suggesting a chronic ureteropelvic junction stricture. Preoperative laboratory results showed a white blood cell count of 10.4x10^3 cells/µL.\nPre-operative diagnosis\nBased on the patient’s history of present illness and associated investigations the preoperative diagnosis was chronic appendicitis.\nTreatment\nLaparoscopic appendectomy was performed on the patient. The appendix was identified and found to be chronically scarred and chronically inflamed in appearance. The mesoappendix was taken down with Sonicision, the base transected with Endo-GIA, and delivered from the peritoneal cavity in an Endobag and sent to pathology.\nPost-operative diagnosis\nThe post-operative diagnosis was chronic appendicitis.\nOutcome/progress\nAfter recovering from anesthesia, the patient was discharged home on the same day as his procedure with no complaints and is currently being followed in an outpatient setting. The patient continues to report complete resolution of his symptoms. Postoperative histopathology report on the specimen taken from the patient (Figure ) showed chronic lymphoplasmacytic inflammation with associated histiocytes and granulation tissue, further supporting the diagnosis of chronic appendicitis.","A 50-year-old man came to the clinic with ongoing pain in his lower right abdomen and a mild fever, which he’d been experiencing for about a month. Initial tests showed enlarged lymph nodes near his appendix, and a CT scan revealed a small stone in his kidney and a slightly enlarged pelvis. Doctors believe he has chronic appendicitis, a long-term inflammation of the appendix, and performed a laparoscopic surgery to remove it. The surgery was successful, and he is now recovering well at home with no pain and a full return of his symptoms."
325,"A 38-year-old woman presented with a 16-year history of ulcerative colitis being treated with mesalazine and infliximab. She had been undergoing routine colonoscopy examinations. Multiple random biopsies performed from the transverse colon to the rectum during colonoscopy suggested rectal cancer, for which she was referred to our hospital for surgery. A follow-up colonoscopy revealed the absence of the haustra between the transverse colon and rectum, without any obvious tumors. Histopathological examination of multiple random biopsy specimens obtained from the transverse colon to the rectum showed dysplasia with p53 overexpression in the rectum, which suggested cancer.\nWe performed laparoscopic proctocolectomy and D2 lymphadenectomy concomitantly with TaTME. The procedure involved two surgical teams. We inserted five abdominal ports, and the colon was mobilized from the ileocecal region to the rectum along with laparoscopy-guided dissection of blood vessels. The rectum was mobilized in the TME plane, and the left and right neurovascular bundles were incised. Transanal surgery was performed simultaneously using the laparoscopic procedure. We used the Lone Star Retractor System (Cooper Surgical, Trumbull, CT, USA), GelPOINT path transanal access platform (Applied Medical, Rancho Santa Margarita, CA, USA), and AirSeal system (ConMed, Utica, NY, USA) to ensure active smoke evacuation to aid in the visualization of the operative field. Circumferential mucosectomy was performed with preservation of the anal sphincter muscle. We used a purse-string suture and closed the rectal lumen to prevent mucus leakage and cancer cell dissemination. Mucosectomy was performed starting from the dentate line and extending into the anal canal. The circular and longitudinal muscles were incised, and the abdominal cavity was opened (Figure ). The specimen was extracted after a slight extension of the umbilical port site. We created an ileal pouch (J-pouch) and performed an ileal pouch-anal anastomosis. Finally, we performed a diverting-loop ileostomy. The operation time was 286 minutes, and the estimated blood loss was 52 mL.\nHistopathological examination of the resected specimen revealed low-grade rectal dysplasia without any evidence of malignancy. It took some time to adjust to the ostomy pouch, and the patient was discharged 21 days postoperatively without any complications. The patient underwent loop ileostomy closure four months postoperatively and recovered without significant loss of the anal sphincter function. The anal sphincter function remained at four months after the second surgery. At four months after the second surgery, the patient is doing well.","This 38-year-old woman has had ulcerative colitis for 16 years and has been regularly monitored with colonoscopies. Recent tests showed signs of possible rectal cancer, leading to a more detailed examination and surgery. During surgery, doctors removed the rectum and a small amount of surrounding tissue, creating an ileal pouch and connecting it to the anus. The patient is now recovering well with a diverting loop ileostomy and is expected to regain normal anal function over time."
326,"A 77-year-old male, with a history of chronic obstructive pulmonary disease (COPD), diagnosed with left shoulder melanoma in 2015, treated by wide excision along with radiation therapy, had a relapse locally at the original site with lung metastasis (stage IIA; pT4, N0, M1b) and was BRAF negative in 2017. He was started on pembrolizumab in August 2017 with a cycle of every three weeks for treatment. Two years later, in 2019, he presented with generalized weakness, productive cough that was clear, acute on chronic worsening dyspnea, and diarrhea for the past week, with his last dose of pembrolizumab three weeks back. He denied any fever, orthopnea, or lower extremity swelling. He admitted to paroxysmal nocturnal dyspnea. He uses 3 L of home oxygen presently requiring high-flow oxygen. On exam, he had audible crackles on the right side along with diminished lung sounds throughout the right lung. His chest X-ray (CXR) showed right middle and lower lobe infiltrates along with interstitial changes. The white blood cell count was normal. His chest computerized tomography (CT), as seen in Figure , showed severe bullous changes in bilateral lungs, a small right pleural effusion, a spiculated nodule in the left lower lobe measuring 1.1 cm by 2.2 cm, thickening of the interlobular area, and interval interstitial infiltrate in the posterior segment of the right upper lobe, right middle lobe, and right lower lobe. At this time, his pembrolizumab was discontinued, and he was started on broad-spectrum antibiotics including atypical coverage. Methylprednisolone was also started. He underwent bronchoscopy with bronchoalveolar lavage to rule out infection, which showed mucosa inflammation of the right lower lobe, right middle lobe, and the anterior segment of the right upper lobe with dark yellow mucus plug seen. Bronchoalveolar lavage (BAL) cultures, viral panel, and Pneumocystis carinii smear were negative. He was discharged home in October 2019 on prednisone 40 mg b.i.d. with a plan to taper down over the next six weeks in the outpatient setting. He developed two consecutive episodes of secondary spontaneous pneumothorax in which he required chest tube placement and missed at least one week of his steroids, which lead to a flare-up of his pneumonitis and getting readmitted to the hospital in November 2019. He was restarted on steroids and followed up with an outpatient pulmonologist where his steroids were weaned in the next eight weeks. Repeat chest CT, as seen in Figure , done three months later showed near resolution of the interstitial infiltrate in the right upper, middle, and lower lobes and unchanged left lower lobe spiculated nodule. In June 2021, he was tapered down to prednisone 2.5 mg daily. His respiratory status was stable, with chest CT showing resolution of pneumonitis. In September 2021, he expired in his sleep.","67-year-old man with COPD and a history of melanoma was admitted to the hospital because of worsening shortness of breath, cough, and diarrhea. Tests showed he had fluid in his lungs and an infection, likely related to his COPD. He was treated with antibiotics and steroids, and his lung condition improved. Sadly, he passed away in September "
327,"A 64-year-old female undergoing chemotherapy and radiation for malignant mucosal lentiginous melanoma of the left gingiva (stage III; pT3, N1, M0) presented in February 2020 with fatigue and worsening shortness of breath for the last two months. She had a left modified radical neck dissection with resection of the left oral cavity. She then underwent 30 treatments of external beam radiation. After radiation was completed, she was started on nivolumab 240 mg IV every two weeks in August 2019. In January 2020, her nivolumab was held due to worsening transaminitis and thyroiditis, which presented as hypothyroidism and then hyperthyroidism after starting levothyroxine. CT abdomen was done at that time, which showed increased heterogeneous attenuation of the liver. She was started on prednisone 40 mg daily. With her history of being a current smoker of 1 ppd for the last 30 years, she admitted to a productive cough that was clear in nature, chest tightness, and orthopnea but denied paroxysmal dyspnea and fever. Chest CT without contrast, done in February 2020, as seen in Figure , showed multiple pulmonary nodules with some interstitial/ground-glass changes in the upper lobes bilaterally, right middle lobe, and right lower lobe, which were worse than her previous chest CT two weeks prior, as seen in Figure . She was diagnosed with nivolumab-induced pneumonitis. Her lab work showed a very low thyroid-stimulating hormone (TSH) level of < 0.07 along with her free triiodothyronine (T3) being normal, free thyroxine (T4) of 4.6, and negative thyroid peroxidase antibody (TPO) antibody. Levothyroxine was then stopped. She still had transaminitis. She was started on ceftriaxone and azithromycin for possible pneumonia. A higher intravenous dose of steroids was initiated. She underwent bronchoscopy with BAL with findings of erythematous bronchial mucosa of the right lower lobe. BAL results were negative, including a viral panel, Gram stain and culture, and fungal stain and culture. After increasing the steroids, she felt better, and her breathing improved. She was discharged on prednisone 60 mg daily and was recommended to follow up on an outpatient basis with oncology and pulmonology. Unfortunately, she did not follow up with either oncology or pulmonology and was lost to follow-up.","This 64-year-old woman is undergoing treatment for melanoma and has been experiencing fatigue and shortness of breath. She had surgery and radiation therapy, and then started a medication called nivolumab. Her treatment was temporarily paused due to thyroid problems, and she developed pneumonia, which was treated with antibiotics and steroids. Despite the treatment, she didn't follow up with her doctors, and we have lost contact with her."
328,"A 65-year-old female diagnosed with non-small cell adenocarcinoma of the right middle lobe, as seen on chest CT (stage IIA, pT2B, pN0, M0) in 2017, underwent right middle lobectomy and mediastinal node dissection three months later. The pathology report showed moderately differentiated adenocarcinoma with visceral pleural invasion and transcription termination factor 1 positive. She completed carboplatin/Alimta in 2018. In January 2019, her repeat chest CT (Figure ) showed multiple nodular masses in the right lower lobe where her original lung cancer was. A positron emission tomography (PET)/CT scan was done, which showed subpleural nodules with moderate fluorodeoxyglucose (FDG) activity, indicating relapse. At that time, she had declined a biopsy. She was started on palliative chemotherapy of carboplatin, taxol, and pembrolizumab in March 2019 and finished the regimen in May 2019. A repeat chest CT done in May 2019 showed a decreased size of her right lower lobe pulmonary nodules without evidence of progression or new distant lesions. She started pembrolizumab for maintenance therapy in June 2019. Then, her chest CT in October 2019 (Figure ) showed complete resolution of the pulmonary nodules with no evidence of progression or new lesions. However, in March 2020, her creatinine (Cr) level started to increase to 3.07 even though she was asymptomatic with no lower extremities edema. Her baseline Cr level was around 1.0-1.2. Her Cr level had increased to 5.53 in April 2020 and further workup was initiated. Her protein: Cr ratio was 1315, no monoclonal protein was identified, and antinuclear antibodies (ANA) and vasculitis profile were negative. Complement levels were normal. She subsequently underwent a kidney biopsy with pathology (Figure ) showing acute and chronic interstitial nephritis grade 3 and tubulitis. Further workup with the kidney was unremarkable. Her pembrolizumab was held toward the end of March, and she was started on a prednisone 40 mg taper. Her Cr did improve to 1.19, close to her baseline, in May 2020. She completed the steroid taper in July 2020. With the improvement of her Cr, she resumed pembrolizumab in July 2020; however, her Cr level started to increase up to 2.09. She was restarted on prednisone 20 mg daily, and after discussion with oncology, a decision was made to stop pembrolizumab indefinitely. In September 2020, she was tapered down to 10 mg daily. She eventually expired a few months later.","This 65-year-old woman had lung cancer that initially responded to treatment but then returned in her lower lung. After completing chemotherapy, her cancer appeared to be gone, and she was receiving maintenance medication. However, her kidney function started to decline, leading to inflammation and requiring a kidney biopsy. Despite treatment, her kidney problems persisted, and she ultimately passed away a few months later."
329,"A 53-year-old female was diagnosed with metastatic melanoma (cTx, pN1b, M1) in February 2019. She originally presented with left inguinal swelling of four months duration in October 2018. Her ultrasound at that time had only shown a prominent left inguinal lymph node for which core biopsy was done in February 2019, confirming it to be melanoma. Her abdomen pelvis CT also showed bilateral inguinal lymph nodes with a lesion at the splenic lower pole. Follow-up chest CT had shown a neoplastic solid mass in the spleen. She had a left inguinal node dissection in March 2019, which was positive for melanoma with 1/22 nodes positive, the largest one being 5 cm. The PET/CT scan seen in Figure in March 2019 was positive for multiple discrete hypermetabolic foci within the spleen without abnormal foci of increased fluorodeoxyglucose (FDG) in either the liver or lungs. She underwent splenectomy in April 2019 with pathology reporting multiple foci of metastatic melanoma. She was started on pembrolizumab in April 2019. However, in January 2020, she presented to outpatient oncology with progressive generalized weakness, extreme fatigue, lethargy, myalgia, poor appetite, weight loss, and mood changes over a period of 6-8 weeks. Labs in February 2020 showed low free cortisol of 0.2 ug/dL and low adrenocorticotropic hormone (ACTH) of < 1.1 pg/mL. Her luteinizing hormone, follicle-stimulating hormone, and prolactin were normal. She was diagnosed with pembrolizumab-induced hypophysitis, and the decision was to hold her pembrolizumab. She was started on prednisone 1 mg/kg/day with gradual tapering to the maintenance dose of 10 mg daily. Repeat cortisol level done in March 2020 was within the normal range of 3.0 ug/dL. Her cortisol response to adrenocorticotropic hormone (ACTH) was also normal at 3.2 ug/dL. She then resumed pembrolizumab in March 2020. Her repeat chest CT in May 2020 was negative for any recurrent cancer. She is currently tolerating pembrolizumab, reporting good energy levels without any fatigue, myalgia, or mood changes while still on prednisone 7.5 mg daily. She finished her treatment course of pembrolizumab in April 2021. With her latest follow-up in August 2021, she did not have any recurrent disease and is in remission, remaining on prednisone 7.5 mg daily.","A 53-year-old woman was originally diagnosed with metastatic melanoma in 2019 and has since been treated with pembrolizumab. Recently, she experienced symptoms like fatigue and weakness, which led to tests revealing she had an inflammation of her pituitary gland (hypophysitis) caused by the medication. Her doctors stopped the medication and started her on steroids to manage the inflammation, and after a period of improvement, she was able to restart pembrolizumab. Currently, she is in remission with no signs of the melanoma returning, and she is continuing to take a low dose of steroids."
330,"A 71-year-old lady initially presented with gradual onset of painless lower limb weakness for one week “off legs”, lower limb paresthesias and increased urinary frequency. Patient denied any recent symptoms of infective etiology. There was no recent or past history of trauma or accidents. Patient’s past medical history was unremarkable and also had no family history of significant concern. Patient was a non-smoker and teetotaler and there was no exposure to environmental factors such as industrial chemicals, radiation, heavy metals or any toxin exposures reported. Patient led an active life as a housewife (Eastern Cooperative Oncology Group [ECOG] performance status 1 before admission) and used to live with her partner. On examination she had symmetrical lower limb motor weakness in an upper motor neuron pattern with Medical Research Council (MRC) muscle power 4/5 both proximally and distally on admission. There were reduced sensations in lower limbs symmetrically to fine touch, pinprick, temperature and proprioception, initially mild but progressed to significant sensory loss over next couple of weeks with sensory level slightly below umbilicus at T11. There was relative sparing of saddle area and anal tone was patulous. There was no spinal tenderness. Her deep tendon reflexes in lower limbs were exaggerated with extensor plantar responses. Rest of the general and systemic examination was unremarkable. Her blood tests including full blood count, inflammatory markers, B12, folate, thyroid functions, calcium, liver function test and myeloma screen came back normal. Cerebrospinal fluid (CSF) analysis done on second day after admission was unyielding and non-specific with normal protein, glucose and cytology etc. She was evaluated with non-contrast MRI spine which showed focal myelopathic cord signal at the conus and at the level of T10 and T11 vertebrae (radiological differential diagnosis on MRI were B12 deficiency/inflammatory/infection) (Figure ).\nWhile being evaluated for same, she underwent a chest x-ray which was suggestive of suspicious nodules in the right lower and mid zone. This triggered further computed tomography (CT) scans with contrast which showed disseminated malignancy with metastatic lesions to anterior chest wall, liver, lungs, and a necrotic 3.5 cm nodal mass in the left gastric region. There was no obvious primary identified on the CT scan. Tumor markers cancer antigen (CA)-125, CA 19-9, carcinoembryonic antigen (CEA) and CA 15-3 were all negative. A repeat MRI spine with contrast was done afterward with suspicion of spinal metastasis which has led to lower limb weakness. MRI contrast showed a 20*10*19 mm enhancing soft tissue metastatic mass lesion seen within conus in comparison with plain MRI done one week earlier (Figure ).\nThere was no obvious primary identified on the CT. This was discussed in upper GI MDT (Gastrointestinal multidisciplinary team) and was thought to be a disseminated malignancy arising from a gastric primary. An endoscopic ultrasound (EUS) showed a deep, malignant-looking gastric ulcer on the high anterior greater curve of stomach. This was seen with EUS as a hypoechoic mass extending into the serosal margin. Biopsy from the stomach lesion surprisingly showed a metastatic malignant melanoma. Histopathology showed scanty strips of benign columnar epithelium with most of the tissue representing tumour, focally necrotic (Figure ). Immunohistochemical assays for focal Melan-A, human melanoma black-45 and SOX10 confirmed the diagnosis of malignant melanoma. Real-time polymerase chain reaction (PCR) analysis of the BRAF gene was done which revealed the presence of a mutation within codon 600.\nThis case was discussed again in dermatology-oncology MDT after biopsy results and was decided that the best course of action would be palliative treatment, considering the advanced stage of the disease and poor performance status. Management options and prognosis were discussed with patient who agreed to palliative management. After discharge patient contracted coronavirus disease 2019 (COVID-19) pneumonia for which she was again hospitalized and treated with oral dexamethasone 6mg (milligrams) once a day and oxygen inhalation via nasal cannula between 2-4lit/min for 10 days. She further had developed bilateral segmental pulmonary emboli and was started on anticoagulation with apixaban. Her neurological symptoms worsened and progressed to paraplegia, urinary and fecal incontinence over a period of three to four weeks since initial presentation. Patient passed away three months after diagnosis of metastatic melanoma under community palliative care.","This 71-year-old woman initially experienced weakness in her legs and numbness, along with increased urination. After further tests, doctors discovered she had a metastatic melanoma, a type of skin cancer, that had spread to her stomach and other organs. Imaging revealed a spinal tumor causing the leg weakness. Despite treatment, her condition worsened, and she eventually passed away three months after her initial diagnosis."
331,"The patient was a 79-year-old Caucasian male with a 7-year history of myelodysplastic syndrome (MDS) and a complaint of abdominal pain. The pain started in the evening of the day before and was accompanied by fever, oral intolerance, nausea, and vomiting. He did not complain of bowel habit changes, although he had a history of chronic constipation. Past medical and surgical history was positive for MDS and cholecystectomy. MDS was controlled with daily thalidomide, deferasirox, dimethicone, pantoprazole, gabapentin, vitamin B12, and folic acid. He also took erythropoietin and rivaroxaban three times a week and filgrastim every fifth day. The family history and habitual history of the patient were unremarkable.\nThe patient was awake and oriented on admission, yet he was ill, dehydrated, and mildly agitated. He had blood pressure 95/60 mmHg, pulse rate 105/min, respiratory rate 17/min, Temperature 37.9 °C, and O2 Saturation 90% on ambient air. Tenderness of the epigastrium and right upper quadrant of the abdomen was noted, without abdominal distension, rebound tenderness, or guarding. Laboratory results were as mentioned in Table . The patient’s electrocardiogram was unremarkable.\nPlain thoracic and abdominopelvic radiographs were normal. In abdominopelvic ultrasonography, mild fluid in subhepatic and inflamed echogenic fat in the upper abdomen and around the pancreas was found, along with duodenal wall thickening. With the impression of pancreatitis, intravenous Ciprofloxacin, Metronidazole, Ondansetron, normal saline, and one unit of packed red blood cells were administered. As the patient did not have a proper urinary output (200ml since urinary catheterization), the computed tomography (CT) scan was performed without intravenous contrast. The abdominopelvic CT scan demonstrated edematous wall thickening of the entire duodenum with water halo and significant adjacent fat stranding and swelling of the pancreas. Pancreatitis was a potential etiology of secondary duodenal wall thickening []. However, observing the bulk of the pathologic changes at the duodenum and severe duodenal wall edema also raised the possibility of primary duodenal pathology. Accordingly, a sign of ischemia was suggested, and a contrast-enhanced CT scan was advised to evaluate the related vessels further.\nThe patient remained hypotensive and oliguric regardless of intravenous fluid resuscitation. Nine hours after admission, norepinephrine was initiated. However, blood pressure remained low and fluctuated during the next five hours, with the minimum being 60/40 mmHg. Despite supportive treatment, the patient’s condition deteriorated to the extent of severe electrolyte imbalance, gasping, severe acidosis (arterial pH = 6.8), and GCS 4/15. Due to the mentioned deterioration, another abdominopelvic CT scan with intravenous contrast was requested at the fourteenth hour of admission. The second CT scan demonstrated a marked decrease in the enhancement of the edematous duodenum and target sign. Since the related visible vessels were patent, microvascular ischemia or necrosis of the duodenum was highly suggested (Fig. ).\nThe patient was immediately transferred to the operating room in a critical condition. With a midline laparotomy incision, 400 mL serous fluid was drained. Kocher’s maneuver was performed to explore the duodenum and the retroperitoneal structures. Duodenum (D2–D3–D4) was grossly ischemic, the stomach was dilated, and the small bowel distal to the Treitz ligament appeared normal (Fig. ). The Pancreas was normal in view, without any saponification, inflammation, or edema in favor of pancreatitis. Exploration of the superior mesenteric artery and vein did not reveal any pathologic findings. Whipple’s procedure was planned initially. However, the patient’s cardiac arrest during the operation directed the plan to a damage control surgery. The overall operation time was approximately one hour and a half. To do the pyloric exclusion in the shortest time, the pylorus was isolated, and a polyester surgical tape was passed behind that. Tight ligation of the tape around the pylorus temporarily excluded the ischemic duodenum. To achieve a damage control surgery, decompression of the stomach was done by a red (18 French) nasogastric tube (Fig. ). Lastly, an open (corrugated sheet) drain was placed near the duodenum, and the patient was then transferred to the intensive care unit. Despite two hours of supportive therapy, blood pressure remained 40 mmHg/pulse, leading to a cardiac arrest with asystole rhythm and patient expiration. According to the refusal of next of kin to consent, an autopsy was not performed.","This 79-year-old man was admitted to the hospital with severe abdominal pain, fever, and nausea. He has a history of a blood disorder and was experiencing dehydration and agitation. Tests revealed inflammation and swelling in his upper abdomen, suggesting a serious problem with his duodenum (the first part of his small intestine). Despite treatment, his condition rapidly worsened, leading to a cardiac arrest and ultimately, his death."
332,"A 72-year-old man presented to emergency room with 8-h fatigue and 4-h mild exertional dyspnea, palpitation, and blurred vision. He was a nonsmoker and denied previous history of cardiovascular disease. Physical examination showed sinus tachycardia (117 bpm) and normal blood pressure (120/70 mmHg). The position and range of apical impulse were normal. There were no heart murmurs, no crackles or wheezes on chest auscultation. In ECG, the ST-segment was elevated by 2–3 mm in leads II, III, and aVF, with Q-waves (Fig. A). Cardiac troponin T was 3.33 ng/mL (normal range 0–0.04). A diagnosis of acute inferior myocardial infarction was established.\nDual anti-platelet therapy (loading doses: aspirin 300 mg and clopidogrel 300 mg) was initiated to prepare for primary PCI. Coronary angiography (CAG) showed multi-vessel lesions, including a total occlusion of the distal portion of a dominant right coronary artery (RCA), 90% stenosis of the proximal portion of the left anterior descending artery (LAD), and diffuse stenosis (50–60%) of the left circumflex artery (LCX) (Fig. A–C). The culprit lesion was in the distal portion of the RCA. The patient received a loading dose of glycoprotein IIb/IIIa inhibitor (tirofiban) after the angiography. Percutaneous balloon angioplasty was then conducted; one BuMATM 2.5 × 20 mm sirolimus-eluting stent was placed to restore blood flow in RCA (TIMI grade 3) (Fig. D). ST-segment elevation and depths of the Q-waves were attenuated after the primary PCI (Fig. B). Secondary PCI was planned for LAD lesions 5 days later.\nImmediately prior to transfer to the cardiac intensive care unit (CCU), the patient developed severe dyspnea. Heart rate (HR) was 120 bpm, and blood pressure (BP) decreased to 90/60 mmHg. Auscultation revealed a loud harsh holosystolic murmur along the left sternal border and crackles throughout both lungs. NYHA functional class of heart failure was IV. A mechanical complication was suspected, but not investigated immediately. We immediately placed an IABP (1:1 augmentation ratio) from femoral access, and the patient received 3-mg morphine, 20-mg furosemide, and 0.1-mg recombinant human brain natriuretic peptide (rhBNP). Once the IABP was inserted, symptoms became alleviated. The BP stabilized at 110/70 mmHg and the augmentation pressure was 120 mmHg. Then the patient was transferred to CCU safely.\nTransthoracic echocardiography (TTE) was performed the next day and revealed a left-to-right shunt in the posterior portion of the interventricular septum with a size of 11.4 mm (Fig. ). The left ventricle end-diastolic diameter (LVED) was 50 mm, and the diameter of the right ventricle was 20 mm. We also detected regional wall-motion akinesia of the left ventricle in the inferior and posterior sections. Left ventricular ejection fraction (LVEF) was 59%. The estimated pulmonary arterial pressure and right ventricular systolic pressure were 48 mmHg. A calculated Qp/Qs was 2.905. There was no regurgitation of the mitral valve or pericardial effusion. An updated diagnosis of ventricular septal rupture was made. We decided to continue IABP to support cardiac function. Medications included aspirin (100 mg orally once a day), clopidogrel (75 mg orally once a day), atorvastatin (20 mg orally once a day), furosemide, spironolactone, and nitrates, as well as an intravenous infusion of rhBNP. The patient received a subcutaneous injection of 40-mg enoxaparin every 12 h to prevent deep vein thrombosis. Omeprazole (40 mg per day) was used to prevent gastrointestinal mucosal injury.\nAn attempt to wean IABP was made on day 12 on the ground of stable hemodynamics and disappearance of all symptoms. Five minutes after reducing the augmentation ratio from 1:1 to 1:2, the patient felt dyspnea, and the BP decreased from 116/68 mmHg to 71/50 mmHg. The augmentation ratio was increased back to 1:1. Dyspnea gradually dissipated, and the BP normalized. We realized the shunt was in a large amount according to the Qp/Qs. IABP can decrease the shunt, so we decided to implement a prolonged use of IABP, which continued at 1:1 ratio for another 16 days. During the use of IABP, we enhanced the medical and nursing concern including checking the circulatory status of the lower limb of the puncture with Doppler ultrasound every day, limiting its movement to avoid dislocation of the balloon catheter and bleeding, and continuous administration of anticoagulation by subcutaneous injection of enoxaparin to prevent deep vein thrombosis. The aspirin, clopidogrel, atorvastatin, spironolactone, and rhBNP were administered during the month when IABP was used. An antibiotic (cefamandole 2000 mg intravenously every 12 h) was given for prophylaxis for bacteremia during invasive IABP insertion from day 21 to 27. Psychological therapy was adopted to enable the patient to cooperate on prolonged use of IABP.\nSurgical repair was offered to the patient on day 27 since the friable tissue in the ischemic myocardium should be sufficiently mature at this time to allow repair. However, the surgeons considered that it was too risky to perform the surgical repair due to the limitation of technique at that time. Firstly, the VSR located at the posterior muscular part of the ventricular septum was lower and deeper than a perimembranous ventricular septal defect approached through a right atriotomy and the tricuspid valve, so the surgical repair was more difficult and beyond the techniques of the surgeons, when they only had experience in the treatment of perimembranous ventricular septal defects at that time. Secondly, the EuroSCORE [] of this case they calculated was 16, and the predicted mortality was 59.07%, which was too high for them to operate safely. Meanwhile, we also consulted the anesthesiologist to evaluate the patients for general anesthetization, and the anesthesiologists deemed there would probably be high risk in the process of a general anesthesia for the open surgery and especially under extracorporeal circulation support. Surgery was also declined by the patient and his family due to the high risk of open surgery and under general anesthesia and extracorporeal circulation support. Instead, the patient opted to receive percutaneous closure of the VSR. So we decided to choose percutaneous VSR closure for this specific high-risk senior patient.\nOn day 28 (4 weeks after VSR), the patient didn’t feel dyspnea or any other discomfort with stable vital signs (HR 90 bpm, BP 98/67 mmHg, augmentation pressure 112 mmHg). We believed the waiting period for the infarcted myocardium to recover was enough according to the documented experiences [, ]. Left ventriculography confirmed an 11-mm left-to-right shunt (Fig. A). Transcatheter closure was conducted via the femoral artery and subclavian vein. Upon establishment of the transseptal wire loop as a rail, the patient developed ventricular fibrillation, and he lost consciousness followed by. A 200-J electrical shock was delivered immediately and restored sinus rhythm. Then the patient regained consciousness. After the wire loop through the rupture was established, the HR suddenly decreased to 30 bpm. A temporary pacemaker was placed, and we proceeded to implant a 24-mm double-umbrella AGA AMPLATZERTM occluder to close the VSR (Fig. B). The vital signs were stable (HR 78 bpm, BP 110/72 mmHg). Echocardiography after the procedure revealed no residual shunt, and no interference of valve functions by the occluder. The estimated Qp/Qs was 1.086. The temporary pacemaker was removed after the closure operation.\nTwo days later, the augmentation ratio was decreased to 1:2 and then to 1:3. IABP was weaned off on day 31. Secondary PCI was conducted on day 35 for LAD lesions (Fig. ). The patient was discharged on day 41.\nAt the last follow-up 6 years later, CAG and TTE revealed no in-stent restenosis, no left-to-right shunt, no mitral regurgitation, and 51% LVEF. He kept taking aspirin, atorvastatin, and metoprolol regularly as the medication therapy.\nAll procedures performed in this study involving human participants were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient.","This 72-year-old man experienced a sudden heart attack that caused a serious complication – a rupture between the chambers of his heart. He was rushed to the hospital and received immediate treatment, including medications, a heart pump, and a procedure to open up blocked arteries. After the initial treatment, he developed a significant leak between the heart chambers, which was repaired with a device. He was closely monitored and received ongoing care to ensure his heart function remained stable."
333,"In 2017, an 83-year-old Japanese woman presented to our department with a chief complaint of a mass in her right maxillary premolar region. She had initially noticed a painless mass in her right maxillary premolar region in 2002. After she had first noticed of the mass, it grew gradually in size, but she sought no treatment for it. She was referred to us for examination and treatment because it was difficult to perform tracheal intubation for surgery of sigmoid colon cancer at another hospital. Her history included sigmoid colon cancer, subarachnoid hemorrhaging, bronchitic asthma, and cardiac insufficiency. Regarding the intra- and extra-oral findings, a massive pedunculated mass in the right maxillary premolar region measuring 83 × 58 × 35 mm was palpable (Fig. ). Furthermore, it covered the front of the right palate, and it protruded to the extra-oral region from the right maxillary premolar alveolar region. Its surface was almost entirely smooth, and some erosions and ulcerations were seen. It was elastic and hard and showed no tenderness on palpation. There was no palpable regional lymphadenopathy, and a laboratory examination revealed no abnormal values.\nPanoramic X-ray revealed the shadow of the mass in the right maxillary premolar region, which included some hard tissue (Fig. ). Computed tomography (CT) showed scattering calcified images in the mass (Fig. ). Magnetic resonance imaging was not performed because she had vertebral artery clips and screws in her forehead. Given the above findings, we suspected benign gingival tumor in the right maxillary premolar region and performed a biopsy under local anesthesia (Fig. a, b).\nHistologically, proliferation of dysplastic squamous epithelia was observed (Fig. ). We noted subepithelial mild dysplastic spindle-shaped cells and collagenous fibers, and scattered calcification and ossification were also observed (Fig. ). Immunohistochemically, the spindle cells were negative for pan-cytokeratin (AE1/AE3), and nuclear translocation of β-catenin was not observed in the spindle cells (data not shown). Therefore, we excluded a diagnosis of carcinosarcoma and fibromatosis. However, we were unable to diagnose absolutely whether the dysplastic squamous epithelia were pseudocarcinomatous hyperplasia of the gingiva or well-differentiated squamous cell carcinoma. In addition, positron emission tomography with computed tomography (PET/CT) revealed that the maximum standard unit value (SUVmax) of the sigmoid colon and the oral lesion were 15.27 and 14.99, respectively, and there were no other obvious metastases (Fig. ). Therefore, we performed tumorectomy under general anesthesia. The pedicle of the tumor was located at the right maxillary premolar area, and the tumor—including the tissue surrounding the lesion—was resected as one mass together with the periosteum (Fig. a–d). At that time, partial destruction of the maxillary bone was seen. The exposed bone surface was slightly curetted. After resection, the wounded area was covered with artificial dermis (TERDERMIS). Finally, tie-over dressing by gauze with ointment was performed.\nThe microscopic findings of the surgically removed tumor were similar to those of the biopsy specimen. The body of the tumor was composed of spindle-shaped cells that were proliferating with collagenous fiber, and scattered bone formation was also observed (Fig. ). The destruction of the basement membrane by atypical squamous epithelia was observed in the surgically removed specimen, suggesting stromal invasion. In addition, immunohistochemical analysis revealed that the dysplastic squamous epithelia were positive for Ki67 and CK17, suggesting that they were squamous cell carcinoma, not pseudocarcinomatous hyperplasia. Furthermore, some spindle cells were positive for smooth muscle action (SMA), indicating myofibroblastic differentiation. Therefore, the epithelial component of the tumor was considered to be well-differentiated squamous cell carcinoma. Because most of the tumor was occupied by spindle-shaped cells and marked ossification histopathologically, we diagnosed the tumor as POF with squamous cell carcinoma (pT1N0M0).\nOne week after surgery, we removed the gauze and covered the wound with an oral appliance for protection (Fig. a, b). There have been no signs of local recurrence or metastasis during follow-up as of 2 years after surgery.","An 83-year-old woman came to the hospital because she had a growing mass in her mouth that was making it difficult to have surgery for a different condition. The mass had been present for over 20 years and was causing problems with her mouth and palate. After a biopsy, doctors determined that the mass was a type of squamous cell carcinoma, a form of cancer. The mass was surgically removed, and she is now being followed closely to ensure there are no signs of recurrence."
334,"A 19-years-old female was first found with occupation about 6.2 cm × 5.7 cm in the right adrenal five years ago based on CT scan at a regular physical examination, accompanied with fever and headache with high blood pressure. Tumor resection was performed, and pathological diagnosis was right pheochromocytoma. The patient was regularly subjected to follow-up.\nDuring this hospitalization, CT scan found multiple tumor occupation in left adrenal and paraganglion region zones with round nodular shadows. Meanwhile, she had fever and headache with high blood pressure. Based on medical history and examination, she was diagnosed as left pheochromocytoma. Meanwhile, the occupying lesions in right lung and pancreas were found, located in extrabasal segment of inferior lobar and head respectively.\nThe case characteristics were summarized: 1. A 12y female; 2. Tumor occupation was first found in right adrenal; 3. Tumor resection was performed and the pathological diagnosis was pheochromocytoma; 4. Multiple round nodular shadows were found in left adrenal and paraganglion region zones after five years; 6. The symptoms were fever and headache with high blood pressure; 7. The diagnosis was pheochromocytoma (PCC) and paraganglioma (PGL) (Combined PPGLs); 8. The occupying lesions located in right lung and pancreas were scanned and diagnosed with masses.","This 19-year-old woman has a history of a pheochromocytoma (a tumor on the adrenal gland) that was found five years ago. Recent scans have revealed additional tumors in her left adrenal gland, a region near her adrenal gland, and in her lung and pancreas. She is experiencing symptoms like fever, headache, and high blood pressure related to these tumors. Based on these findings, she has been diagnosed with a combined pheochromocytoma and paraganglioma, and further evaluation is needed to determine the best course of treatment."
335,"A 57-year-old Caucasian woman was recently diagnosed with advanced stage ovarian cancer. Otherwise healthy, she had initially consulted her general practitioner simply for abdominal distension. Further investigations [abdominal computed tomography (CT), serum CA125determination and laparoscopy] revealed a peritoneal carcinomatosis. The diagnosis of stage III (FIGO classification) ovarian cancer was established and the patient received a carboplatin-paclitaxel based chemotherapy regimen in a neoadjuvant setting. The indication of a debulking surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) was retained by our institutional tumor board. A CVL was inserted via the right jugular vein into the superior vena cava in prevision of surgery and the appropriate positioning of the catheter was verified by chest x-ray. An implantable central venous catheter (Port-a-Cath) was already in place (Fig. ).\nThe first 48 postoperative hours were marked by difficult pain management, hypotension, and transient hyperlactatemia responding to fluid replacement and norepinephrine. On postoperative day 3, she presented acute onset dyspnea when transferred from the bed to a chair, and a major drop in pulse oxygen saturation (from SpO2 96% to 83%) justified the administration of oxygen (5 L/min) via a nasal cannula. On physical examination, her body temperature was 37.4 °C, blood pressure 135/81 mmHg, heart rate 122 beats/min, respiratory rate 20/min. No chest pain was reported. Pulmonary examination revealed a bilateral reduction of basal breath sounds with dullness at percussion. Chest x-ray examination (Fig. ) showed bilateral pleural effusions that could largely explain patient’s dyspnea and oxygen desaturation. After switching from a nasal cannula to a nonrebreather mask (FiO2 0.40), the patient’s condition seemed to stabilize in supine position. A measurement of the arteriovenous oxygen difference was obtained via sampling through the arterial and central venous line after the patient was again lying in supine position (Table ).\nA sampling error or a wrong positioning of the recent CVL was suspected, but a sample taken from the Port-a-Cath confirmed the venous value. A transthoracic echocardiography (TTE) with bubble test failed to show a right-to-left shunt, atrial septum was intact. No pulmonary hypertension was detected and right ventricle was not dilated. A CT pulmonary angiography (Fig. ) showed large bilateral pleural effusions with atelectasis of both lower lobes, acute pulmonary embolism in the right middle and upper lobe pulmonary arteries, and ultimately an anatomical variant of the left upper pulmonary vein draining into the left innominate vein. Following bilateral chest drainage, the patient was treated with low molecular weight heparin twice daily. There was no evidence for deep venous thrombosis. This was followed by a significant clinical improvement, a disappearance of the platypnea–orthodeoxia complaints and a reduction of oxygen requirement over the following days.\nAt 1-year follow-up, TTE showed no right ventricular dilation and absence of pulmonary hypertension. From an oncological perspective, stability was also observed during her niraparib maintenance therapy, with absence of new lesions at a 1-year follow-up abdominal CT.","This 57-year-old woman was recently diagnosed with advanced ovarian cancer that has spread to her abdomen. She started seeing her doctor because of stomach pain, and doctors found the cancer through tests like CT scans and a laparoscopy. She received chemotherapy to shrink the tumor before surgery, and a special catheter was placed in her arm to help with the surgery and future treatments. After surgery, she experienced some breathing difficulties and low oxygen levels, which were caused by fluid buildup in her lungs. After treatment, her breathing improved, and she is now doing well with ongoing cancer treatment and regular checkups."
336,"A 31-year-old man categorized as New York Heart Association Class IV was admitted to the hospital after suffering from anorexia for 2 weeks and dyspnoea for 1 week in August 2001. He had previously undergone surgery for appendicitis and had on other past medical history. He exhibited mild intellectual disability, and electrocardiography (ECG) depicted a short PR interval with pre-excitation and negative T waves (). Echocardiography revealed a hypertrophic dilated left ventricle with poor systolic function [left ventricular ejection fraction (LVEF) 24%] and signs suggestive of non-compaction myocardium (). Doppler parameters indicated reduced e′ velocity at the mitral valve annulus and increased tricuspid regurgitation velocity. A restrictive filling pattern was evident via pulse Doppler of the mitral valve. According to the American Society of Echocardiography/European Association of Cardiovascular Imaging guidelines and references, his left ventricular diastolic dysfunction was in Grade 3. There was severe mitral and tricuspid regurgitation. Abnormal laboratory parameters included elevated total bilirubin (85.3 µmol/L), indirect bilirubin (55.4 µmol/L), and aspartate aminotransferase (284 IU/L). He experienced sudden cardiac death at the age of 33 years.","This 31-year-old man was admitted to the hospital because he was having trouble breathing and wasn’t eating well. Tests showed he had a weakened heart with reduced pumping ability and some unusual heart structure. He also had problems with how his heart filled with blood, leading to a restrictive filling pattern. Sadly, he had a sudden cardiac death at a young age, and his condition was severe."
337,"A 62-year-old Caucasian man was admitted to our department in June 2020. He was diagnosed with follicular lymphoma in 2010 and treated with an R-CHOP regimen (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone) for six cycles, achieving complete response; in 2017, because of a disease recurrence, he was treated with six chemotherapy cycles with R-bendamustine, followed by maintenance therapy only with rituximab for 2 years. The last administration was in February 2020. Clinical and radiological follow-up was negative; during the maintenance therapy, the patient had urinary tract recurring infections, gingivitis, and herpes zoster cutaneous reactivation.\nHe sought medical attention because of progressive vision loss. His pharmacological therapy included atorvastatin, amlodipine, and pantoprazole. He was afebrile; his vital signs were in range. General and neurological physical examination was negative, except for right homonymous hemianopia. Brain computed tomography (CT) showed an uneven cortical and subcortical hypodense lesion in the left posterior temporal and occipital areas, with no contrast enhancement. Magnetic resonance imaging (MRI) confirmed the left parenchymal lesion, composed of two parts: a periventricular one showing inhomogeneous signal (decrease in T1-weighted, increase in T2-weighted sequences); and a more uniform one, involving white matter and characterized by strong T2-weighted and fluid-attenuated inversion recovery (FLAIR) hyperintensity and T1 hypointensity. Restricted diffusion was noted in both components, especially in the white matter, but no gadolinium enhancement was observed. Magnetic resonance (MR) spectroscopy pointed out a reduction of N-acetyl aspartate peak, an elevated choline peak, and a double peak of lactic acid.\nTotal body contrast CT did not document hematological disease activity and confirmed the two previously known lymph nodes, a left axillary and a right mediastinal para-esophageal one, stable in dimensions (16 × 10 and 11 × 6 mm, respectively). Neurological findings were interpreted as a consequence of intracranial relapsed lymphoma. So, after a multidisciplinary assessment, brain biopsy was planned. Histological samples showed a large number of macrophages (CD68+, CD14+) overshadowing any other cell, except for isolated reactive astrocytes and mild perivascular T-lymphocyte infiltrates. No neoplastic cell was clearly individuated. No microbiological test was performed on brain tissue.\nThe patient, discharged after brain biopsy, returned to the hospital because of worsening eyesight, mental confusion, and psychomotor slowing. Laboratory investigation on blood was not significant: peripheral blood cell count was normal with 4.61 × 109 white blood cells and mild reduction in hemoglobin level (13.9 g/dL); C-reactive protein was < 5 mg/L. Electrolyte levels, renal function, liver enzyme levels, and coagulation examinations were in range.\nBrain MR was repeated (Figs. , ) and showed, especially on FLAIR/T2-weighted images, the hyperintense left temporal–parietal–occipital and peritrigonal lesion notably increased in dimension and extended from the left to the right hemisphere through the corpus callous. It was also characterized by vasogenic edema with a slight mass effect on cortex gyri. There was no significant contrast enhancement; diffusion-weighted imaging (DWI) showed restricted diffusion around lesion edges. Despite the rapid progression of clinical and imaging findings, the absence of significant contrast enhancement and, above all, the inconclusive response of previous histological report excluded the hypothesis of an expanding neoplastic lesion; MR features were instead considered suggestive for inflammatory demyelinating process, and progressive multifocal leukoencephalopathy was finally suspected. Therefore, a diagnostic lumbar puncture was performed. Cerebrospinal fluid appeared clear and colorless. Chemical–physical analysis showed presence of 10 leukocytes/μL (0.00–5.00) and mild elevation in protein level (albumin 0.350 g/L); glucose level was normal (cerebrospinal-fluid-to-serum ratio 82%). There was an immunoglobulin G (IgG) level of 0.025 g/L and an IgG index (Link index) of 0.66 (< 0.7). Oligoclonal bands were negative. Bacterioscopic and microscopic examination for Cryptococcus neoformans was negative; cultural examinations for mycobacteria, Listeria monocytogenes, Borrelia burgdorferi, and Toxoplasma were negative; galactomannan antigen was absent; polymerase chain reaction for Epstein–Barr virus (EBV), cytomegalovirus (CMV), herpes simplex 1 and 2, HHV6, HHV7, HHV8, varicella–zoster, measles, BK virus, adenovirus, enterovirus, Toscana virus, and rubella was negative; virus JC quantitative real-time PCR was positive with detection of numerous viral genomes (9,548,473 units/μL).\nCSF serology for aforementioned infectious agents was negative. CFS immunophenotype, despite the low amount of cells after centrifugation, was characterized by 18.0% of lymphocyte, predominantly T, without any B population.\nBlood serology for HIV Ag/Ab was negative. Coronavirus disease 2019 (COVID-19) swab test was negative.\nHe was treated supportively with trazodone and mirtazapine. The outcome was poor: neurological findings continued to worsen, and he progressively became blind, confused, and disorientated. He was finally transferred to a hospice for palliative care (Table ).","This 62-year-old man has a history of lymphoma that he’s been managing since Recently, he started experiencing vision problems and confusion, and tests revealed a growing area of damage in his brain. Although initial scans didn’t show signs of cancer returning, further tests suggested a possible inflammatory condition, and a spinal tap confirmed the presence of a virus in his cerebrospinal fluid. Despite treatment, his condition continued to decline, and he was ultimately transferred to a hospice for comfort care."
338,"In December 2017, a 19-year-old female was referred to our hospital with a recent diagnosis of histiocytic sarcoma. The patient had presented as pharyngeal pain and left neck mass with fever for 2 months. She had received tracheotomy because of airway obstruction resulting from the large mass arising from the left parapharyngeal space at local clinic. PET/CT scan showed a large solid mass located in the left parapharyngeal space with compression of pharyngeal cavity and multiple enlarged lymph nodes in the left neck (). The histopathological review confirmed diagnosis of HS. Histologically, HS is composed of large polygonal cells with epithelioid-to-pleomorphic morphology, abundant eosinophilic to vacuolated or foamy cytoplasm, ovoid to irregularly shaped nuclei, and variably prominent nucleoli (). For immunohistochemical markers, most HS express CD68 and CD163 and partially express S100 (). Chemotherapy (cyclophosphamide, doxorubicin, vincristine, prednisone, and etoposide (CHOEP)) was initially started on December 8, 2017 with the aim of stabilization of the fulminate disease course; however, the lesions did not shrink and pain and dysphagia were heavier caused by the compression of the pharynx (). In order to relieve the compression, irradiation to the lesions of pharyngeal and neck was started on day 11 of chemotherapy. After irradiation of 20 Gy/10 F, the size of lesions became a little bit smaller (pharyngeal mass: from 6.0 cm × 4.4 cm to 5.6 cm × 4.0 cm; neck mass: from 3.5 cm × 2.8 cm to 2.9 cm × 2.6 cm).\nMeantime, next-generation sequencing (NGS) of tumor tissue was performed using a panel of 93 genes (Gene+ OncoLym). This analysis revealed the presence of oncogenic mutation c.2888-1G>T in the MET gene, exon 14 (allele frequency, 5.58%), as well as an activated mutation c.361T>A (C121S) in the MAP2K1 gene, exon 3 (allele frequency, 17.23%). MEK inhibitor trametinib has been reported to be effective in HS patients with MAP2K1 mutation (); however, trametinib was not available in China at that time. It has been reported that patients with MET exon 14 skipping mutation-positive nonsmall cell lung cancer are sensitive to MET inhibitor crizotinib (); therefore, this histiocytic sarcoma patient began to take crizotinib (250 mg, twice daily) after irradiation of 20 Gy/10 F. Partial response (PR) was observed after radiotherapy of a total dose of 60 Gy/30 F and 1-month treatment of crizotinib, with sum of the product of the longest perpendicular dimensions (SPD) decreased by 57% (). The lesions kept shrinking () after radiotherapy, and crizotinib was still taken daily for 1 month more. However, 2-month treatment of crizotinib cost her family RMB 100,000 Yuan. The patient could no longer afford such an expensive drug.\nIn order to search for new targetable therapeutic drugs, NGS was done again with a panel of 1,021 genes (Gene+ Onco-C1021T). The most frequently mutated genes were mutation c.410G>A (G137D) in the DUSP2 gene, exon 2 (allele frequency, 18.9%), mutation c.290G>A (C97Y) in the HIST1H3B gene, exon 1 (allele frequency, 15.9%), and mutation c.3646A>T (S1216C) in the GRIN2A gene, exon 13 (allele frequency, 15.7%). Both DUSP2 and GRIN2A are in the RET signaling pathway. Imatinib is a tyrosine kinase inhibitor (TKI) that inhibits RET, PDGFR, and KIT. It has been reported to be effective in some HS cases (). The patient was subsequently treated with imatinib (400 mg daily) and thalidomide (100 mg daily) since March 2018. The cost of imatinib and thalidomide was RMB 2,600 Yuan/month. Two months after the treatment, excellent PR was observed () compared with tumor size in March 2018. Four months later the re-evaluation by CT scans showed a nearly complete remission (CR) (). The patient took maintenance of imatinib and thalidomide for 2 years and stopped the treatment in March 2020. To date (September 2021), 45 months after HS diagnosis, she is still alive without tumor recurrence.\nTo explore the possible underlying mechanism of imatinib plus thalidomide in this HS patient, experiments in vitro were performed in a canine HS cell line DH82. Results of cell counting kit-8 (CCK8) assays showed that the proliferation activity of DH82 was significantly inhibited by imatinib but not thalidomide (). Combined thalidomide and imatinib treatment did not improve the inhibitory effects of imatinib to DH82 (). We speculated that no synergistic effect existed between imatinib and thalidomide, but each of them might have its own specific antitumor activity.\nAs mentioned above, mutations of DUSP2 and GRIN2A are involved in the RET signaling pathway. RET signaling leads to the activation of the RAS/MAPK and the PI3K/AKT pathways and has key roles in cell growth, differentiation, and survival (). Further KEGG pathway enrichment analysis of NGS results from patient’s tissue also revealed that PI3K/AKT and MAPK pathways were activated in this HS patient (). Immunohistochemistry staining on the patient’s tissue was performed to detect phosphorylated ERK (p-ERK) and phosphorylated JNK (p-JNK) of MAPK pathway and phosphorylated AKT (p-AKT) of PI3K/AKT pathway. Results showed that p-AKT and p-ERK were strongly positive, while p-JNK was almost negative (), indicating the patient actually harbored the activation of MAPK and PI3K/AKT pathways. Treatment of DH82 with imatinib demonstrated that p-ERK and p-AKT were substantially inhibited with imatinib while p-JNK was slightly elevated in a dose-dependent manner, which confirmed the inhibitory effects of imatinib on DH82 by targeting activation of MAPK and PI3K/AKT pathways ().","A 19-year-old woman was diagnosed with a rare type of cancer called histiocytic sarcoma, which started with pain and a lump in her neck. After surgery to relieve pressure on her airway, doctors used radiation therapy and then started her on a new medication called crizotinib, which was found to be effective in shrinking the tumor. Further testing revealed specific genetic mutations in her cancer cells, leading to the selection of imatinib and thalidomide as a maintenance treatment. After two years of treatment, she achieved a complete remission and remains cancer-free today."
339,"A 43-year-old primipara presented with severe hypertensive disorder of pregnancy and fetal growth restriction (FGR) at 19 gestational weeks. At 23 6/7 gestational weeks, an emergency cesarean section was conducted due to worsened hypertensive disorders of pregnancy (HDP) and a non-reassuring fetal status. “En caul” delivery could not be achieved because of the thick uterine wall. The caul refers to the amniotic membrane. To be born in a caul (en caul) means to be born with the head covered by the amnion (or be born within an intact unruptured amnion). A male infant with a birthweight of 293 g was born without any apparent trauma. Endotracheal intubation and surfactant replacement were performed immediately after birth in the delivery room. The umbilical venous catheter (UVC) and the peripheral arterial catheter were successfully placed, while umbilical arterial catheter (UAC) insertion was unsuccessful. Initial examination revealed anemia (hemoglobin level: 10.8 g/dl), disseminated intravascular coagulation (DIC), and the presence of slight ascites on ultrasonography, suggesting intra-abdominal bleeding. Intensive treatment, including HFOV, inotropes, steroids, and massive blood and plasma transfusion for the progressive anemia and DIC, was started. However, liver and adrenal bleeding gradually became evident on ultrasonography () and he continued to suffer from refractory hypotension and further progressive anemia. Throughout the course, his parents hoped to switch to palliative care, seeing his irreversible worsening clinical condition. He died of hemorrhagic shock at 3 DOL.","This woman was admitted to the hospital at 19 weeks of pregnancy because of dangerously high blood pressure and a problem with the baby’s growth. She needed an emergency C-section at 23 weeks due to worsening complications and a concerning situation with the baby. A very small baby, weighing only 293 grams, was delivered without any apparent injury. Unfortunately, the baby had severe health problems, including anemia and bleeding, and despite intensive treatment, he passed away a few days later."
340,"A 40-year-old primipara woman was diagnosed with FGR at 19 gestational weeks. She was transferred to our hospital at 22 gestational weeks due to severe HDP. A female infant was born at 22+6/7 gestational weeks with a birthweight of 279 g after an emergency en caul cesarean section due to maternal HDP. Endotracheal intubation and surfactant administration were performed in the delivery room. The UAC and the PICC were successfully placed, while UVC insertion was unsuccessful. Prophylactic indomethacin was administered once, causing PDA closure at 1 DOL. The circulatory status of the patient was successfully stabilized within 72 h of life without IVH. Mean fluid intake during the first weeks of life was 143.0 ml/kg/day. Enteral feeding with breast milk was started at 3 DOL. The breast milk secretion was insufficient; therefore, we increased enteral feeding slowly and started to use hydrolyzed formula milk at 23 DOL. She did not develop NEC throughout her clinical course. Although HFOV, five-time repeated surfactant replacement, and systemic hydrocortisone were required to manage the severe bronchopulmonary dysplasia, she was successfully extubated at 73 DOL. Oral feeding was started at 101 DOL and the patient was discharged at 146 DOL with home oxygen therapy, without tube feeding. She did not develop IVH or PVL. Although ophthalmologists diagnosed her ROP (right; stage II, left; stage III), no therapy was required. She is now 8 months of corrected age and stays healthy in the outpatient clinic without signs of developmental delay; she already sits, rolls over, and pulls up to standing.","This woman was diagnosed with complications during her pregnancy, leading to premature labor and a very small baby. After a difficult delivery, she received intensive care to help her baby breathe and stay stable. Over the next few months, she and her baby received ongoing support to help her baby grow and develop, and she was eventually able to go home without needing a feeding tube."
341,"A 53-year-old female participant in the BCG-DENMARK-COVID trial was included in early June 2020 and randomized to BCG, which was applied intradermally in the right deltoid region. A rather strong local skin reaction to the vaccine followed, with clear, yellowish serous secretion from the injection site lasting 4 to 5 months. Additionally, swollen and sore lymph nodes in the axil on the vaccinated side were noted. The lymph node symptoms led to her being examined for breast cancer in January 2021. The participant had been BCG-vaccinated as a child at school entry, and a scar from the childhood vaccination was noted on her right shoulder at the trial inclusion procedure. According to her mother, she had also reacted strongly to the childhood BCG vaccination. As far as she (and her mother) knows, she has never been exposed to TB. The participant is healthy and takes no medication.\nBy the end of January 2021, she received the first Moderna Covid-19 vaccination in the left arm. She received no other vaccines during follow-up. The participant reacted to the Covid-19 vaccine with fever, muscle pain, and a large local reaction (the area being red and inflamed) which subsided within a few days. One to two days after vaccination, the trial BCG vaccination site scar began to itch and she experienced renewed secretion from the site, and the lymph nodes felt sorer. The itching and the secretion lasted for a week.\nThree weeks later, she received the second Moderna vaccination, after which she felt ill again. Also, this time the BCG site began to itch during the following day. There were no other symptoms, and the secretion from the BCG scar did not reappear. The itching lasted for 2 weeks. No treatment was needed or provided. Again, she did not notice symptoms from the childhood BCG scar.","This 53-year-old woman participated in a study about the BCG vaccine and experienced a strong reaction at the injection site, including a yellow discharge and swollen lymph nodes. Because of these symptoms, she was checked for breast cancer, but thankfully, nothing was found. She had also received the BCG vaccine as a child and had a scar from that vaccination. After receiving the Moderna COVID-19 vaccines, she experienced similar reactions, including itching and discharge from the BCG scar, but these symptoms resolved without needing any treatment."
342,"A 49-year-old female participant was enrolled and randomized to BCG in June 2020. The vaccine was applied intradermally in the right deltoid region. She was not aware of having received a BCG vaccination as a child. However, when she was included in the trial, a BCG scar was noted on the right arm. In October 2020, she received an influenza vaccination (Vaxigriptetra®). No reaction at the trial BCG scar was noted. The participant takes omeprazole daily as a treatment for her gastroesophageal reflux disease and levocetirizin (Xyzal®) for her chronic urticaria.\nLate December 2020, she received the Pfizer-BioNTech Covid-19 vaccine in the left arm with no subsequent reaction at any of the BCG scar sites. After her second dose of Pfizer-BioNTech vaccine late January 2021, also in the left arm, she noted itching, clear yellowish secretion, and some bleeding from the BCG scar site on the right arm. She did not experience swollen lymph nodes. The symptoms lasted for 2 weeks and resolved without treatment. There was no reaction at the site of the childhood BCG scar. She had no other symptoms after the Pfizer-BioNTech vaccine.","This 49-year-old woman received a BCG vaccine in June 2020, which left a scar on her right arm. She later received an influenza and then a Pfizer-BioNTech COVID-19 vaccine, with no problems at the scar site. However, after her second COVID-19 vaccine, she developed a localized reaction at the BCG scar, including itching and some bleeding, which resolved on its own after two weeks. She has no other symptoms and continues to feel well."
343,"We hereby report the case of a 16-year-old Caucasian female who has been followed at our Allergy Unit of Meyer Children's University Hospital in Florence for allergic asthma since the age of 6. At the physical examination, she presented a history of respiratory clinical manifestations such as cough and shortness of breath. During her asthma history, she also reported two hospitalizations: the first one during a wheezing episode triggered by an airway infection and the second one during an asthmatic attack without an infection. She required treatment with short-acting β2-agonist and systemic corticosteroids during her asthma exacerbations. The patient presented positive skin prick tests to house dust mites and cat fur from the first clinical evaluation and a positive skin prick test to pollen (grass, mugwort, hazel, birch, and poplar) during the follow-up.\nShe also suffered from food allergy, i.e., to nuts, with sensitization to lipid transfer protein and profilin. At the age of 2, the patient had anaphylaxis after eating cashew and adrenaline autoinjectors were prescribed. She had skin prick tests, prick by prick tests, and blood tests for nuts, and they resulted positive not only for cashew but also for peanut, almond, hazelnut, walnut, pine nut, and pistachio, which were all excluded from the diet. Moreover, with carrots and fennels, she presented itch in her throat and dyspnea. For this reason, following the positive skin prick tests, the patient also excluded these foods from the diet. At 16 years old, the patient presented anaphylaxis twice after eating a pear and shrimps, which were then excluded from her diet.\nApart from asthma and food allergy, she did not suffer from other illnesses. The patient reported a parental history of atopic disease: her mother suffered from nickel contact allergy and her father from rhinoconjunctivitis with grass and Parietaria pollen sensitization.\nWe have evaluated all the possible differential diagnoses with asthma or additional factors, which were eventually ruled out. For example, no clinical features of chronic bronchitis, cystic fibrosis, or gastroesophageal reflux were detected. The patient also underwent an electrocardiogram, which did not reveal any rhythm abnormalities.\nAfter the diagnosis of asthma, she attended periodic follow-up visits at our Allergy Unit, where spirometry was performed each time as well. Afterwards, at 16 years old, her asthma clinical manifestations worsened progressively, becoming severe despite treatment with high-dose inhaled corticosteroid, long-acting β2-agonist, and anti-leukotriene (fluticasone/salmeterol and montelukast) (). Indeed, she had frequent asthma exacerbations, especially in the evenings, about once every month, and dyspnea for minimal physical efforts while under these treatments. In addition, the patient presented a spirometry with a reversible lung obstruction. Indeed, the patient presented a basal FEV1 of 79% with a positive bronchodilatation test equal to 290 ml (+12%).\nThus, treatment with subcutaneous injections of the anti-IgE antibody omalizumab, 600 mg every 2 weeks, was started at the age of 16 years, although it was used as off-label due to her high total IgE serum concentration (2,003 kU/L). The patient's clinical condition benefitted from the treatment with omalizumab (, ), with clinical improvements after the first injection and with an improvement of the spirometry (FEV1 = 94%, with a negative bronchodilatation test) performed after the seventh injection.\nDuring the SARS-CoV-2 pandemic, the patient did not change her habits, including going to school, and on October 20, 2020, one of her classmates resulted positive for SARS-CoV-2 from real-time polymerase chain reaction (RT-PCR) on a nasopharyngeal swab. Therefore, she was sent home for quarantine. Indeed, she did not suffer from clinical manifestations typical of COVID-19, nor her asthmatic signs or symptoms did worsen at the time. However, after 7 days, she performed RT-PCR on a nasopharyngeal swab, which resulted positive for SARS-CoV-2. The patient was still without clinical manifestations at the time, but after 2 days she developed diarrhea, asthenia, myalgia, epistaxis, and maximum body temperature 37.5°C treated successfully with paracetamol. At that time, the patient had been undergoing therapy with subcutaneous omalizumab every 2 weeks for about 3 months, as well as fluticasone/salmeterol and montelukast daily (). Moreover, during quarantine, omalizumab treatment was easily continued at home, and it was switched to self-administration through telephonic support and digital material available online (), such as educational videos and the Asthma Control Test. It is worth mentioning that the patient remained free of asthma clinical manifestations the whole time she was positive of SARS-CoV-2, without significant differences in asthma management during this period (). Furthermore, no drug adverse events have been recorded. Finally, she reported good self-confidence with the administration of omalizumab at home.","This 16-year-old girl has had severe asthma and food allergies since she was a young child, including reactions to nuts, shellfish, carrots, and fennel. She’s had multiple hospitalizations for asthma attacks and has a family history of allergies. Despite taking medications like inhaled steroids and a new antibody, her asthma has gotten worse recently, leading to frequent flare-ups. After trying different treatments, including an anti-allergy injection, her condition improved significantly. During the COVID-19 pandemic, she tested positive for the virus but didn't experience any worsening of her asthma symptoms, and she successfully managed her medication at home with support."
344,"Our case concerns a 48-year-old male with a history of syphilis and heterozygous AS sickle cell trait. Mantle cell lymphoma with multiple lymphomatous polyposis had been diagnosed based on a bone marrow biopsy. The patient received three cycles of R-Maxi CHOP and three cycles of R-High Dose-Ara-C (Nordic protocol\n) with complete response on the CT scan. His peripheral blood stem cells (PBSC) were mobilized and harvested on his last cycle of R-High Dose Ara-C with granulocyte-colony stimulating factor (G-CSF) and plerixafor. Seven months after his diagnosis and two months after his last chemoimmunotherapy, he underwent the BeEAM protocol for an autologous HCT, which consists of bendamustine 200 mg/m2 intravenously given on days –8 and –7, cytarabine 200 mg/m2 intravenously twice a day on days –6 to –3, etoposide 100 mg/m2 intravenously twice a day on days –6 to –3, and melphalan 140 mg/m2 intravenously on day –2.\n He was only taking vitamin D supplements at the time.\nBaseline laboratory workup was unremarkable, as shown in . On day –8, he began the BeEAM protocol and received his first dose of intravenous bendamustine 440 mg (200 mg/m2). He also received intravenous fluids as part of the BeEAM protocol. From day –6, his urine output, serum sodium and serum creatinine started to increase (). His urine output markedly increased from 3725 mL on day –8 to 7425 mL on day –3 and the serum creatinine increased from 75 µmol/L on day –7 to 155 µmol/L on day –3. His serum sodium also increased to a peak of 155 mmol/L on day –2. During this time, the patient only complained of mild nausea. Vital signs were stable. Forced diuresis with furosemide was suspended and oral hydration was optimized.\nOn day –1, the patient started complaining of polyuria, nocturia and thirst. However, he could not drink, since he had severe mucositis and odynophagia due to his chemotherapy, which led him to be put on total parenteral nutrition a few days after. A nephrology consultation request was made. Laboratory studies revealed the following values: random urine sodium 56 mmol/L; random urine osmolality 307 mOsm/kg and specific urine gravity from urinalysis 1.006. Despite the normal range of urine osmolality, the nephrologist team suspected a nephrogenic diabetes insipidus (NDI) along with concomitant dehydration in absence of other identified causes of polyuria. However, no water deprivation test or desmopressin challenge was done since the patient had developed an acute kidney injury (AKI), and required fluids for his hypernatremia and his upcoming HCT. After the HCT, the patient’s infusion was first switched to 0.45% sodium chloride and subsequently to dextrose 5%. Serum sodium levels showed little improvement while fluid balance improved but remained negative. On day 5, desmopressin 1 mcg was administered subcutaneously with no effect on diuresis. The following day, a desmopressin (4 mcg) challenge was administered intravenously and showed a 33% increase from baseline urine osmolality of 204 mOsm/kg to an average of 277 mOsm/kg with a decrease in diuresis. These values were compatible with a diagnosis of diabetes insipidus. The patient remained polyuric and highly dependent on fluids due to his hypernatremia. Since he was partially responsive to the desmopressin challenge, intranasal desmopressin was started on day 8. The dose was increased until it reached desmopressin 40 mcg twice daily intranasally on day 11 (). A brain magnetic resonance imaging (MRI) was then performed on day 12 to rule out central lymphomatous infiltrates, which came back negative and therefore reinforced a diagnosis of partial NDI. On day 13, his natremia returned to normal range along with diuresis decreased to 600-800 cc/8 h, intravenous dextrose 5% was stopped. Desmopressin was weaned off and then stopped on day 15. By then, the patient’s nocturia had markedly improved and NDI was considered resolved. On day 18, the patient was discharged from the hospital. To this day, he remains in remission of his lymphoma.","This 48-year-old man was diagnosed with mantle cell lymphoma and had successfully completed chemotherapy. After a stem cell transplant, he developed a condition causing excessive urination and high sodium levels, which the doctors called nephrogenic diabetes insipidus. They treated this with desmopressin, a medication that helps the body regulate fluid balance, and he was able to return home in remission."
345,"A 38-year-old woman, 32+ 2 weeks pregnant, previous smoker with a past history of one miscarriage and livedo reticularis was admitted to the emergency department (ED) of her local hospital due to a sudden onset of pain, cold and functional impotence of the lower limbs. During the obstetric evaluation, fetal death was observed.\nGiven the suspicion of lower limb ischemia, low weight molecular heparin was started, and the patient was transferred to our hospital, which has vascular surgery. On admission in our ED, she was alert and oriented, hemodynamically stable and presented with tachypnea. Absence of pulses, cold and pallor of the lower limbs, with minimal neurosensory deficit and muscle weakness were observed. Laboratory workup showed hypocapnia, thrombocytopenia, elevation of liver and pancreatic enzymes, elevated total creatinine kinase (CK) and lactate dehydrogenase (LDH). Table resumes clinical and laboratory evolution during hospitalization. The Computed Tomography angiography (Angio CT) showed bilateral (central and lobar) pulmonary embolism (PE), deep venous thrombosis of the inferior vena cava and left iliac axis, areas of splenic and right kidney infarction and multiple arterial and venous thrombosis. Juxta-renal aortic thrombosis (Fig. ) was also observed as well as thrombosis of the left renal artery (with hypocaptation of the left kidney), right common iliac artery, left hypogastric artery, left common femoral artery, right deep femoral artery and right tibioperoneal trunk (Fig. ).\nAs lower limb ischemia was tolerated (acute limb ischemia - grade IIA), surgery was postponed. The patient was admitted to the intensive care unit (ICU) where presumptive CAPS diagnosis was made and anticoagulation with unfractioned heparin infusion was started. Despite anticoagulation, the patient presented worsening of neurosensory deficit and muscle weakness (acute limb ischemia - grade IIB), with the need of urgent revascularization surgery. Under general anesthesia, a cesarean section was performed to extract the dead fetus, and intrauterine balloon tamponade was placed for control of postpartum hemorrhage, followed by bilateral thromboembolectomy of the lower limbs and axillary-bifemoral bypass with synthetic prosthesis. Surgery was uneventful. Fetus had no anatomical abnormalities and weighed 1540 g. The patient recovered both lower limb perfusion as well as bilateral femoral, popliteal and distal pulses and was extubated the next day. During her stay in the ICU, the patient maintained therapeutic anticoagulation, underwent a total of six sessions of plasmapheresis and the first dose of rituximab (1000 mg) was administered. Under this treatment, an evident improvement was observed: the platelet count, hepatic enzymes and serum creatinine normalized. On the sixth day of hospitalization, she was transferred to an intermediate care unit at the hospital of her residence area where she continued TPE until the 14th day, when the second dose of rituximab was administered. After those treatments, the patient was discharged with a vitamin K antagonist. Regarding the initial laboratory workup, before plasmapheresis or rituximab infusions, antiphospholipid antibodies were negative (LA, aCL and β2GPI) as well as screening for thrombophilia (including protein C and protein S deficiency, antithrombin III deficiency, Leiden Factor V mutation and prothrombin mutations), and other autoimmune diseases. Histological examination of the placenta showed areas of small vessel infarction.\nSix months after discharge, the patient has resumed her day-to-day life activities. She remains under warfarin therapy and the bypass is functional, with distal pulses maintained and without intermittent claudication. Additional workup was repeated and continued to be negative for aPL (even with the patient taking warfarin, which could cause a false positive lupus anticoagulant) and other autoimmune diseases (including SLE, vasculitis and sarcoidosis), with normal complement counts (C3, C4 and CH50) as well as negative for thrombophilia, cryoglobulinemia, cold agglutinin disease, autoimmune hemolytic anemia, paroxysmal nocturnal hemoglobinuria and infectious diseases (negative for hepatitis B virus, hepatitis C virus, cytomegalovirus, Epstein Barr and toxoplasmosis). CD19 positive cells are still depleted (0,1%; normal 7–23%), due to previous rituximab infusions. Other extra-criteria antibodies testing was not available in the laboratory and was not performed [Table ].","This 38-year-old woman, who was pregnant, experienced sudden pain and numbness in her legs, which turned out to be caused by blood clots in her legs and lungs. Doctors discovered these clots were blocking blood flow to her kidneys and spleen, and she needed emergency surgery to restore circulation. During her hospital stay, she received treatments to dissolve the clots and manage complications, including a cesarean section to remove the stillborn baby. After several weeks of care, she is now recovering well and is being monitored to ensure the blood clots don't return."
346,"A 24-year-old man was admitted to our hospital 48 h after developing an acute retrosternal chest pain that was radiated to the upper back, associated with multiple episodes of vomit (food content), and progressive dyspnea. Additionally, he describes intermittent difficulty swallowing solid food. His medical history is significant for well-controlled asthma using salbutamol as needed.\nInitial evaluation revealed a temperature of 38.4 degrees; blood pressure, 100/60 mmHg; heart rate, 118/min; respiratory rate, 26/min, and SpO2, 94% on room air. Additionally, the physical examination showed subcutaneous emphysema in the cervical and thoracic regions, shallow breathing, dullness to percussion in both lung bases. His investigation results on admission revealed white blood cell count of 21,000/μL (91% Neutrophils), C-reactive protein (CRP) and procalcitonin were 39.2 mg/dL and 9 ng/mL respectively. Liver and renal function were normal. The patient was admitted with sepsis of unclear etiology, although there was a suspicious for esophageal perforation predisposing mediastinitis and sepsis.\nA contrast-enhanced thoracic computed tomography (CT), showed a pneumomediastinum, cervical emphysema, bilateral pleural effusion, as well as extraluminal oral contrast surrounding the distal portion of the gastro-esophageal junction region and fluid-air level indicating a collection in the posterior mediastinum (Fig. a–d). Initial management included intravenous fluid, nothing per oral (NPO), broad spectrum antibiotics, and analgesia. Due to the clinical condition of the patient, time of the rupture and inaccessibility to an intensive care unit due to the Covid 19 pandemic situation, the thoracic surgery and gastroenterology teams decided a nonoperative approach based on endoscopic therapy. The patient underwent endoscopy that showed a distal esophageal lineal tear just above Z line of approximately 4 cm with irregular edges. Irrigation and drainage of food debris of the cavity was performed before a distal auto-expandable esophageal prosthesis SX-ELLA (ELLA-CS) of 25 mm × 18 mm × 15 cm with antimigration technology and anti-reflux valve was placed (Fig. ). The thoracic surgeon decided to put a bilateral pleural tube oriented toward the perforation preventing future complications. Biopsies of the esophageal mucosa were obtained confirming the clinical suspicion of eosinophilic esophagitis (Fig. a).\nFive days after admission, a new endoscopic procedure was performed, showing migration of the esophageal stent into the stomach, a persistent perforation defect in the lower third of the esophagus with granulation tissue and two small cavities suggesting a fistulous tract. A fistulogram was performed confirming the fistulous tract between the esophagus and the pleura (Fig. a, b). Based on these findings and previous case reports found in the literature it was decided to place an endoluminal vacuum-assisted closure with sponge in the area of perforation with the fistula (Fig. c–e) to control both complications. The sponge was cutted to 7 cm, adjusted and grasped with a tripod equipped endoscope and introduced in the cavity under direct visualization. After placement of the sponge, a vacuum device was connected and set to a continuous 125 mmHg sub-atmospheric, moderate intensity pressure.\nIn the second intervention for dressing change of the sponge, two OVESCO clips (OTSC®) were placed as a strategy to reduce the size of the tear and closure of the fistula, reducing up to 30% of the longitudinal size. The patient required four additional dressing changes of endoscopic vacuum-assisted closure with sponge, each one performed every 72 h, until the fistulogram showed resolution of the esophago-pleural fistulous tract (Fig. a–c).\nDespite good progress of the esophago-pleural fistula, the patient condition got worse due Clostridium difficile colitis. Despite appropriate antibiotic treatment, and resucitacion with fluids the patient developed shock with severe acute respiratory distress syndrome requiring vasopressor support and mechanical ventilation with neuromuscular blockade. The gastroenterology team decided to place a new esophageal prothesis in order to avoid dressing changes of the sponge and worsening of the clinical condition. Considering Clostridium difficile infection, a recto-sigmoidoscopy was performed which reported ischemic colitis and pseudomembranes. Due to lack of improvement despite treatment due to uncontrolled foci of infectious (colon), an emergency left hemicolectomy and a Hartmann’s procedure were performed.\nOver the next 7 days, the patient condition improved, allowing to perform an endoscopy with fistulogram that showed a recurrent fistulous esophago-pleural tract (Fig. a). The esophageal prothesis was removed and a new vacuum-assisted closure with sponge was placed. Three dressing changes of endoscopic vacuum-assisted closure with sponge were performed before esophago-pleural fistula resolution was evident (Fig. b, c). The sponge and the OTSC® was retired due to a complete fistula resolution.\nA follow-up endoscopy performed 3 days after the last vacuum-assisted closure with sponge was removed, that showed epithelized esophageal mucosa with granulation tissue (Fig. d). After 46 days the patient was discharged with outpatient follow-up.\nTwo months later, the patient was asymptomatic, tolerating solids in the diet. A prednisone base therapy and food elimination diet were initiated as a measure of control of his eosinophilic esophagitis.","This man was admitted to the hospital after experiencing severe chest pain, vomiting, and difficulty breathing. Doctors found that he had an infection in his chest and esophagus, which was causing a hole to form between his esophagus and the space around his lungs. To help heal the hole, doctors placed a special tube in his esophagus to hold it open and prevent further complications. After several procedures and treatments, the infection cleared up, and he was able to go home with a plan to manage his underlying condition."
347,"A 50 years old male long-distance truck driver presented to our Hospital in Addis Ababa, Ethiopia with severe constant retrosternal chest pain, diaphoresis and vomiting of ingested matter for the previous two days. The patient had a history of hypertension and elevated blood cholesterol levels. He was brought to the emergency department after he experienced an acute loss of consciousness of short duration. He reported that, after an episode of severe chest pain, he was not aware of his surroundings and lost control over his truck for a few seconds. The truck went off the road but fortunately no one was injured. The patient had no previous history of heart disease, and no cough or pleuritic pain. There was no history of alcohol or cigarette use.\nPhysical examination was normal except for hypertension (blood pressure 160/100 mm Hg) and low-grade fever with axillary temperature of 37.5 °C. He was admitted to hospital. Initially he was investigated for an acute coronary syndrome. Echocardiogram findings and serum troponin levels were normal. On the second days of his admission, he experienced one episode of bloody vomiting. On further questioning, his physicians learned that the patient had pain on swallowing. They also discovered that he was taking ceftriaxone injections and Doxycycline 100 mg capsules twice per day for four days. The medications were prescribed in another health facility for a febrile illness. The patient had no prior history of esophageal disease.\nA gastroenterologist was consulted and esophagogastroduodenoscopy (EGD) was performed. There were multiple mucosal ulcerations in the proximal and middle esophagus (Figs. and ) as well as at the lower esophageal sphincter. In addition, hyperemia and erosions were seen in both stomach and duodenum. Mild bleeding was noted.\nLaboratory tests were normal except for a mild transient elevation of liver transaminases and a triglyceride level of 243 mg per deciliter. Serum albumin and bilirubin were normal. Hepatitis B surface antigen and Weil flex test were positive. Ultrasound of the abdomen showed increased echogenicity of the liver consistent with liver steatosis and fatty liver. There were no features of cirrhosis or portal hypertension. Chest X-ray, Complete blood count, blood film, and fasting blood sugar were all normal. H.Pylori stool antigen test, Hepatitis C. Virus and HIV antibody tests were negative. Diagnosis of Doxycycline-induced esophageal ulcerations was made, and doxycycline was discontinued. The patient was treated with ceftriaxone one gram intravenous twice daily to complete the course of treatment for the acute febrile illness and omeprazole 40 mg intravenous twice daily. He was also given antacid suspension orally. Parenteral analgesics were added as required. The pain and fever subsided gradually and the patient was discharged improved after 6 days, on omeprazole 20 mg orally twice daily for four weeks. He was advised to swallow pills in upright position and with water to prevent recurrence of similar problems. After 3 weeks, the patient returned for follow-up. He was asymptomatic. Liver transaminases were normal. Hepatitis B. Viral DNA level was 99 international unit per milliliter. Hepatitis e antigen was negative. Antiviral treatment was not indicated and the patient was linked to care for his hypertension and chronic hepatitis B infection.","This 50-year-old truck driver came to the hospital with chest pain, nausea, and a brief loss of consciousness after his truck went off the road. He has high blood pressure and high cholesterol, and it turned out that the medication he was taking for a recent fever was causing ulcers in his esophagus, leading to the pain and bleeding. After an endoscopy, doctors found several ulcers in his esophagus and stomach and stopped the medication causing the problem. He is now taking medication to protect his stomach and is being monitored for his high blood pressure and hepatitis B."
348,"A 55-year-old man presented with sudden onset of dizziness, diplopia, difficulty of closing eyes, and trouble of chewing after lifting heavy goods 3 days prior to admission. He had a history of mild hypertension for 1 year with treatment of amlodipine 2.5 mg/d and poorly-controlled asthma. On examination, he showed completed bilateral horizontal gaze palsy which was uncorrected by vestibuloocular reflex. Gazed-evoked upbeat nystagmus (UBN) was observed on attempted upward gaze but not on straight-ahead gaze position. In addition, he had bilateral peripheral facial paralysis with predominance on the left. Examination on other cranial nerves, including facial sensation, taste, hearing, and pharyngeal reflex, were normal. Mild ataxia was noticed on the left upper extremity when performing finger-to-nose test. His muscle strength was 5 on four limbs and he had normal pinprick sensation and brisk tendon reflexes. Brain MRI revealed hyperintensity in right middle cerebellar peduncle and bilateral dorsal pontine tegmentum on diffusion-weighted image, indicating new infarction (Fig. a). No periventricular white matter lesions were observed. CT angiography of vertebrobasilar artery showed no evidence of significant stenosis (Fig. b). CSF examination showed normal protein level and CSF analysis for oligoclonal band, myelin oligodendrocyte glycoprotein antibody, and aquaporin-4 antibody were negative. Contrast-enhanced transcranial doppler showed > 50 microbubbles during the Valsava maneuver, suggesting potential cardiac right-to-left shunt. Further transoesophageal echocardiography revealed patent foramen ovale (PFO; Fig. c) with a tunnel length of 12.6 mm. Transthoracic echocardiography revealed left atrial diameter of 32 mm and left ventricular ejection fraction of 70.4%. No left ventricle hypertrophy or atrial septal aneurysm was observed. Holter monitor examination in hospital did not capture remarkable arrhythmias, i.e. atrial flutter or atrial fibrillation. Rrivaroxaban 15 mg/d was prescribed at discharge and closure of PFO was then administrated. His symptoms relieved and follow-up MRI at 6 month showed an old infarction of the same region on T1 weighted image (Fig. d) without other evidence of demyelination.","A 55-year-old man experienced sudden dizziness, blurry vision, and difficulty with eye movements after lifting heavy objects. Tests revealed a stroke in a part of his brain that controls movement and balance, and a hole in his heart. The stroke was likely caused by a blood clot traveling from his heart to his brain, and he was treated with medication to prevent blood clots. His symptoms improved after addressing the heart issue and he is now being followed up to monitor his recovery."
349,"A 69-year-old Han man complained of chest distress and shortness of breath after stress for four months. He was diagnosed with hypertension for four months, and he felt that these symptoms were accompanied by high blood pressure. The maximum blood pressure measured was 200/110 mmHg. Benidipine hydrochloride (4 mg twice daily), metoprolol succinate (47.5 mg Qd), furosemide (20 mg Qd) and spironolactone (20 mg Qd) were applied to control blood pressure. In addition, he had a dull pain in his left waist for four months. Abdominal enhanced computerised tomography (CT) demonstrated a suspected left renal infarction. His past medical history included 10 years of diabetes mellitus and hyperlipidemia. During this hospitalization, he was diagnosed with resistant hypertension with chronic renal disease and renal dysfunction (creatinine 122 µmol/L, eGFR = 51.80 mL/min × 1.73 m2). Abdominal enhanced CT was reperformed and showed that his left renal artery was nearly occluded, his right renal artery had mild to moderate stenosis and his left kidney had atrophied. An invasive angiography with angiographic catheter JR 4.0 further demonstrated a 95% stenosis of the proximal segment of the left renal artery, and the middle segment was blurred with multi-channel-like blood flow (Fig. , Additional file ). An RDC guiding catheter was used and run through across the lesion. The proximal lesion was pre-dilated by a 4.0 mm × 15 mm balloon at 10 atm. A commercially available IVUS system (iLAB, Boston Scientific Corporation, Marlborough, Massachusetts) was used to acquire IVUS images. A 40 MHz, 2.6 F imaging catheter (Atlantis SR Pro or Pro 2, Boston Scientific) was advanced distal to the lesion, and an automated pullback was performed at a speed of 0.5 mm/s. Multiple lumens and HLS were demonstrated by IVUS, and the cavity was surrounded by fibrous tissue (Fig. , Additional file ). A lesion of the middle segment of the left renal artery was sequentially dilated by a 1.5 × 15 mm balloon, 2.5 × 10 mm balloon and 4.0 × 15 mm balloon at 8–12 atm, and an express SD 5.0 × 19 mm stent was implanted at 12 atm successfully, overlapping with the proximal segment of the left renal artery (Fig. , Additional file ). The final angiogram showed restored flow to the distal renal artery. The patient recovered well and was discharged with atorvastatin (20 mg Qd), ezetimibe (10 mg Qd), aspirin (100 mg Qd), clopidogrel (75 mg Qd), Sacubitril Valsartan Sodium (25 mg Bid) and metoprolol succinate (47.5 mg Qd). Over three months of follow-up, the patient achieved complete remission of chest distress, shortness of breath and waist pain. Blood pressure was well controlled around 130/75 mmHg and renal function was recovered (creatinine 96 µmol/L, eGFR 69.21 mL/ (min × 1.73 m2).","69-year-old man came to the hospital because of chest pain and shortness of breath that had been bothering him for months. He also had high blood pressure and a history of diabetes. Tests revealed a blocked artery in his left kidney, which was causing the pain and contributing to his high blood pressure. After a procedure to open the blocked artery with a stent, his symptoms improved, and his blood pressure and kidney function returned to normal."
350,"A 14-month-old Congolese male child with oculocutaneous albinism visited the Mbujimayi pediatric clinic with fever and fatigue. He was originally from Kasai Oriental, a region of the Democratic Republic of the Congo. He was the youngest in a family of five children, three of whom had oculocutaneous albinism. He himself has sickle cell disease and the rest of the siblings have a sickle cell trait (Fig. ). He was born at term with a birth weight of 3000 g, and presented with spontaneously resolutive neonatal jaundice. The other significant history was episodes of fever with a monthly frequency of two episodes. No vaso-occlusive crisis was clearly diagnosed, in particular no dactylitis episode was reported. The vaccination schedule according to the expanded program of immunization in the DRC was respected with Bacille Calmette et Guérin (BCG) vaccine against tuberculosis; diphtheria, tetanus and pertussis (DTP) vaccine; oral polio vaccine (OPV) against polio; hepatitis B and haemophilus influenzae vaccine (HepB-HiB1); rotavirus vaccine (Rotasiil1); pneumococcal vaccine (Prevenar); measles vaccine (VAR); and yellow fever vaccine (AAV). The patient has never been hospitalized and has always been treated on an outpatient basis in health centers during febrile episodes such as malaria or typhoid fever. The siblings reported no particular clinical history.\nHis parents are not albinos and have no specific medical history; they never benefited from a prenuptial test. They reported their fear of the judgment of others because they have albino children when they are not affected.\nThe patient was wide awake with good contact and interaction. Generalized pallor was noted with subicteric conjunctivae, and the irises were bluish gray and translucent, thus appearing red with nystagmus (Fig. ). The patient’s skin was pinkish white, depigmented, without any particular lesions or bruises.\nOn physical examination, weight and height were at the third and tenth percentile for age, respectively. There was no fever (temperature 36.5 °C), the respiratory rate was high (53 cycles/minute), as well as the heart rate (176 beats/minute), but the latter was regular with the presence of a 1/6 systolic murmur at the mitral focus. The oxygen saturation was 94%. The oral examination was normal. The lymph nodes were free, without lymphadenopathy. The lung examination was normal. The abdomen was supple and painless. There was stage III splenomegaly according to the Hackett classification. Laboratory tests showed a hemoglobin level of 48 g/L and white blood cells at 13,200/μl (complete blood count performed with the Sysmex poch-100i; Sysmex, Norderstedt, Germany).\nThe diagnosis of SCD has been suggested on the basis of pallor, jaundice, and severe anemia. It was confirmed first by a positive rapid test (BioMedomics, Inc, Morrisville, USA) and then by hemoglobin electrophoresis.\nThe diagnosis of albinism was proven by molecular genetics on a blood sample and identification of the mutation involved, that is homozygosity for the 2.7 kb deletion of OCA2 (laboratory “Centro Nacional de Biotecnologia CNB-CSIC Campus de Cantoblanco, Darwin 3, 28049 Madrid, Spain). A family investigation was then performed (see Fig. ).\nGiven the very low hemoglobin level, the patient’s age, and SCD, a blood transfusion was indicated. Antibiotic treatment (ceftriaxone and amikacin) was started in the hospital. The evolution was favorable. On discharge from hospital, routine prophylaxis with folic acid and oral penicillin was initiated, and advice on crisis prevention and medical monitoring was given to the parents.\nA dedicated patient monitoring program has been set up for sickle cell anemia and albinism with hygiene advice and sun protection measures. The patient received a hat, sunglasses, and sun protection cream. Ophthalmologic follow-up has also been set up. The evolution remains dermatologically stable, no skin lesion was observed. For SCD, the patient received treatment with hydroxyurea since early 2020. It is still too early to objectify the benefit of this therapy but he had only one infectious episode, one vaso-occlusive crisis, and did not require blood transfusion for over a year.","This 14-month-old boy from the Democratic Republic of the Congo has two rare conditions: oculocutaneous albinism and sickle cell disease. He’s been experiencing fevers and fatigue, and his skin and eyes appear pale and bluish. Tests confirmed his diagnoses, and he received a blood transfusion and antibiotics to help him feel better. The doctors are closely monitoring his condition and providing him with sun protection and ongoing care to manage his health."
351,"A girl presented with asymptomatic proteinuria, which was revealed by a urinary screening test performed in Japan when she was 3 years old. She had no family history of renal or neurological disorders. The urine protein to creatinine ratio (UPCR) was 1.0–1.5 g/gCr (reference range < 0.2 g/gCr) at that time. Ultrasonography revealed normal echogenicity in both kidneys. She developed nephrotic syndrome, without systemic edema, at the age of 8 years. Polyuria and polydipsia with a urine output of 3L in a day were also documented. UPCR was 11.5 g/gCr, and the serum albumin level was 2.1 g/dL (reference range 3.7–5.5 g/dL). The serum creatinine level was 0.97 mg/dL (eGFR was 46.8 mL/min/1.73m2). The urine specific gravity was 1.008, and urinary beta 2-microglobulin increased to 9,269 µg/L (reference range ≤ 150 μg/L). A kidney biopsy revealed that 13 (52%) of 25 glomeruli showed segmental or global sclerosis. Furthermore, two glomeruli showed cellular lesions, which were characterized by swollen, vacuolated, and proliferative glomerular epithelial cells, throughout Bowman’s space. The underlying glomerular capillaries were partially collapsed and occluded by swollen endothelial cells and karyorrhexis, which was consistent with a pathological diagnosis of FSGS (Fig. A) []. No glomeruli with collapse and overlying podocyte hypertrophy and hyperplasia were not observed. Cystic dilatations of the tubules and interstitial fibrosis were also observed (Fig. B). The patient presented with no neurological manifestations, such as seizures or developmental delays. Brain magnetic resonance imaging (MRI) and electroencephalogram detected no abnormalities. She could hold her head up at 4 months old, sit at 8 months old, pull up to stand at 9 moths and speak single words at 1 year and 6 months old. She did not need special support to attend school. Her renal function continued to deteriorate, and she eventually developed ESRD, despite the administration of angiotensin receptor blockers. At 9 years of age, pre-emptive kidney transplantation was performed, with a kidney donated by her mother. No recurrence of proteinuria has been observed for 1 year and 9 months after transplantation.\nWe performed whole-exome analysis using a previously described method [], focusing on variants in the genes that are currently known to cause FSGS or nephronophthisis (Tables S and S), and identified a de novo novel heterozygous C to A transition (c.1461C > A) in the last exon of TRIM8, resulting in a premature stop codon (p.Tyr487*). The alternative and reference allele counts were 68 (46%) and 80 (54%), respectively. Sanger sequencing showed that the individual had the variant but that her parents did not. This variant was absent in population databases including the Exome Aggregation Consortium database (ExAC, ), Genome Aggregation Database (gnomAD, ), 1000 Genomes (1000G, ), ESP6500 (). No additional pathogenic variants in the genes that are currently known to cause FSGS or nephronophthisis were identified (Tables S and S). This variant was classified as pathogenic (PVS1, PM1, PM2, PM6, and PP4) based on the criteria developed by the American College of Medical Genetics and Genomics [].\nThe sequence analysis of mRNA was performed as previouly reported methods []. RNA was extracted from peripheral blood mononuclear cells with the RNeasy Mini Kit (QIAGEN), according to the manufacturer’s instructions. The RNA was treated with DNase (QIAGEN) to avoid genomic DNA contamination, and 200 ng of total RNA was reverse transcribed, using the SuperScript VILO cDNA Synthesis Kit (Thermo Fisher Scientific) for the mRNA analysis. The following primers were used to amplify and sequence exon 6 of TRIM8 from cDNA: 5’-GAGTGTCCCCCTGTACCCTT -3’ (forward) and 5’-CTACAGGGTGTATGGGCAGC-3’ (reverse). Polymerase chain reaction experiments were performed, using Invitrogen Platinum II Taq Hot-Start DNA Polymerase (Thermo Fisher Scientific) and T100TM Thermal Cycler (Bio-Rad Laboratories) identifing mRNA sequences transcribed from the TRIM8 mutant allele (Fig. ), which confirmed the escape from nonsense-mediated mRNA decay (NMD) [].\nImmunohistochemical analysis were performed using the formalin-fixed paraffin-embedded kidney biopsy specimens obtained from the present patient and nine control individuals consisting of three living kidney transplantation donors who served as normal controls, five patients with primary FSGS and one patient with nephronophthisis who served as disease controls. We performed autoclave-based antigen retrieval, for 15 min at 105 °C, in Bond Epitope Retrieval Solution 2 (Leica Biosystems Newcastle, Ltd., Newcastle Upon Tyne, UK). Specimens were incubated with goat polyclonal antibody against an epitope corresponding to amino acids 540–551, at the C-terminus of human TRIM8 (Abcam, Cambridge, MA, USA; catalog no. ab4302), overnight, at a dilution of 1:500 []. Immunofluorescence staining, using the anti-TRIM8 antibody in normal control specimens, is shown in Fig. (Fig. A–E). TRIM8 expression was observed in the nuclei of all glomerular cells (Fig. A–C). Double immunostaining with mouse anti-human podocalyxin monoclonal antibody (PHM5, Merck Millipore, Darmstadt, Germany), at a dilution of 1:100 which was used as a podocyte marker (Fig. B) [] and mouse anti-human cluster of differentiation 34 (CD34) monoclonal antibody (QBEND/10, Leica Microsystems, Wetzlar, Germany), at a dilution of 1:40 which was used as an endothelial cell marker (Fig. C) [] showed that TRIM8 was expressed in the nuclei of podocytes and endothelial cells, respectively. Proximal tubular cells that were identified by mouse anti-human cluster of differentiation 10 (CD10) monoclonal antibody (56C6, Leica Microsystems, Wetzlar, Germany) without dilution [] and distal tubular cells that were identified by mouse anti-human epithelial membrane antigen (EMA) monoclonal antibody (Clone E29, Dako, Santa Clara, California, USA) without dilution [] also showed the nuclear expression of TRIM8 protein (Fig. D and ). Similar findings were observed in specimens from disease controls (Fig. S). In contrast, the present patient showed a lack of TRIM8 protein expression in any cells in the glomeruli and tubules (Fig. F–J). The tubules showing cystic dilatation were positive for EMA, but negative for CD10, indicating that cystic dilatation was evident in the distal tubules (Fg. I and ). IHC staining, using anti-SOCS1 goat polyclonal antibody (Abcam, catalog no. ab9870), at a dilution of 1:500 [] of the kidney biopsy specimens derived from the present patient showed stronger cytoplasmic SOCS1 expression in glomerular and tubular cells than observed in control samples (Fig. S).","This young girl started experiencing problems with her kidneys when she was 3 years old, first with a slight increase in protein in her urine. By age 8, she developed a serious kidney disease called nephrotic syndrome, which caused swelling and other symptoms. Over time, her kidney function continued to decline, eventually leading to kidney failure. At age 9, she received a kidney transplant from her mother, which has been successful so far. Recent genetic testing revealed a new variation in a gene called TRIM8 that may have contributed to her kidney disease, and researchers are continuing to study this finding."
352,"A 71-year-old male was admitted to the hospital due to productive cough along with low grade fever, chest pain and shortness of breath. His past medical history included hypertension and glaucoma, and he took irbesartan regularly (150 mg per day). The patient did not smoke cigarettes, drink alcohol or use recreational drugs. No relevant travel history or contact history were detected. The patient had no food or drug allergies.\nThree weeks before admission, the patient began to have productive cough, with chest tightness and a temperature of 38 °C. After 2 weeks of progressive symptoms, the patient visited the local hospital. He reported pleuritic chest pain of visual analogue scale score 2. His vital signs and other physical examination results were reported as normal. Initial blood test showed elevated white blood cell (WBC) count (14.8 × 109/L) and C-reactive protein (CRP) level (86 mg/L) as well as liver enzyme elevation. Other laboratory test results were normal. Chest computed tomography (CT) revealed patchy opacities in both lower lobes and a small amount of right-sided pleural effusion. He was then admitted to the local hospital and received intravenous sulperazon (cefperazone–sulbactam) 2.0 g once every 8 h, but symptomatic improvement was not noted. Repeated chest CT scan revealed increased pleural effusion in the right. Subsequently, the patient was transferred to our hospital for treatment.\nOn the admission, his temperature was 37.8 °C, pulse rate 109 beats/min, respiratory rate 18 breaths/min, blood pressure 145/87 mmHg, and oxygen saturation 98% on room air. The patient reported no night sweats, weight loss, joint pains, or myalgias. Pulmonary auscultation found decreased breath sounds on both lower fields. No icterus or lymphadenopathy was detected. Thoracocentesis was performed immediately and a chest tube was introduced. Purulent and hemorrhagic fluid was aspirated (Fig. A), and laboratory tests of the pleural effusion revealed an exudate (fluid protein 25.70 g/L, serum protein 28.70 g/L [normal: 35.0–55.0 g/L], fluid lactate dehydrogenase 621 U/L, serum lactate dehydrogenase 355 U/L [normal < 243 U/L]). Other pleural fluid analysis showed: WBC 5000/µL with 88% neutrophils, red blood cells 21,000/µL, fluid glucose 7.31 mmol/L, and fluid adenosine deaminase 18 U/L. Pleural fluid smear, gram stain, bacterial culture, acid-fast bacilli culture and smear, and cytology were all negative. Blood culture was also negative. He was diagnosed as empyema and intravenous tazocin (piperacillin-tazobactam) 4.5 g once every 8 h was initiated.\nDuring the treatment, repeated blood and fluid cultures were all negative, so the pleural effusion was sent for NGS. Deoxyribonucleic acid (DNA) was extracted directly from the pleural fluid. The extracted DNA was amplified, purified, and sonicated to a size of 200–300 bp. Fragmented DNA was end repaired to construct DNA libraries, and supplemented with adapter overnight followed by polymerase chain reaction (PCR) amplification. Qualified DNA libraries were sequenced using the BGISEQ-100 platform. We generated high-quality sequencing data by removing low-quality reads, low-complexity reads, and reads shorter than 35 bp. Human data were mapped to a human reference (hg19) and excluded. The remaining sequencing data were aligned to the bacterial, virus, and fungal databases. All pathogen sequence reference databases were from National Center for Biotechnology Information (NCBI) ().\nNGS identified 14 out of 19317 reads uniquely corresponding to S. constellatus. Although the number of the reads was small, S. constellatus was the only pathogen detected. Tazocin was used continuously for 12 days, then followed by moxifoxacin 400 mg per day for a total of 14 days. Meanwhile, the pleural effusion was drained continuously through the chest tube. The patient claimed gradually resolved symptoms under antibiotic treatment and effusion drainage, and subsequent CT scanning confirmed the improvement (Fig. B, C).","This 71-year-old man was admitted to the hospital because of a worsening cough, fever, and chest pain. Initial tests showed an infection in his lungs, with fluid buildup around his lungs, and elevated white blood cell count. After drainage of the fluid, tests revealed a bacterial infection, and he was treated with antibiotics and drainage. The tests also identified the specific bacteria causing the infection, which helped guide his treatment."
353,"A 13-year-old male patient had a history of progressive subungual melanosis in the hallux of the right foot for over 4 years, but no pseudo-Hutchinson signs (Fig. A). After resection, the tumor was pathologically diagnosed as a subungual melanocytic nevus. The defect of the nail matrix was repaired with a lateral toe pulp island flap based on the plantar digital artery (Fig. B and C). The patient was monitored for 16 months and made a full recovery after surgery (Fig. D–F).","A 13-year-old boy had a dark spot growing under his toenail for several years. Doctors removed the spot, which was found to be a benign mole. They then repaired the area where the nail used to be with a flap of skin from his foot, and the toe healed well. The patient is being monitored to ensure the repair is successful, and he is recovering fully."
354,"A 5-year-old female patient had a history of progressive subungual melanosis for more than 2 years and intermittent pain in the fifth toe of her right foot for more than half a year (Fig. A). After resection, the tumor (total nail matrix) was pathologically diagnosed as a subungual melanocytic nevus of the fifth toe. The defect of the total nail matrix was repaired with a lateral toe pulp island flap based on the plantar digital artery (Fig. B and C). The patient was monitored for five months and had a satisfactory outcome (Fig. D and E).","This 5-year-old girl has had darkening of her nails for a while, and recently started experiencing pain in her fifth toe. A small growth was found under her nail, and doctors removed it completely. They then used a piece of skin from her toe to cover the area where the growth was removed. After checking her toe for five months, the doctors are happy with how it’s healing and the skin looks good."
355,"A 1-year-old boy was pathologically diagnosed with a subungual melanocytic nevus on his right index finger (Fig. A). The patient had been suffering from progressive subungual melanosis for over half a year. After removing the nail plate, split-thickness excision of the pigmented nail bed lesions was performed under a microscope (Fig. B and C). Furthermore, the residual nail bed was flattened under a microscope (Fig. D). The patient was monitored for 20 months and had a satisfactory outcome (Fig. E).","A one-year-old boy had a dark spot growing under his fingernail that was getting bigger over time. To remove the spot and prevent it from spreading, doctors carefully took it out and smoothed the area underneath. They monitored the area for 20 months, and it has remained healthy and without any further changes. The doctors are confident that the treatment was successful."
356,"This boy, born at 30+ 1 weeks of gestation to a 32-year-old mother, was the first child of non-consanguineous Caucasian parents. The mother was overweight before pregnancy, with a BMI of 30. Elevated maternal serum biochemical markers in the first trimester of pregnancy prompted a prenatal noninvasive test for trisomy 21, which was negative.\nThe mother consulted at her local hospital, a type 2 perinatal centre, for headaches that had worsened over 48 h. The examination found arterial hypertension (145/95 mmHg) with no other sign of preeclampsia. Foetal heart rate monitoring was non-reassuring, with reduced variability and decelerations. Ultrasound and Doppler assessment showed decreased active foetal movements, absent diastolic flow in the umbilical artery, and cerebral vasodilation (resistance index = 0.5). Intramuscular betamethasone (12 mg) was administered to the mother, and caesarean delivery was decided on 1 h later in a context of more pronounced decelerations.\nApgar scores were 4/7/10 at 1, 5 and 10 min, respectively; arterial cord blood pH was 6.97 and cord lactate was 16 mmol/L. Birthweight was 930 g (<3rd centile, according to Olsen curves []), and head circumference was 25.5 cm (3rd-10th centile). Pathological examination of the placenta found four foci of infarction, with size varying between 7 and 12 mm in the major axis, representing < 10% of placental volume. Examination outside these areas was considered normal. The neonate was bagged with 30% oxygen for a few minutes and then supported with nasal continuous positive airway pressure (CPAP). Peripheral venous catheterization was performed to provide standard hydration, vitamin K (1 mg), and a bolus dose of caffeine (20 mg/kg). The newborn was then transferred to a type 3 NICU.\nOn admission, 3 h after birth, the fraction of inspired oxygen (FiO2) required to maintain adequate oxygenation had increased to 70%, prompting surfactant administration (200 mg/kg of poractant alfa), according to the less invasive surfactant administration procedure with propofol (1 mg/kg) for premedication, as stated in our service protocol []. Soon after, the FiO2 required to maintain adequate oxygenation decreased to 25% and capillary blood gases showed improved pH (7.28) and lactate (6.8 mmol/L) compared to cord blood values. An epicutaneocaval catheter was inserted into the left basilica vein at the 7th postnatal hour to perfuse PN with a separate glucose-amino acid solution (2.7 ml/h of Pediaven NN2, Fresenius Kabi, Sèvres, France), an additional solute of amino acids (0.3 ml/h of Primène 10%, Baxter, Maurepas, France), and ILE (0.2 ml/h of Medialipide 20%, B. Braun Medical, Boulogne, France), the whole providing 7 g/kg/day of carbohydrates, 2 g/kg/day of proteins, and 1 g/kg/day of lipids, with carnitine (8 mg/kg), multivitamins (Cernevit, Baxter, Maurepas, France) and trace elements (Nutryelt, Aguettant, Lyon, France). Enteral feeding was initiated, with 16 ml per day of donor human milk administered continuously through a gastric tube. Thrombocytopenia (33,000/mm3, see reference ranges for newborns and infants used by our laboratory in the Table (Table )) and impaired coagulation tests (prolonged prothrombin time – with a decrease in all vitamin K-dependent factors – and partial thromboplastin time, fibrinogen < 0.35 g/L) prompted the administration of 20 ml/kg of fresh frozen plasma.\nTwenty-four hours after birth, the respiratory state was stabilized with CPAP + 6 cm H2O and FiO2 24%; haemodynamics was adequate with heart rate: 152 bpm, mean arterial blood pressure: 35 mmHg, and diuresis: 5.2 ml/kg/h over the first 24 h. Five episodes of hypoglycaemia, ranging between 1.4 mmol/L and 2.5 mmol/L and occurring after PN initiation, had required 10% glucose intravenous boluses. The PN prescription was changed to (i) a preparation in the department of a mixture of glucose, proteins and electrolytes corresponding to respective glucose and protein intakes of 10 g/kg/day and 3 g/kg/day, infused at a rate of 4.1 ml/h, and (ii) continuation of the same ILE at a rate 7 ml/day, i.e. 0.3 ml/h, corresponding to lipid intake of 1.5 g/kg/day. The exact wording for the parenteral nutrition over the next 24 h was: (i) Preparation (with details on the different solutes of the mixture): total 98 ml, at a flow rate of 4.1 ml/h, and (ii) Medialipide 20%: 7 ml, given separately from the main mixture. The prescription, however, was misinterpreted by the nurse, who administered ILE at a rate of 7 ml/h. The error was identified 4 h later by the physician who visited the infant for an increase in oxygen requirements. The infant was found to have tachypnoea, with a respiratory rate of 60–70 breaths per minute compared to 40–50 in the preceding hours. Other vital signs were normal, lungs were clear to auscultation, there was no cardiac murmur, and neurologic examination was normal. ILE was immediately stopped, but it was estimated that the infant had received 5.6 g of this emulsion, i.e. 25 mg/kg/min of lipids over 4 h, before cessation.\nThe infant’s condition rapidly deteriorated. Four hours after stopping ILE, the infant was still tachypnoeic, and the FiO2 required to maintain adequate oxygenation had increased to 50%. The infant’s alertness, muscle tone and motor skills were preserved. Values for systolic, diastolic, and mean blood pressure (respectively 49, 27, and 35 mmHg) were comparable to those observed in the previous hours. Capillary blood gases showed pH: 6.98, PCO2: 56 mmHg, PO2: 45 mmHg, bicarbonate: 13 mmol/L, and lactate: 11.4 mmol/L. Echocardiography found markers of pulmonary hypertension (PH), with peak tricuspid regurgitant jet velocity reaching 3 m/s – for an estimated systolic pulmonary artery pressure of 35–40 mmHg − and a predominant right-to-left shunt in the ductus arteriosus. Nitric oxide (20 ppm) was administered on the inspiratory branch of the CPAP circuit, and transcutaneous blood gas monitoring was implemented. After a transient improvement lasting a few hours, with FiO2 reduced to 35%, acute respiratory distress syndrome developed (Fig. ). This required intubation and high-frequency oscillatory ventilation 10 h after the lipid overdose. Chest X-ray following intubation showed reticular infiltrates with a positive air bronchogram and reduced lung volume (Fig. ), prompting the administration of a second dose of surfactant.\nShock occurred in the following hours, with collapsed blood pressure and anuria, refractory to continuous alkalization with 4.2% bicarbonate, albumin, norepinephrine, and an attempt to restart diuresis with furosemide (Fig. ). Successive echocardiographs while the infant was still being treated with inhaled nitric oxide showed persistent PH, as previously described, normal left ventricular function (fractional shortening 30–35%), adequate cardiac output (estimated at 200–250 ml/kg/min), and absence of cardiac thrombus or pericardial effusion. Bleeding in the gastric and tracheal tubes and at the entry of the epicutaneocaval catheter, associated with profound thrombocytopenia (< 10,000/mm3), prompted the transfusion of platelets, red blood cells, and fresh frozen plasma.\nSeventeen hours after the end of lipid overload, biological assays revealed a very high serum triglyceride level, 51.4 g/L, moderate cytolysis (alanine aminotransferase: 91 IU/L, aspartate aminotransferase: 151 IU/L, creatine kinase: 1002 IU/L, lactate dehydrogenase: 1936 IU/L), renal failure (creatinine: 122 μmol/L), haemodilution (protein: 20 g/L, albumin: 11 g/L, sodium: 130 mmol/L), hyperglycaemia (26 mmol/L), hypophosphataemia (0.86 mmol/L), normal calcium (2.36 mmol/L), and increased lactate (12.2 mmol/L). Persistent thrombocytopaenia was associated with decreased neutrophil count (340/mm3). Coagulation tests could not be performed at this time point because of extreme lipaemia.\nThe first exchange transfusion (ET) was started 5 h later, i.e. 22 h after stopping ILE, a delay required for the placement of a double lumen umbilical venous catheter and the reception of blood products. After the exchange of 210 ml, i.e. approximately a 3-volume exchange transfusion, this technique reduced the triglyceride level to 13.72 g/L. The same technique with the same exchanged volume was repeated a few hours later, and the last analysis 43 h after the lipid overload showed a reduction in the triglyceride peak by 14- to 15-fold (Fig. ).\nThe infant’s condition nevertheless remained critical, with persistent bleeding and shock despite repeated transfusions of platelets and fresh frozen plasma, catecholamines, the vasopressin analogue terlipressin and broad-spectrum antibiotics (cefotaxime, amoxicillin and amikacin). Glycaemia was constantly > 25 mmol/L despite continuous insulin infusion (0.1 IU/kg/h). A peritoneal catheter was placed, but the renal replacement therapy was ineffective in producing significant ultrafiltration and reversing oedema and acidosis. Two blood cultures and a peritoneal fluid culture did not identify any pathogens. Hypofibrinogenaemia (0.5 g/L), with prolonged coagulation times beyond the measured thresholds, was observed. Factors II, V, VII and X ranged from 11 to 19%. The infant died 96 h after birth, i.e. 69 h after lipid overload, in a context of refractory lactic acidosis (Fig. ).\nA few blood tests could be carried out a posteriori on samples preceding the lipid overload kept in the biochemistry laboratory. The acylcarnitine profile found increased free carnitine (235 μmol/L) and increases in short- and medium-chain acylcarnitines, which could be attributed to supplementation. We observed no increase in long-chain acylcarnitines, potentially suggestive of carnitine palmitoyl transferase 1 deficiency, but classically observed with carnitine supplementation. Amino acid chromatography showed an increase in alanine and proline, as frequently observed in relation with lactic acidosis and birth asphyxia. In addition, a decreased ratio of branched-chain amino acids/(phenylalanine + tyrosine) was suggestive of hepatic dysfunction.\nParents were immediately informed of this serious adverse event associated with care. They did not want an autopsy to be carried out, but they gave their consent for whole-exome sequencing for themselves and their infant to search for abnormalities, particularly metabolic diseases, which might have favoured such a severe clinical expression of lipid overload. Whole-exome sequencing did not identify a pathogenic variant that could be related to the infant’s symptoms. The event was reported to the quality and risk management department of our institution, to the regional pharmacovigilance center and to the regional health agency. It was entered in the national pharmacovigilance database, for transmission to the European and global pharmacovigilance databases (EudraVigilance and VigiBase).","This baby was born prematurely at 30+1 weeks and had a difficult start to life, with low blood oxygen levels and signs of infection. Despite receiving multiple medications and treatments, including specialized feeding and oxygen, the baby developed a severe reaction to a medication called ILE, leading to dangerously high levels of fat in the blood and ultimately, a fatal outcome. Throughout his stay in the hospital, doctors carefully monitored his condition and tried various interventions to support his breathing and overall health. A thorough investigation was conducted to understand the cause of the baby’s illness and prevent similar events in the future."
357,"A 25-year-old woman, without previous medical history, presented for infertility lasting for more than one year. Clinical examination was normal but abdominal and pelvic computed tomodensitometry (CT) revealed a cyst of the left ovary associated with abundant peritoneal ascites that could correspond to mucinous material. Pelvic magnetic resonance imaging (MRI) confirmed ascites and showed a heterogeneous mass of the left ovary measuring 8.4 × 6.8 cm with adipose, solid and cystic regions that were suggestive of a dermoid cyst. The right ovary and uterus seemed normal. No other lesion was seen in the rest of the body, notably in the digestive system. In this context, surgery by left oophorectomy with appendicectomy and omentectomy was performed 3 months after the first consultation, without resorting to additional hyperthermic intraperitoneal chemotherapy (HIPEC). Intra-operative examination revealed mucinous material inside the peritoneal cavity and a normal digestive tract with a normal appendix. There was no complication of the surgery. The 5-month follow-up based on clinical and imaging surveillance revealed no complaints. Without relapse, the patient was able to pursue her plan to have a child.\nMacroscopically, the left ovary was cystic measuring 9.5 × 7 × 7 cm and weighing 305 g. It was ruptured on 4 cm. Its cut section revealed a heterogeneous and viscous mass with hair. The appendix, measuring 6 cm in length, and the omentum were macroscopically normal. Histologically, the ovarian cyst corresponded to a mature pluritissular teratoma with intermingled skin and pilosebaceous annexes, serous and mucinous glands, respiratory epithelium, adipose tissue and smooth muscle (Fig. ). The organoid areas with the aspect of a colon, representing about 20% of the ovarian cyst, were composed of colonic mucosa, muscularis mucosae, and submucosa from the surface to the depth. A thick muscularis propria was also observed. In the colonic mucosa, some glands were elongated and layered with moderate proliferating epithelial cells with minimal atypia, near to mucin pools stained with Alcian blue. The colonic epithelial cells were immunohistochemistry stained with CK20 and CDX2, and showed heterogeneous staining for CK7. These cells were negative for estrogen and progesterone receptors (Fig. ). The ovarian surface was covered with hyperplastic mesothelial cells and presented acellular mucinous pools, also found in the omentum. The left fallopian tube was normal. The appendix examined in totality was histologically normal besides mucin deposits on the surface of the serosa. It did not present any mucocele or LAMN/HAMN. All together, these data suggested a diagnosis of acellular PMP (according to Carr classification []) caused by a ruptured appendiceal-like mucocele associated with LAMN, in a left ovarian teratoma.\nNext generation sequencing of the LAMN of the teratomatous mucocele revealed an activating mutation of KRAS gene c.35G > A corresponding to the p.(Gly12Asp) substitution (Fig. ). Complementary molecular analysis by SNaPshot showed an associated mutation of GNAS c.602G > A resulting in p.(Arg201His). No mutation was found by these two techniques on the other tissues of the ovarian teratoma (squamous, respiratory, adipose or smooth muscle elements) or in the normal appendix and ovarian parenchyma (Fig. ).","A 25-year-old woman who had been trying to conceive for over a year came to the hospital to investigate the cause of her infertility. Tests revealed a large cyst on her left ovary with some fluid around it, and further imaging confirmed a complex mass within the cyst. Surgery to remove the cyst, appendix, and a small part of her abdomen was performed, and the tissue samples showed it was a type of mature teratoma with a small area resembling a colon. The genetic testing revealed specific mutations in genes that could explain the condition, and thankfully, the patient is now able to pursue her plans to have a child."
358,"A 45-year-old woman presented to pulmonary clinic for evaluation of worsening dyspnea, cough, and hypoxemia. Her medical history was significant for limited cutaneous systemic sclerosis (lcSSc), pulmonary arterial hypertension (PAH), and interstitial lung disease (ILD) (Fig. A). She had no history of atopy or food allergies and used an albuterol inhaler as needed. PAH had been diagnosed seven years prior to presentation when she had a reported pulmonary artery (PA) pressure of 80/39 mmHg, mean PA pressure of 56 mmHg, pulmonary capillary wedge pressure (PCWP) of 12 mmHg, and cardiac output by thermodilution of 3.17 L/min. Her pulmonary function tests (PFTs) at that time showed a forced vital capacity (FVC) of 3.06 L (75% predicted) and a diffusing capacity for carbon monoxide (DLCO) of 19.61 mL/min/mm Hg (66% predicted). Per her medical records and history, the patient had been treated with dual therapy (tadalafil and macitentan) for PAH and was subsequently able to wean from oxygen supplementation that she had previously required, indicating a therapeutic response. Her ILD was considered mild, and she did not require supplemental oxygen at rest or with exertion. She was treated with mycophenolate mofetil for a year after her initial diagnosis, but the patient discontinued this medication about 6 years prior to presentation due to fear of reactivating remote Lyme infection after she had read about chronic Lyme disease on the internet. The patient had been stable on her regimen of tadalafil and macitentan until 3 months prior to presentation when she began to experience rapidly progressive dyspnea and new onset hypoxemia. Right heart catheterization (RHC) revealed that her PAH had worsened with a mean PA pressure of 72 mmHg and a new drop in cardiac index (1.7 L/min/m2), requiring initiation of subcutaneous treprostinil.\nAdditional history revealed she had taken oral helminth therapy as an alternative treatment for lcSSc. She ingested Necator americanus larvae on 3 separate occasions, 12 weeks apart, with an increasing number of helminths with each dose (3, 5, and 15 larvae). She experienced abdominal pain and diarrhea after the first ingestion. Her respiratory symptoms started 6 weeks after the third and largest dose of helminths.\nIn the clinic, patient appeared to be in mild respiratory distress with a respiratory rate of 24 breaths per minute, and she required 6–8 L/min of oxygen via nasal cannula to maintain saturations above 90%. She was tachycardic (104 beats/min) with a loud second heart sound, jugular venous distension, and severe lower extremity pitting edema up to her thighs. Auscultation of the chest revealed bibasilar rales. Dermatologic exam was notable for scattered telangiectasias over her face, arms, and hands.\nComplete blood count showed leukocytosis (13.5 × 103/uL) with 10% eosinophils (absolute count of 0.98 × 103 cells/uL). Of note, prior blood counts from 2017 had shown normal eosinophil counts (291 × 103 cells/uL). Basic metabolic profile was significant for an elevated creatinine (1.31 mg/dL) but was otherwise normal. Serum brain natriuretic peptide level was elevated at 578 ng/L. IgE was also elevated at 1372.9 IU/mL, and IgG antibodies to Strongyloides were detected. Multiple stool analyses were negative for ova and parasites. PFTs at that time revealed an FVC of 2.03 L (49% predicted), a decline of over 1.0 L since her prior testing; complete testing was not possible due to her respiratory symptoms.\nHigh resolution computed tomography (HRCT) of the chest showed worsened subpleural reticulations, traction bronchiectasis, and diffuse ground glass opacities suggesting chronic pulmonary fibrosis in a pattern consistent with non-specific interstitial pneumonia. The patient’s CT scan revealed progressive ILD and showed new diffuse ground glass opacities compared imaging from four years prior (Fig. A–D). There were also signs of decompensated right heart failure, including markedly enlarged dilated right heart chambers, pericardial effusion, and flattening of the interventricular septum (Fig. E). Though some of the ground glass opacities could have been consistent with pulmonary edema or parenchymal inflammation, additional areas had tract-like abnormalities leading to pleural edge, atypical for either of those entities [] and not conforming to normal pulmonary vasculature (Fig. D).\nImmediately after her disclosure of the ingestion of parasites, the patient was treated with albendazole and corticosteroids for presumed Löffler’s syndrome with transient improvement in her dyspnea and hypoxemia. However, due to progressive hypoxemia and signs of decompensated right heart failure on her examination at her outpatient visit, she was admitted to the hospital.\nTransthoracic echocardiogram revealed preserved left ventricular function but severely dilated right sided chambers, with a right ventricular systolic pressure estimated at 90 mmHg. Repeat RHC revealed persistently elevated mean PA pressure of 73 mmHg, worsened PCWP of 17 mmHg, and elevated pulmonary vascular resistance of 11.5 Wood units. Due to rapid decompensation and severity of hypoxemia, bronchoscopy was not able to be performed.\nRespiratory failure progressed, with the development of refractory hypoxemia and right heart failure that did not respond to aggressive medical therapy in the intensive care unit, including intravenous diuresis and treprostinil as well as inhaled epoprostenol. She was emergently evaluated for lung transplantation but was not offered listing due to her comorbidities, which included severe esophageal dysmotility and renal dysfunction. Her respiratory status continued to deteriorate despite aggressive medical intervetion, and the patient decided to transition to comfort-focused care. She passed away shortly thereafter, and post-mortem examination was declined by her family.","This 45-year-old woman has a complex medical history including scleroderma, high blood pressure in her lungs, and lung scarring. She recently developed worsening shortness of breath and low oxygen levels, likely due to a reaction to taking parasite medication she had ingested. Tests revealed her lung condition had worsened significantly, and her heart was struggling to keep up. Despite treatment, her condition continued to decline, and she ultimately passed away peacefully while receiving comfort care."
359,"A 36-year-old woman presented to a local hospital with a history of neck pain. Computed tomography (CT) and magnetic resonance imaging (MRI) demonstrated a tumor arising from the anterior elements of the C1 and C2 vertebrae. Cervical spine fusion and mass reduction surgery were performed. The resected specimen showed diffuse proliferation of plasma cells and was diagnosed as “plasmacytoma” at that time. Additional radiotherapy was performed, but the patient later dropped out from the treatment course.\nSixteen years later, at the age of 52 years, the patient returned complaining of dysarthria. CT and MRI showed a similar but much larger mass at the same location, and recurrence of the tumor was diagnosed (Fig. ). The mass compressed the spinal cord and was thought to be responsible for the dysarthria. The patient was referred to our hospital for further examination and treatment. Quantitative serum Ig analysis showed an increased level of IgG (2096 mg/dl; reference range 870–1700 mg/dl) and normal levels of IgM (203 mg/dl; reference range 46–260 mg/dl) and IgA (293 mg/dl; reference range 110–410 mg/dl). A serum Ig-free light chain study revealed increased levels of both free kappa light chain (61.5 mg/l; reference range 2.42–18.92 mg/l) and free lambda light chain (88.1 mg/l; reference range 4.44–26.18 mg/l) (kappa/lambda: 0.70).\nExcisional biopsy of the tumor was performed. Flow cytometry analysis demonstrated a distinct population of abnormal plasma cells which were positive for CD56 (96%), CD38 (70%), CD45 (9%), and CD19 (1%). Approximately 96% of these tumor cells co-expressed cytoplasmic kappa and lambda light chain based on a CD38-positive gate strategy (Fig. ). Histologically, the specimen showed diffuse proliferation of plasmacytoid tumor cells with pale paranuclear area and dense chromatin, and immunohistochemistry showed that these tumor cells were strongly and diffusely positive for CD138 (Fig. a, b) and MUM1, and negative for CD3, CD20, and CD56. Immunohistochemistry for IgL demonstrated co-expression of kappa and lambda light chain (Fig. c, d). Additionally, we demonstrated in situ hybridization (ISH) targeting IgL mRNA using FFPE specimen, and ISH also revealed that tumor cells co-expressed kappa and lambda light chain (Fig. e, f). Bone marrow biopsy showed no evidence of plasmacytes showing dual expression or deviation of kappa and lambda light chain, and additional CT and MRI revealed no skeletal abnormality except for the primary lesion, and absence of end-organ damage attributable to a proliferative plasma cell disorder.\nWe reviewed the previous specimen and performed additional IgL immunohistochemistry. The sample revealed a histological pattern similar to that of the later sample as well as co-expression of kappa and lambda light chain (Additional file : Fig. S1).\nTaking these findings together, the tumor was diagnosed as SPB with dual expression of the kappa and lambda light chains. After diagnosis, the patient underwent nine courses of VRD (bortezomib, lenalidomide, and dexamethasone) therapy, which reduced the size of the lesion. Two years after diagnosis, the lesions have not increased in size, and bone marrow examinations have shown no progression to PCM.","This 36-year-old woman has a history of a tumor in her neck that returned 16 years later. The tumor is a type of cancer called multiple myeloma, which causes her to produce too much of a certain type of antibody. Blood tests showed high levels of IgG and abnormal light chains, confirming the diagnosis. After receiving treatment, the tumor has remained stable, and she is being closely monitored."
360,"In May 2021, a 19-year-old caucasian male patient was referred to the university hospital of Brandenburg owing to a severe decline of excretory kidney function. The serum creatinine concentration was 649 µmol/l (normal range 62–106 µmol/l) at the time of admission (Fig. ). The patient suffered from mild dyspnea, lack of appetite, and moderate itchiness.\nThe patient did not report any known diseases, nor did he take any medication on a regular basis. Approximately 10 weeks earlier, he had an upper respiratory tract infection that did not require antibiotics or other medications such as nonsteroidal anti-inflammatory drugs (NSAIDs). Since then, he did not recover completely but instead suffered from persistent fatigue. Also, he lost 8 kg of body weight until admission. He denied fever, nausea/vomiting, myalgia, arthralgia, skin abnormalities, photosensitivity, Raynaud symptoms, and hair loss. He did not report morning stiffness or lower back pain. Three weeks before admission, he noticed pain in his right eye, accompanied by redness and blurred vision. A prompt ophthalmological examination led to the diagnosis of anterior uveitis. The ocular inflammatory process was not treated in a systemic manner, particularly not with systemic steroids, antibiotics, or NSAIDs. He exclusively received steroid-containing eye-drops. One day before admission, he underwent outpatient control of several blood parameters to identify the etiology of ocular inflammation. Serum analysis showed severely deteriorated kidney function.\nAt the time of admission, he presented an overall reduced physical condition. His height was 180 cm and body weight 93 kg (BMI: 28.7). His initial blood pressure was 144/114 mmHg and heart rate 124 beats per minute. Respiratory rate was 14 breaths per minute, and peripheral oxygen saturation was 99%. He had no increased body temperature. Examination of heart, lungs, and abdomen did not reveal any pathological findings, the same applied for both the central and peripheral nervous system. Abdominal skin was moderately affected by striae distensae.\nBesides impaired excretory kidney function, the patient showed moderately increased C-reactive protein (CRP) (45.7 mg/l; normal range < 5 mg/l) and elevated haptoglobin (2.6 g/l; normal range 0.3–2.0 g/l). Also, parathormone (PTH) was mildly elevated (72.8 pg/ml; normal range 15–65 pg/ml). Immune diagnostics revealed the following positive findings: anti-nuclear antibodies (ANA) titer (1:160; normal range < 1:160) and anti-La (56.6; normal range < 46). Both cytoplasmic and perinuclear Anti-Neutrophil Cytoplasmic Antibodies (c- and pANCA) were negative, anti-proteinase 3 was 2.3 U/mL (normal range < 10 U/mL). Light chain (LC) diagnostics showed increases of both, kappa- and lambda-LC (121 mg/l; normal range 3.3–19.4 mg/l, and 60.1 mg/l; normal range 5.71–26.3 mg/l), and the ratio differed from the normal range as well (2.01; normal range 0.26–1.65). Total serum immunoglobulin-G (IgG) was mildly elevated (22.1 g/L; normal range 5.49–15.8 g/L). Chlamydia pneumoniae-IgG (21 RE/ml; normal range < 16 RE/ml) was positive, as was serological testing for Epstein–Barr virus (EBV) [virus-capsid antigen (VCA) EBV-IgG-antikoerper (Ak) (enzyme-linked immunosorbent assay) 137 RE/mL (normal range < 16 RE/mL), Epstein-Barr Nuclear Antigen 1 (EBNA 1)-IgG-Ak 1.02 (normal range < 80)]. Differential blood cell count showed an eosinophil percentage of 3.6% (normal range 0.5–7%). Other non-aberrant findings were monocytes, platelet count, and serum and urine calcium.\nSemiquantitative urine analysis showed a proteinuria of 0.25 g/l and few erythrocytes (25/µl; normal: negative). The daily proteinuria was determined to be 0.77 g (normal range < 0.15 g). Urinary eosinophils were negative.\nTransthoracic echocardiography showed a mildly reduced left ventricular ejection fraction (50%; normal range > 60%). Diastolic function was impaired, although mild as well. The inferior part of the left ventricle was akinetic. Visually, the right ventricular function was slightly reduced. Computed tomography of thorax and abdomen revealed diffuse intraabdominal lymph node expansion. The initial ophthalmological investigation confirmed the diagnosis of unilateral anterior uveitis of the right eye. Specifically, the right conjunctiva showed perilimbical hyperemia, and the cornea was unaffected. The anterior chamber was not flattened and did not contain relevant cell numbers. Retinal investigation did not reveal any signs of inflammation.\nDue to AKI of unknown origin, we performed kidney biopsy (6 days after admission). Two samples were obtained from the left kidney. Initial ultrasound analysis showed normal organ dimensions and no signs of obstruction. The pathological investigation by an experienced renal pathologist showed interstitial inflammatory infiltrates around the tubuli mainly composed of lymphocytes (Fig. ). The findings led to the diagnosis of acute interstitial nephritis []. Signs of glomerular inflammation were absent. The diagnosis was tubulointerstitial nephritis with anterior uveitis (TINU) syndrome of no specific or suspected origin.\nImmediately after admission, the patient received intravenous glucocorticoids (prednisolone 250 mg daily) on three consecutive days, followed by oral prednisolone (1 mg/kg daily for 7 days, dose reduction of 10 mg daily every 7 days thereafter) since we initially suspected an ANCA-associated autoinflammatory disease. Also, we started the patient on renal replacement therapy (RR, hemodialysis) after central vein catheter insertion into the right femoral vein. Volume depletion during individual dialysis session was not mandatory since urine production was not affected. One week after admission, the patient suffered from fever and general weakness. He received intravenous antibiotics (piperacillin and tazobactam) and was transferred to the local intensive care unit (ICU). The central vein catheter was removed since the patient showed localized pain around the insertion area, that is, signs of catheter-related blood infection. RRT was continued after establishing a new central vein catheter at the ICU. Two days after the initial fever attack, the patient developed generalized rash including moderate itchiness. The antibiotics therapy was adapted to meropenem. During the ICU stay, oral prednisolone therapy was continued as initiated. Discharge from the ICU was initiated after 3 days. The last dialysis treatment session was performed 1 week before discharge from the hospital (Fig. ). Kidney excretory function continuously improved, with a last serum creatinine concentration of 214 µmol/l. Also, the ocular manifestation resolved almost completely after local corticosteroid eye drop treatment for 7 days. The in-hospital stay lasted for nearly 3 weeks, and the further management was planned in the outpatient area.","This 19-year-old man was admitted to the hospital because his kidney function suddenly dropped, and he was experiencing symptoms like fatigue, itching, and eye pain. Tests revealed he had an inflammation of the kidney and an eye condition called uveitis, along with signs of an infection. He also had a rash and elevated levels of certain antibodies. After receiving treatment with medications and dialysis, his kidney function improved, and his eye problems resolved, allowing him to be discharged home with ongoing monitoring and medication."
361,"A 58-year-old Japanese woman with a 3-year history of genital ulcers and oral aphthae was admitted to our hospital. She had a history of uterine fibroids and thrombocytopenia during pregnancy. Her family history included hypertension and diabetes in her mother and MDS in her offspring. A year ago on admission, she developed abdominal pain and persistent diarrhea. Colonoscopy revealed multiple colonic ulcers, and she was referred to our hospital. On physical examination, we found erythema nodosum without uveitis on the left forearm. Laboratory tests revealed macrocytic anemia (red blood cell count, 251 × 104/μl; hemoglobin level, 9.3 g/dl). White blood cell and platelet counts were 4800/μl and 14.3 × 104/μl, respectively. Serum C reactive protein levels were 0.35 mg/dl and anti-nuclear antibody was negative. Human leukocyte antigen analysis was positive for B51 and A26. Colonoscopy showed multiple, round, punched-out ulcers from the terminal ileum to the descending colon (Fig. a, ). Intestinal Behçet’s disease (BD) was diagnosed, and she received 3600 mg of mesalazine, 0.5 mg of colchicine, and 30 mg of oral prednisolone per day. Adalimumab, a TNF inhibitor was also added for maintenance therapy. However, during steroid tapering, her abdominal symptoms relapsed. Persistent anemia was observed and bone marrow examination was performed. The results revealed the presence of trisomy 8, trisomy 9, and X chromosome abnormalities (48, + 8, + 9, X, i(X) (q10) in 12 out of the 20 cells examined; Fig. ). The patient’s bone marrow was hypoplastic with the appearance of micromegakaryocytes and < 1% of atypical cells, resulting in the diagnosis of low-risk MDS (refractory anemia). At the age of 60, Infliximab (5 mg/kg) against refractory intestinal BD was initiated instead of Adalimumab. Infliximab was temporary effective for abdominal symptoms, however, she developed pneumonia with fever at the age of 61. Chest X-ray and lung computed tomography (CT) showed diffuse ground-glass opacities in both lungs (Fig. a, ). High-resolution chest CT and histopathology via transbronchial lung biopsy revealed the presence of pulmonary alveolar proteinosis (Fig. ). Her serum GM-CSF concentration was 4.3 pg/ml (normal range, < 5 pg/ml) and an anti-GM-CSF antibody was negative. Based on findings with the underlying disease, a diagnosis of SPAP was established. She was treated with infliximab (5 mg/kg) for active intestinal BD for every 4 weeks and received whole lung lavage to improve respiratory symptoms with SPAP. She is now preparing to receive bone marrow transplantation as a curative treatment.","60-year-old Japanese woman with a history of skin problems and digestive issues was admitted to the hospital after experiencing abdominal pain and diarrhea. Tests revealed she had intestinal Behçet’s disease, a rare condition causing ulcers in her colon, and also developed a type of blood disorder called myelodysplastic syndrome (MDS). Further examination showed abnormalities in her bone marrow, leading to a diagnosis of low-risk MDS. Finally, she was diagnosed with pulmonary alveolar proteinosis, a lung condition, and is now preparing for a bone marrow transplant to treat her MDS."
362,"A 36-year old woman was referred to a tertiary unit at 29+5 weeks of gestation due to fetal cardiomegaly and mild ascites (Fig. a, b), which was detected on a scan undertaken for suspected small for gestational age. The woman was nulliparous with a low-risk first trimester combined screening test and unremarkable anomaly scan. She was rhesus B negative and underwent non-invasive prenatal testing that determined the rhesus genotype of the fetus to be rhesus B negative. Otherwise, no red blood cell antibodies were reported. Of note, the maternal grandmother was known to have sideroblastic anaemia that was diagnosed at 17 years of age. The mother of the unborn fetus and her brother had undergone testing as children but were told that no further follow-up was needed. Unfortunately, additional information regarding and genetic testing for this family history was not available. The woman had mild macrocytic anaemia with haemoglobin of 10.4 g/dl and a mean corpuscular volume of 104 fl. The woman had not received any preconceptional or genetic counselling.\nUpon arrival, the first scan in our department demonstrated a middle cerebral arterial (MCA) peak systolic velocity (PSV) value above 1.5 Multiples of the Median (MoM). Fetal biometry was normal, as was the amniotic fluid index and umbilical artery doppler. Dexamethasone for fetal lung maturation was administered, and an uncomplicated in utero fetal blood transfusion (IUT) was performed at 30+3 weeks of gestation. The pre-transfusion fetal haemoglobin was 4.4 g/dl, which was increased to 14.1 g/dl following 120 mL of blood transfusion through the intrahepatic portion of the umbilical vein (Fig. ). Investigations to determine the underlying cause of fetal anaemia included microarray comparative genomic hybridization, serology to exclude congenital infection, a peripheral blood film and fetal bilirubin to detect haemolysis. These preliminary investigations were normal. A fetal MRI brain was performed, which showed no abnormalities. In the subsequent weeks, the MCA-PSV improved, as did the cardiomegaly.\nAt 35+3 weeks of gestation, the MCA-PSV increased to above 1.5 MoM. The options of delivery and ex-utero transfusion versus IUT were discussed with the multidisciplinary team and with parents. As it was considered that an IUT would allow pregnancy to proceed to term, it was decided to proceed with IUT. A second IUT took place with a pre-and post-transfusion fetal haemoglobin of 9.7 and 15 g/dl, respectively. The MCA-PSV remained stable until 37 weeks and three days when a caesarean section for breech presentation was planned. Fetal biometry was consistent with previous measurements, and the amniotic fluid index and umbilical artery doppler were normal. The woman was counselled regarding the need for delivery and likely need for exchange transfusion in the neonatal period. Delivery of a female infant via caesarean section due to breech presentation was performed at 37 + 4 weeks. The birth weight was 3150 g, and apgar scores were 8 and 10 at 1 and 5 minutes, respectively.\nSubsequently, the infant again became anaemic, requiring regular 3–4 monthly blood transfusions but she is making good developmental progress. Her anaemia was unresponsive to pyridoxine. Iron levels should be closely monitored to detect the need for chelation therapy in future care plans, since iron toxicity is a major cause of morbidity and mortality in XLSA [].\nFetal and maternal blood was collected for DNA extraction, at the time of the first IUT. DNA was sequenced by next-generation sequencing for 11 genes associated with sideroblastic anaemia. Analysis was performed using Agilent SureSelect XT custom enrichment technology and Illumina DNA sequencing. Significant maternal cell contamination of the fetal blood sample was excluded using the ABI AMPFLSTR Identifiler PCR Amplification Kit. A heterozygous variant in the ALAS2 (NM_000032.4) gene was identified in both the mother and the fetus. The c.488G > A; p.(Arg163His) variant identified affects a conserved amino acid and is absent from the gnomAD controls database []. The variant was therefore classified as pathogenic according to the American College of Medical Genetics (ACMG) variant interpretation guidelines []. The findings for both the mother and the fetus were confirmed by Sanger sequencing. These results were available two days following delivery and verified a heterozygous ALAS2 c.488G > A; p.(Arg163His) and SLC4A1 c.876 + 5G > A mutations in both the baby (Fig. a) and the mother (Fig. b). X-inactivation studies [, ] were undertaken. There was no significantly skewed X-inactivation in the sample provided from the baby. The level of X-inactivation in the mother could not be determined, as she was uninformative for the AR locus [].","This baby was born with a low amount of red blood cells, which was discovered during pregnancy scans. The mother has a family history of a rare blood disorder called sideroblastic anemia, and tests revealed that she and her baby both carry a gene mutation that causes this condition. To help the baby, a blood transfusion was performed during pregnancy, and the baby was delivered by C-section at 37 weeks. The baby is now receiving regular blood transfusions and is doing well, but her iron levels need to be closely monitored to prevent potential problems."
363,"We report a 31-year-old Saudi male patient who presented initially to the neurology clinic at King Faisal Specialist Hospital and Research Centre, Jeddah, to evaluate reversible recurrent rhabdomyolysis. The patient was healthy and had no medical background prior to this presentation. He denied the use of any medications or herbal agents. His family history was negative for any neurological illnesses, and his parents were not related. The patient was unemployed at the time of the first presentation. He habitually smoked one pack of cigarettes per day for 10 years, however, he had no history of alcohol intake or illicit drug use.","A 31-year-old man from Saudi Arabia came to the hospital because his muscles were breaking down, causing weakness. He had never had any health problems before, and he didn’t take any medications or use any herbal remedies. He was a smoker and had no family history of neurological diseases. He was currently unemployed."
364,"An 80-year-old Japanese woman presented with a history of TTS complicated by ischemic enteritis (Fig. ). She was previously admitted to our hospital, where she presented with bloody stools due to ischemic enteritis, and was treated with hydration of 1500–2500 mL/day and dobutamine. The patient was subsequently discharged without cardioprotective drugs. She was married and had one daughter. She had no family history of cardiovascular disease. She used to cook at a nursing home but she retired. She had smoking history and a drinking habit. She presented to our hospital with upper abdominal pain and bloody stools, 4 months after her first hospital admission for TTS.\nAt the examination, her general condition was good, she was conscious alert, with a temperature of 36.6 °C. Her height and weight were 148 cm and 42 kg (body mass index 19), respectively. Her blood pressure was 114/80 mmHg, heart rate was 90 bpm, and arterial oxygen saturation on room air was 97%. Her abdominal pain improved upon admission. Her abdomen was flat, soft, tender, and had good gurgling,however, digital rectal examination showed blood on the examining finger, indicating a possible relapse of ischemic enteritis. Physical examination showed a regular cardiac rhythm with normal S1 and S2, no detectable murmurs, and clear lungs. Although the patient did not experience chest pain, her electrocardiogram revealed negative T waves in many leads (I, II, III, aVL, aVF, V2, V3, V4, V5, V6). Blood examination showed that her brain natriuretic peptide (BNP) and troponin I levels had risen to 1578 pg/mL (healthy upper limit 18.4 pg/mL) and 357.2 pg/mL (healthy upper limit 15.6 pg/mL), respectively. Her blood urea nitrogen (BUN) and creatinine levels had risen to 26.1 mg/dL (healthy upper limit 20.0 mg/dL) and 0.87 mg/dL (healthy upper limit 0.79 mg/dL), respectively. There were no findings of liver dysfunction. Echocardiography showed wall motion abnormality centered on the left central ventricle, with ballooning of the apical portion. We suspected the recurrence of TTS. We believe that the abdominal pain and dehydration due to ischemic enteritis may have contributed to the development of TTS.\nAs a treatment, we gave the patient a small amount of oxygen (2 L/min). She also received 10,000 units/day of continuous intravenous heparin for 2 days to prevent left ventricular thrombosis, and fluid replacement of 1500 mL/day to treat TTS (Fig. ). Although her body weight increased temporarily, the urine volume was normal, oxygenation was stable, and exacerbation of heart failure was not observed. The BNP level also showed a downward trend; hence, diuretics were not administered and hydration was continued. The BNP level and myocardial wall motion were normalized on the fourth day after admission (Fig. ).\nFor differentiation of ischemic heart disease, a coronary angiography and an acetylcholine provocation test were conducted on the 22nd day after admission. Although no significant stenosis was found in the coronary artery, the acetylcholine provocation test revealed a considerable multivessel coronary spasm in the left coronary artery, suggesting coronary vasospastic angina pectoris associated with TTS, which was treated using β-blockers, calcium channel blockers, and nicorandil. She was discharged on the 22nd day. The negative T-wave on the electrocardiogram normalized on the 60th day after discharge. (Fig. ). She was also treated in gastroenterology and psychiatry, and was prescribed probiotics and anxiolytics. Her ischemic enteritis remained stable without abdominal pain. Subsequent follow-up showed no recurrence of TTS over 3 years.","This 80-year-old woman was readmitted to the hospital after experiencing a recurrence of a heart condition (TTS) complicated by intestinal problems. She had previously been treated for a similar issue and now presented with abdominal pain and bloody stools. Tests revealed signs of heart failure and a possible blood clot, and she received treatment with oxygen, blood thinners, and fluids. After several days, her condition improved, and she was discharged home with medications to manage her heart condition and intestinal issues."
365,"A 63-year-old female patient was admitted to our hospital with intermittent epigastric abdominal pain for the past three months. Results of the physical examination on admission indicated no icteric sclera. The abdomen was soft with no palpable abdominal mass. The patient had experienced a weight loss of about five kg in the past two months and had no history of hepatitis B or C. She had been exposed to dogs and sheep and denied any history of exposure to infected cases from the epidemic area. After admission, CA19-9 was measured and was 796.20 U/mL. No serological examination for echinococcosis was performed because of the limited conditions of our hospital. Test results from pelvic ultrasound indicated menopausal uterus and uterine fibroids. Test results from gastroscopy showed chronic non atrophic gastritis. Colonoscopy revealed multiple polyps in the large intestine (basically removed); intestinal histopathology (cecum, biopsy) showed severe chronic inflammation of the mucosa and adenomatous hyperplasia of the individual glands. Contrast-enhanced computed tomography of the upper abdomen (Fig. A) revealed a more homogeneous thickening of the gastric wall in the antrum. Round unenhanced low-density foci with a diameter of 4.6 cm was seen in the S7 segment of the liver. Nodular calcifications were also observed. No significant dilatation was noted in the intrahepatic and extrahepatic bile ducts. The size and shape of the gallbladder were normal, the wall was not thick, and no significant abnormal density was observed in the cavity. The pancreas, spleen, and adrenal glands showed no significant abnormalities. Test results from computed tomography indicated liver cyst and intrahepatic calcifications. Contrast-enhanced magnetic resonance imaging of the liver and gallbladder (Fig. B, ) revealed a normal size and shape of the liver and proportion of each lobe, and the intrahepatic and extrahepatic bile ducts and flow vessels ran naturally. A long T1 and long T2 cystic signal with a diameter of about 4.7 cm was observed in the right lobe of the liver, with liquid level, short T1 high signal intensity in the lower layer, high signal intensity on diffusion-weighted imaging sequence, and enhancement of the cyst wall on the enhanced scan. Test results from magnetic resonance imaging showed that the space-occupying lesion of the right lobe of the liver had been considered to be more likely a hepatic hydatid cyst. Preoperatively, three-dimensional reconstruction demonstrated the location of the tumor and its relationship with the surrounding vessels (Fig. A).\nThe patient underwent laparoscopic S7 segmentectomy. Intraoperative findings showed that the tumor was located at the S7 segment of the liver and was about 5 × 4 cm in size and partially protruding from the surface of the liver, with an intact capsule and clear boundary with normal liver tissue. The tumor compressed the right hepatic vein and its tributaries and densely adhered to the right hepatic vein (Fig. B). On postoperative pathology, a mass was observed immediately adjacent to the liver capsule, with a volume of 5 × 5 × 4.5 cm. The section surface showed a brown turbid fluid, a smooth inner wall, and greyish red, greyish yellow, and soft section surfaces of other liver tissues. The pathological section showed a low-grade mucinous cystic neoplasm (volume 5 × 5 × 4.5 cm) in the S7 segment of the liver, with steatosis in the surrounding hepatic tissue area, chronic inflammatory cell infiltration in the portal area, and no tumor cell involvement in the margin of the liver resection.\nImmunohistochemistry demonstrated tumour cells CK7 (+), CK19 (+), and CEA (−); stromal cells ER (+), PR (+), α-inhibin (a small amount +), vimentin (+), desmin (+), and actin (+) (Fig. A–C). This study was approved by the Ethics Committee of Yantai Affiliated Hospital of Binzhou Medical University.","A 63-year-old woman was admitted to the hospital because she’s been experiencing stomach pain for three months and has lost some weight. Tests revealed several polyps in her colon, which were removed, and showed signs of chronic inflammation. Imaging scans showed a possible liver cyst, and further tests confirmed this diagnosis. The patient underwent surgery to remove the cyst from her liver, and the pathology report showed it was a benign, low-grade tumor."
366,"In 2009, a 29-year-old Peruvian male presented with a 3-month history of a chronic cough productive of yellow/green coloured sputum with occasional haemoptysis, associated with significant fatigue, diminished appetite, weight loss (7 kg in two months), night sweats and back pain.\nA chest X-ray demonstrated right-sided apical cavitation with prominent bilateral hilar lymphadenopathy. A sputum specimen was positive for acid fast bacilli. Microscopic Observed Drug Susceptibility (MODS) testing did not indicate drug resistance.\nThe patient was treated with a 4-drug (rifampicin, isoniazid, pyrazinamide and ethambutol) anti-TB antibiotic regimen for a course of two months. Rifampicin and isoniazid was continued for a further four months. Sputum smears became negative after one month of treatment suggestive of a favourable disease progression and six subsequent sputum smear samples were negative. A repeat chest radiograph demonstrated right-sided apical fibrous reticular infiltrates consistent with treated inactive TB. The patient was considered to be in remission and remained asymptomatic thereafter.\nThe patient was born in the Callao region of Peru (population size 800,000). The patient shared one bedroom with his wife, son and parents in law. The patient’s locality is known for a pig farm that employs many of the region’s residents. Our patient’s bother worked on this pig fam and had regular contact with our patient. 1-month prior to our patient’s presentation the patient’s brother was successfully treated for TB, the causative MTBC agent was not identified. The patient did not report any other contacts with domestic or wild animals and denies ingesting unpasteurised dairy products. There was no further household transmission of TB between our patient and the other members of his household.\nThe patient’s sputum sample was processed on both liquid (MODS) and solid Ogawa medium. An aliquot was sub-cultured and underwent Spoligotyping after DNA extraction at the Universidad Peruana Cayetano Heredia (Lime, Peru) [, , ]. The isolate was identified as M. caprae and was further genotyped using a 15-loci MIRU-VNTR analysis at the Kobe Institute (Kobe, Japan) following established protocols []. Subsequent whole genome sequencing identified the sample as M. caprae.","A man from Peru came to the hospital after struggling with a persistent cough, fatigue, and weight loss for three months. Initial tests revealed he had tuberculosis (TB) in his lungs, and he was treated with a strong medication regimen. After several months of treatment, the tests showed the TB was clearing up, and his lungs appeared healthier on an X-ray. Further investigation revealed he had a rare type of TB, Mycobacterium caprae, which is often linked to contact with pigs."
367,"In 2008, a 64-year-old Peruvian male presented with a 3-month history of a productive cough with haemoptysis and shortness of breath. A sputum smear was positive for acid fast bacilli. MODS testing did not indicate drug resistance. A chest radiograph demonstrated apical cavitation of the left upper and middle lung lobe with blunting of the left costo-diaphagmatic angle.\nThe patient was treated with the standard 4-drug regimen for a total course of 6 months. Sputum smears became negative after one month of treatment suggested favourable disease progression and five subsequent sputum smears were negative.\nHowever, 1 month after the patient stopped treatment he deteriorated clinically and had three positive smears. He was treated with a second line anti-TB regimen of ethambutol, pyrazinamide, ethionamide, ciprofloxacin, cycloserine, kanamycin and para-aminosalicylic acid for a total duration of 18 months. Despite initial improvements in symptoms, the patient relapsed again on 2nd line therapy and died of respiratory failure in 2012.\nThe patient lived alone in the region of Lima South (population size 1,200,000). Prior to his initial hospitalisation the patient had spent two months visiting family in the city of Huánuco in central Peru. This region has the greatest density of cattle farms and grazing cows in the country. While the consumption of unpasteurised milk in Huánuco is commonplace, our patient denies consuming unpasteurised dairy products.\nThe patient’s sputum sample was processed on both liquid (MODS) and solid Ogawa medium. An aliquot was sub-cultured and underwent Spoligotyping after DNA extraction at the Universidad Peruana Cayetano Heredia (Lime, Peru) [, , ]. The isolate was identified as M. bovis and was further genotyped using a 15-loci MIRU-VNTR analysis at the Kobe Institute (Kobe, Japan) following established protocols []. Subsequent whole genome sequencing identified the sample as M. bovis.","A man from Peru was admitted to the hospital in 2008 after struggling with a persistent cough, bloody mucus, and difficulty breathing for three months. Tests revealed he had tuberculosis (TB) and the infection was in his lungs. He received a long course of medication, but unfortunately, he relapsed and eventually died from breathing problems. Further investigation showed he had contracted M. bovis, a type of TB found in cattle, likely from visiting a region with a high number of cattle farms."
368,"The proband, a 2-year-old Russian girl at the time of the last clinical evaluation, was admittedto the neurological department with repeated, prolonged myoclonic, and generalized seizures responsive only to intravenous injection of diazepam. She was the fourth child of healthy parents from a non-consanguineous marriage. The proband has three healthy siblings (). During the pregnancy, a risk of miscarriage was observed at 12 weeks of gestation. Delivery and neonatal period were unremarkable. At the age of 4 months, during obstructive bronchitis, she developed an absence seizure with apnea 3–5 s long that repeated daily afterward. At the age of 5 months, during hot water bathing, the proband had a prolonged generalized myoclonic seizure for 40 min that was responsive only to diazepam injection. Similar episodes repeated every 7–10 days without any provoking factors.\nOn brain magnetic resonance imaging (MRI), periventricular leukomalacia was noted and was considered as a result of ischemic brain injury with no relevance to the epileptic phenotype of the patient. Routine electroencephalography (EEG) did not show any epileptiform activity in the interictal period. Antiepileptic therapy included carbamazepine (300 mg/day), topiramate (87.5 mg/day), and clonazepam (0.75 mg/day), which had no effect on seizure frequency and duration. In the evaluation, the proband had prolonged myoclonic, tonic-clonic, and atonic seizures once a week. Early motor milestones were normal, but language development was delayed. Neurological examination at the age of 2 revealed moderate hypotonia with brisk tendon reflexes and mild gait ataxia. Based on the clinical picture, the proband was diagnosed with DS.","This young girl has been experiencing frequent and long seizures since she was a baby. These seizures are difficult to control and only respond to a specific medication. Brain scans show some damage to the brain, but it doesn't seem to be the main cause of her seizures. She is receiving medication to help manage her seizures, but her development, particularly her language skills, is slightly delayed."
369,"The patient is a 7-year-old boy born and raised in the Central-West region of Brazil (Cuiabá) from nonconsanguineous parents. He exhibited odontogenic abscesses associated with unexplained sinusitis at age 4 with resolution of the condition after standard treatment. At the age of 5 years, a low-impact trauma in the right elbow, evolved to a local disproportional inflammation within a few hours, was treated with immobilization with plaster splint. Over the following two weeks, the patient developed fever and persistent, painful edema diagnosed as pyoarthritis in the right elbow. At that time, laboratory analysis revealed mild anemia (hemoglobin (Hb) = 10.0 g/dL), hematocrit (Ht) = 30.5%), high levels of acute reactant markers (erythrocyte sedimentation rate (ESR) = 21 mm, and C-reactive protein (CRP) = 15.3 mg/dL (reference value (RV) < 1 mg/dL)). Also, a computed tomography (CT) of the right elbow revealed the presence of diffuse periosteal reactions affecting the proximal metaphyseal regions of the radius and ulna, as well as of the distal metaphyseal region of the humerus plus voluminous joint effusion and diffuse soft tissue elbow enlargement, especially in the medial aspect. With the diagnostic impression of probable sepsis of the elbow, the patient underwent arthrotomy with surgical drainage of a large amount of purulent fluid with lumps, and broad-spectrum antibiotic therapy was initiated. After 72 hours of antibiotic therapy and arthrotomy, the patient maintained the marked painful edema in his right elbow and culture of the synovial fluid came out negative. The patient remained hospitalized until the 53rd postoperative day when he presented with sudden and pronounced edema and pain in the left knee after trauma caused by falling from his own height while playing in the corridor of the ward. He underwent to another arthrotomy of the left knee with discharge of abundant purulent liquid. Due to persistent painful edema, the patient underwent another arthrotomy of the left knee for drainage of a joint effusion that was repeatedly purulent, all with negative cultures.\nOf note, a magnetic resonance imaging of the left knee () showed significant inflammatory arthropathy, with joint effusion associated with diffuse synovitis; extension of the inflammatory process into the periarticular soft parts affecting the popliteus and quadriceps muscle; extrusion of the medial meniscus; and small foci of inflammatory edema affecting the subcortical bone marrow of the trochlear sulcus and the medial femoral condyle, which may have corresponded, according to the radiological report, to the focus of incipient osteomyelitis.\nAfter 60th day of hospitalization, the patient was discharged, but two weeks after, there was another need for hospitalization due to another arthritis. At that time, laboratory analysis demonstrated high levels of acute reactants markers, and he never evaluated to sepsis. Another arthrotomy was necessary again with pus drainage.\nBecause of the recalcitrant situation and the suspicion of an inborn error o immunity, a target gene panel (Invitae panel, 407 genes, Jeffrey Modell Foundation Partnership Program) was requested, and an already reported mutation in the PSTPIP1 (c.688 G > A (p. Ala230Thr)) gene was found in the patient and in the mother.\nCuriously, just after genetic founding, the mother discovered similar cases in the family and reported of herself having a prolonged episode of arthritis in the right knee, which began after trauma during a soccer game at the beginning of her adult life. During the reported occasion, she fully recovered, and other members of the family have not yet been genetically investigated. She also reported of having had cystic acne throughout adolescence and young adulthood, with nowadays facial skin marked by scar depression. Finally, genetic sequencing was not just essential for the diagnosis but also for genetic counseling and clarification of the underlying condition. During 2 years of follow-up, no episodes of pyoderma gangrenosum were noted, neither in the mother nor in the index patient.\nAfter being discharged from his last hospital stay, the patient presented at the age of 8 with two more subsequently episodes of left knee arthritis triggered by low-impact local trauma and a new episode of arthritis, this one in the right ankle, due to trauma secondary to the use of inadequate footwear (). In these episodes, the response to treatment with prednisone 1 mg/kg over 3 weeks was satisfactory, followed by gradual withdrawal. However, no specific measure could be accessed to infer the impact of the nonpreventive treatment with anti-IL1, as suggested in the literature. Unfortunately, anti-IL1 blockers are not available, in both public and private systems in Brazil, and that is the reason the patient did not receive it.","This young boy has had a series of difficult infections in his elbows and knees, starting when he was 4 years old. These infections have caused significant swelling and pain, and doctors have had trouble figuring out what’s causing them. Tests revealed he has a genetic condition that makes him more prone to these infections, and the doctors found a specific gene mutation responsible. Despite multiple treatments, the infections kept coming back, and the doctors are continuing to monitor his condition closely."
370,"A 45-year-old Caucasian male visited his gastroenterologist for follow-up on Crohn's disease, which was limited to the terminal ileum. Three months prior to the present episode, treatment with 6-mercaptopurine was discontinued due to elicited leukopenia. There were no clinical signs of activity of Crohn's disease, but he reported an acute worsening of chronic lower back pain for three weeks, which was accompanied by chills for two days. Due to the absence of fever, he was discharged after blood cultures were drawn. After 48 hours, a Gram-positive coccus (Granulicatella adiacens) was detected, and he was requested to come to our emergency department. He reported no recent history of dental treatment, signs of respiratory infection, or gastrointestinal complaints. On physical examination, he did not appear acutely ill. The blood pressure was 130/93 mmHg, his pulse 120 beats per minute, and his auricular temperature 37.5°C. Except for a holosystolic heart murmur in the apical region, no abnormalities were found on examination. Specifically, no spinal percussion tenderness or focal neurological deficit was detected. His hemoglobin level was 11.0 g/dL (ref. 14–18 g/dL), and his white blood cell count was within the normal range. The erythrocyte sedimentation rate (46 mm/h; ref. 0–10 mm/h) and C-reactive protein (45 mg/L; ref <8 mg/L) were elevated. He received penicillin 12 ∗ 106 U/24 h and gentamicin 3 mg/kg/24 h intravenously for possible endocarditis. Both transthoracic and transesophageal echocardiograms were performed, which showed mitral valve insufficiency based on a prolapse, but no vegetation or other echocardiographic signs of infectious endocarditis. Therefore, the Dukes criteria were not met, and endocarditis was ruled out with reasonable certainty. Gentamicin was discontinued, and the dose of penicillin lowered to 6 ∗ 106 U/24 h. Lumbar MRI showed vertebral osteomyelitis at discus L2 and L3 (white arrow, ). In total, our patient was treated with intravenous penicillin for Granulicatella adiacens osteomyelitis for three weeks, followed by two weeks of oral clindamycin (600 mg three times daily). Clindamycin was chosen for its high bone tissue penetration. Six weeks after cessation of antibiotic treatment, the patient had fully recovered. In addition, this was supported by low inflammation markers and negative follow-up blood cultures.","6 This 45-year-old man came to the hospital because of a new, severe back pain and fever. Tests revealed a bacterial infection in his spine, specifically in the discs of his lower back, caused by a type of bacteria called Granulicatella adiacens. After ruling out other possible causes like endocarditis, doctors treated the infection with antibiotics and found that the patient was recovering well. He was discharged after three weeks of antibiotics and is now fully recovered."
371,"A 72-year-old female presented to the emergency room after injuring her left eye during a syncopal episode. A computed tomography scan performed in the emergency department revealed disorganization of the left globe with blood in the vitreous humor. Her medical history included hypertension, systemic lupus erythematosus, and coronary artery disease.\nHer vital signs were within normal limits with the exception of mild systolic hypertension. She underwent immediate globe rupture repair under general anesthesia with successful reapproximation of a large 20 mm scleral laceration. In the immediate postoperative period, rather than performing subtenon injection due to the possibility of a more posterior rupture, the surgeon chose to perform a retrobulbar block using 5 mL of bupivacaine 0.5% for postoperative pain management and akinesis (to prevent extrusion of additional intraocular contents). This was done using a 31 mm 25-gauge blunt needle.\nNo blood was aspirated back into the syringe, and the needle advanced without incident. Prior to injection, the patient was breathing spontaneously via the anesthesia machine circuit and had not received any additional narcotics/muscle relaxants for 2.5 hours. There was full recovery of neuromuscular blocking agent after reversal, as demonstrated by the use of a nerve stimulator. Over a duration of 7 minutes, however, the anesthesiologist noted a steady increase in end-tidal CO2, resulting in apnea. She remained intubated and was transported to the postanesthesia care unit for an additional 1.5 hours, after which she was successfully extubated. She was admitted to medicine and monitored for an additional time of 48 hours. No new neurologic or cardiac deficits were found, and she was discharged without event.","This 72-year-old woman went to the emergency room after injuring her eye during a fainting spell. A scan showed damage to her eye, and she needed immediate surgery to repair a large tear in her eye. To prevent further problems, the surgeon used a retrobulbar block to numb the area and prevent her eye from moving. After a period of monitoring, she was successfully taken off her breathing tube and discharged home without any new problems."
372,"An 11-year-old female soccer player presented to the office with left knee pain that she first noticed 5 years ago after falling off a bike. Since that time she had a low level of pain in the knee, which was now significantly worse over the last month as soccer activities increased, the pain was worse with stairs, running, squatting, and kneeling; it was located in the anterior medial aspect of her knee. She denied radiation of the pain, numbness, tingling, popping, or locking. She had infrequent effusions and was using ice and NSAIDS as needed for pain. She had also tried a course of physical therapy with no improvement. An X-ray showed an osteochondritis dissecans (OCD) lesion of the lateral trochlear groove (), and an MRI was obtained to stage the lesion. The MRI showed a stable OCD lesion of the trochlea (). Given the stability of the lesion and patient age, the decision was made to proceed nonoperatively with weight bearing in a locked knee brace for 8 weeks for activities of daily living and restriction from athletics/sports. At her 8th week follow-up, she still had occasional pain with mild flexion and the X-ray showed bone formation of the trochlear groove without any subchondral collapse or loose bodies (). She was continued in the locked knee brace for another 4 weeks. At her 12th week follow-up, she was pain free and was taken out of the knee brace. Physical therapy was started to strengthen her left lower extremity which had undergone atrophy in the brace, and by 16 weeks, she had regained her strength and was participating in soccer drills. She was transitioned from formal therapy to a home exercise program, and at 20 weeks, she was cleared to return to all activity, doing so without complication.","This 11-year-old girl has had knee pain for five years, which has gotten worse recently due to playing soccer. An X-ray and MRI showed a small problem with the bone in her knee, called osteochondritis dissecans, but it was stable. She wore a brace for several months to protect her knee and allow the bone to heal, and she has now fully recovered and is able to return to all activities, including soccer."
373,"The patient was a previously healthy 6-year-old girl of northern European descent whose only concerns had been enlarged tonsils, chronic constipation, and slow growth. She had no other health problems. There was no family history of endocrine or immunologic diseases. While watching television, she developed a grand mal seizure and became apneic. She was emergently transported to Children's Mercy Hospital. Initial physical examination was unremarkable except for short stature, with weight 20 kg (37.7 percentile) and height 104.6 cm (less than the 1st percentile). Family history revealed that the patient's mother is 162.6 cm tall and father is 177.8 cm tall; midparental height is 167.7 cm (50th percentile).\nShe was found to have a critically low total calcium of 1.1 mmol/L (normal range 2.2–2.5 mmol/L) and a blood glucose of 3.6 mmol/L (normal range 3.6–6.1 mmol/L). Her phosphorus was elevated at 3.6 mmol/L (1–1.9 mmol/L), and magnesium was low at 0.49 mmol/L (0.66–0.94 mmol/L). Initial iPTH level was low at 7 ng/L (10–89 ng/L), and subsequent iPTH levels remained low. She was diagnosed with primary hypoparathyroidism. She received intravenous calcium chloride and magnesium sulfate. Computed tomography of the head was normal. An extensive endocrine workup revealed that she had Howell-Jolly bodies consistent with autoimmune hyposplenism, a condition frequently seen in APS1 []. No other autoimmune deficiencies were noted at that time. Karyotype was 46, XX. Evaluation of 22 q 11 variants was normal. A growth hormone (GH) stimulation test was performed during initial admission. Her peak GH level was 12.8 ng/mL (normal >10 ng/mL).\nWith the documentation of two unusual autoimmune findings, genetic testing for AIRE gene was performed. The patient was found to be a compound heterozygote for 2 known disease-causing variants. The first was a nucleotide change of C > T in exon 6 of the AIRE gene resulting in the substitution of the normal arginine codon with a stop codon at position 257. This mutation is denoted R257X or Arg257Term. The second mutation was a 13 base-pair deletion in exon 8, beginning in codon leucine 323 and resulting in a change from leucine to serine, followed by a frameshift and premature stop codon 50 residues downstream (denoted c.967 979del13 and p.Leu323SerfsX50). Thus, she was heterozygous for R257X and c.967 979del13. Both are common, independently recurring mutations in APS1 []. The 13-base deletion has been published with various nomenclature (c.965 977del13 or p.Cys322fsX5l).\nAfter testing positive for AIRE gene variants, additional serologic testing revealed seropositivity for 21-hydroxylase antibodies (a marker for adrenal autoimmunity) and positivity for intrinsic factor autoantibodies (a marker for atrophic gastritis). shows her positive serology over time, along with her immunosuppressive medications. Antibody testing for thyroid disease, type 1 diabetes mellitus, and celiac disease yielded negative results at that time.\nAt the age of 6 and a half years, a low-dose ACTH stimulation test showed a borderline peak cortisol of 433 nmol/L (normal >500 nmol/L). ACTH stimulation testing was repeated a year later, at which time, she demonstrated a peak cortisol level of 334 nmol/L, in addition to an elevated renin level. Subsequently, hydrocortisone and fludrocortisone replacement therapy were initiated. shows the number of autoimmune conditions over time along with her immunosuppressive medications.\nBecause of persistent short stature, she underwent repeat GH stimulation testing around age 9. Her peak GH level was 11.8 ng/mL, demonstrating GH sufficiency again. However, as her height was below the 3rd percentile, growth hormone therapy was initiated with excellent response. She ultimately achieved an adult height of 161.5 cm, within the range of her midparental height.\nHer liver enzymes were modestly elevated at this time. Serologic testing revealed positive smooth muscle antibodies, which are associated with autoimmune hepatitis. At age 10, she developed hypertension and nephrocalcinosis and was placed on thiazide diuretics. At the age of 11 years, serologic testing revealed positive glutamic acid dehydrogenase (GAD), antinuclear antibodies (ANA), and Sjögren syndrome antibodies (SSA). Her thyroid antibodies have remained negative throughout her course. Over time, her smooth muscle antibodies and SSA antibody levels have gradually normalized ().\nAt age 12 years, she began to develop patches of alopecia on her scalp, which was distressing to her. We referred her to rheumatology for aggressive management of her APS1. She was begun on rituximab, monoclonal antibody therapy directed at CD20, a B cell epitope. Unfortunately, soon after she received 2 doses of rituximab, her hair loss progressed rapidly to alopecia totalis and then progressed to alopecia universalis.\nThe patient was referred to pediatric dermatology, who began with intralesional triamcinolone injections but soon added oral methotrexate. She was begun on 20 mg weekly, but this was increased to 25 mg weekly at age 16, which she continues to date. She was empirically placed on 1 mg folic acid daily and vitamin B12 with the methotrexate therapy. Complete hair regrowth was achieved within a year. denotes progressive hair loss ( and ) followed by hair regrowth (). The patient has tolerated the methotrexate well.\nAt the age of 15, she was referred to gynecology for fertility discussion. Periods had been regular throughout, and gonadotropins were normal. Ovarian antibodies, known to be nonspecific, were measured at that time and were positive. Midcycle LH was 25 IU/L, and FSH was 7.1 IU/L. Anti-Müllerian hormone (AMH) level was 21.3 pmol/L (normal range for AMH 7.5–91.8 pmol/L). Six months later, the AMH level was noted to be 6.9 pmol/L, suggestive of low ovarian reserve [, ]. She then underwent fertility preservation. The procedure was highly successful, with 21 eggs harvested and stored. Surprisingly, five months after the retrieval, AMH levels were found to have normalized at 152.4 pmol/L. Her LH and FSH normalized. Her most recent LH is 0.5 IU/L, and her LH is 1.9 IUL. Cycles have remained regular throughout.\nAt age 18, a bone mineral density study noted focal areas of severely decreased bone mineral density in the distal femurs. Plain films showed ill-defined lucencies with adjacent sclerosis in the distal femurs, which we attribute to metaphyseal dysplasia, a rare bone condition previously described in 2003 in 2 unrelated patients with APS1 [].\nSix months later, she suddenly developed profound hypokalemia, which was thought to relate to apparent mineralocorticoid excess that has been described in APS1 []. This was managed with spironolactone and a reduction in fludrocortisone. A few weeks later, she experienced sudden mental status changes. She was seen emergently and was thought to be in septic shock. Testing for active COVID-infection was negative, but she had IgG antibodies to COVID-and met the diagnostic criteria for multisystem inflammatory syndrome in children (MIS-C). She required fluids and intensive care support. She recovered uneventfully from MIS-C, although she remains with elevated brain natriuretic peptide.\nShe is currently doing well as a college student at a major university and undergoes frequent laboratory monitoring.","This 6-year-old girl has a complex medical history with several unusual conditions. Initially, she was diagnosed with primary hypoparathyroidism, a condition where her body doesn't make enough of a hormone needed for healthy calcium levels. Further testing revealed she has a rare genetic condition called APS1, which causes her immune system to attack her own tissues, leading to problems with her adrenal glands, kidneys, and ovaries. Over time, she has needed medications to replace hormones and manage her symptoms, and she has experienced hair loss and bone problems. Recently, she was diagnosed with MIS-C, a serious illness affecting multiple organs, but she is now recovering well and continuing her studies."
374,"A 3-year-old boy was admitted with severe respiratory distress and tachypnea after upper respiratory tract infection. He had dyspnea, cough, orthopnea, respiratory rate about 43 /min, and mild plethora of the face.\nHe was the only child of family, and his parents were not relatives. He was born through normal vaginal delivery, and his Apgar score was 10 at birth and 5 minutes after birth. He had no developmental delay, but his weight and height were under 3 percentiles of growth, which was also evident on physical examination. We did not find significant data in his past medical history.\nOn physical examination, we found a 5 x 4 cm mass on the left supraclavicular area and neck. The mass was firm and non-tender. Blood pressure was normal. Skin examination revealed multiple café-au-lait spots ().\nLaboratory data showed hemoglobin (Hb) 10.9 g/dL, WBC 13300 /µL, neutrophil 29%, eosinophil 7%, monocyte 8%, lymphocyte 56%, platelet count 262000/µL, urea 21mg/dL, creatinine 0.6 mg/dL, uric acid 3.9 mg/dL, and lactate dehydrogenase (LDH) 754 U/L. Beta-HCG was 0.66 m IU/ml and alpha fetoprotein was 1.01 IU/ml.\nChest radiograph was obtained, which showed a large mediastinal mass (), and chest CT scan revealed a heterogenous mass on the left mediastinum () with extension from thoracic inlet to the neck (). The mass compressed the neck vessels and airway.\nCorticosteroids were started because of respiratory distress and superior vena cava syndrome. After three days, the patient became stable, and incisional biopsy was taken from the neck mass, indicating proliferation of spindle cells within wire-like collagen fibrils in loose background, in favor of neurofibromatosis ().","This 3-year-old boy was admitted to the hospital because he was having trouble breathing and coughing after a cold. He also had a noticeable lump in his neck and was smaller than expected for his age. Tests showed a large mass in his chest and neck, which was pressing on his airways and blood vessels. After starting treatment, he stabilized, and a biopsy confirmed he has neurofibromatosis, a condition that causes tumors to grow on nerves."
375,"The patient was a 7-year old boy from Miandoab, a city in the south of West Azarbaijan province, Iran. He exhibited no history of prenatal and postnatal diseases and had a normal growth. His healthy parents were cousins. He had successfully passed the first grade of the primary school. The patient suddenly experienced febrile serial generalized tonic-clonic seizures and the hospitalized in Shahid Abbasi teaching Hospital in Miandoab.\nAfter controlling his seizures using the bolus doses of phenobarbital and phenytoin, his consciousness level decreased, thereby necessitating endotracheal intubation and assisted ventilation. Then the patient was transferred to our tertiary subspecialty ward in Urmia’s Motahari Hospital. When we visited the patient for the first time, he was suffering from a deep coma; however, his vital signs were normal.\nOn physical examinations, his pupils were dilated with inadequate response to light, the liver was palpable about 4cm below the costal margin; his muscular tone severely diminished, and deep tendon reflexes were undetectable. His examination was otherwise normal.\nRoutine laboratory tests revealed a nonketotic hypoglycemia () and elevated liver enzymes (). According to the endocrinology consultation, a blood and urine sample was obtained to further evaluation into the exact cause of hypoglycemia. The obtained results ruled out ethiologies such as lipid malabsorbtion (TG=92 mg/dl¸ cholesterol 73 mg/dl¸ LDL=37 mg/dl; disorder of respiratory chain (lactate =13 ng/dl)¸ adrenal insufficiency ( cortisol=62.4µg/dl)¸ hypopituitarism (ACTH=411 pg/ml), and hyper insulinemia (insulin= 0.7µIU/ml)[Figure 3]\nLaboratory tests suggested by our pediatric gastroenterologist to detect the viral or immune cause of hepatitis revealed nothing, and the urine toxicology screening test was negative. Since his older male sibling died five years ago with the similar symptoms, the inherited inborn errors of metabolism were highly likely; thus, we delivered dried blood spot samples to a lab in Germany for tandem mass spectrometry. The brain computerized tomography revealed no edema. Unfortunately, the patient died two days after admission due to multiple organ system failures. The acylcarnitine analysis showed significantly elevated levels of medium-chain acylcarnitines (hexanoylcarnitine(c6)1.3µmol/lit (0- 0.15) and octanoylcarnitine(c8)0.73 µmol/lit (0- 0.23), which is compatible with medium-chain acyl-coA dehydrogenase deficiency ().\nThe filter paper screening also revealed no indication of congenital hypothyroidism ¸ adrenal hyperplasia¸ galactosemia ¸biotinidase deficiency¸ amino acid metabolism disorders and tyrosinemia typ1. Molecular genetics verification was impossible due to the patient's death.","A 7-year-old boy from Iran suddenly had seizures and was rushed to the hospital. While doctors stabilized him with medication and breathing support, he fell into a deep coma and sadly passed away. Tests revealed he had low blood sugar and elevated liver enzymes, and an analysis of his blood suggested a rare metabolic disorder. Because of his death, genetic testing couldn't be done, but the results pointed to a condition called medium-chain acyl-coA dehydrogenase deficiency."
376,"A 10-year-old girl was referred to our center due to a lack of balance and urinary incontinence from three weeks ago. The patient was the third child of consanguineous parents. Nervous development of the patient was normal before the onset of disease. The disequilibrium had progressed gradually and was consistent with the symptoms of cerebellar involvement and urinary incontinence. Due to prior low-grade fever, the patient had been treated with the suspicion of viral cerebellitis in the previous center and then referred to us owing to the worsening of symptoms.\nDuring the physical examination, the patient was unable to sit and walk independently, and cerebellar tests, including finger to nose and tandem gait, were abnormal, deep tendon reflexes were diminished, and Babinski sign was detected bilaterally\nBrain and cervico-thoraco-lumbar magnetic resonance imaging was performed for further investigation. These tests were normal. Lumbar puncture was also normal (glucose=50 mg/dl, protein= 30 mg/dl white blood cell = 3, and red blood cell = 0). High concentration of lactate dehydrogenase (LDH=4775) and anemia (Hb=8.8 gr/dl, mean corpuscular volume=104 fL, Red blood cell= 2540000, platelet = 163000) were detected in biochemical tests. Thus, the possibility of malignancy was raised. Organomegaly and lymphadenopathy were not seen in abdominal sonography, and hypercellular marrow with megaloblastic changes was observed in bone marrow examination. In addition to these data, elevated mean corpuscular volume (MCV=104 fL) with hyper segmented neutrophil in peripheral blood smear was noted, and the diagnosis of megaloblastic anemia was established. Consequently, vitamin B12 and folate levels were assessed. The serum level of vitamin B12 was found to be 70.41 pg/ml., which was significantly lower than the normal range (160-970 pg/ml). The range of folate was also normal.\nRegarding the low prevalence of vitamin B12 deficiency in healthy persons, extensive studies have been performed to find out the cause. In order to rule out atrophic gastritis, gastric and duodenal endoscopy and biopsy were performed. The data from the stomach and upper gastrointestinal tract were normal. There was no evidence for celiac disease in duodenal biopsy, and serum anti-tissue transglutaminase (TTG) was within the normal range. Gastrointestinal transit evaluation showed no defect in the mucous membranes of jejunum and ileum. Thus, gastrointestinal disorders were rejected. In urinalysis, 2+ proteinuria was observed with no leukocyturia and hematuria. Further nephrological evaluations, including renal sonography and other specific tests, demonstrated proteinuria (random urine protein/creatine ratio was 1) with an unknown etiology. To rule out the metabolic disorders that can interfere with the metabolism of vitamin B12, chromatography of blood amino acids, urine organic acids assessment, and tandem mass spectrometry were carried out, but no abnormality was detected.\nFinally, based on the investigations and hematologic and nephrological findings, the diagnosis of Imerslund-Gräsbeck syndrome was established. The patient was treated with a high dose of vitamin B12 daily injection (1000 microgram intramuscular) for one week, followed by 1000 microgram weekly, leading to improved balance. In one-month follow-up, she was able to walk, her cerebellar symptoms had greatly disappeared, and the patient had no incontinency; however, proteinuria persisted.","This 10-year-old girl has been having trouble with balance and leaking urine for the past three weeks. Tests revealed a problem with her cerebellum, the part of the brain that controls balance and movement. Blood tests showed low levels of vitamin B12, leading to a diagnosis of Imerslund-Gräsbeck syndrome, which explains the vitamin deficiency. After starting vitamin B12 injections, she has improved her balance and stopped leaking urine, but she still has some protein in her urine that needs further monitoring."
377,"A 22-year-old male presented to the Department of Emergency Medicine with complaints of sudden onset right flank pain accompanied by gross hematuria. Pain was moderate in intensity, nonradiating with no history of trauma or fever. Vitals including pulse, blood pressure, and respiratory rate were within normal limits. Urine microscopy demonstrated multiple red blood cells suggesting hematuria. The patient was referred to the Department of Radiology for ultrasonography of the abdomen which revealed a well-circumscribed, heteroechoic, cortical based lesion measuring 2.0 × 1.8 cm located in the upper pole of right kidney. Color Doppler demonstrated no significant internal vascularity within the lesion (). Furthermore, a well-defined heteroechoic mass was noted at the dependent portion of the urinary bladder, suggestive of a giant retained clot (). Chest radiograph, chest computed tomography (CT), and bone scans were all negative for metastasis. Based on the radiological findings, a diagnosis of renal oncocytoma with symptomatic hematuria leading to giant clot retention in the urinary bladder was made. The patient was referred to the Department of Urology for further management where she underwent laparoscopic partial nephrectomy of the right kidney and tumor resection. The resection margins were free of tumor, and there was no evidence of perinephric invasion or lymphadenopathy. Histopathological examination of the resected specimen revealed round and polygonal cells within a loose stromal background, eosinophilic granular cytoplasm, round nuclei with inconspicuous nucleoli, and absent or rare mitotic figures consistent with a diagnosis of renal oncocytoma (). The patient was further catheterized, and urinary bladder irrigation with clot retraction was performed. The patient recovered well with no complications and was discharged home in good condition on the seventh day post operation. At 3 months follow-up, the patient was free of symptoms and had no signs of recurrence.\nThe patient has given written informed consent to publish his case and clinical images.","A 22-year-old man went to the hospital because of sudden, sharp pain in his side and blood in his urine. Tests showed a small, non-cancerous growth in his right kidney and a large blood clot in his bladder. Doctors removed the kidney growth and the clot through surgery, and the margins of the surgery were clear. The patient is now symptom-free and has been closely monitored for three months with no signs of the problem returning."
378,"A 50-year-old male presented with hematuria, increased frequency, and burning sensation during micturition along with left flank pain for 2 months. There was a history of generalized weakness, weight loss, and loss of appetite during this period. On examination, the patient was poorly nourished with the presence of a palpable left-sided abdominal mass.\nUltrasonography (USG) of the abdomen showed the presence of right ectopic kidney and left-sided hydronephrosis. A heterogeneous mass was noted in the left kidney. Another polypoidal mass was also seen in the lumen of the urinary bladder, attached to its posterolateral wall.\nContrast-enhanced computerized tomography (CECT) of the abdomen helped in the renal anatomy and characterization of the mass, which revealed L-shaped crossed fused renal ectopia. The right kidney was not present in the right renal fossa and was in the midline, anterior to the aortic bifurcation at the L4-L5 level. It was malrotated and fused with the lower pole of the left kidney. The left kidney was enlarged, with a large soft heterogeneous tissue density mass involving the interpolar and lower pole regions that exhibited heterogeneous enhancement with central non-enhancing areas. Few calcified foci were seen in the mass, along with moderate hydronephrosis. In the delayed phase (15 minutes), no contrast excretion from the left kidney was recorded. The interpolar region of the right kidney was contiguously infiltrated by the left lower pole renal mass. Small tumor thrombi were present in the segmental right renal veins draining the interpolar region. Aortocaval, para-aortic and left renal hilar lymphadenopathy were also noted.\nAlong with these findings, a well-defined polypoidal mass was seen in the left posterolateral wall of the urinary bladder, infiltrating the left vesicoureteral junction. The middle and distal parts of the left ureter were contiguously involved by this urinary bladder mass. A peripheral rim of calcification was present.\nBecause of the involvement of multifocal enhancing masses of the moieties of crossed fused renal ectopia, urinary bladder, and left ureter, the radiological differential diagnoses offered were multifocal transitional cell carcinoma, renal cell carcinoma (RCC)- mucinous adenocarcinoma variant with multifocal spread, and renal sarcoma.\nNo distant lesion was found on metastatic work-up.\nInitially, transurethral resection of the bladder lesion was done. Microscopic examination showed features of noninvasive papillary urothelial carcinoma, predominantly low-grade with high-grade focal areas, along with extensive dystrophic calcification and necrosis, and focal osseous metaplasia ().\nThe patient was then taken up for surgery for resection of the renal mass, and a left nephrectomy with partial right nephrectomy was also performed. The specimen was submitted for histopathological examination. The results showed that the capsule was intact. Cut section of the left kidney showed a tumor measuring 14x11x10 cm, replacing the entire normal structure. The renal pelvis was not identified, and part of the right kidney consisted of cystic and solid areas, with tumor measuring 3x2.5x2 cm ().\nMultiple sections examined from both the kidneys showed a tumor composed of large areas of cartilaginous differentiation along with tumor cells arranged in diffuse sheets and fascicles. Marked pleomorphism and mitotic activity were noted, 18-19/10hpf. There was an abrupt transition to well-differentiated nodules of hyaline cartilage. Intervening stroma showed consistent focal areas of myxoid change with chronic inflammatory cell infiltrate. The focal osteoid formation was present, with numerous giant cells and apoptotic debris. Areas of chicken-wire calcification and hemorrhage were also identified, along with large necrotic sections ().\nThe tumor cells were immunopositive for CD99 with strong S100 protein expression in the areas of cartilaginous differentiation, immunonegative for pan-cytokeratin, CK7, CK20, p63, desmin, and myogenin, and with retention of INI1 expression ( and ). These morphological and immunohistochemical features suggested the presence of primary chondrosarcoma in crossed fused renal ectopia involving both moieties.\nThe patient was lost to follow-up after discharge from the hospital after an uneventful postsurgical period of 2 weeks.","This 50-year-old man has been experiencing problems with his urinary system for two months, including blood in his urine, frequent urination, and pain in his lower back. Tests revealed he has a rare condition where his kidneys are fused together and one kidney is misplaced. A tumor was found in both kidneys and the bladder, and after surgery to remove the affected kidneys, the pathology revealed it was a type of cartilage cancer called primary chondrosarcoma."
379,"A 25-month-old boy with fever and maculopapular rashes was admitted to Mofid Children's Hospital. His parents explained that fever began three days ago and rashes developed after one day. The rashes started with mild itching in the feet, spreading to the thighs and the genital area on the second day. Despite taking antihistamines, the fever and rashes continued on the third day. The patient presented to the hospital with fever, malaise, poor feeding, mucosal involvement of the mouth, lips, conjunctiva, and maculopapular rashes, which resulted in ulcer and bulla formation (). On arrival, he had the following vital signs. Temperature: 39.5°C, blood pressure = 82/10 mmHg, respiratory rate = 28, and pulse rate = 86. The patient's clinical course, blistered skin lesions, and mucosal involvement led to the primary diagnosis of SJS/TEN. Reviewing his medical history revealed that ranitidine was the only medication he had used in the past three weeks. Moreover, his mother had a history of upper respiratory infection (URI) three weeks ago, which was accompanied by low-grade fever and resolved in three days. The patient received supportive care, steroids, and intravenous immune globin (IVIG) based on the primary diagnosis of SJS/TEN. In the meantime, laboratory work up and a COVID-19 PCR test were performed. The results showed white blood cells (WBC) count = 3200/μl (Polymorphonuclear (PMN): 58% and lymph: 41%), hemoglobin (Hgb) = 12.3 gr/dl, and platelet count = 29000/μl. Additionally, the erythrocyte sedimentation rate (ESR) was 36 mm/hr and the CRP level was 58 mg/l. Liver function test, blood urea nitrogen (BUN), creatinine (Cr), albumin, and lactate dehydrogenase (LDH) were within the normal ranges. However, ferritin and fibrinogen levels were elevated (517 μg/L and 615 mg/dL, respectively) and the COVID-19 PCR result was positive. The findings of the chest CT scan were unremarkable. Considering the patient's general conditions, fever, and laboratory findings, MIS-C was diagnosed and atazanavir was added to his treatment. On the following day, his fever subsided and he began to eat and drink. After four days, he was discharged from the hospital with minimal skin lesions and a normal condition (). The patient was followed after one week, indicating that his laboratory test results were within the normal ranges and he was doing great.","A 25-month-old boy was admitted to the hospital because he had a high fever and rashes that were spreading across his body. The rashes started with itching and then became more severe, causing blisters and sores on his skin and inside his mouth. Tests showed he had a viral infection (COVID-and a condition called SJS/TEN, which causes severe skin reactions. He received treatment with medications and supportive care, and his fever and symptoms improved, allowing him to go home after four days."
380,"A 53-year-old woman was referred to our clinic with waist and back pain and numbness of the lower limbs for more than 1 month. The pain was not related to her posture and became more prominent when she moved. She had a medical history of lumbar disc herniation and no history of trauma. On initial evaluation, her vital signs were stable. Apart from the pain of the waist and back, physical examination revealed unremarkable findings. Routine blood tests were obtained. Further, liver function tests revealed normal results. The blood CA199, CA125, CEA, and AFP levels were also within normal limits.\nComputed tomography of the chest revealed scattered pulmonary nodules with calcifications associated with a soft tissue mass measuring 3.3 cm × 2.4 cm and without pleural thickening at the superior lobe of the right lung () (SOMATOM definition, Siemens Healthcare, Erlangen, Germany; tube voltage, 100-120 kVp; tube current, 450 mA; slice thickness, 0.625 mm; pitch, 0.992:1; rotation speed: 0.5 s/rot; ASIR-V:30%.). Enlarged lymph nodes of the right hilar were also evident. Abdominal contrast-enhanced CT revealed diffuse lesions with massive calcifications in the liver, which shows faint peripheral enhancement in the arterial phase and low enhancement in the portal phase (Iopromide Injection, Bayer Pharma AG; the arterial phase and portal venous phase were obtained at 25 s and 60 s after contrast injection.). The largest lesion measuring 10.2 cm × 5.9 cm was located in the right lobe of the liver and (). CT examination also revealed osteolytic lesions with a massive thick sclerotic rim in the right second rib, 11th thoracic vertebra, and first lumbar spine. Bone scintigraphy with 99mTc-methylene diphosphonate showed multiple hypermetabolic activities in the involved bones (). Cerebral magnetic resonance imaging (MRI) revealed no anomalies. The patient underwent transthoracic needle biopsy of the largest pulmonary lesion located in the right superior lobe. Histopathological analysis revealed epithelioid cells arranged in a glandular pattern with clear cytoplasm (). Immunohistochemical staining showed that the neoplastic cells were positive for CD31, CD34, CAMTA1, and EMA, but negative for ERG, TFE3, PCK, and desmin, with a Ki-67 index rate of 10%. Histopathological examination indicated a rare low-grade malignant vascular neoplasm, confirming the diagnosis of EHE.\nConsidering the multiple intra-pulmonary, right hilar lymph node, liver, and bone metastases, the patient was treated with chemotherapy with paclitaxel liposome (240 mg/m2; day 1) and carboplatin (550 mg/m2; day 1). At 8 months, the patient had completed four cycles of combination therapy. There were no changes in the patient’s disease status on CT at the 8-month follow-up visit.","A 53-year-old woman came to the clinic complaining of persistent pain in her back and legs, along with numbness. After some tests, including CT scans and biopsies, doctors discovered several concerning findings, including lung nodules, liver lesions, and bone tumors. A rare type of cancer called EHE was identified through the biopsy results. The patient is currently undergoing chemotherapy and will continue to be monitored closely."
381,"The following case is presented to illustrate, using a biopsychosocial-ecological perspective, three different approaches to serving persons with dementia and their caregiver(s) in clinical practice.\nPresenting concerns: Janice is an 85-year-old woman who lives independently in senior housing in the Canadian province of Alberta. In response to Janice's increasing needs for support, Gwen, her daughter and primary caregiver, scheduled an appointment for them to meet with her mother's Geriatrician to discuss changes in Janice's health and function related to her progressing dementia, and planned to discuss her own needs for support as well.\nGwen reported to the geriatrician that her mother's decline had been steady since her last appointment, most notably in her short term memory such that she was increasingly losing items, struggling to recall recent events, forgetting names, and having difficulty finding words, managing complex tasks, and planning. She shared that her mother had developed paranoia and visual hallucinations over the past year during which she imagines that strangers are trying to get into her home to steal her treasured belongings. The hallucinations had increased steadily and had worsened over the past month now occurring multiple times per week usually at night. Gwen also reported that Janice calls her frequently asking for help, and she noticed her mother being more irritable, angry, and frustrated than she used to be. She shared that her mother wanders out of her room but has not gotten lost.\nGwen also noted a “quite rapid” decline in Janice's function. Because she was no longer able to use the stove and had burned pots, she ultimately stopped cooking and depends on microwave-ready meals and easy snacks. Even with Gwen bringing her meals, however, Janice has had a 20 pound weight loss over the past year. Janice can still perform basic activities of daily living such as dressing, grooming, bathing, feeding, toileting, transfers and mobilization. She can still use the phone and does housekeeping and laundry on her own, but Gwen finds clothes soaked in urine in the laundry and believes that her mother has not bathed in a month. Gwen now manages her mother's money, medical appointments, and medications, and does her shopping and other errands as well.\nJanice's neighbors and building management started to raise concerns to Gwen about her mother's safety, which Gwen reported has greatly increased her own anxiety about her mother's living situation. They reported that Janice is seen wandering around the facility at all hours and often checks in with other residents when she gets confused about day and time. There are times when she will knock on her neighbors' doors asking for help while experiencing hallucinations. They know her well and reassure and redirect her but Gwen wonders how long they will be willing to do this. Janice adamantly denies needing assistance but Gwen was finally able to get her to accept homecare for help with medications. The agency recently informed Gwen, however, that Janice does not always open the door for the homecare attendants and that she sometimes calls them derogatory names and yells at them to “get out.”\nConcurrent problems: While Janice has experienced urinary incontinence for years, she was managing on her own with pads and then protective underwear as the incontinence worsened. Gwen describes her mother's bladder control as “good during the day” but notes that she “occasionally soaks her night clothes and bed during the night.” Janice also has occasional bowel incontinence and Gwen noticed that her pericare had declined and shared that she had found smeared stool around the toilet. The geriatrician also expressed concern about Janice's sensory deprivation noting that she is legally blind due to macular degeneration and that she suffers from bilateral hearing loss and has been unable to manage hearing aids on her own. Janice's other medical conditions include hypertension, osteoporosis, osteoarthritis, and hypothyroidism. She never smoked, rarely consumes alcohol, and gave up driving 3 years ago because of her vision loss.\nMental exam: The geriatrician noted that Janice was alert and cooperative and that she needed a pocket talker to hear. She scored 24/30 on the Mini-Mental State Exam () and 18/30 on the Montreal Cognitive Assessment (), both of which indicate “mild dementia.” The Clock Drawing Test (), a measure of spatial dysfunction and neglect, was abnormal. She correctly placed the numbers on the clock face but could not tell time. She had problems with orientation and displayed both short and long term memory deficits. Language skills were intact other than occasional word finding problems. She appeared anxious and got easily irritated. She needed reassurance to complete the assessment. She was occasionally distracted by visual hallucinations (e.g., she saw people in the room and wanted them chased away). She denied symptoms of depression. She had poor insight into her cognitive and functional decline and displayed poor mental reasoning when it came to supports needed to help her with her health and housing. She overestimated her abilities and did not recognize the degree of supports being provided to her. She acknowledged that her daughter provides some help but said she could manage without it. She expressed annoyance with having homecare.\nPhysical exam: No apparent distress.\nFamily and social history: Janice completed education through Grade 8 and worked as a secretary until she had children. She has been widowed for 20 years after having been a caregiver to her husband who died of cancer. She has 3 daughters, 1 son, and 8 grandchildren. Gwen, the youngest, her primary caregiver, and “the baby” of the family, is married, has 2 children, and lives 10 min away. Janice's son, Jack, is an accountant who lives out of town, helps with higher level financial management such as taxes, and is a source of emotional support for Gwen. Janice often mentions that Jack “leads a busy life with work and family” as an explanation for his infrequent visits. Her two older daughters are both married, retired, and live in other provinces. They check in about their mother periodically and visit once a year. Neither of the two older daughters is close to Janice or Gwen with the emotional distance rooted in their shared belief that their mother favored their two younger siblings when they were growing up. Gwen and Jack have remained close and frequently discuss their mother's deteriorating health and function. Janice has lived in her current residence, a subsidized senior housing facility, for the past 30 years. She has limited finances, including her husband's pension and her own, and she relies on her children to assist with money as needed.\nPatient's values and beliefs: Janice does not want to leave her home. She is feisty and wishes to remain independent. She is fond of her belongings and takes pride in them– e.g., furniture, paintings, pictures, collectibles, etc. She believes that she raised her children well and gave them a good education, and she now expects reciprocity. She acknowledges the support provided by her daughter but is not particularly empathic toward her stress.\nMedical and legal issues: Janice designated Gwen and Jack as the agents in her Personal Directives and Enduring Power of Attorney (EPOA), respectively. The EPOA was activated at the time it was established. Janice's Goals of Care Designation, a medical order used in Alberta to describe and communicate the general focus of care including the preferred care location, indicates that goals and interventions are for cure or control of illness. Her goals exclude the option of ICU care, while transfer to an acute care facility may be considered if required for diagnosis and treatment.\nCaregiver stress: Gwen is committed to caring for her mother and determined to support her at home. She reported that she had promised not to relocate her to a “nursing home.” However, she admits to feeling “very stressed” caring for her mother. She is the only one in town and has taken over the majority of the responsibilities. Janice is quite demanding and calls her day and night asking for help. She gets easily irritated and angry with Gwen who has already reduced her hours at work by going part-time. Gwen believes at this rate she will have to quit work all together. This adds to her stress because she feels guilty about harming her family's financial situation. She and her husband annually spend $6,000 subsidizing her mother's housing, food, and health care supplies. Gwen is keenly aware that their daughters are approaching college age and that this is not the time to leave the workforce. She feels that her life is “on hold.” Her husband and children are supportive and help however they are able. She resents the lack of support from her sisters but finds her brother more supportive as he provides her with emotional support and helps to support their mother financially. At the same time she feels he could visit more often. She shared that caregiving is taking a toll on her health as she is experiencing panic attacks, insomnia, poor concentration, feelings of guilt, and chronic migraines, in addition to having emotional and physical symptoms associated with perimenopause.","85-year-old Janice is experiencing a decline in her memory and thinking skills, which is causing confusion, paranoia, and hallucinations. She’s having trouble remembering things, recognizing people, and managing daily tasks, and she’s experiencing visual hallucinations. Her daughter, Gwen, is the primary caregiver and is struggling to balance her own life with caring for her mother. Gwen is worried about Janice’s safety and well-being, and she’s feeling overwhelmed by the demands of caregiving. Janice also has several other health issues, including urinary and bowel incontinence, vision loss, and hearing loss, which are contributing to her difficulties and increasing Gwen’s stress. Doctors are exploring options to help Janice, including potential deep brain stimulation surgery, and are working with Gwen to find ways to support her and ensure her safety and comfort."
382,"We present a case of a 58-year-old female patient with a large recurrent ventral hernia. Six years before, the patient had been operated on for the umbilical hernia, with the simple repair without a mesh. The patient was an active smoker who suffered from morbid obesity with a body mass index of 43 kg/m2 and COPD as comorbidities relevant for this case report.\nThe patient was introduced to the surgeon during hospitalization at the gastroenterology department where a diagnostic workup due to a clinical picture of chronic small bowel obstruction was conducted. While taking the anamnesis, the patient reported frequent abdominal cramps, swelling, and pain in the area of the hernia that had intensified in the last few weeks. The physical examination revealed a large irreducible ventral hernia in the lower abdomen that was quite painful on palpation, but soft and, at that time, without signs of incarceration or strangulation. Taking into account the clinical picture with threatening hernia incarceration, the surgeon did not opt for preoperative optimization of the patient in terms of smoking cessation and starting a weight loss program but made an indication for semielective surgery.\nOn operative procedure, greater omentum, part of the transverse colon, and a cluster of small bowel loops with signs of chronic obstruction were found as hernial content. After adhesiolysis hernial content was reduced into the abdominal cavity. Hernial defect measuring about 7 cm in diameter and about 15 cm in the vertical line with significant rectus diastase in the supraumbilical part of the abdomen was revealed. Using the Rives-Stoppa technique a wide retromuscular space was created. Lateral dissection boundaries of this space were perforating neurovascular bundles in the area of the lateral edges of the rectus muscle on both sides. The posterior fascia was easily closed using also a portion of the hernia sac to bridge the defect between the posterior rectus sheaths. A 30 × 25 cm polypropylene mesh was placed in the retromuscular space ensuring adequate mesh overlap over the edges of the hernia defect of a minimum of 5 cm in all directions.\nWhen we observed that the anterior fascia, due to the size of the defect and decreased abdominal wall elasticity, would not close entirely and cover the mesh, we opted for rectus mobilization by the ACS method to avoid bridging. Upon extensive dissection of the anterior abdominal wall subcutaneous space without preservation of the rectus perforator vessels, relaxing incisions of the external oblique muscle aponeurosis were performed. Using the Ramirez technique, long longitudinal incisions of aponeurosis were made bilaterally, adjacently to the semilunar line, extending from the costal arch to the groin. This procedure resulted in the considerable mobilization of the vital musculofascial flap medially, and the hernial defect was closed at the midline without tension. Then, four redon drains were placed, i.e., 2 in the retrorectus space and another 2 in the subcutaneous space.\nThe postoperative course was complicated by skin ischemia. Ischemic lesions of the abdominal wall skin on the right with signs of necrosis along the midline were observed already on day 8 (). On postoperative day 11, multi-slice computed tomography (MSCT) of the abdomen was performed because of the ever more abundant wound discharge. MSCT findings showed a large subcutaneous seroma, a normal musculofascial component of the abdominal wall, appropriate mesh position, and normal intra-abdominal status. Percutaneous puncture of seroma was performed and about 800 ml of clear seroma was evacuated. During the next 10 days, ischemia progressed, along with the development of another two full-thickness skin necrotic foci paramedially (). Considering the relatively strict demarcation area of necrosis, we opted for the operative procedure of necrosectomy.\nFollowing abdominal wall necrosectomy with a safety margin of healthy tissue and considering an appropriate amount of vital residual abdominal skin, as well as the absence of signs of local tissue infection or mesh infection, primary wound closure was performed in consultation with a plastic surgeon (). As early as day 4 of the second operation, increased wound discharge and signs of skin wound dehiscence occurred, which required removal of skin sutures (). Then, a wound dressing with a hypertonic solution was applied for a week.\nWhen inflammation subsided, negative pressure wound therapy (NPWT) with the “Renasis Ez Max VAC® system” (Smith & Nephew, Mississauga, Canada) was initiated (). NPWT was delivered in continuous mode with negative pressure maintained at −100 mm Hg. Dressing in the form of a sponge of polyurethane black hydrophobic foam was changed every third day. After 2 weeks of NPWT administration, considerable improvement was recorded in wound cleaning and formation of healthy granulation tissue (). NPWT was continued for the next 2 months, which resulted in further improvement of condition of the patient, along with decreased wound discharge and cavity reduction. The wound swab obtained twice during dressing change was sterile. The patient was discharged from the hospital and regular changing of silver-impregnated antimicrobial wound dressing (Aquacel Ag, ConvaTec, Reading, United Kingdom) was continued in ambulatory care that led to complete wound closure in 7 months ().","This 58-year-old woman was admitted to the hospital because of a large, painful hernia in her abdomen that was threatening to become trapped. She had this hernia repaired years ago, but it had returned. The surgeon found that the hernia contained part of her small intestine and carefully removed it, closing the defect with a mesh to prevent it from happening again. However, the surgery caused some skin problems and tissue damage, requiring further treatment with drainage and wound care."
383,"A 16-year-old male with DOCK8 deficiency (homozygous for DOCK8 variant NM_203447.3:c.4153+1G>A) enrolled on an IRB-approved National Cancer Institute HSCT trial for patients with DOCK8 (NCT01176006). His disease, diagnosed at age 8 years, manifested with recurrent sinopulmonary infections, chronic molluscum contagiosum, and eczematous dermatitis. Recent complications included diagnosis of Diffuse Large B-Cell Lymphoma (DLBCL) 4 months pre-HSCT. Treatment with rituximab (4 doses) and LMB regimen (, ) (2 cycles), including vincristine, doxorubicin, corticosteroids, methotrexate and with cumulative cyclophosphamide dose of 3300 mg/m2 as per ANHL1131, Group B (R-COPADM), was complicated by chemotherapy-associated grade III BKV-HC managed with cidofovir and 2 infusions of third-party donor-derived quadrivalent anti-cytomegalovirus (CMV), -Epstein-Barr virus (EBV), -adenovirus (ADV), and -BK virus specific T-cells (VSTs) at 5x107 cells/m2 (NCT02532452) (). VSTs were well tolerated without any infusion reaction. HC symptoms subsequently resolved while asymptomatic BK viremia persisted. Achieving a complete remission, he proceeded to HSCT with his father as the haploidentical bone marrow donor. Reduced intensity (RIC) conditioning was comprised of fludarabine 30 mg/m2 x 5 days (days -6 to -2), busulfan dosed with target area under the curve (AUC) of 3600-4000 uM.min/day x 3 days (days -4 to -2), cyclophosphamide 14.5 mg/kg x 2 days (days -6 and -5), and low-dose total body irradiation (TBI, 200 cGy) on day -1. Graft-versus-host disease (GVHD) prophylaxis was comprised of post-transplant cyclophosphamide (PT/Cy) on days +3 and + 4 along with mycophenolate mofetil (MMF) x 30 days and tacrolimus x 6 months, both of which started on day +5 (). Mild cystitis without hematuria developed on HSCT day 0. BK viremia simultaneously increased from 588,844 copies/mL (3 days pre-HSCT) to 6,456,542 copies/mL on day +4 ().\nHis immediate post-HSCT course was complicated by gross hematuria and painful bladder spasms with PT/Cy (50 mg/kg) on days +3 and +4. Grade IV gross hematuria, managed with daily blood product transfusions, persisted alongside worsening acute kidney injury (AKI, day +14) (). Continued transfusion dependence led to significant fluid retention and 11 kg weight gain over 11 days. Daily platelet infusions were used to maintain platelet count ≥ 30 K/mcL, and platelet engraftment ≥ 50 K/mcL was not attained. Neutrophil engraftment was achieved at day +21; chimerism studies demonstrated 100% donor-derived cells without evidence of GVHD.\nProgressive hydronephrosis and hydroureter prompted Foley catheter and bilateral nephrostomy tube placement by day +30. Gross hematuria from the left nephrostomy tube occasioned left renal arteriography which revealed active bleeding unrelated to the nephrostomy at multiple sites and features suggestive of vasculitis. Coil embolization performed on two separate occasions (days +35 and +38) provided only transient stabilization. Ongoing bleeding and transfusion needs were accompanied by increasing total and direct hyperbilirubinemia (2.9 mg/dL and 2.7 mg/dL, respectively (day +43)). Complete left renal artery embolization on day +42, followed by initiation of intravenous cidofovir (), stabilized the patient for transport to receive an additional infusion of third-party VSTs (day +43) produced using the same donor as his second pre-HSCT infusion (NCT02532452). BK viremia measured 5,754,399 copies/mL at third infusion, nearly a full log increase from viral load with pre-transplant VSTs ().\nTwo days after VST infusion (day +45), onset of cytokine release syndrome (CRS) was evidenced by fever, hypotension, worsening lung opacities, and bilateral pleural effusions. Inflammatory markers indicative of CRS were also elevated. CRP peaked at 268.7 mg/L on day +43 following VST infusion. Interestingly, CRP had been rising in the days leading up to VST infusion, potentially in the context of significant bleeding and interventional procedures. A rapid rise in ferritin was seen from 4,641 mcg/mL pre-VSTs (day +40) to 5,313 mcg/mL post-infusion (day +45) and 17,456 mcg/mL on day +47 (). Plasma IL-6 rose from 360 pg/mL pre-infusion (day +41) to 2,182 pg/mL on day +45 with CRS onset and prior to initiation of the IL-6R (receptor) blocker tocilizumab (). Concurrent blood, urine, and viral testing remained negative for any signs of new infection. Aggressive fluid resuscitation, bilateral chest tube placement, vasopressor support, continuous renal replacement therapy (CRRT), and 4 doses of tocilizumab (8 mg/kg) were given over 48 hours. Following fluid resuscitation for CRS, liver studies (day +47) demonstrated worsening hyperbilirubinemia. Liver ultrasound showed hepatosplenomegaly and sluggish flow through the main portal vein, raising concern for late-onset sinusoidal obstruction syndrome (SOS)/veno-occlusive disease (VOD). Given prior life-threatening hemorrhage, defibrotide was contraindicated. Multiorgan failure and worsening coagulopathy led to hypoxic arrest on day +63. Autopsy was declined but limited postmortem single core liver, kidney, and lung biopsies demonstrated hepatic SOS/VOD with zone 3 hemorrhagic necrosis, acute renal tubular injury, and early pulmonary exudative phase diffuse alveolar damage (). Limited tissue SV40 immunostain for polyomavirus was negative at all 3 sites.","This 16-year-old boy has a rare condition called DOCK8 deficiency that caused him to have frequent infections and skin problems since he was young. Recently, he developed lymphoma, and doctors started him on a high-dose chemotherapy treatment with a bone marrow transplant from his father. While the transplant was successful in removing the lymphoma, he experienced some complications, including significant bleeding and kidney problems. He also developed a severe reaction to the stem cells used in the transplant, requiring intensive treatment. Sadly, despite all the efforts, he passed away due to complications from the transplant."
384,"A 77-year-old man had CAD and underwent percutaneous coronary intervention (PCI) in November 2017 and October 2019. The patient had severe AS with trans-aortic valve mean pressure gradient of 47 mmHg and received TAVR with a 29 mm Edwards Sapien 3 valve uneventfully in November 2019. A day after TAVR, echocardiographic-derived mean trans-aortic valve pressure gradient of the patient was 15 mmHg (). Dyspnea was improved, and the patient had good exercise tolerance thereafter. One year later (October 2020), the patient suffered from worsening heart failure with pulmonary edema. ECG of the patient revealed a new-onset Af and the echocardiogram disclosed an increased mean THV pressure gradient to 48 mmHg (), worsening mitral regurgitation (MR), and pulmonary hypertension (PH). Response of the patient to standard heart failure treatment, such as intravenous inotropics and diuretics, was poor. MDCT revealed HALT and RLM (). On the top of the concurrent single antiplatelet medication for CAD of the patient, a NOAC (rivaroxaban) was added for the new-onset Af and leaflet thrombosis. A series of follow-up echocardiograms within 3 months showed a progressive drop in trans-aortic valve pressure gradient to 17 mmHg (), together with reduced MR and PH. MDCT showed resolution of HALT and RLM () 4 months after NOAC treatment. Heart failure symptoms improved gradually but Af persisted. Unfortunately, the patient had a passage of tarry stool and drop of hemoglobin to 7.2 mg/dl in the fifth month after concomitant use of clopidogrel and rivaroxaban. The patient received a therapeutic endoscope, proton pump inhibitors, and blood transfusion for stopping the upper gastrointestinal bleeding. The patient discontinued clopidogrel but kept on using rivaroxaban and was free from heart failure symptoms and bleeding events thereafter.","This 77-year-old man had a history of heart problems, including a heart attack and a new valve replacement. After the valve replacement, he initially felt much better, but then his heart failure got worse, leading to fluid buildup in his lungs. Tests showed a blood clot in his valve and other issues, and he started taking a new medication to prevent blood clots. Eventually, the clot resolved, and his heart failure symptoms improved, but he continued to have a heart rhythm problem."
385,"A 70-year-old man presented shortness of breath in July 2017. He did not report any oncological or autoimmune familial medical history, but had a personal history of type 1 diabetes, dyslipidemia, and arterial hypertension. A thoracoscopy allowed pleural fluid evacuation and the diagnosis of epithelioid malignant pleural mesothelioma. Frontline chemotherapy by cisplatin-pemetrexed was started and was switched to carboplatin-pemetrexed due to deterioration of renal function (6 cycles). In November 2017, he started vinorelbine due to pleural effusion relapse.\nIn March 2021, as he presented an increase of dyspnea and needed several thoracentesis, CT scan showed a nodular thickening of pleura. The tumor board decided to treat him with nivolumab in 3rd line (240 mg every 2 weeks). After the 1st infusion (March 18, 2021), he presented with grade 2 asthenia, grade 1 vomiting, and gastroesophageal reflux disease (GERD) with a 3-kg weight loss. Two days after the second infusion (March 31, 2021), the patient contacted us for asthenia, vomiting, and grade 3 diarrhea, limiting his quality of life (treated at home by diosmectite, loperamide, and racecadotril). The 3rd infusion was reported by 2 weeks. He was hospitalized just before the 3rd infusion because of watery and foul-smelling diarrhea, without blood, GERD, fluctuating nausea, and vomiting, complicated by dehydration and hypotension. Physical examination revealed a grade 1 sinus tachycardia, a known pleural effusion, and a normal abdomen. Biologically, he had normal plasmatic values of ionogram, TSH, ACTH, and cortisol. The dosage of total immunoglobulins was normal, and the serum protein electrophoresis only showed an inflammatory profile. Stool culture, Clostridium difficile research, and parasitological examination of the stool were negative.\nTo progress toward a diagnosis, we performed endoscopic evaluation. Ileocolonoscopy with ileal and colic biopsies were normal, eliminating Crohn’s disease, ulcerative colitis, or ICI-induced colitis. Esophago-gastroduodenoscopy (EGD) showed a major duodenitis with erythematous aspect and diffuse superficial ulcerations (). To eliminate infectious enteropathy, we performed intestinal biopsies with normal bacteriological and virological examination. Surprisingly, histological examination revealed elementary lesions of CeD: increased intraepithelial T lymphocytes (IEL) (>30 IEL/100 enterocytes), crypt hyperplasia, marked villous atrophy, and alteration of normal crypt/villous ratio (3:1) (). The CeD was graded type 3b according to the modified Marsh classification of histologic findings in CeD (Oberhuber). Serum IgA antibodies to tissue transglutaminase were positive (128 U/ml, with a lab norm of 10 U/ml) and anti-endomysial IgA were positive (80 U/ml, with a lab norm of 10 U/ml). Altogether, the endoscopic, histological, and biological results led us to the diagnosis of CeD induced by immune checkpoint inhibitors (anti PD-1 nivolumab).\nHe was initially treated with proton pump inhibitors (PPI) intravenously 200 mg/day for 48 h and then 120 mg/day (40 mg 3 times a day). He received PPI at 80 mg for 1 month. In the face of persistent diarrhea despite anti-diarrheal medication, methylprednisolone 1 mg/kg/day was introduced initially intravenously for 3 days, then prednisolone per os at a dose of 1 mg/kg/day for 2 months, followed by a progressive decrease until 10 mg/day. A test to eliminate adrenal insufficiency was performed, before stopping corticosteroid. A gluten-free diet was also introduced in hospitalization for a long-term perspective. The patient remained in the hospital for 2 weeks and nivolumab was stopped. Ten days after the end of hospitalization, he only had three diarrheas per day.\nTwo months later in the consultation, he mentioned a good clinical improvement and a weight gain with a good adherence of gluten-free diet. A control EGD was realized, finding a complete healing of the inflammatory aspect and of the ulcerations of the duodenum (). Duodenal biopsies showed grade I villus atrophy and mild chronic aspecific gastritis (without atrophy, metaplasia, dysplasia, or Helicobacter pylori). A control EGD is planned 6 months later and a lifelong gluten-free diet is recommended.\nIn an oncological perspective, 7 months after the last nivolumab infusion, the patient still had a stable disease, without requiring any additional line of systemic treatment ().","This 70-year-old man has been battling mesothelioma, a type of cancer, since He’s had several rounds of chemotherapy and then another treatment with nivolumab, a drug that targets the immune system. Unfortunately, this treatment caused side effects like diarrhea and nausea, which were difficult for him to manage. After a thorough investigation, doctors discovered that the diarrhea was actually caused by the nivolumab drug, leading to a condition called Celiac disease. He’s now following a strict gluten-free diet and taking medication to manage his symptoms, and his condition has stabilized."
386,"A 78-year-old female with past medical history including autoimmune hepatitis, hypothyroidism. She presented to the hospital via emergency medical services with left arm weakness and jerky movements. This event was witnessed by family while she was eating. No recent trauma or fall. No earache, hearing loss, or discharge. No loss of consciousness reported. Of note, she is on azathioprine for autoimmune hepatitis. She was evaluated by the stroke team upon arrival. Vital signs included: elevated blood pressure at 153/72 mmHg, normal pulse 91, and normal respiratory rate at 17. She was afebrile. Laboratory work up revealed normal white cell count (WBC) of 7.2 109/L, and normal hemoglobin of 12 gm/dL. Platelets noted to be low at 80 109/L. Serum chemistry was unremarkable except for low sodium of 129 mEq/L. Urine toxicology drug screen was negative. COVID-19 PCR (polymerase chain reaction) test was negative.\nA Computed Tomography (CT) head on admission revealed left temporoparietal intraparenchymal hemorrhage, right frontal sulcal subarachnoid hemorrhage, and left parietal sulcal subarachnoid hemorrhage. Vessel images with Computed Tomography Angiogram (CTA) head and neck revealed extensive venous sinus thrombosis involving superior sagittal sinus, bilateral transverse, and sigmoid sinuses. She subsequently underwent Magnetic Resonance Imaging (MRI) of the brain with and without contrast and Magnetic Resonance Venogram (MRV) which confirmed extensive venous sinus thrombosis and multicompartment bleeding. No restricted diffusion noted ().\nPatient was evaluated by interventional neurology, neurosurgery, and neuro critical care team. She was started on levetiracetam for symptomatic treatment of focal seizures with left upper extremity shaking. She was started on anticoagulation with heparin drip and was admitted to neuro ICU for close neurological monitoring.\nDesired therapeutic level of activated Partial Thromboplastin Time (aPTT) at 67.2 s was achieved at 24 h and patient remained in the neuro critical intensive care unit. After a thorough multidisciplinary team discussion due to persistent left-sided weakness, diffuse CVST, multicompartment bleeding while being on anticoagulation, low platelets, and anticipation of moderate to high risk of unfavorable outcome; the decision was to perform endovascular mechanical venous thrombectomy (Approximately 48 h after admission). She underwent a successful mechanical venous thrombectomy using the INARI FlowTriever system with large clot burden extracted. She remained clinically stable after the procedure and her left upper extremity weakness improved at day 5. No new symptomatic ICH. The 22 French (7.33 mm) venous access was sutured with figure of 8 technique followed by manual pressure. No post-procedure groin complications noted. She was switched to novel oral anticoagulation prior to discharge. During the 3 months follow-up–MRI brain with and without contrast revealed near complete resolution of the clot burden in superior sagittal sinus and left transverse-sigmoid complex. Her 3 months modified Rankin score was at 0. She was resumed on apixaban for 12 months with a follow-up brain magnetic resonance venogram planned.","This 78-year-old woman was brought to the hospital after experiencing sudden weakness and jerky movements in her arm. Tests revealed a bleeding in her brain and a blood clot in her sinuses, which were treated with a procedure to remove the clot. She is now recovering well and her arm strength is improving, and she is being monitored closely to ensure her condition remains stable."
387,"A 44-year-old man with acute lymphoblastic leukemia (ALL) underwent induction chemotherapy (IC) (day 0). An outline of the episodes is showed in . Bone marrow suppression with fever and septic shock occurred on day 10. Patient suffered serious infection, and empirical treatments were used with imipenem (IPI, 1 g, every 8 h, intravenous injection), vancomycin (VAN, 1 g, every 12 h, intravenous injection) and voriconazole (VRC, 200 mg, every 12 h, intravenous injection) as broad-spectrum antibacterial and antifungal prophylaxis and empirical treatments were used with broad-spectrum antibiotics including antifungal prophylaxis. The patient's blood pressure recovered but recurrent fever occurred after 3 days. At that time, a series of cultures of peripheral blood (PB) were negative from day 10 to day 28. No pathogen could be detected, and the patient experienced neutropenia from day 10 to day 25 (). However, hemiplegia and hemiconvulsions suddenly occurred on patient's right limb, and computed tomography (CT) scans of patient's brain showed a hyperdense lesion with surrounding edema, which was highly suspected as cerebral infarction in the right parietal lobe and small hypodense areas in the left and right parietal lobes. No obvious abnormality was showed by magnetic resonance angiography (MRA) of patient's brain, but CT scans of the lung showed multiple hyperdense lesions on day 18 (). Febrile neutropenia in patients after chemotherapy with cerebral symptoms may be highly indicative of infections in the brain (, ).\nTreatment was continued VRC and IPI, and changed to teicoplanin (TEC, 400 mg, daily, intravenous injection) as antibacterial and antifungal drugs Treatment was changed to voriconazole (VRC) and imipenem as antifungal and antibacterial methods after a positive result for the test of (1,3)- β-D-glucan (100.70 pg/mL, Guangzhou Zhaokang Biotechnology Co., Ltd) on day 22. However, the fever was unresponsive and the lesions in the brain and lung were more serious a week later. The procalcitonin (PCT) level was elevated to 4.40 ng/mL, and c-reactive protein (CRP) reached 218.33 mg/L. Aminoleucine transferase (ALT) and aspartate transaminase (AST) reached 145 U/L and 242 U/L, respectively. Other drugs were applied to protect patient's liver function. Rhizomucor miehei infection was shown by mNGS (Genskey Medical Technology Co., Ltd, Beijing, China. NextSeq 500) of PB with high relative abundance about 99.94% on day 25 (), and liposome-associated amphotericin B (AmBL, 100 mg, daily, intravenous injection) was immediately used as antifungal therapy from day 25 to day 55. The patient's body temperature returned to normal (36–37°C) after 3 days of treatment and his complete blood count (CBC) recovered from neutropenia on day 28. Infection with Rhizomucor miehei was also proven by mNGS both in CSF with relative abundance about 0.35% on day 28 and BAL with relative abundance about 28% on day 35 (), and the routine and biochemical examinations of CSF were negative (). After 2 and 3 weeks of antifungal treatment, CT scans showed that lesions were obviously absorbed both in the brain () and the lung (), and PCT and CRP were also recovered following antifungal therapy (). Finally, the patient's general condition improved, and his right limb function partly recovered on day 55. He requested to go back to the local hospital to continue antifungal therapy due to his family reasons.\nAfter 2 months of follow-up by telephone, we learned that patient changed to receive antifungal treatment with amphotericin B (AmB, 150 mg, daily, intravenous injection) and posaconazole (Pos, 300 mg, daily, orally) for other 2 months, because patient's liver had recovered to normal function. Besides, significant reduction in the size of the lesions on imaging tests of this patient were reported. The next chemotherapy for ALL was also prepared to carried out. The antifungal drug regimen was well-tolerated and achieved a remarkable effect.","This 44-year-old man with leukemia experienced a serious infection that led to fever, low blood counts, and neurological problems. During treatment, he developed a brain infection that caused weakness and seizures, and later a lung infection was identified. Despite aggressive treatment with multiple antibiotics and antifungals, the infections were difficult to treat, but eventually resolved. He is now continuing his leukemia treatment and recovering, with significant improvement in his condition."
388,"In January 2021, a 2-year-old boy of white Caucasian origin presented to his local ophthalmologist for acute unilateral sixth nerve palsy and was subsequently transferred to our pediatric emergency department for further evaluation. The patient, generally being fit and well, had developed a sudden dysfunction in lateral movement of his left eye, resulting in a continuous abduction deficit with consecutive fixated turn of the head to the left side. His medical history was unremarkable for trauma, headache, vomiting or fever. He had not received any vaccinations within the last few weeks. Apart from a mild gait instability, there were no concomitant symptoms or other focal neurological deficits on clinical examination. The patient did not suffer from any chronic diseases and did not take any regular medication; his vaccination status was complete according to national recommendations.\nThree weeks prior to onset of symptoms the patient had experienced a respiratory tract infection resulting in an increased respiratory rate, dry cough, intermittent fever and loss of appetite, lasting for 2 weeks. Symptomatic treatment was initiated by his local pediatrician, who attributed the patient's symptoms to a common cold rather than COVID-19. Thus, no oropharyngeal swab for SARS-CoV-2 or other viruses was obtained. At the same time, the patient's father and his uncle developed cough, dyspnea, sore throat and muscle aches; the uncle tested positive for SARS-CoV-2 on PCR from oropharyngeal swab (). The child's uncle does not live in the same household but had been in close contact to the patient 4 days prior to his positive test for several hours due to an indoor-birthday party. The patient's relatives were unvaccinated as at that time the COVID-19 vaccines were still unavailable for the general public.\nOn admission, laboratory inflammatory markers including C-reactive protein were negative. Full blood count showed mild thrombocytopenia (186 109/) but was unremarkable otherwise. Cranial contrast-enhanced magnetic resonance imaging (MRI) showed an hypoplastic left abducens nerve and atrophy of the corresponding left lateral rectus muscle compared to the contralateral side (). There were no signs suggesting any inflammatory intracranial process or elevated intracranial pressure, no papilledema. A lumbar puncture was performed. The cerebrospinal fluid (CSF) opening pressure was 24 cmH2O corresponding to the upper limit of normal range () thus diagnostic lumbar puncture was followed by therapeutic drainage of 8 ml CSF. Routine CSF laboratory parameters yielded a normal result; no oligoclonal bands were detected on CSF/serum. Multiplex-PCR (Filmarray, BioFire, Biomerieux Lyon, France) from CSF was negative for cytomegalovirus (CMV), enterovirus, herpes simplex viruses 1 and 2, human herpesvirus 6, human parechovirus, varicella zoster virus, Cryptococcus neoformans and gattii, E. coli K1, Haemophilus influenzae, Listeria monocytogenes, Neisseria meningitides as well as Streptococcus agalactiae and pneumoniae. An additional multiplex-PCR performed on an oropharyngeal swab sample yielded a negative result for adenovirus, coronaviruses 229E, HKU1, NL63 and OC43, human metapneumovirus, human rhino-/ enterovirus, influenza virus A and B, Middle East Respiratory Syndrome Coronavirus (MERS-CoV), SARS-CoV-2, parainfluenza virus 1–4, respiratory syncytial virus, Bordetella pertussis, Bordetella parapertusssis, Chlamydophila pneumonia and Mycoplasma pneumoniae. Testing for Borrelia burgdorferi showed no antibodies in neither serum nor CSF. An EEG was unremarkable. Repeated ophthalmologic examinations revealed incomitant squint angles due to left-sided sixth nerve palsy and a significant abduction deficit of the left eye, consistent with the diagnosis of left abducens nerve palsy. An underlying retraction syndrome was considered unlikely due to the sudden onset of symptoms and absent globe retraction. Optic nerve examination was unremarkable.\nReal-time reverse transcriptase PCR (rRT-PCR) test for SARS-CoV-2 (oropharyngeal swab sample) was negative on admission, while serology turned out to be positive for SARS-CoV-2 anti-spike IgG (Euroimmune, Germany). Of particular note in this context, SARS-CoV-2 specific IgG was also detected in CSF. Pathogen-specific antibody index as an indicator for potential intrathecal antibody production was negative, suggesting involvement of central nervous system being secondary to systemic infection rather than direct viral infection (). An rRT-PCR for SARS-CoV-2 from CSF was negative.\nGiven the boy's history of recent respiratory tract infection, COVID-19 very likely in his father and proven in his uncle, and detection of SARS-CoV-2-IgG antibodies in the patient's serum and CSF, post-infectious abducens nerve palsy appeared to be the most likely diagnosis. During inpatient stay, symptoms already showed spontaneous mild improvement without therapeutic measures. Following discharge, the boy was regularly seen for ophthalmologic follow-ups. Three months following onset of abducens nerve palsy, the family noticed a distinct improvement in eye movement and the child eventually made a full recovery 2 weeks later.","A 2-year-old boy suddenly started having trouble moving his left eye, and his parents brought him to the hospital for an evaluation. He had recently had a cold, and his father and uncle had also been sick with COVID-Although initial tests for COVID-19 were negative, the doctors found antibodies to the virus in his blood and cerebrospinal fluid, suggesting he had been infected. After further testing, they determined that the eye problem was likely a temporary issue that developed after his illness, and his eye movement gradually improved over the next few months."
389,"A 42-year-old Chinese man presented to the outpatient clinic of otorhinolaryngology at Xijing Hospital, Fourth Military Medical University, in September 2018, complaining of right ear discharge accompanied by discomfort for 3 years. The patient had a history of seawater contact in August 2015, including underwater diving near the coast of the Yellow Sea near Qingdao City, Shandong Province. After a couple of days, the patient noticed a small volume of clear drainage from both ears, accompanied by mild discomfort and ear pruritus. As the condition progressed, drainage from the left ear gradually vanished within half a month, whereas the drainage from the right side increased and became a thick, purulent exudate containing debris, eventually turning a dark brown or chocolate color. As the ear canal became almost entirely obstructed by the thick excretion, the patient experienced mild hearing loss, coupled with intermittent tinnitus, and experienced a sensation of ear fullness. Without the use of any medications, he removed debris from the right ear using cotton swabs, resulting in a return to normal hearing and the relief of symptoms. Within a few days, new thick drainage would obstruct the ear canal, and the symptoms would reappear. The patient sought health care twice at local hospitals in Xi’an City from Shaanxi Province; however, the underlying illness remained unidentified and did not improve. The discharge from the right ear gradually reduced and turned from brown to white until March 2018, when a low-concentration hydrogen peroxide solution was used to rinse the right ear canal at a local hospital. During the course of the illness, the patient denied any accompanying symptoms, such as fever, chills, headache, vertigo, diarrhea, or flatulence, and he did not have a history of infectious diseases, diabetes, or any immunocompromising condition. Before the diagnosis, the patient underwent audiometric and otoscopic examinations, in addition to radiological examinations using computed tomography imaging to examine the temporal bone. The exudate was collected from his right ear using a sterile swab and was transferred to the clinical microbiology laboratory for examination. All specimen processing and bacteriological analysis procedures were performed with approval from the ethics committee of the Xijing Hospital, Fourth Military Medical University, with assigned number KY20183304-1. The patient provided written informed consent prior to participation in this project.","This man has been dealing with a persistent drainage problem in his right ear for three years, which started after swimming in the sea. Over time, the drainage worsened, becoming thick and dark, and he experienced hearing loss and a feeling of fullness in his ear. He tried cleaning the ear himself, which temporarily relieved the symptoms, but the problem kept returning. After several visits to doctors, tests including imaging and a sample of the ear drainage were taken to determine the cause of the infection, which is still being investigated."
390,"The patient is a 52-year-old white post-menopausal woman and a non-smoker. Eight years prior to the study start in 1997, the diagnosis SLE was made by a senior internist (P.K.) and a senior dermatologist (N.S.) according to the following ACR criteria: kidney involvement (histological evaluation of chronic mesangial proliferative glomerulonephritis, WHO classification IIIa) with microscopic hematuria; arthralgia; urticarial vasculitis; oral ulcers; facial rash. Moreover, she showed decreased complement C4 (hypocomplementemia), leukopenia and enhanced antinuclear antibodies (ANA, 1:2560); analyses of antinuclear anti-double-stranded DNA antibodies (ds DNA) were negative.\nPharmacologic treatment lasted three years and consisted primarily of steroids (4–20mg) in combination with other non-steroidal anti-inflammatory medication (paracetamol). The patient did not tolerate antimalarials; moreover, she refused further immune suppressive therapy (e.g. azathioprine, mycophenolate, cyclophosphamide) although her disease fulfilled WHO classification IIIa for SLE. Nevertheless, her laboratory values improved (no proteinuria, no pathological urine sediment) during pharmacologic treatment. The patient attended psychotherapy for three years following diagnosis.\nDuring regular check-ups between first diagnosis in 1989 and study start in 1997, the following minor clinical disease manifestations related to SLE had been identified: oral ulcers, urticarial vasculitis lesions at various body sites (e.g. facial rash), small joint pain, fatigue, tiredness and fever. These symptoms did not require steroidal or immunosuppressive drug therapy and were treated by the patient symptomatically (e.g. mouth rinsing with hexetidin solution). At study start, the patient presented with elevated ANA (1:160, ds DNA negative, SS-Ro-antibody positive) with the above-mentioned mild clinical symptoms, which did not require steroid treatment.","This woman was diagnosed with lupus (SLE) eight years ago, which causes inflammation in the kidneys and other parts of the body. She received treatment with steroids and other medications for three years, which helped to improve her condition. Although she didn't tolerate other immune-suppressing drugs, she continued to experience some symptoms like mouth sores and fatigue. Currently, she has a slightly elevated antibody level and mild symptoms, but doesn't need steroid treatment at this time."
391,"A 60-year-old woman was admitted with a continuously enlarged neck mass for 1 year and hoarseness for 1 week. In addition, she presented with dyspnea for 5 months. The patient had no family history of parathyroid diseases or hyperparathyroidism-jaw tumor syndrome. Physical examination showed a firm left neck mass of approximately 6.0 cm * 5.0 cm. Laboratory findings revealed elevated serum PTH (188.1 pg/ml, reference range: 15–65 pg/ml) and hypercalcemia (total serum calcium: 3.29 mmol/L, reference range: 2.1–2.6 mmol/L). Indicators related to thyroid function were within normal limits. Laryngoscopy showed left vocal cord paralysis. Ultrasonography showed that the left thyroid lobe was enlarged significantly, a hypoechoic lesion nearly occupied the whole lobe, and comparable signs were presented on the neck CT (). Tc-99m sestamibi scintigraphy demonstrated two-phase nuclide accumulation on the left thyroid (). Chest CT showed multiple micro pulmonary nodules ().\nDuring the surgical exploration, we found that the tumor invaded the anterior cervical muscle group and left recurrent laryngeal nerve. Only the superior parathyroid was found in the left neck. En bloc resection (including part of the invaded recurrent laryngeal nerve and muscle tissue and entire thyroid) and left central lymph node dissection were performed to completely remove the affected tissue. The tumor profile showed that the thyroid was markedly infiltrated, and the normal gland was almost invisible (). Postoperative histopathological findings revealed that SaPC widely invaded the ipsilateral thyroid, and 1/6 of the lymph nodes showed metastasis. Immunohistochemical staining was further performed to confirm the diagnosis (); results were presented below: (1) Carcinomatous components: Some PC cells show negative nuclear staining of parafibromin (); Cytokeratin (AE1/AE3) (+); Chromogranin A (+); E-Cadherin (+); PTH (+); Calcitonin (–); Thyroglobulin (-); Desmin (-); KI-67 index 10%; (2) Spindle cell components: Desmin (+; ); Cytokeratin (AE1/AE3) (-); Chromogranin A (-); E-Cadherin (-); Calcitonin (-); KI-67 index 30%. In addition, the existence of transition zones () and positive N-cad staining in both carcinomatous and sarcomatoid components () was found during pathological examination.\nThe patient recovered soon postoperatively and remained hoarse. She did not experience choking when drinking water, and dyspnea significantly improved. Three months later, the patient complained of progressively aggravating dyspnea and a gradually growing neck mass. Serum calcium and PTH levels were without abnormal elevation during this time (). Clinical examinations suggested regional relapse and multiple pulmonary metastases (). In contrast to the chest CT before, it seemed that pulmonary metastasis had occurred before the first surgery. Enhanced MRI showed extensive local organ and tissue invasion by the recurred tumor (). At last, the patient gave up the medical treatments.","60-year-old woman was admitted due to a growing lump in her neck, hoarseness, and difficulty breathing. Tests showed high levels of calcium and a tumor in her thyroid gland that was invading nearby nerves and muscles. Surgery to remove the tumor was successful, but she remained hoarse. Unfortunately, the tumor returned and spread to her lungs, and she chose not to continue treatment."
392,"The patient was a 75-year-old man with a body mass index of 23.4 kg/m2 and an American Society of Anesthesiologists (ASA) class of three. He had a history of end-stage renal disease on hemodialysis and was initially transferred to our hospital from an outside facility for subacute functional decline and encephalopathy. His admission work-up was notable for a leukocytosis of 12,800 cells/μL, Enterococcus faecalis bacteremia, and bilateral pleural effusions. His 12-lead electrocardiogram showed sinus rhythm with first-degree atrioventricular block and a right bundle branch block. The patient was started on broad spectrum antibiotics for bacteremia and later narrowed to ampicillin. Repeat blood cultures were negative. On hospital day one, the patient underwent a transthoracic echocardiogram which demonstrated a left ventricular ejection fraction of 45-50% with global systolic dysfunction, moderate RV dysfunction, mild pulmonary hypertension with a pulmonary artery systolic pressure (PASP) of 41 mmHg, and no visible valvular vegetations.\nThe patient underwent thoracentesis of the right sided pleural effusion, and bacterial cultures of the pleural fluid grew Enterococcus Faecalis. Given this multifocal enterococcus infection, TEE was pursued.\nOn hospital day nine, the patient underwent TEE under monitored anesthesia care. The anesthesia service was consulted due to pulmonary hypertension as per the institutional guideline. The patient was connected to standard monitoring as recommended by the ASA. Supplemental oxygen was provided by a face mask, with end-tidal carbon dioxide monitoring. The patient received a total of 70 mg of propofol administered over the first ten minutes of the procedure in boluses of 10 mg-20 mg. Hemodynamically, the patient's blood pressure at the beginning of the procedure was 140/50 mmHg with a pulse in the low 60 s. His blood pressure dropped precipitously after receiving propofol down to 60/40 mmHg fifteen minutes into the procedure. At that point, the patient became bradycardic and went into a PEA cardiac arrest. The TEE probe was withdrawn, and chest compressions were initiated under the advanced cardiac life support algorithm. The patient was intubated, and return of spontaneous circulation (ROSC) was achieved after four rounds of chest compressions and epinephrine administration. The patient's cardiac rhythm then deteriorated into ventricular fibrillation requiring defibrillation and further chest compressions. ROSC was achieved once again, and the patient was transferred to the intensive care unit. Upon review of the images captured by TEE at the onset of PEA arrest, it was noted that the RV was severely dilated with minimal contractile function, and a small pericardial effusion was found (Video ). Due to the patient's critical condition, his family decided to transition him to comfort measures only.","This 75-year-old man was admitted to the hospital after experiencing confusion and shortness of breath, and was found to have a bacterial infection in his blood and fluid around his lungs. Tests revealed he had a weakened heart and high blood pressure, and his condition worsened rapidly after receiving anesthesia. Despite efforts to stabilize him, he suffered cardiac arrest and passed away. His family chose to focus on comfort care during his final days."
393,"One of the authors (TS Tsai, a 48-year-old man) was bitten on the right palm near the base of the index finger by D. siamensis () in the Reptile and Amphibian Facility at the National Pingtung University of Science and Technology in Pingtung, Taiwan. The accident might have occurred when the patient's fingers stuck to the skin of the snake when he grasped the neck of the snake to inspect its mouth and nostrils for symptoms and signs of infection. After inspection, the patient attempted to release the snake back into the snake box, and it then turned its head around and bit the patient's hand while he was releasing it. Two fang bite marks, scratches, and bruises were visible on the spot.\nThe patient started to feel numbness and tingling of the ankles and experienced slight difficulty in walking approximately 30 min after the snakebite. The patient visited a local hospital 30 min later and was administered 4 vials of monovalent antivenom for D. siamensis at the emergency department within 1 h of being bitten. However, the swelling and redness had progressed to the wrist () approximately 9 h after the snakebite. Therefore, 4 more vials of antivenom were administered, for a total of 8 vials within 10 h. Blood tests showed acute kidney injury, disseminated intravascular coagulation, and coagulopathy (). No leukocytosis or anemia was observed, and antibiotics, such as ceftazidime, were prescribed.\nThe patient was admitted after which the right hand showed the most obvious swelling and pain for the first 2-3 days. The swelling and pain even extended to the right elbow. The patients' fingers were almost unable to bend. Ice pillow packing was used to relieve the pain, local heat, and swelling. Fortunately, there were no blisters, hemorrhagic bullae, or finger ischemia. The patient also received two courses of hyperbaric oxygen treatment on days 3 and 4, and the swelling of the hand quickly subsided after that. Then, the patient's fingers were able to bend slightly on the third night and were able to bend more than 90° on day 4. During hospitalization, the patient's daily stool was soft or fluid and dark blue-green but not black, whereas the urine color was normal. The patient was discharged from the hospital on day 5, and on day 8, a large area of elevated red skin rash and itching of the body occurred at home. After administration of antihistamines, the symptoms disappeared. The patient still sometimes experienced mild numbness and painful sensations at the bite site for up to one month after the snake envenomation.","A man was bitten by a snake in Taiwan and initially experienced numbness and difficulty walking. He received antivenom and underwent treatment for kidney problems and blood clotting issues. Despite the initial severity, his hand swelling and pain gradually improved with ice packs, heat, and hyperbaric oxygen therapy. He developed a rash at home, which resolved with antihistamines, and experienced lingering numbness at the bite site for several weeks."
394,"A 41-year-old gravida 6 para 3 female (uncompleted pregnancies due to miscarriages) presented to the emergency department with a chief complaint of chest pain. Pain started two hours prior to presentation, was dull, left sided, substernal, radiated to her left arm, and with a severity of 7/10. One month prior to presentation the patient was complaining of an abnormal uterine bleeding and was started on oral norgestimate/ethinyl estradiol pills.\nThe patient has no significant past medical history. Her social history is significant for smoking 1/2 a pack per day for the past 10 years. Her family history is not significant for premature coronary artery disease or thrombosis. Her past surgical history is significant for dilatation and curettage two weeks prior to presentation. Home medications include daily oral norgestimate/ethinyl estradiol pills. Her only cardiac risk factors were smoking and the use of oral contraceptives.\nUpon arrival to the emergency department, the patient was vitally stable. Her physical exam was noncontributory. Cardiac and lung exams were unremarkable. A twelve-lead ECG showed 1 mm ST elevations in inferior leads with no reciprocal changes and a sinus rhythm at a rate of 60 beats/minute (). A STEMI code was called, and the patient was taken emergently to the Cath Lab. She was started on acute coronary syndrome (ACS) treatment including oral aspirin 324 milligrams, oral clopidogrel 600 milligrams, and intravenous heparin 5000 U. Significant laboratory data showed a troponin of 0.615 ng/mL (0-0.04 ng/mL), hemoglobin of 8.7 g/dL (12.0-15.5 g/dL), and white blood cell count of 13.7 × 109/L (4.5 − 11.0 × 109/L).\nCoronary angiogram revealed a saddle clot (thrombus) involving the distal left main artery, left circumflex, and proximal LAD (). TIMI flow grade was 3 throughout, and no intervention took place during the coronary angiogram. There was also an occlusion in the apical portion of the LAD. The right coronary artery did not have any abnormalities. An echocardiogram revealed an estimated ejection fraction of 55-60% with apical septal hypokinesis and a normal diastolic function. There was no evidence of an intra-atrial shunt or left atrial appendage. Patient was admitted to the cardiac care unit (CCU) and was started on intravenous tirofiban for 10 hours, oral aspirin 81 milligrams daily, oral clopidogrel 75 milligrams daily, and intravenous heparin drip as per ACS protocol. Troponin peaked at 11 ng/mL. The patient was given 2 units of PRBC due to persistent vaginal bleeding. Doppler of the lower extremities did not reveal any evidence of a DVT. Telemetry over 48 hours of the hospital stay did not show any evidence of cardiac arrhythmia.\nThrombophilia testing lab results showed antithrombin activity of 88% (80-120%), antithrombin III AG 79%, cardiolipin AB IGA < 9.4 APL (<20.0 APL), beta − 2 glycoprotein IGG < 9.4 U/mL (<20.0 U/mL), beta − 2 glycoprotein IGM < 9.4 U/mL (<20.0 U/mL), cardiolipin AB IGM MCLIP < 9.4 MPL (<15 MPL), cardiolipin AB IGG GCLIP < 9.4 GPL (<15 GPL), prothrombin G20210A gene negative, beta − 2 glycoprotein AB IGA < 9.4 U/mL (<15.0 U/mL), PS/PT IGG < 9.4 U (<30.0 U), PS/PT IGM 20.0 U (<30.0 U), and platelet count of 376 K/μL (149-400 K/μL).\nOBGYN were consulted, and placement of IUD was recommended to help prevent bleeding. After 48 hours, heparin was discontinued. EKG prior to discharge showed resolution of ST elevations (). A repeat angiography was not done as symptoms had resolved and EKG showed resolution of STEMI. OCPs were held, and patient was discharged on oral anticoagulation.","A 41-year-old woman came to the hospital with sudden, severe chest pain. Tests revealed a blood clot in a major artery of her heart, causing a heart attack, and she was treated immediately. She had a history of irregular bleeding and smoking, which contributed to her risk. Blood tests showed elevated levels of a protein called troponin, indicating heart damage. Doctors performed a procedure to clear the blockage and found no other problems with her heart. She was started on medications to prevent further clots and will continue to take them long-term. Finally, the OBGYN recommended an IUD to help prevent future bleeding episodes."
395,"A 29-year-old man presented from a psychiatric hospital with 2 days of sudden onset, progressive chest pain, and troponin elevation after recently initiating clozapine therapy.\nThe patient had a history of alcohol abuse and treatment-resistant schizoaffective disorder with multiple suicide attempts. After intentional ingestion of acetaminophen and lithium, he was involuntarily hospitalized for intensive treatment, including initiation of clozapine therapy.\nThe differential diagnosis included acute coronary syndrome, myopericarditis from viral or drug-induced etiology, pulmonary embolism, and pneumonia. The patient first noted substernal chest pain worse with deep inspiration 8 days after clozapine initiation. Symptoms worsened over the following two days. Electrocardiogram (EKG) demonstrated ST elevations most prominent in V2-V3 (). Serum labs were notable for leukocytosis and elevated troponin, brain natriuretic peptide, and inflammatory markers ().\nChest pain persisted, and he was transferred to the cardiology service. Cardiovascular exam was notable for tachycardia without murmurs or rubs. He had no peripheral edema or elevated jugular venous pressure. Lungs were clear. Cardiac magnetic resonance (CMR) imaging showed global hypokinesis with a left ventricular (LV) ejection fraction (EF) of 45% (Figure ) and subtle late gadolinium enhancement of the epicardial lateral wall and lateral pericardium with abnormal parametric mapping () consistent with myopericarditis. Respiratory viral panel was negative. Clozapine was discontinued. Metoprolol was initiated for impaired LV function with colchicine and ibuprofen for pericarditis with prompt symptomatic relief. Repeat EKG showed resolution of ST-elevation and no arrhythmic events on telemetry. He was discharged back to the psychiatric hospital with planned cardiology follow-up for echocardiogram and repeat laboratory testing.","This 29-year-old man was admitted to the hospital after experiencing sudden chest pain and elevated heart markers while starting a new medication called clozapine. He has a history of mental health challenges and had previously taken acetaminophen and lithium, which led to a hospitalization. Tests revealed he had developed myopericarditis, an inflammation of the lining around the heart, likely caused by the clozapine medication. He is now receiving treatment for this condition and will continue to be monitored by both cardiology and psychiatry teams."
396,"A 60-year-old man presented with sudden left leg pain in L4 nerve root area. The patient demonstrated neurogenic paralysis on left quadriceps femoris muscle with manual muscle testing 3, hyporeflexia on left patella tendon, and positive left straight leg raising test. MR images revealed left L4-5 foraminal herniated disc without fragment in the spinal canal (). Left L4 selective nerve root block remarkably affect. According to the above results, the patients was diagnosed as left L4 nerve root entrapment by left L4-5 foraminal disc herniation.\nThe patient underwent discography for the presurgical imaging diagnosis. The double-needle was inserted to the L4-5 disc, and tip of the needle was located at middle of the nucleus pulposus. Finally, 3.0 cc of Iohexol was injected cautiously without reproduction of left L4 radicular pain. After discography, the patient complained deterioration of left leg numbness without neurological deficit. On disco-CT and postdiscogram MR images, herniated fragment was migrated from neural foramen to cranial central canal which was not detected in previous images (Figures and ).\nHerniotomy was performed by osteoplastic approach [, ]. Observing the neural foramen, the herniated disc fragment was migrated from neural foramen to cranial central canal. 2.5 g-weighted disc fragment with annulus fibrosus was extirpated with one piece (). After surgery, neurological status was fully recovered immediately.","60-year-old man came to the hospital with sudden pain in his left leg, which was caused by a problem with a disc in his spine. Tests showed he had weakness in his leg and numbness, and a doctor performed a nerve block to confirm the diagnosis. Further imaging revealed that the disc fragment had moved into the spinal canal, and a herniotomy was performed to remove the fragment. After the surgery, his leg pain and numbness disappeared, and he fully recovered."
397,"A 74-year-old man presented with right leg pain in L4 nerve root area. He was treated in previous clinic with diagnose of right L4-5 foraminal disc herniation (). After disc block with 1.9 mg of Dexamethasone sodium phosphate and 1.5 ml of mepivacaine hydrochloride, the patients complained of deterioration of right leg pain.\nThe patient was introduced to our facility, and reexamined MR images showed herniated fragment migrated from neural foramen to cranial central canal which was not detected in previous images (). No neurological deficit was observed. Transforaminal lumbar interbody fusion with right L4-5 facetectomy was performed to extirpate the herniated fragment.","This 74-year-old man came to us because he was still experiencing pain in his right leg, even after a previous treatment for a disc problem. We reviewed his MRI scans and found that a piece of the disc had moved into the spinal canal, which wasn't seen before. To remove this piece and relieve the pain, we performed a surgery called a transforaminal lumbar interbody fusion, which involved removing the fragment and fusing the bones in his lower back. He is currently recovering from the surgery and will continue to be monitored closely."
398,"A 65-year-old man with continuous irritable cough over 15 days was admitted to our department for a mass lesion in the right hilum. He presented with no face swelling and superficial varicose veins. He was a former smoker, and medical history included hypertension and diabetes mellitus. At bronchoscopy, the tumor was found occluding the right upper lobar bronchus and infiltrating the bronchus intermedius. It was diagnosed as squamous cell carcinoma by transbronchial tumor biopsy. Chest contrast computed tomography (CT) revealed that the mass of 80 mm in the maximum diameter located in the right upper lobe. The tumor directly invaded the arch of the right PA and a wide range of SVC. It was discovered that the left brachiocephalic vein (BCV) along the left margin of mediastinum walking down the line directly into the coronary sinus (). A venous flow was detected in the anterolateral descending aorta with dilatation of the coronary sinus (diameter of 21 mm) without other heart abnormalities by transthoracic echocardiography. Based on these findings, the diagnosis of PLSVC was made. There were no detectable metastases in other organs through brain magnetic resonance imaging (MRI), upper abdomen CT scan and bone nuclear scan.\nThe right posterolateral thoracotomy was performed on October 31, 2006. As expected, the tumor invaded the wall of SVC and no left brachial cephalic vein bifurcation was observed. The SVC was separated, clamped and resected following intravenous injection of 5, 000 IU of sodium heparin. The ringed PTFE graft of 12 mm in diameter was interpositioned between the right brachial cephalic vein and the origin of SVC by running sutures with 4-0 polypropylene. The tumor resection was completed by double sleeve lobectomy of right upper and middle lobes. The anastomosis between the right main bronchus and the cut end of the lower lobe bronchus was performed by interrupted suture using 3-0 Vicryl (Ethicon, USA). Right PA trunk and right lower PA reconstruction was completed by the running suture with 4-0 polypropylene (). And systematic mediastinal lymph node dissection was performed. All excised margins were tested microscopically negative for malignancy. As anticoagulation treatment, we began with low molecular Heparin on the operative day, then switched and continued to warfarin when thoracic drains removed. The postoperative course was uneventful, and the patient was discharged from the hospital on the 12th postoperative day. Final pathological diagnosis was poorly differentiated squamous cell carcinoma invaded the right SVC, right PA and mediastinal nodes were positive for malignancy.\nAfter 4 cycles of chemotherapy (Gemzar and cisplatin) and 1 cycle of radiotherapy, Chest MRI showed patency of the graft and PLSVC (). He died 21 months after operation because of remote metastasis.","A 65-year-old man with a persistent cough was admitted to the hospital after doctors found a tumor in his lung that was blocking a major airway. The tumor was identified as squamous cell carcinoma and was causing the tumor to invade the major blood vessels in his chest. The tumor was surgically removed, and the blood vessels were repaired, but the cancer had spread to nearby lymph nodes. Despite chemotherapy and radiation, the patient eventually passed away 21 months after the surgery due to the cancer spreading to other parts of his body."
399,"A 15-year-old boy was admitted with a 2-month history of fever, decreased appetite and nonproductive cough. Cough and fever progressed despite of antibiotics prescription for presumed community-acquired pneumonia a few weeks prior to admission.\nAt the time of admission, he had fever, tachycardia, respiratory distress and splenomegaly. In laboratory findings, he had pancytopenia; urine analysis and ESR was normal, blood and urine culture was negative. Gram stain of his sputum showed gram positive cocci. Acid fast bacillus was not found in sputum smear. HBsAg and Anti-HCV and Anti-HIV were negative. ANA, Anti ds-DNA, p-ANCA and c-ANCA were requested but all of them were negative. Peripheral blood smear and bone marrow aspiration were normal. Echocardiography also was normal. His chest X-ray and CT-scan of thorax showed bilateral and peripheral nodular and alveolar pattern, predominantly in lower lobes of the lung. Mediastinal lymphadenopathy and pleural effusion were not seen (). Abdominal sonography and CT showed hepatosplenomegaly. Splenomegaly was more prominent than hepatomegaly. Para-aortic lymphadenopathy was not seen. Bronchoscopy and open lung biopsy were not performed because the patient's guardian did not permit. Two days later, we saw a few small brown nodular lesions on the left leg (). Skin excisional biopsy was taken. One day later, he developed right hemiplegia, seizure and aphasia. Brain CT scan showed a broad hypo dense area on the left temporal and basal ganglia that had little enhancement after injection of Ⅳ contrast (). At this time, the result of skin biopsy was prepared. There was severe infiltration of lymphocytes and histiocytes and atypical lymphocytes on the subcutaneous and deep parts of the derma. The main aggregation of the cells was around the vessels and nerves and appendices of the skin. The endothelial layer of the vessels had inflammation, but there was no morphologic pattern of vasculitis. Lymphoid cells had positive reaction with CD45RO: so lymphomatoid granulomatosis (LG) was diagnosed (). He was treated with steroid pulse therapy and oral cyclophosphamide but after 12 days he expired without any clinical improvement and any response to the treatment.","This 15-year-old boy was admitted to the hospital after a couple of weeks of fever, cough, and loss of appetite. Initial tests showed he had a lung infection, but the cause wasn't clear. As his condition worsened, he developed weakness on one side of his body, seizures, and difficulty speaking. Doctors discovered he had a rare condition called lymphomatoid granulomatosis, which caused inflammation in his skin and organs. Sadly, despite treatment, he passed away after 12 days."
400,"A 51-year-old Thai woman was hospitalized in July 2012 with edema at the left lower extremities and painful left inguinal mass for 6 months.\nPhysical examination revealed matted bilateral inguinal lymph nodes up to 9 cm in size with hard consistency, erythema, and tenderness without fluctuation or ulcer. Marked swelling at both lower extremities was observed. There was no other superficial lymphadenopathy. Otherwise, physical examinations were normal.\nIncisional biopsy of the left inguinal lymph node revealed metastatic round cell tumor which is immunohistochemistry positive for vimentin, S100, and HMB-45. The immunophenotype is consistent with malignant melanoma ().\nTherefore, primary tumors in the lower extremities, abdominal cavity, and anogenital organ were suspected. By complete skin examination, no cutaneous lesion was identified. Ophthalmoscopy, gastroscopy, colonoscopy, and cystoscopy were completely normal. Genital and pelvic examinations did not show any evidence of lesion. She denied previous abnormal or removal of cutaneous lesion. Computer tomography of the whole abdomen showed multiple enlarged lymph nodes throughout the abdominal and pelvic cavity up to 9.5 cm, along with compression of both iliac veins without an organ-specific lesion (). CT chest was unremarkable. The patient was diagnosed with metastatic melanoma of unknown primary. The molecular testing had not been done due to the patient's reimbursement issue, and the specimen was poor in quality for further testing. During the investigation, she developed severe pain requiring high-dose opioid, so she has undergone 20 Gy of palliative radiotherapy for bilateral inguinal lymph nodes. Despite radiotherapy, the remaining tumors were up to 7.4 cm based on the CT scan. For the subsequent systemic therapy, according to a national reimbursement policy, she could not access an immune checkpoint inhibitor or targeted drug. Chemotherapy was prescribed with carboplatin (AUC5) and paclitaxel 175 mg/m2 for 6 cycles. After completion of the planned chemotherapy, the symptom was slightly improved. The CT scan at the first 3 months showed that the response was stable disease, but the following CT scan demonstrated a gradual decrease in size over time from August 2012 to November 2017 (). During the follow-up period, the patient developed multiple depigmented patches around the lips, trunk, and periorbital and inguinal area, which are typical of vitiligo.","A 51-year-old woman was admitted to the hospital in 2012 because of swelling in her legs and a painful lump in her groin, which turned out to be a cancerous tumor. A biopsy confirmed that it was metastatic melanoma, meaning it had spread from an unknown primary location. After a thorough examination, doctors found enlarged lymph nodes throughout her abdomen and pelvis, but no other signs of the cancer. Despite treatment with radiation and chemotherapy, the cancer continued to grow, and she eventually developed skin changes called vitiligo."
401,"A 50-year-old male patient presented with a one-day history of right leg weakness, numbness, and urinary retention. There was mild back pain and right leg weakness for two weeks which worsened significantly during the last 24 hours. On neurological examination the patient‘s right leg was weaker than the left (2/5 and 4/5 on Lovett test, respectively), the patellar reflex was exaggerated, and Babinski sign was positive bilaterally. Also, on the right side, there was sensory loss in the leg and below the Th8 dermatome. On the left, there was sensory loss below Th10 dermatome and distal loss of temperature sensation.\nPast medical history revealed a cervical trauma which occurred 30 years ago when a glass chip lodged into the left side of the patient's neck. The patient removed the visible glass shard from his neck and did not seek medical attention; therefore no clinical and radiological investigations were carried out. No neurological symptoms followed this incident.\nComputed tomography (CT) of the lumbosacral and thoracic regions showed a mild convexity of the L5-S1 intervertebral disc with no other clinically significant findings (“Siemens SOMATOM Emotion 6”) (images not shown). To further explore the possible causes of the patient's symptoms, magnetic resonance imaging (MRI) of the C1–L2 segments was performed (“Siemens MAGNETOM Avanto 1.5 T”). At the level of C4–Th3 the spinal cord was thickened and hyperintense on T2W images, features consistent with myelitis; however there was no appreciable contrast uptake (Figures and ). At the level of C6–C7 an oblong (1.6 x 0.4 cm), T1 and T2 hypointense lesion was found (). Because a foreign body was suspected, CT scan of the C1–Th3 levels was performed and demonstrated a hyperdense lesion occupying the spinal canal and the left intervertebral foramen ().\nCombining the CT and MRI results with the past medical history of an old injury with a glass fragment, it was determined that the lesion represented a glass foreign body in the spinal canal. The patient also had an X-ray of the cervical spine but the foreign body could not be visualized, most likely due to being located at the level of C6-C7, where it was obscured by the surrounding structures ().\nTaking into account the clinical picture, an extensive period of time between the trauma and current presentation, and MR imaging findings, an acute demyelinating episode rather than traumatic spinal cord injury was suspected. Further diagnostic work-up would typically have included a lumbar puncture to identify oligoclonal bands, cells, and protein, but it was contraindicated due to the risk of disturbing the foreign body and causing it to migrate upon a sudden decrease in pressure during puncture. Serum Aquaporin-4-specific antibodies could not be performed at the time and were planned for a later time.\nThe patient fulfilled the inclusion criteria for acute transverse myelitis: bilateral and not necessarily symmetrical sensory, motor, and autonomic spinal cord dysfunction, a clear sensory level, peak of symptoms within 4 hours and 21 days after onset of symptoms, and exclusion of other causes (neoplastic, vascular, and compressive) []. Compressive cause was excluded because the spinal cord pathology seen on MRI extends far from the location of the glass shard, which would be unlikely given the size of the foreign object and its possible effect upon the spinal cord if it migrated within the spinal canal. Thus, because the glass fragment lay dormant for the last 30 years, it was deemed not to be the direct cause of the patients' symptoms.\nTreatment with methylprednisolone 500 mg intravenously daily for 6 days was initiated.\nAfter consultation with the neurosurgeons it was decided not to remove the foreign body from the spinal canal, because the risks of surgery would outweigh the benefits. At the time of consultation, the patient was already showing improvement on medical management. Given that the situation was not hyperacute, the symptoms were better explained by the inflammatory and demyelinating reaction within the spinal cord rather than direct contact with the foreign body. Also, it could not be guaranteed that removing the glass shard would result in symptomatic improvement. Upon removal of the foreign body some diffuse bleeding would be expected, which combined with the already inflamed spinal cord parenchyma would likely further compromise the spinal cord, potentially causing vascular complications and myelomalacia, all of which would further decrease the chance of clinical improvement. Risk of general surgical complications (postoperative infection, bleeding, and thromboembolism) further argued against surgical treatment. An absolute indication for surgical treatment would be an infectious complication of the foreign body, which was not present. The patient agreed with the treating physicians that surgery would not be the best option and did not want the operation. If current medical treatment would have proven unsuccessful, and the patient's clinical condition worsened, surgery would have been indicated.\nDuring the course of treatment the patient's condition improved. Sensory loss diminished, and the right leg strength improved to 4/5 on Lovett test, but urinary retention remained. Intermittent catheterization was prescribed.\nThe patient returned for a follow-up visit 10 months later with a stable and improved neurological state. Lower limb strength was 3/5 proximally and 4/5 distally, with positive bilateral Babinski sign. Minimal intermittent urinary retention remained but did not significantly impair the patients' quality of life. The patient resumed his activities of daily living and continues to work as a security guard. Follow-up MRI of the cervical spine shows the same oblong hypointense object and normal spinal cord after the resolution of myelitis (Figures and ).\nFurther follow-up is scheduled every 6 to 12 months, with an outpatient brain MRI to identify any other demyelinating lesions that may be present in case this episode was part of neuromyelitis optica (NMO), acute demyelinating encephalomyelitis (ADEM), or multiple sclerosis (MS).","This 50-year-old man experienced a sudden onset of weakness, numbness, and difficulty urinating in his right leg. Doctors found that a glass shard he had removed from his neck 30 years ago was causing inflammation and damage to his spinal cord. Although he had a history of a spinal injury, the current symptoms were likely due to an inflammatory reaction in his spinal cord rather than direct contact with the glass. After receiving treatment with medication, his symptoms improved, and he was able to return to his normal activities."
402,"A 51-year-old woman presented with fatigue, nausea, and vomiting for three days. She was diagnosed with metastatic melanoma to pelvic nodes in July 2014 and was treated with ipilimumab (anti-CTLA-4) in combination with nivolumab (PD-1 inhibitor) between September 2014 and April 2015, receiving a total of 4 cycles of combination therapy and 9 cycles of nivolumab monotherapy with an initial mixed response followed by slow progression of disease. In April 2015 she was enrolled on a study of radiation (to pelvic mass) in combination with pembrolizumab (PD-1 inhibitor), receiving a total of 9 cycles of pembrolizumab with no toxicities. In December 2016 she was found to have new brain metastases and in January 2017 pembrolizumab was added. The day following her second dose of pembrolizumab, she developed fatigue and nausea and began having intermittent vomiting and diarrhea. Abdominal CT scan demonstrated diffuse colitis. Infectious studies including C. difficile antigen, stool culture, viral PCR, and ova and parasites exam were all negative. She was started on methylprednisolone 2m/kg/day. Over four days of hospitalization, her abdominal pain worsened and she developed melena, which progressed to bright red blood per rectum. She was given infliximab at 10mg/kg. Repeat imaging performed 48 hours later due to severe abdominal distension showed large amounts of free air with gaseous distention of large and small bowel loops, consistent with perforation in the context of colitis with ileus. She was taken to the operating room for emergent bowel resection and a perforation site was identified at the transverse colon. The resected transverse colon serosa was congested and dusky with site of perforation identified (). The colonic mucosa revealed diffusely edematous folds as well as confluent areas of yellowish exudate and multifocal ulcers (). Histologic sections confirmed the presence of transmural necroinflammation and multifocal ulceration (Figures and ). The findings were of a fulminant colitis with multifocal ulceration and perforation. No evidence of metastatic melanoma to the bowel was identified.","This 51-year-old woman has a history of melanoma that has spread to her lymph nodes and brain. She had been receiving immunotherapy medications to treat her cancer, but recently developed severe nausea, vomiting, and diarrhea. After tests, doctors found that she had a serious infection in her colon that led to a perforation (a hole) in her bowel, requiring emergency surgery. The surgery involved removing part of her colon, and the pathology confirmed a severe inflammation of the colon."
403,"A 65-year-old right hand dominant, African American male presented to the ED via emergency medical service. He had just finished smoking crack cocaine when he developed left arm pain that he described as “cramping”. He reported that the pain was so intense that he became weak causing him to fall onto the ground. The pain made him feel like “jumping out of the window.” He denied any head injury and he had no loss of consciousness (LOC). The patient had no chest, shortness of breath, or dyspnea on exertion. He denied any neck, back, or abdominal pain.\nThe patient's past medical history included diabetes, hypertension, hepatitis C, sick sinus syndrome, paroxysmal atrial fibrillation, hyperlipidemia, deep vein thrombosis, chronic kidney disease, hilar mediastinal adenopathy, diastolic heart failure, valvular heart disease, and cardiac arrhythmia of nonsustained ventricular tachycardia with a permanent pacemaker. The patient admitted to intermittent cocaine abuse. His medications include atorvastatin, furosemide, isosorbide mononitrate, acetaminophen with codeine, apixaban, hydralazine, metformin, albuterol sulfate, amlodipine, and tamsulosin.\nVital signs were essentially within normal limits with the exception of a blood pressure of 142/83 mmHg.\nThe patient had a strong left radial pulse and brisk capillary refill of the left hand with no tenderness or deformity. The patient was noted to have left arm weakness and what looked like choreiform or clumsy left arm movements. His left leg was also noted to be weak. There was no numbness. Interestingly, light touch to any part of the left arm produced significant discomfort to the point where he did not want anything touching the left arm. He was noted to have decreased rapid alternating movements on the left upper extremity as well as mild difficulty with fine motor control. His left arm and left leg motor strength was 4/5. His cranial nerves II to XII were grossly intact. There were no visual fields cuts noted. Extraocular motility was intact. The grimace was symmetric. There was no evidence of double simultaneous extinction.\nThere were no pulsatile abdominal masses on exam and the bilateral radial pulses were equal. The patient was unable to tell the exact time of onset of his symptoms. The patient's left arm pain improved with morphine 4 mg intravenously.\nThe electrocardiogram (ECG) showed sinus tachycardia with first degree atria-ventricular block, as well as ST and T wave abnormality suggestive of lateral ischemia []. This is however unchanged compared to his ECG from two years previously []. His cardiac enzyme was negative.\nA computed tomography (CT) scan of the head without contrast showed an acute 2.2 cm intraparenchymal hemorrhage with vasogenic edema in the posterior right parietal lobe [see ]. X-rays of the upper extremity were unremarkable. The chest X-ray showed normal cardiac silhouette and pulmonary vasculature.\nLaboratory data showed a creatinine of 1.34 mg/dL. The urine drug screen showed cocaine.\nThe patient was placed on a continuous nicardipine infusion to maintain a systolic blood pressure of 140 mm Hg as per neurosurgical consultation. He was transferred to a neurointensive care unit. His left arm pain resolved after 24 hours. The carotid ultrasound showed no hemodynamically significant carotid stenosis and antegrade flow was present in the bilateral vertebral arteries. A CT angiography of the head and neck did not show any aneurysms. His serial cardiac enzymes remained negative throughout his hospitalization. A cardiac catheterization was not performed as the patient had it done one year previously showing angiographically normal coronaries. A cardiology consult was obtained and the patient was found to have no evidence of acute coronary syndrome (ACS) or ischemia. He was subsequently discharged to a rehabilitation facility.","This 65-year-old man went to the hospital after experiencing sudden, severe pain in his left arm while using crack cocaine. He felt like he was going to jump out of the window from the pain and became weak, causing him to fall. He has a history of several health problems, including diabetes, heart issues, and cocaine abuse. After tests, doctors found a bleeding in his brain and treated him with medication and monitoring, and he was eventually discharged to a rehabilitation facility."
404,"A 42-year-old man presented with a nine-year history of intermittent productive cough. He also endorsed recurrent episodes of fevers, chills, and night sweats but denied shortness of breath, chest pain, hemoptysis, or weight loss. He presented to the Emergency Department for similar symptoms two months prior and was given a seven-day course of levofloxacin, with temporary improvement in symptoms. Past medical history was significant only for diabetes mellitus. He had no prior surgery and did not take any medications. He worked as a gardener and denied any history of smoking, alcohol, or drug use. On examination, vital signs were stable with normal oxygen saturation on room air. Chest auscultation revealed mildly decreased breath sounds and rhonchi in the left lower lung field. The remainder of the examination was normal.\nA chemistry panel and CBC were unremarkable, including a WBC of 7.0. Sputum AFB smears and bacterial cultures were all negative. Chest radiograph showed left basilar lung consolidation with tree-in-bud opacities and stenosis of the LMSB (). Chest CT scan showed extensive tree-in-bud opacities with confluent consolidation in the left lung base, enlarged hilar and mediastinal lymph nodes, and stenosis of the LMSB ().\nFlexible bronchoscopy revealed near-occlusion and distortion of the mid-LMSB (). Mechanical debulking with endoscopic biopsy forceps allowed for passage of the bronchoscope to the distal LMSB where a metallic foreign body was appreciated (). Histologic analysis of the biopsied endobronchial tissue revealed inflammation and squamous metaplasia, but no evidence of malignancy.\nSubsequently, the patient underwent rigid bronchoscopy and a 2.7 centimeter metal foreign body was retrieved using rigid bronchoscopy forceps (). Granulation tissue occluding the LMSB was treated with argon plasma coagulation. The patient reported immediate improvement in his symptoms. Upon further questioning, he recalled a previous aspiration of a small piece of an aluminum beverage container that he used in lieu of dental floss 12 years before. This object was retained over that time period until we removed it.\nFollow-up surveillance bronchoscopy demonstrated persistent endobronchial stricture in the distal LMSB. Rigid bronchoscopy with radial incisions by electrocautery knife and balloon dilation was used to restore luminal patency. Repeat CT scan one month after endobronchial therapy showed resolution of the left lung tree-in-bud opacities and consolidation and restored patency of the LMSB. The patient's chronic cough resolved and he remains asymptomatic.","A 42-year-old man has been dealing with a persistent cough for nine years, along with occasional fevers and chills. Tests revealed a foreign object – a small piece of an aluminum can – lodged in his lung, which had been causing inflammation and narrowing of the airway. Doctors successfully removed the object and treated the inflammation, leading to a significant improvement in his symptoms. Further treatment, including airway dilation, was needed to fully restore the airway, and he is now doing well and has no cough."
405,"A 66-year-old Caucasian gentleman presented to his GP with a singular, raised, crusted, scaly lesion of 21 × 20mm size with marginal erythema on the dorsum of his left hand (). He reported that the lesion was present for several years but had noted recent growth.\nMedical history included AK, basal cell carcinoma (BCC), and seborrheic keratoses in various distributions over recent years, putting him at a higher risk of keratinocyte carcinoma []. The lesion was diagnosed in the primary care setting as an AK, though possibility of BCC and SCC was considered.\nThe previous BCC had been managed successfully with six weeks of topical imiquimod treatment. Procedural removal of the AK was offered to the patient, but he expressed interest in trying a different approach. The patient was contemporaneously enrolled in a clinical trial examining the use of Kanuka honey on rosacea [] and decided to try using the Kanuka honey topically on his AK.\nHonevo® medical grade Kanuka honey (90% Kanuka honey, 10% glycerin) was topically applied once daily using a small amount on the fingertip rubbed into the lesion and surrounding 5 mm of normal skin for 10–30 seconds. It was left on for 30–60 minutes and then washed off with water. This was done consecutively for five days, after which the patient took a treatment break of two days due to lesion tenderness. During the break, the lesion was gently picked at, thereby debriding it. This process was repeated for a total of three months; there were no other treatments used prior to or during this regimen and there were no adverse reactions. The lesion gradually reduced in size with an initial rapid reduction in its dry, crusted nature.\nAfter three months, residual appearance of the lesion was a 20 mm by 17 mm area of pink skin with no elements of hypertrophy, crusting, or loss of skin integrity (). At six months, there were no signs of recurrence. At nine months, the appearance of the skin had fully returned to normal. A telephone follow-up was conducted at two years after treatment, and the patient reported that his skin in the area was still completely normal and that there were no signs of recurrence. A photograph was taken at this time ().","66-year-old man came to his doctor with a growing, scaly bump on his hand that he’d had for years. The doctor determined it was likely a seborrheic keratosis (a common, harmless skin growth) but also considered the possibility of skin cancer due to his history. Instead of removing it, he decided to try using Kanuka honey, which is currently being studied in a clinical trial, and it worked well, shrinking the bump significantly over three months. After six months, the skin had completely returned to normal, and there were no signs of the bump coming back, even after two years of follow-up."
406,"A 66-year-old woman presented for an outpatient colonoscopy for evaluation of six months of iron deficiency anemia, diarrhea, and rectal bleeding. Her history was significant for gastroesophageal reflux disease and chronic low back pain, on twice-daily naproxen. Serologic studies were notable for hemoglobin of 7.4 g/dL and a mean corpuscular volume of 70.6 fL. Colonoscopy demonstrated four diaphragm-like strictures, with scarring and ulceration, interspaced between normal mucosa in the cecum and ascending colon (). Biopsies obtained from the cecal and ascending strictures revealed ulceration, acute inflammation, and reactive changes (). The patient was instructed to discontinue naproxen and avoid all other NSAIDs. Patient was subsequently scheduled for a repeat colonoscopy in three months to monitor for resolution. Subsequent colonoscopy revealed both endoscopic () and pathologic resolution () of the diaphragm-like proximal colonic strictures.","A 66-year-old woman came in for a colonoscopy to investigate anemia, diarrhea, and rectal bleeding. Her blood tests showed she was very anemic, and the colonoscopy revealed four narrow areas in her colon that were causing problems. The biopsies showed inflammation and scarring in these areas, and it was determined that she had been taking naproxen which was contributing to the problem. The doctor instructed her to stop taking naproxen and scheduled a follow-up colonoscopy to check if the issues were resolving. Thankfully, the second colonoscopy showed that the narrow areas had disappeared."
407,"A 19-year-old Caucasian male presenting with severe primary sclerosing cholangitis underwent orthotopic liver transplantation and required a retransplant 5 weeks later due to a liver insufficiency caused by ligation of ruptured arterial pseudoaneurysm. He received more than 40 blood transfusions. The second donor was a 76-year-old male without a history of liver disease. The patient's postoperative course after retransplant included prolonged hemodialysis (8 weeks) due to acute kidney injury, cytomegalovirus (CMV) infection, hepatitis E infection, and hepatic artery stenosis in the anastomosis area, treated by angioplasty and stent insertion. Of note, hepatic artery stenosis resulted in ischemic-like cholangiopathy and prolonged cholestasis.\nThe patient's condition stabilized eight months after transplantation. Cyclosporin and Myfortic were administered for immunosuppression, in addition to aspirin and ursodeoxycholic acid. A liver biopsy performed at that period revealed numerous hypertrophic, iron-loaded macrophages and severe bile duct damage and loss, consistent with early mild chronic rejection (). Hemosiderosis was attributed to secondary iron overload, considering the numerous risk factors for this complication presenting before and after the retransplant (multiple blood transfusions, kidney injury, and CMV infection).\nEight months later, elevation of liver enzymes was observed: alanine transaminase (ALT), 127 IU/L, aspartate transaminase (AST), 61 IU/L, alkaline phosphatase, 209 IU/L, and gamma-glutamyl-transpeptidase (GGT), 222 IU/ L. Extensive laboratory and radiologic evaluations showed no abnormalities, aside from iron-related parameters: serum iron, 110 ng/ml, ferritin, 3170 mg/dl (versus 29 mg/dL before transplant), transferrin, 119 mg/dL, and transferrin saturation, 66%. Repeated liver biopsy revealed sinusoidal fibrosis with mild cholangiolar proliferation. Iron staining showed significant accumulation of iron in macrophages and hepatocytes, consistent with marked hemosiderosis ().\nThe combination of abnormal laboratory iron parameters and biopsy findings showing clear worsening of iron accumulation, without apparent new risk factors for secondary iron overload, led us to suspect primary rather than secondary hemosiderosis. Genetic testing of the patient's DNA ruled out preexisting HH and did not show any common HFE mutations (C282Y or H63D). Genetic high-resolution melt curve analysis of a biopsy sample revealed compound C282Y/H63D heterozygosity, confirming a genetic defect in the donor tissue, which elicited hereditary hemochromatosis in a recipient without any known HFE mutation.\nMagnetic Resonance Imaging (MRI) performed or iron assessment revealed mild hepatic iron overload, consistent with 5 mg/gr, and did not show accumulation of iron in other organs: pancreas, adrenals, spleen, and heart.\nFollowing the confirmation of the diagnosis, the patient was enrolled in a phlebotomy program.","This 19-year-old man had to undergo two liver transplants after his first one failed due to complications. He received many blood transfusions and battled infections during his recovery, requiring extended dialysis. Despite these challenges, he eventually stabilized, but later developed signs of liver damage and iron buildup. Further testing revealed a genetic condition that caused iron overload in his transplanted liver, leading to a phlebotomy program to remove excess iron."
408,"A five-year-old male began experiencing a popping sensation followed immediately by the sensation of heat and pressure starting medially at the base of the skull which radiated two centimeters bilaterally. He was a product of a nonconsanguineous union, whose birth history did not involve instrumentation and whose past medical, surgical, and developmental histories were all noncontributory. This sensation was experienced simultaneously with bilateral numbness of the posterior tongue and difficulty in speaking, both of which lasted fifteen seconds with gradual attenuation. The above would occur upon abrupt lateral rotation (either direction) of the head approximately five to ten times a year unrelated to trauma, diminishing in frequency as the patient aged.\nBetween the ages of eleven and fifteen, as a competitive fencer, the patient would experience the same symptomology when performing actions involving extreme, abrupt lateral rotation of the head, translating to a frequency of approximately once to twice weekly.\nAt the age of nineteen, during military training and combat, the same symptomology was experienced approximately fifteen times when abrupt lateral rotation of the head prompted by various forms of minor external head trauma was experienced. After discharge from the military, frequency of these episodes was sustained with simple lateral rotation of the head outside the context of trauma for six months with spontaneous abatement.\nAt the age of twenty-one, the first time the patient came to medical attention for the above complaint, the patient's physical and neurologic examinations were completely normal, including cranial nerve exam. Of note, the patient was not hyperelastic, nor was there a family history of the same. Magnetic resonance imaging (MRI) of the cervical spine (Figures –) was performed revealing slight dysplastic enlargement of the anterior arch of C1 vertebrate, mild degenerative changes of the atlantoaxial junction, and spinal canal caliber on the lower end of normal limits. No evidence for abnormal bone spurring or abnormality of the hypoglossal canal was found.\nOf note, upon further query, it was found that the patient's grandmother also had the same condition.","This five-year-old boy has been experiencing unusual sensations – a popping feeling followed by heat and pressure in his head – that happen when he quickly turns his head. These sensations are accompanied by numbness in his tongue and difficulty speaking, and they usually last for a short time. The episodes started when he was young and have become more frequent when he’s been fencing or during military training, especially when his head is turned quickly. An MRI of his neck showed a slight abnormality in the bones of his neck, and it’s thought this may be related to the symptoms, as his grandmother also had the same issue."
409,"A 72-year-old woman was referred to our hospital with pancreatic head and neck carcinoma for more evaluations and surgical operation. She had been suffering from abdominal pain for about 1 year, and the pain has become more intense in the last 6 months. The pain was postprandial and localized to the right upper abdomen. She has become icteric with generalized spread plus itching since past 2 months. The patient had no history of alcohol consumption or smoking. The patient was referred to us with a highly probable diagnosis of pancreatic head and neck carcinoma to be consulted for surgery. Also, a plastic stent was implanted for her in the previous health center due to intra- and extrahepatic duct dilation for the relief of patient symptoms and signs. After admission to our hospital, she was evaluated by abdominal computed tomography (CT), endosonographic imaging, and diagnostic ampullary biopsy. The abdominal CT scan with contrast exhibited an 18 mm × 20 mm hypodense mass at the head and neck of the pancreas (). Also, endosonographic imaging was performed to rule out periampullary lesions (). A 20 mm × 19 mm lesion was seen in the pancreatic head and neck region, and the main pancreatic duct was slightly dilated in the body of the pancreas. Moreover, the common bile duct (CBD) was distally thickened and contained sludge. Subsequently, diagnostic biopsy for pathological assessment was done. The biopsy revealed an irregular gray-creamy soft tissue which had undifferentiated malignant tumor features at microscopic evaluations. During the Whipple procedure, after cutting the neck of the pancreas in the left side of the portal vein, the frozen section revealed more involvement of pancreatic tissue. Although additional 2 cm was resected, the pancreas residue still had tumor involvement, macroscopically. Therefore, the patient was undergone total pancreatectomy.\nPostoperative pathological studies were established and indicated a tumor with the greatest dimension of 4 cm which was extended to the duodenum. Invasion of the venous, lymphatic vessels and perineural sites was seen; however, no exact evidence of distant metastasis was found. The periampullary occlusion had hindered the bile flow over time, leading to chronic cholecystitis and pancreatitis which was confirmed by histopathological assessments. The hematoxylin and eosin staining revealed the presence of two cellular populations including spindle fibroblast-like and pleomorphic cells within the tumor. In addition, the proliferation pattern of the mesenchymal cells was storiform (). There was not any well-differentiated component in the tumor tissue or adjacent tissue. Overall, the pathologic stage II A was assigned to the tumor. The immunohistochemical staining was performed, and the tumor was positive for CD68, lysozyme, alpha 1-antichymotrypsin, and vimentin (). Also, it was negative for S-100P, cytokeratin, epithelial membrane antigen, desmin, CD34, smooth muscle antigen, MDM2, and CDK4. Therefore, the tumor diagnosis was compatible with UPS. The tumor Ki-67 expression was more than 30%. To evaluate metastasis occurrence, contrast-enhanced thoracic high-resolution CT scan and multidetector CT scan of abdominopelvic were done at 6, 12, 18, 24, and 36 months after operation, and no evidence of metastasis was detected. Insulin and Creon were started after surgery for long life. The further follow-up investigations were done by periodic CT scan and ultrasonic imaging. Fortunately, she was disease-free during 5-year follow-up and tolerated total pancreatectomy, well.","A 72-year-old woman was admitted to the hospital after experiencing persistent abdominal pain and jaundice. Tests revealed a tumor in the head and neck of her pancreas, and a biopsy confirmed it was cancerous. To relieve her symptoms, a stent was placed in her bile duct, but the tumor had spread. Ultimately, she underwent a total pancreatectomy, removing her entire pancreas, and the pathology showed the tumor had spread to the duodenum. After surgery, she was closely monitored and is currently doing well with insulin and pancreatic enzyme replacement therapy."
410,"A 64-year-old man with a medical history of micropapillary thyroid cancer and stage IIIb lung adenocarcinoma with no evidence of active disease for 3 years after chemotherapy and radiation presented with subjective complaints of new onset fatigue, nausea, scalp tenderness, and xeroderma. His medications included gabapentin 300 mg four times a day for chemotherapy-induced neuropathy, erlotinib 150 mg once daily for epidermal growth factor receptor- (EGFR-) positive lung adenocarcinoma, and omeprazole 40 mg once daily for subjective gastroesophageal reflux disease. A screening magnetic resonance image of the head revealed a new hypovascular pituitary mass measuring approximately 1 cm by 0.8 cm ().\nOn examination, the patient's vital signs were within normal limits. On physical exam, xeroderma was appreciated in all extremities. Finger size was proportional and no prognathism, acromegaly, or Cushingoid features were appreciated. The cardiopulmonary exam was normal.\nInitial lab values demonstrated normal free triiodothyronine (T3) of 2.4 pg/mL (normal range (NR) 1.8–4.2 pg/mL), normal T3 of 86 ng/dL (NR 70–172 ng/dL), and normal free thyroxine of 1.00 ng/dL (NR 0.84–1.68 ng/dL). Prolactin was elevated at 28.9 ng/mL (NR 2.5–17.0 ng/mL). The patient's morning cortisol was immeasurably low at <1.0 mcg/dL (NR > 10 mcg/dL) as was the patient's testosterone level at <20 ng/dL (280–1100 ng/dL). Luteinizing hormone was low at 0.05 mIU/mL (NR 1.8–12.0 mIU/L).\nThe patient was started on prednisone 20 mg by mouth daily, at which point he noticed immediate improvement in his energy and appetite as well as decrease in his nausea. For chronic steroid replacement therapy, the patient's treatment was changed from prednisone to hydrocortisone 20 mg of hydrocortisone in the morning and 10 mg in the evening. The patient was additionally instructed about the dangers of adrenal crisis and told to increase his hydrocortisone to 90 mg daily if acutely ill.\nWithin a month of initial diagnoses, the patient suffered acute visual bilateral field cut and loss of peripheral vision. A repeat MRI demonstrated rapid enlargement of his pituitary mass, nearly doubled in size and described as a 2.2 cm by 1.2 cm mass impinging on the overlying optic chiasm ().\nThe patient subsequently underwent transsphenoidal resection of his pituitary mass. Gross histology characterized the mass as firm and fibrous. Macroscopic analysis revealed metastatic lung adenocarcinoma described as adenohypophysis fibrosis. Further histologic analysis revealed positive identification of cytokeratin 7, TTF-1, Ki-67, and epidermal growth factor receptor (EGFR) positive with EGFR gene nucleotide change demonstrating T790M and L858R positivity. This histopathology demonstrated further EGFR mutation of the patient's known history of lung adenocarcinoma which initially was only positive for EGFR mutation L585R.\nAfter transsphenoidal resection and subsequent whole-brain radiation, further results demonstrated a continued low morning cortisol at <1.0 mcg/dL (NR > 10 mcg/dL) and testosterone level at <20 ng/dL (280–1100 ng/dL). Luteinizing hormone was low at <0.1 mIU/mL (NR 1.8–12.0 mIU/L) as was follicle-stimulating hormone 0.8 mIU/mL (NR 1.5–12.4 mIU/mL). Free T4 was low at 0.65 ng/dL (NR 0.84–1.68 ng/dL) and thyroid-stimulating hormone was low at 0.019 MCI/mL (NR 0.4–4.0 MCI/mL). Prolactin was lower than previous but still elevated at 14.9 ng/mL (NR 2.5–17.0 ng/mL).","This 64-year-old man has been battling lung cancer and has now developed new symptoms, including fatigue, nausea, and dry skin. Blood tests revealed low cortisol and testosterone levels, suggesting a problem with his pituitary gland. An MRI showed a growing mass in his pituitary gland that was found to be cancerous, originating from his lung cancer. After surgery to remove the mass, further tests showed that the cancer had spread to the pituitary gland, and he will continue with radiation therapy."
411,"A 32-year-old male presented with progressive shortness of breath and leg swelling. He had a past medical history significant for HIV disease and metastatic transitional cell cancer of the renal pelvis and left nephrectomy. He had metastatic involvement of the spinal bones, mediastinal lymph nodes, and lungs. He received gemcitabine and cisplatin followed by salvage treatment with atezolizumab. He was also on combination antiretroviral therapy (elvitegravir/cobicistat/emtricitabine/tenofovir/alafenamide) with undetectable viral load and CD4 count of 444 cells/mm3.\nThe physical exam was remarkable for tachycardia, respiratory distress, decreased breath sounds in the right hemithorax, and lower extremity swelling. A chest X-ray showed a right-sided pleural effusion and an enlarged nodular density in the left upper lobe. Laboratory data revealed anemia and an elevated troponin I. Patient symptoms resolved partially after he received a blood transfusion and underwent therapeutic thoracentesis. Transthoracic echocardiography revealed normal left ventricular function and a large, mobile, cystic mass in the right and left atrium. Transesophageal echocardiography revealed a large mass composed of solid and cystic components. The solid component, a 5 × 2.3 cm2 mass, had invaded the basal half of the interatrial septum and the cystic component was found to protrude into the left atrium (). Multiple enhancing neoplastic masses in the muscular compartment in both calves were detected on MRI of the lower extremities, which were in favor of neoplastic lesions (Figures –). The patient was not a candidate for surgery considering the extent of disease and he expired few days after presentation.","6 This 32-year-old man came to the hospital because he was having trouble breathing and his legs were swollen. He had a history of HIV and cancer that had spread to his bones, lymph nodes, and lungs. After treatment, his symptoms improved somewhat with a blood transfusion and draining fluid from his chest. However, tests revealed a large mass in his heart and cancer spread to his legs, and he unfortunately passed away a few days later."
412,"The subject was a 75-year-old woman who had suffered from postherpetic neuralgia for 8 years. In December 2016, she underwent a Gasserian ganglion nerve block at the Department of Anesthesiology of our hospital. Gasserian ganglion nerve block was performed to treat the first branch neuralgia of the trigeminal nerve in the right at the point which is 2.5 cm lateral to the right angle of the mouth, following a straight line directed toward the pupil. A needle was inserted under guidance of X-ray imaging on the monitor display. When the tip of the guiding needle reached the foramen ovale, the location of the needle tip was confirmed to be slightly inside from the median in the frontal view. Next, after confirming dysesthesia, 2% xylocaine was injected. And then loss of sensation was confirmed in the V1 and V2 regions. After injecting 99.5% ethanol (0.2 mL), radiofrequency thermocoagulation was added continuously for 180 seconds at 90°C.\nOn the following day, the patient noticed a sudden visual acuity (VA) decline and hyperemia in the right eye. She visited a neighborhood ophthalmologist and was diagnosed with conjunctival hyperemia, corneal epithelial defect, and Descemet's folds in the right eye. Because no improvement was achieved, she visited our hospital two days later. The initial VA was 0.03 (n.c.) in the right eye. A slit-lamp examination revealed severe conjunctival hyperemia all around the periphery, remarkable corneal superficial neovascularization, especially, at 11 to 5 o'clock, corneal epithelial defects of the size approximately 3 × 4 mm, Descemet's membrane folds, and mild stromal edema were observed; however, no signs of inflammation were seen in the anterior chamber. The patient did not experience any eye pain and corneal sensitivity measured with Cochet-Bonnet esthesiometer was <10 mm in the right eye and 60 mm in the left, showing remarkable decline of corneal sensitivity in the right. She was diagnosed with NK stage 2 ().\nTreatment was started with 0.3% ofloxacin ophthalmic ointment (Tarivid® ophthalmic ointment 0.3%; Santen Pharmaceutical Co., Ltd., Osaka, Japan) twice daily and rebamipide ophthalmic suspension (Mucosta® ophthalmic suspension unit dose 2%; Otsuka Pharmaceutical Co., Ltd., Tokyo, Japan) 4 times daily. The corneal epithelial defects were gradually alleviated at 1 week after ganglion nerve block (Figures and ).\nBecause severe conjunctival hyperemia persisted, we added fluorometholone ophthalmic suspension (fluorometholone® ophthalmic suspension unit dose 0.1%; Santen Pharmaceutical Co., Ltd.) 3 times daily. At 1 month after ganglion nerve block () severe conjunctival hyperemia, very severe corneal superficial neovascularization at 11 to 5 o'clock, persistent corneal defects with smooth and rolled edges, and stromal swelling in the central cornea occurred. At 2 months (), superficial punctate keratopathy (SPK), conjunctival hyperemia, corneal superficial neovascularization at 11 to 5 o'clock, irregularity of corneal epithelium, stromal scarring in the central cornea, and the right corneal sensation of 10 mm remained. Evaluation with the HRT II RCM was made in April 2017 and no corneal subbasal nerve fibers were observed in the right eye (Figures and ). Five months after the trigeminal nerve block, slight irregularity of corneal epithelium and stromal scarring in the central cornea were observed; however, conjunctival hyperemia and corneal superficial neovascularization were overall improving and the corneal sensation in the right eye also recovered to about 50 mm although SPK remained (). At this point, corneal epithelial defect was overall improving; therefore, ofloxacin eye ointment was stopped. Rebamipide and fluorometholone eye drops were continued and the patient was followed up. According to the patient, she had regained the sense of touch on the cheek. Corneal erosion sometimes recurred but it recovered in about a week. Six months after the trigeminal nerve block, the corneal sensation in the right eye was 60 mm. SPK and conjunctival hyperemia, corneal superficial neovascularization, and corneal stromal edema were all ameliorated (). Therefore, fluorometholone eye drops were tapered off and only rebamipide eye drops were continued. In addition, at this point, regenerated corneal subbasal nerve fibers in the right eye were detected by the HRT II RCM although they appeared to be smaller and thinner than those in the fellow eye (Figures and ).","This 75-year-old woman has been dealing with pain from shingles for eight years. Initially, she received a nerve block to try to relieve the pain, which seemed to help. However, after the nerve block, she developed problems with her right eye, including inflammation, corneal damage, and vision loss. Despite treatment, the eye problems persisted, leading to further complications like scarring and a loss of feeling on her cheek. Over time, with continued medication and careful monitoring, her eye problems improved, and she regained some of her sense of touch."
413,"A 65-year-old man with no relevant family or personal history was admitted due to a 2-day history of mild dysarthria and naming impairment with difficulty finding words and holding a conversation. Furthermore, his family reported at least one-month history of motor awkwardness and gait instability. During the neurological examination, the patient was well oriented, displayed fluent, coherent spontaneous language, and had mild dysnomia. He had remarkable clinical symptoms of parkinsonism: glabellar reflex was persistent; the patient presented a decreased blinking rate, hypomimia, moderate bradykinesia and hypokinesia in the right limbs, and slow gait with reduced right arm swing (Hoehn & Yahr stage I). The examination revealed no further abnormalities. With the clinical suspicion of parkinsonism, we performed a complete blood test performed at baseline, with either normal or negative. A brain MRI scan revealed increased signal intensity in the left putamen with diffusion restriction, suggesting lacunar infarction (). By the initial findings obtained in the MRI, vascular study was completed with echocardiogram, Holter monitoring, and Doppler ultrasound of the supra-aortic trunks which showed no significant alterations. Parkinsonism was studied using 123I-ioflupane SPECT (DaTSCAN), which revealed asymmetrical tracer uptake in the caudate nuclei (decreased uptake in the left caudate nucleus) and a near-complete lack of activity in the putamina, particularly on the left side (). On discharge, the initial working diagnosis was ischemic stroke and idiopathic Parkinson's disease. The patient remained at home. Progression was poor: dysphasia persisted and mobility decreased dramatically until the patient was unable to walk or even hold a standing position. He was readmitted 2 weeks later in a state of stupor. An emergency EEG revealed short-interval (<2 s) lateralized interictal epileptiform discharges in the left hemisphere (triphasic sharp waves and spikes); discharges were of greater amplitude in anterior regions and occasionally spread to the contralateral hemisphere. Within 24 hours, the patient went into coma (Glasgow Coma Scale 3) and was admitted to the intensive care unit. In the following days, and despite administration of several antiepileptic drugs (levetiracetam, valproate, propofol, and midazolam), he remained comatose and displayed clonic movements in the right arm. An additional brain MRI scan performed a month after the initial scan revealed progression of the lesions, with a patchy, gyriform hyperintensity predominantly affecting the cortex of the left hemisphere and the right temporoparietal cortex, visible in diffusion sequences only ().\nOur patient's rapid neurological deterioration, the presence of myoclonus, and MRI findings pointed to a diagnosis of Creutzfeldt-Jakob disease. To rule out other possible causes of rapidly progressive dementia, we conducted a tumour extension study including tumour markers, onconeural antibodies, and a thoracic-abdominal CT scan; the study yielded negative results. The results from a CSF cell count and a biochemical and microbiological analysis were negative; Harrington test for the determination of 14-3-3 protein on CSF was positive (test was performed at Spain's National Microbiology Centre). An additional EEG performed 2 months after the first EEG revealed disorganised slow background activity, with generalized periodic sharp-wave complexes (PSWCs) at a frequency of 1 Hz (). A genetic study found no mutations in the PRNP gene; the study of codon 129 polymorphisms revealed methionine/valine heterozygosity (M129V). The probable diagnosis of sporadic CJD was reached based on the World Health Organization (WHO) diagnostic criteria []. He died 5 months after disease onset; autopsy was not granted.","A 65-year-old man experienced difficulty speaking and moving, along with clumsiness, which led to a neurological evaluation. Tests revealed signs of Parkinson’s disease and a stroke, but further investigation showed a rare and rapidly progressing brain disorder called Creutzfeldt-Jakob disease. The patient’s condition quickly worsened, leading to a coma and requiring intensive care. Despite treatment, he remained unresponsive, and a genetic test confirmed the diagnosis of sporadic CJD."
414,"The patient was a 40-year-old primiparous woman. She was diagnosed with submucosal fibroids by her previous gynecologist 5 years prior, based on chief complaints of atypical genital bleeding and hypermenorrhea. She underwent hysteroscopic myomectomy for one 1 cm sized and one 3 cm sized submucosal fibroid located between 2 and 3 o'clock in the uterine fundus. Asherman's syndrome was suspected after the patient exhibited secondary hypomenorrhea 10 months after surgery. Therefore, hysterosalpingography and magnetic resonance imaging (MRI) were performed. Intrauterine adhesions were suspected based on hysterosalpingography findings, while uterine cavity narrowing was identified using MRI. Hysteroscopy revealed filmy adhesions consistent with myomatous tissue at the excision site, and the patient was diagnosed with Asherman's syndrome.\nEight months after diagnosis, the patient underwent hysteroscopic adhesiolysis. The filmy adhesions observed on the left side of the fundus were easily separated with Hegar cervical dilators, and an intrauterine device was inserted after dilation. The patient was diagnosed with stage I Asherman's syndrome defined by European Society for Hysteroscopy classification of intrauterine adhesions, and menstrual flow returned to normal after the operation.\nWhile the patient had a strong desire to bear children, her inability to conceive for 7 years led her to pursue in vitro fertilization (IVF). After having a miscarriage at 7 weeks of gestation, she underwent cervical dilatation and uterine curettage.\nSix months after the miscarriage, the patient became pregnant again through IVF and was referred to our hospital at 7 weeks of gestation. At 19 weeks of gestation, tissues with a free edge were visualized within the amniotic cavity using obstetric ultrasound and were determined to be amniotic sheets on MRI at 31 weeks of gestation (). The course of pregnancy was uneventful thereafter, and an elective cesarean section was performed at 38 weeks and 2 days of gestation because of a breech presentation.\nThe placenta adhered to the uterine wall after childbirth and could not be easily separated manually. The blood vessels on the uterine surface at the placental implantation site were engorged (), leading us to diagnose the patient with placenta increta. The placenta remained firmly adherent to the uterine wall, and although there was almost no bleeding from the uterine cavity, cesarean hysterectomy was performed after informed consent was obtained from the patient. In the abdominal cavity, 4 cm subserosal uterine fibroids were observed on the left side of the fundus, and adhesions thought to be caused by endometriosis were found in the right adnexa, posterior uterus, and anterior rectum. The operative time was 101 minutes, while the total blood loss was 1,584 ml (including amniotic fluid). Blood transfusion was not required. Macroscopic examination of the uterus after extraction showed the presence of placenta from the fundus to the posterior wall, diffusely adherent to the myometrium (), along with partial thinning of the fundus.\nPlacenta increta was confirmed based on postpartum histological findings of placental villi invading the myometrium, without an interposed decidual plate.\nThe postoperative course was uneventful, and the patient was discharged in good health on the 7th postpartum day.","This patient had a history of uterine fibroids and struggled with irregular bleeding and difficulty conceiving. After several procedures to remove the fibroids, she developed Asherman’s syndrome, which caused scarring inside her uterus and made it hard to get pregnant. She successfully conceived through IVF but experienced a miscarriage, and then discovered she had placenta increta, where the placenta attached too deeply into the uterine wall. Ultimately, a cesarean section was performed, and the uterus was removed due to the placenta’s persistent attachment."
415,"The patient was a 45-year-old Hispanic male who had end-stage renal disease of unknown etiology, hypertension, and hyperlipidemia. His HLA typing was A 2,- B 7, 35, Cw 4, 7, DR 4,- DQ 8,-. His donor was a 46-year-old Hispanic female with history of hyperlipidemia with a measured 24-hour urine creatinine clearance of 151 ml/min. Her HLA typing was A 2,31, B 35,44, Cw 4,5, DR 4,-, DQ 7,8. The patient had been on intermittent hemodialysis for two years prior to undergoing living related kidney transplant. Induction therapy consisted of basiliximab and solumedrol. Maintenance therapy was with tacrolimus, mycophenolate mofetil, and prednisone. His two-year course after transplant had been unremarkable, with a baseline serum creatinine of 1.5–1.7 mg/dL (134–150 μmol/L), without proteinuria or hematuria.\nTwo years after transplant he presented to the renal transplant clinic with complaints of lower extremity edema that had appeared over the previous three days. He stated he had experienced a flu-like illness a week prior. In addition, he admitted to inadvertently taking tacrolimus 1 mg q12h, rather than his prescribed dose of 3 mg twice a day for almost one month. He had corrected the dose approximately 3 weeks prior to presentation. On examination, he was normotensive and afebrile. Cardiovascular and respiratory examinations were normal. He had periorbital edema and 6 mm pitting edema in lower extremities. He did not have graft tenderness or bruit.\nLaboratory data was remarkable for creatinine of 2.2 mg/dL (194 μmol/L). Urinalysis showed moderate blood and 3+ protein (previously no proteinuria), urinary sediment of more than 50 red blood cells (RBCs), 11–20 white blood cells (WBCs) per high power field (HPF), and urine protein/creatinine ratio of 8.2 g (previously 100 mg). Tacrolimus trough was 4.9 ng/mL.\nDue to acute kidney injury, proteinuria, and hematuria in the setting of suboptimal immunosuppression, there was a high concern for acute rejection versus rapidly progressive glomerulonephritis perhaps due to recurrence of the unknown primary disease. Renal ultrasound and a renal biopsy were ordered. Given the risk of acute rejection due to inadvertent medication noncompliance, prednisone was increased from 10 mg daily to 50 mg daily, tacrolimus was increased from 3 mg twice a day to 5 mg twice a day, and mycophenolate mofetil was increased to 1500 mg twice a day. Of note, BK virus and donor specific antibodies were negative a month prior.\nThe renal US was negative for hydronephrosis or calculi. Three days later, a biopsy was performed.\nPreliminary biopsy report was consistent with postinfectious glomerulonephritis (). Due to the recent infection, anti-streptolysin O (ASO) antibodies, C3, and C4 were ordered. Since initial biopsy did not have any glomeruli for immunofluorescence (IF), he was scheduled for repeat biopsy.\nIn the interim, C3 and C4 were reported. C3 was low at 59 mg/dL with a normal C4 at 35 mg/dL (). Tacrolimus trough was 8.6 ng/mL.\nSecond kidney biopsy one week later revealed minimal residual subendothelial electron dense deposits, but no evidence of large subepithelial electron dense deposits (). IF showed nonspecific patchy staining with C3 in glomeruli and some tubules. All other reagents were negative, including C4d in peritubular capillaries, BK, and SV40 in tubular cells. There is no evidence of cell-mediated or antibody mediated glomerulonephritis. Overall, biopsy was consistent with resolving postinfectious glomerulonephritis. Anti-streptolysin O (ASO) was elevated at 603 IU/mL (), highly indicative of Streptococcus being the causative agent.\nGiven that his AKI did not appear to be due to rejection, tacrolimus was decreased back to his basal dose of 3 mg twice a day and prednisone was tapered to 10 mg daily. Fluid management was achieved with furosemide. He was not prescribed any antibiotics. A month later, creatinine had decreased to 1.9 mg/dL (168 μmol/L), and in a 3-month period, it had returned to baseline and proteinuria and hematuria had completely resolved ( and ).","This 45-year-old man with kidney failure had been doing well after a kidney transplant for two years. Recently, he developed swelling in his legs, blood in his urine, and some protein in his urine, which raised concerns about a possible rejection of the transplant. It turned out he accidentally took too little of his medication, which caused the kidney problems. After a series of tests, including kidney biopsies, doctors determined that he had developed an infection that caused inflammation in his kidneys. The doctors increased his medication and treated the infection, and his kidney function has now returned to normal. He will continue to monitor his kidney function closely and take his medications as prescribed."
416,"A 2-year-old healthy boy underwent an office-based surgical circumcision by his physician. During the procedure, profound bleeding was observed that was not controlled by applying direct pressure. The physician attempted to control the bleeding by multiple deep suturing; the bleeding was stopped successfully and the patient was discharged home. During the postoperative period, the child had progressive painful and difficult voiding with only few drops of urine after straining. During this period the child was prescribed painkillers for his discomfort and no additional evaluation was done. After about a week he was referred to a district hospital with fever (temperature=38.1C), agitation, vomiting, urinary retention, and significant abdominal distension. Abdominal examination showed decreased bowel sounds, dull percussion, and severe guarding. Urgent abdominopelvic ultrasound revealed large volume of free fluid in the abdomen and pelvis, with small amount of urine in the bladder. The initial lab tests also showed leukocytosis with left shift and increased blood creatinine and blood urea nitrogen. Catheterization with a 6-Fr feeding tube failed because it did not pass beyond the subcoronal urethra. The patient underwent emergency midline laparotomy with the diagnosis of acute abdomen and the fluid was drained. A small intraperitoneal bladder rupture was noticed at the dome of bladder. The gastrointestinal tract was inspected precisely and was intact. The diagnosis of intraperitoneal bladder rupture with urinary ascites was made probably due to near-total urethral obstruction. The rupture site was repaired in two layers and a suprapubic cystostomy catheter was fixed.\nTwo months later, the patient was referred to our center for further evaluation and treatment. Antegrade voiding cystourethrography (VCUG) was performed via the suprapubic catheter that showed terminal urethral obstruction (). Urethroscopy was attempted under general anesthesia that failed due to complete obstruction at 1 cm from the meatus.\nDecision was made to explore the area and to repair the urethra. Through a circumferential incision, distal urethra was elevated from the corpus spongiosum. A 3-Fr ureteric catheter also did not pass the obstructed part (). A 5-mm fibrotic tissue was encountered at the site of obstruction (). The corpus spongiosum was dissected free from the corpora cavernosa to prevent iatrogenic chordee after end-to-end urethral anastomosis. The obstructed fibrotic part of urethra was completely resected and an end-to-end urethral anastomosis was performed along with spongioplasty over an 8-Fr silicon catheter in two layers; dartos pedicled flap was used to cover the site of anastomosis. The postoperative period was uneventful and the patient was discharged home with suprapubic and urethral catheter. The urethral catheter was removed seven days following the surgery. The suprapubic catheter was removed four weeks after the surgery following normal urethral voiding and normal ultrasound. A VCUG was performed six weeks after the surgery that showed normal bladder and urethra with acceptable voiding per urethra (). During a 2.5-year follow-up period, the patient was asymptomatic with normal renal function, ultrasound, and voiding pattern. He had a maximum flow rate of 15.3 ml/sec in uroflowmetry study.","This 2-year-old boy had a circumcision that unfortunately caused a lot of bleeding. After the procedure, he had trouble peeing and developed a fever and abdominal pain, requiring emergency surgery to find a bladder rupture. Surgeons repaired the rupture and performed a complex procedure to clear the blockage in his urethra, ultimately restoring normal urination. He has been doing well for over two years with no problems."
417,"Donor 1 was a 6-year-old boy from Guangxi province in China. The initial symptoms on 13 May 2015 were fever of unknown cause with insomnia and refusal to eat or drink. He was sent to a local hospital due to subsequent agitation, screaming and incoherent speech. Three days later, he suffered from dysphagia and hypersalivation. His condition subsequently worsened despite treatment with ribavirin. Finally, he died on 26 May after receiving an initial diagnosis of viral encephalitis of unknown cause (Fig. ). Cerebrospinal fluid (CSF) analysis revealed an opening pressure of 60 drops/min, transparent and limpid fluid, a glucose level of 4.7 mmol/L, and a protein level of 265 mg/L. Computed tomography (CT) revealed a slightly decreased density in the bilateral temporal lobes. Tests for HIV, hepatitis B, hepatitis C, and syphilis yielded negative results. Earlier, the donor had frequent contact with domestic dogs but no longer had this type of exposure since moving to live with his grandmother in another city. His family members denied either exposure to potentially rabid animals or history of rabies vaccinations. His kidneys and corneas were donated for transplantation.\nDonor 2 was an 11-year-old girl who lived in Hebei province in China. Her initial symptoms were nausea, chills and vomiting on 22 Sep 2016. One day later, her condition worsened, and she suffered from fever, disorder of consciousness, coma, respiratory failure and decreased blood pressure, followed by insipidus and myasthenia gravis on 5 Oct. She died on 11 Oct after an initial diagnosis of acute disseminated encephalomyelitis (Fig. ). The results of CSF analysis were normal. Magnetic resonance imaging (MRI) revealed diffuse signal abnormalities throughout the brain and cervical spinal cord. Tests for HIV, hepatitis B, hepatitis C, syphilis, cytomegalovirus, Epstein Barr virus, coxsackie virus, herpes simplex virus, adenovirus and rubella virus yielded negative results. Her family members denied exposure to potentially rabid animals or history of rabies vaccinations. Her kidneys and liver were donated for transplantation.\nRecipient 1 was a 55-year-old male who received a kidney transplant from donor 1 on 27 May 2015. The allograft recovered successfully, and the immunosuppressive regimen included tacrolimus, mycophenolate sodium and prednisone. The patient initially became symptomatic on 10 Jul 2015 (Fig. ). MRI revealed mottled signal abnormalities around the bilateral ventricles and deep white matter (low or equal signal on T1WI and high signal on T2WI and Flair), which indicated white matter demyelination. He was clinically diagnosed with rabies on 24 Jul according to the typical symptoms; this diagnosis was confirmed 4 days later by detectable rabies virus-specific nucleic acids in saliva, urine, and sputum samples. The patient died on 23 Aug. His family members denied exposure to potentially rabid animals or history of rabies vaccinations.\nRecipient 2 was a 43-year-old male who received a kidney transplant from donor 1 on 27 May 2015. The allograft recovered successfully, and the immunosuppressive regimen included tacrolimus, mycophenolate sodium and prednisone. The patient initially became symptomatic on 14 Jul 2015 (Fig. ). CT revealed a suspicious mottled low-density region in the right basal ganglia region. He was clinically diagnosed with rabies on 24 Jul according to the typical symptoms, which was confirmed 4 days later by detectable rabies virus-specific nucleic acids in saliva and urine samples. The patient died on 17 Aug. His family members denied exposure to potentially rabid animals or history of rabies vaccinations.\nRecipient 3 was a 57-year-old female who received a liver transplant from donor 2 on 11 Oct 2016. The allograft recovered successfully, and the immunosuppressive regimen included tacrolimus, mycophenolate mofetil and methylprednisolone. The patient initially became symptomatic on 18 Mar 2017 (Fig. ). CT revealed a suspicious mottled low-density region in the left basal ganglia region. She was clinically diagnosed with rabies on 24 Mar according to the typical symptoms, which was confirmed 2 days later by detectable rabies virus-specific nucleic acids in a saliva sample. The patient died on 26 Mar without invasive mechanical ventilation. Her family members disclosed that she had contact with a domestic pet half a year before transplantation but denied exposure to potentially rabid animals or history of rabies vaccinations.\nRecipient 4 was a 50-year-old male who received a kidney transplant from donor 2 on 11 Oct 2016. The allograft recovered successfully, and the immunosuppressive regimen included tacrolimus, mycophenolate mofetil and prednisone. The patient received post-exposure prophylaxis (PEP) immediately after confirmation of rabies in deceased recipient 3 on 26 Mar 2017, which consisted of a 5-dose vaccination regimen (5 doses of purified chick embryo cell rabies vaccine, the first given directly after suspected exposure and subsequently on days 3, 7, 14, and 28) with 1 dose of rabies immunoglobulin (20 IU/kg). However, he still became initially symptomatic on 10 Aug 2017 (Fig. ). He was clinically diagnosed with rabies on 14 Aug according to the typical symptoms, which was confirmed 1 day later by detectable rabies virus-specific nucleic acids in saliva, urine, and sputum samples. The patient died on 16 Aug without invasive mechanical ventilation. His family members denied exposure to potentially rabid animals or history of rabies vaccinations.\nRecipient 5 was a 46-year-old male who received a kidney transplant from donor 2 on 11 Oct 2016. The allograft recovered successfully, and the immunosuppressive regimen included tacrolimus, mycophenolate sodium and prednisone. The patient received PEP immediately after confirmation of rabies in deceased recipient 3 on 26 Mar 2017, which consisted of a 5-dose vaccination regimen with 1 dose of rabies immunoglobulin. The recipient currently remains asymptomatic (Fig. ). Unfortunately, the level of rabies-specific neutralizing antibody was not quantified.\nRabies virus-specific nucleic acids were detectable in saliva, urine, and sputum samples from recipient 1 and were also detectable in saliva and urine samples from recipient 2. The same results were found in a saliva sample from recipient 3 and in saliva, urine, and sputum samples from recipient 4. According to diagnostic criteria in China [], these recipients were laboratory-confirmed as positive for rabies.","This report details five cases of adults who developed rabies after receiving kidney or liver transplants from donors in China. The patients initially showed symptoms like fever, confusion, and difficulty swallowing, and were later confirmed to have rabies through testing of their saliva and urine. In several cases, the virus was detected in the donors as well, suggesting a possible source of infection. While some patients received post-exposure treatment, unfortunately, four of them died from the disease."
418,"A 51-year-old man was admitted to the Department of Nephrology of the First Affiliated Hospital of Nanjing Medical University (Nanjing, China) in June 2016 because of hypercalcemia and renal failure. The patient was well until he developed persistent leg and low back pain 20 days before admission. Prior testing at a local hospital showed progressive abnormal renal function (serum creatinine, 304.2 μmol/L), a high serum calcium level (4.86 mmol/L), and an extremely high level of parathyroid hormone (PTH) (1551 ng/L; reference level, 12–88 ng/L). After hydration with saline and diuresis with loop diuretics and hemodialysis, the patient was transferred to our hospital. He had no unusual issues with the exception of a 1-year history of stage 3 chronic kidney disease.\nOn admission, the patient reported fatigue, bone pain, and polyuria. A physical examination on admission revealed blood pressure of 119/80 mmHg and heart rate of 71 beats per min, but no specific findings of the lymph nodes, head, neck, heart, lung, abdomen, or joints. Laboratory testing revealed corrected serum calcium of 3.39 mmol/L, phosphorus of 0.84 mmol/L, PTH of 540 ng/mL, serum creatinine of 151 μmol/L (after hemodialysis), and hemoglobin of 120 g/L. Urine calcium was 9.2 mmol/24 h. An electrocardiogram (ECG) showed complete right bundle branch block and a precordial abnormal ST segment and normal corrected QT (QTc) interval (Fig. ). Myocardial marker analysis showed serum myoglobin of 31 μg/L and high-sensitivity troponin T of 826 ng/L. Ultrasonography revealed a nodule at the left lower pole of the parathyroid gland. Single-photon emission computed tomography results indicated an adenoma of the left lower pole of the parathyroid gland. Due to no family history of such disease and no medication history of thiazide or lithium, a diagnosis of primary hyperparathyroidism caused by a parathyroid adenoma was made. Because of the high level of troponin T and changes to the ST segment on an ECG, acute myocardium injury was considered, thus parathyroidectomy was deferred for multidisciplinary consultation. The final diagnoses were hypercalcemia, primary hyperparathyroidism, parathyroid adenoma, acute renal injury, chronic kidney disease, and acute myocardium injury.\nHydration with 3000 mL of fluid [60% saline (0.9%) and 40% dextrose (5%)] per day, furosemide at 40 mg three times per day, cinacalcet at 25 mg per day, and RRT were utilized to reduce serum calcium levels. Double lumen catheterization of the right femoral vein was used for vascular access. Slow extended dialysis with low-calcium dialysate (1.25 mmol) at bedside was started using a commercially available continuous RRT device (multiFiltrate, Fresenius Medical Care Deutschland GmbH, Bad Homburg, Germany). The dialysate flow was 4800 mL/h for 8 h each session. Changes in serum corrected calcium levels are shown in Fig. . Due to an unsatisfactory lowering of serum calcium and preparation for parathyroidectomy, slow extended dialysis was switched to 8-h RCA-predilutional hemofiltration at a replacement rate of 4300 mL/h. The replacement solution was prepared by a local pharmacy (Na, 105 mmol/L; bicarbonate, 21.5 mmol/L; zero calcium; magnesium, 0.5 mmol/L; and glucose, 6.7 mmol/L). The infusion rate of 4% trisodium citrate (citrate dose 4.2 mmol/L) was 220 mL/h and the blood flow was 120 mL/min when hematocrit was 32%.\nGiven the time limit of treatment, we gradually reduced the rate of calcium supplementation from 5.6 to 2.2 mmol/h in the first session of RCA-hemofiltration (Fig. ). The post-filter ionized calcium was 0.34–0.53 mmol/L during RCA-hemofiltration. The corrected serum calcium level decreased from 3.5 to 3.2 mmol/L and ionized calcium decreased from 1.97 to 1.67 mmol/L. This result was not inspiring, despite estimated 30 mmol calcium loss during RCA-hemofiltration.\nA lower infusion rate of calcium (1.1 mmol/h) was started at the second session of RCA-hemofiltration (Fig. ). However, chest discomfort and palpitations were reported by the patient after treatment for 15 min. At that time, his blood pressure was 110/64 mmHg and heart rate was 125 beats per min. Blood gas analysis showed potassium of 3.8 mmol/L and a rapid serum ionized calcium decrease (from 1.91 mmol/L to 1.71 mmol/L). Hence, calcium supplementation immediately increased to 5.6 mmol/h and 2 min later, the chest discomfort and palpitation disappeared. However, the 12-lead ECG demonstrated sinus tachycardia with a prolonged QTc interval as compared to baseline at 3 days before (Fig. and Table ). One hour later, the calcium infusion was gradually reduced to 2.2 mmol/h to reduce the level of serum calcium without inducing sinus tachycardia. The ionized calcium decreased from 1.91 to 1.61 mmol/L with unaltered corrected serum calcium.\nThe third RCA-hemofiltration session began at a calcium infusion rate of 2.2 mmol/h, which resulted in a decrease in corrected serum calcium from 3.5 to 2.7 mmol/L and in ionized calcium from 1.91 to 1.18 mmol/L without arrhythmia (Fig. ).\nAfter the third RCA-hemofiltration session, high-sensitivity troponin T decreased to 89.8 ng/L and the patient was deemed sufficiently stable to undergo parathyroidectomy. An untypical parathyroid adenoma was pathologically confirmed. Postoperatively, PTH dropped to 16.9 ng/L and serum calcium dropped to 2.25 mmol/L. Serum calcium was 2.11 mmol/L and serum creatinine was 173 μmol/L at discharge. The patient received a telephone follow-up at 2 months after discharge and reported serum calcium of 2.13 mmol/L and serum creatinine of 112 μmol/L without calcium supplementation.","This 51-year-old man was admitted to the hospital because of high calcium levels and kidney problems. He was found to have a small, non-cancerous growth on his parathyroid gland causing his calcium levels to be too high. Initially, doctors treated him with fluids and medications to lower his calcium, but he also experienced some heart problems during the process. After a series of dialysis treatments and a parathyroidectomy, his calcium levels and kidney function improved, and he was discharged home with a plan for continued monitoring."
419,"A 42 year old male presented to his primary care physician with a 20 pound unintentional weight loss over a 3 month period and new left axillary lymphadenopathy. A core biopsy of his axillary mass revealed metastatic malignant melanoma. He had no prior history of a primary melanoma. A staging PET/CT revealed abnormal FDG uptake in his left axilla and small bowel. A left axillary lymph node dissection was performed and revealed 2 of 19 lymph nodes involved with metastatic melanoma, BRAF wild type, the largest of which measured 10.1 cm. His medical history was significant for oligodendroglioma, which was surgically resected eight years prior to presentation, followed by radiation therapy for recurrence five years prior to presentation. He received four doses of systemic ipilimumab for his metastatic melanoma without incident. Post-treatment imaging revealed disease progression with new diffuse subcutaneous, lung, liver and bilateral axillary lymph node metastases.\nHe subsequently enrolled on a genetically engineered T-cell trial, targeting tyrosinase. He received fludarabine and cyclophosphamide as a conditioning regimen, then his engineered T-cells were infused, followed by one week of low dose IL-2, (72,000 U/kg IV q8 hours). Per the treatment protocol, unfractionated creatinine kinase (CK) levels were obtained just prior to and for two weeks after infusion of the genetically engineered T-cells. The CK levels were within normal limits during the course of this treatment. He initially experienced disease response, however, four months after his T-cell therapy, he again developed diffuse progression with new hilar lymphadenopathy and progression of his lung and axillary lymph node metastases.\nHe was next treated with three doses of pembrolizumab but post-treatment imaging again revealed disease progression in his lungs. CK levels were not checked during treatment with either ipilumumab or pembrolizumab. Despite multiple lines of therapy, the patient continued to have an excellent performance status, so he thus proceeded to treatment with HD IL-2 (600,000 IU/kg IV over 15 min every 8 h day 1–5 and day 15–19), which began nine months after receiving his engineered T-cell infusion.\nDuring cycle one of course 1 (day 1–5) of HD IL2, he received 10 out of 14 possible doses and experienced the expected adverse effects of hypotension, sinus tachycardia, oliguria, metabolic acidosis, and acute kidney injury. Serum CK was monitored per protocol and was initially normal but peaked at 641 (50–320 IU/L) during the fourth day of treatment without associated symptoms or cardiac findings on EKG.\nHe had an uncomplicated recovery and was re-admitted to the hospital for cycle 2 of course 1 (day 15–19) of HD IL-2, without complaints and a normal serum CK level of 133 (50–320 IU/L). After 6 doses of HD-IL2, he began to experience diffuse myalgias and rigors. He was noted to have a rapid rise in CK to 2700 and increase in his serum creatinine from 2.5 to 4.4 (0.6–1.4 mg/dL). An EKG revealed sinus tachycardia and his serum troponin level was normal at 0.02 (0.00–0.04 ng/mL). The rise in CK was attributed to rigors and he was continued on therapy. He went on to receive 2 additional doses of HD IL-2. When his CK rose further to 3900 and the myalgias became more severe despite resolution of his rigors, subsequent doses were held.\nFurther investigation revealed an elevated serum aldolase of 32.7 (1.2–7.6 U/L), and elevated urine myoglobin of 132 (< 28 mcg/L). MB fractionation of CK was not performed. Urinalysis demonstrated large blood without red blood cells. Serial EKGs demonstrated sinus tachycardia but not sequelae of hyperkalemia, such as peaked T waves. Other labs for serologic autoimmunity, e.g. anti-nuclear antibody, anti-double stranded DNA anti-striated muscle antibody and anti-smooth muscle antibody, were not checked. Muscle function was not assessed with electromyography (EMG). He received supportive care and aggressive intravenous hydration with normal saline. He recovered fully from this episode of rhabdomyolysis.\nSubsequent staging with a PET/CT revealed a mixed response in his pulmonary nodules with mild improvement in his hilar and axillary lymphadenopathy. Given his full recovery from previous toxicity and the mixed response on imaging, the decision was made to proceed with a second course of HD-IL2 therapy with close monitoring of CK levels and a low threshold for discontinuation of therapy.\nHe received only two doses of HD IL-2 and again developed diffuse myalgias with a rapid rise in his serum CK level from 184 to 1680. Serum aldolase and urine myoglobin were again significantly elevated, at 16.2 and 3430, respectively. All further doses of HD IL-2 were held and he was again supported with aggressive intravenous hydration. His clinical symptoms resolved and his CK level trended down to the normal range.\nDue to this unusual toxicity, a muscle biopsy was performed to further evaluate for rhabdomyolysis and to ascertain if his engineered T-cells were present in his muscle tissue. The biopsy revealed rare myofiber necrosis and myophagocytosis and scant endomysial infiltrate. The infiltrate consisted of a mixture of CD3 and CD4 positive T-lymphocytes, CD68 positive macrophages and lesser numbers of CD8 positive T-lymphocytes, suggestive of an immune-mediated toxicity causing necrotizing myopathy (Fig. ). It was difficult to ascertain if the T-lymphocyte present represented his engineered T-cells. Of note, the engineered T-cells remained detectable in the circulation at this time. He was discharged home and follow up PET/CT imaging revealed a near complete response. At the time of this follow up, he was found to have new onset vitiligo involving his neck, upper back, chest, and upper arms (Fig. ). A skin biopsy at the edge of his neck vitiligo again revealed CD3+ T-cell infiltration (Fig. ).\nNo further HD-IL2 therapy has been administered and he continues to experience a durable response on imaging nearly two and a half years since completion of his HD IL-2 therapy. Of note, CK levels had not been checked when the patient had received prior therapy with ipilumumab or pembrolizumab. CK levels were, however, monitored at the time of treatment with engineered T-cells and were noted to be within the reference range.","Mr. Jones has been receiving several different treatments for his advanced melanoma, including immunotherapy drugs and a new type of T-cell therapy. Unfortunately, he has experienced some side effects, particularly muscle damage (rhabdomyolysis), which caused a significant rise in a blood test called CK. Despite these challenges, he has shown a good response to treatment and is currently stable. Recently, he developed vitiligo, a skin condition that causes patches of hairlessness, and his doctors are continuing to monitor his progress closely."
420,"A 32-year-old healthy pregnant woman was referred to the Medical Genetic Centre of Ganzhou Maternal and Child Health Care Hospital. The pregnant woman was 160 cm tall and weighed 53 kg with normal hallmark developmental milestones. She delivered a boy 10 years ago. During the second trimester maternal serum screening in another hospital, she was notified that the foetus had an increased risk of developing T18 syndrome. The study participant was at 16 weeks’ gestation. As is routine practice, an ultrasound was conducted to monitor the developmental status of the foetus. The ultrasound examination at a gestational age of 16 weeks showed that the foetus was normal. To avoid invasive prenatal diagnosis procedures, an NIPT was offered to further screen for common foetal chromosomal abnormalities. This project was approved by the Research Ethics Committee of Ganzhou Maternal and Child Health Care Hospital. Written informed consent was obtained from all of the participants or guardians that participated in this research.","This woman is 32 years old and currently pregnant. She was referred to a genetic specialist because a previous screening test suggested a possible risk of a chromosomal condition in her baby. However, a recent ultrasound showed the baby is developing normally. To get more information, the doctor offered a non-invasive test called NIPT, which is now being used to screen for common problems. All the necessary steps have been taken to ensure the safety and well-being of both the mother and the baby."
421,"A 56-year-old Japanese male was referred to our hospital with dyspnea and hypotension. He had a history of Parkinson’s disease with psychosis. He complained of left-sided chest pain and productive cough in the 2 months before admission. He reported no risk factors for HIV infection, occasional alcohol consumption, and was a current smoker with a 30-pack/year smoking history. Initial vital signs were as follows: blood pressure, 77/56 mmHg; heart rate, 106/min; body temperature, 36.1 °C (97.0 °F). There was no lymphadenopathy or hepatosplenomegaly. Examination of the oral cavity revealed poor dentition and inadequate hygiene. Jugular venous pressure was elevated to the angle of the jaw at 45° and did not change with respirations. The breath sounds attenuated at both sides, and dullness was present at the base of the bilateral lung. Cardiovascular examination revealed regular rhythm, tachycardia, and distant heart sounds. Laboratory test values were as follows: white blood cells, 29,950/mm3 with a left shift; hemoglobin, 10.6 g/dl; platelets, 453,000/mm3; random serum glucose, 125 mg/dl (normal, 75–115 mg/dl); serum lactate dehydrogenase (LDH), 260 U/l (normal,119–229 U/l); serum aspartate and alanine aminotransferase (AST and ALT), 80 U/l and 24 U/l (normal, 0–35 U/l); serum albumin, 3.1 g/dl (normal, 4–5 g/dl); serum C-reactive protein (CRP), 17.25 mg/dl (normal, < 0.2 mg/dl). The chest radiograph (Fig. ) revealed a mass in the left upper lung, bilateral pleural effusions and cardiac enlargement. The chest computed tomography (CT) scan showed a wedge-shaped and pleural-based mass in the left upper lobe (LUL), a thick-walled cavitary lesion containing only water density in the left lower lobe (LLL), bilateral pleural effusions and pericardial effusion (Fig. ). An electrocardiogram was normal. The patient received acute pericardiocentesis and pericardial drainage tube placement, which yielded approximately 800 ml of dark yellow fluid and restored blood pressure. Thoracentesis on both sides was performed and revealed yellow and turbid pleural fluid. Gram stain and cytologic examination of both pericardial and pleural fluid showed no organisms and also malignant cells. Examination of sputum showed no pathogen on staining. Two sets of blood culture specimens drawn at the time of admission did not yield any organisms. The patient was initially diagnosed with a bacterial pericarditis and lung abscess in the LLL. Intravenous ceftriaxone was started as an empirical antimicrobial treatment.\nAfter the admission, the patient continued to be afebrile and his respiratory and cardiovascular status was stable. On day 6, there was minimal pericardial fluid in the drainage tube so the drain was removed. In the morning of day 9, a fever of 39.0 °C (102.2 °F) and sinus tachycardia (150/min) occurred. The tachycardia continued for 4 h, and he experienced atrial fibrillation (Af). Immediately after the onset of Af, a cardiac arrest with asystole occurred. He underwent an immediate cardiopulmonary resuscitation, which restored sinus rhythm and blood pressure. Chest CT scan obtained on the same day revealed the remaining thick-walled cavitary lesion in the LLL and the reduced pericardial effusion. After the cardiopulmonary state was stabilized, he underwent ultrasound-guided pneumocentesis of the cavitary lesion in the LLL and 35 ml of purulent fluid was obtained (Fig. ). Gram stain of the fluid revealed Gram-positive filamentous rods, and cultures of the fluid grew Actinomyces species (Fig. ). We analyzed the fluid using a method for clone library sequencing of the 16S ribosomal DNA (rDNA) gene and Actinomyces meyeri along with other anaerobes (Fusobacterium species) were detected []. Transbronchial biopsy and bronchial washings of the mass lesion in both the LUL and LLL were performed. The biopsy revealed non-specific inflammation and organization of the lung tissue with no bacteria. On day 11, antibiotics were changed to intravenous penicillin, and his condition continued to be stable. After 4 weeks of intravenous penicillin therapy, antibiotics were switched to oral doxycycline therapy and he was discharged. Echocardiogram before the discharge showed no evidence of pericardial effusion or constrictive physiology. The patient completed the total six-month antibiotic therapy. At follow up, 6 months after discharge, the patient was gaining weight, felt well, and his CT images had continued to show improvement (Fig. ).","This 56-year-old man was admitted to the hospital because he was having trouble breathing and his blood pressure was very low. He has Parkinson’s disease and has a history of smoking, which contributed to his symptoms. Tests revealed a lung infection and fluid around his heart, and he needed a procedure to drain the fluid. After treatment, his condition improved, but he then developed a fever and heart problems, leading to a cardiac arrest. Fortunately, he was revived, and further tests revealed the infection was caused by a bacteria called Actinomyces. After completing a course of antibiotics, he was discharged and is now feeling better."
422,"Nivolumab was initiated to treat a 56-year-old woman, with unresectable NSCLC who initially received an adjuvant chemotherapy consisting of 4 cycles of cisplatin-pemetrexed. New lesions appeared and were classified pT3N0M0. Nivolumab therapy was then initiated. Figure summarizes the key clinical and immunological data of the index case as well as the timeline of immune checkpoint analysis.\nNo sign of SLR was detectable before the treatment with nivolumab (Fig. and ). A partial response was observed after 5 infusions of nivolumab as suggested by CT scan (Fig. and ). Skin lesions appeared after 5 nivolumab infusions (Fig. ), then mediastinal nodes size started to increase and a micronodular interstitial syndrome was observed (Fig. and ) after 20 nivolumab infusions. Biopsies showed epithelioid cells and Langhans multinucleated giant cells without necrosis, microorganisms or refringent bodies, compatible with sarcoid-like inflammation (Fig. ). Tumor cells, alcohol-acid resistant bacilli and fungus or parasite were not detected (data not shown). Nivolumab was then discontinued. According to RECIST criteria, the patient had a partial response at this time.\nMediastinoscopy revealed sarcoid lesions. Mediastinal nodes sizes (Fig. ) and skin lesions were decreased 155 days later (data not shown), while the interstitial syndrome had deteriorated (Fig. ). Bronchoalveolar lavage showed hypercellularity comprising 41% of lymphocytes without pathogens or cancer cells (data not shown). 348 days later, CT scan showed normal mediastinal nodes and regression of the interstitial syndrome (Fig. ).\nThe expression of PD-1, PD-L1 and PD-L2 was analyzed on PBMC at various time-points after stopping nivolumab (defined as day 0). An important increase of PD-L1 expression was observed on B and T cells at d56 with a peak at d147, compared to other patients treated with nivolumab without relevant immune related reactions (Fig. ). An elevated expression of PD-L1 was observed on monocytes at d147 (Fig. ). PD-L1 expression by PBMC of the index case returned to basal levels at d251 (Fig. ). If we hypothesize that the increase of PD-L1 expression was consecutive to a rebound effect after stopping the treatment, we would have observed a similar increase in patients treated with nivolumab that did not exhibit immune related events. Moreover, expression of PD-1 on T cells was punctually undetectable at d147 at a time when PD-L1 expression was maximal (Fig. ). No marked change of PD-L2 expression was observed. Intriguingly, the increased PD-L1 expression was evidenced only from day 56 to day 147 after nivolumab arrest (Fig. ). This observation suggests that an elevated expression of PD-L1 consecutive to blocking PD-1/PD-L1 interaction can be associated with sarcoid-like reaction (SLR).","A 56-year-old woman with lung cancer received an initial chemotherapy treatment and then started on nivolumab, an immunotherapy drug. Initially, the treatment showed a positive response, with a reduction in tumor size. However, as the treatment continued, she developed a related inflammatory reaction resembling sarcoidosis, which affected her skin and lungs. Eventually, the inflammation subsided, and her condition improved, with the tumor size decreasing further, and the PD-L1 expression in her blood returning to normal levels."
423,"A 56-year-old lady presented with a 6 month history of rectal bleeding, passage of mucus and a change in bowel habit to more frequent stools. She had no abdominal pains and her weight was maintained. The patient had been born with bladder exstrophy and had multiple surgeries culminating in a cystectomy with ileal conduit formation at 5 years of age. She had suffered with recurrent urinary tract infections for most of her childhood and adult life but was otherwise well with no other major co-morbidities or risk factors for colorectal malignancy and had no family history of colorectal disease.\nColonoscopy revealed two large sessile polyps in the sigmoid colon in close proximity to each other and adjacent to a diverticulum-like structure (Fig. ). Each polyp was approximately 3 cm in size and both exhibited a type IV pit pattern with areas of irregularity suggestive of focally advanced disease. Histological examination confirmed both polyps were adenomas comprising both low and high grade dysplasia, without submucosal invasion (Fig. ). On closer inspection the mucosa around the diverticulum was also atypical but not adenomatous. The remainder of the colonoscopy was unremarkable with no other evidence of diverticular disease or polyps elsewhere.\nEndoscopic resection was considered as a therapeutic option however in view of the above characteristics as well as difficult endoscopic access surgery was preferred. Furthermore, radiological imaging had initially raised the possibility of invasive disease in view of sigmoid thickening. The patient underwent high anterior resection and an open approach was chosen because of suspected intra-abdominal adhesions following extensive pelvic surgery. An end colostomy was formed at the patient’s pre-operative request. At laparotomy the right fallopian tube was adherent to the sigmoid colon and adjacent to this a blind ending tube was noted to emerge from the anti-mesenteric border of the colon. This was marked for pathological identification.\nOur patient went on to have an uneventful recovery and her quality of life following surgery was good. Her wish to have a permanent colostomy stemmed from the fact that she had always suffered from an erratic bowel habit and that she was already knowledgeable with regards to stoma care in view of her pre-existing ileal conduit.","A 56-year-old woman came to the doctor because she had been experiencing rectal bleeding, mucus discharge, and changes in her bowel habits for six months. She has a history of bladder surgery from when she was a young child and has had frequent urinary infections. During a colonoscopy, doctors found two large polyps in her colon that were concerning and required surgical removal. After surgery, a colostomy was created as she had always struggled with her bowel movements and was familiar with stoma care."
424,"We report a case of a 23-year-old female who presented with a 4-month history of fluctuating weakness of the right upper and lower extremities associated with a band-like sensation of the trunk along with bowel and bladder disturbances. There was no consult done. One month prior to her admission, she noted electric-like sensation on her nape, which was worsened by neck movement. The weakness eventually progressed to the left lower extremity causing difficulty in ambulation. The patient eventually became quadriparetic and needed to be assisted in all activities of daily living. She also developed dysphonia, dysphagia, difficulty of breathing and projectile vomiting that prompted consult to our institution.\nThe patient was hemodynamically stable upon admission. There were no spinal deformities noted. The sphincter tone was lax. Higher cortical function was normal. On cranial nerve examination, there was weak gag and shoulder shrug bilaterally. Manual motor testing revealed 2–3/5 on both upper extremities and left lower extremity and strength of 4/5 on the right lower extremity. Hypesthesia was also described at the C4 dermatomal level and dissociated sensory loss was also noted. The patient was hyper-reflexic on both the upper and lower extremities along with bilateral extensor toe sign. The patient's neck was paratonic.\nInitial work-up included a cranio-spinal magnetic resonance imaging (MRI) revealing a long expansile contrast-enhancing mass involving the entire spinal cord, from the level of the cervical region to the conus medullaris with extension to the lower medulla oblongata (A–D). The patient was started on dexamethasone, which provided minimal relief of symptoms. She was also referred to the neurosurgery service. The patient was advised surgery, however, on the 20th hospital day, the patient's symptoms progressed to quadriplegia and respiratory failure. She subsequently developed healthcare-associated pneumonia succumbing to septicemia.\nOn autopsy, all lobes of the lungs were consolidated secondary to pneumonia (A & B). There was swelling of the entire spinal cord with white to yellow mucoid material coming out from the central canal (C). Cut sections showed that the entire spinal cord and caudal medullary region were replaced by mucoid material (D). The rest of the examination was unremarkable. Histopathologic examination of the spinal cord revealed a cellular tumor arranged around the central canal of the cord. The cells are generally small, with round to slightly ovoid nuclei. The chromatin materials are generally vesicular with occasional clumping seen. Fibrillary processes were noted in the cytoplasm (E & F). Immunohistochemical studies were done. S100 showed staining of the cytoplasmic globules with weak staining of intact cytoplasms. GFAP, synaptophysin, κ, λ and leucocyte common antigen had negative results. Further genetic characterization of the tumor such as IDH, 1p/19q codeletion, MGMT, EGFR and TP53 mutations was not done because of its unavailability in the institution but its histologic and immunochemical features are consistent with holocord oligodendroglioma.","This patient presented with a gradual worsening of weakness in her arms and legs, along with problems with her bladder and bowels, over a period of four months. She also experienced unusual electrical sensations in her neck and eventually became unable to walk without assistance. Further tests revealed a large tumor pressing on her spinal cord, which caused her symptoms to rapidly worsen, leading to difficulty breathing and pneumonia. Sadly, despite treatment, the patient’s condition deteriorated, and she passed away."
425,"A 28-year-old female was referred to our dermatology clinic because of persistent swelling of the earlobes several months after ear piercing. Clinical examination showed symmetrical nodes on both earlobes (Figure ). Histology showed epithelioid granulomas with a lymphocytic infiltrate, as seen in sarcoidosis and foreign body reactions. There were no further signs of sarcoidosis (normal chest X-ray and normal angiotensin-converting enzyme findings) or foreign material. Patch testing with the European baseline series and a dental series (including various metals) was performed. Positive reactions to nickel sulfate 5% pet. [+ on day (D) 2 and D3] and palladium chloride 1% pet. (+ on D3) were observed. Four weeks after the patch test, a persistent reaction on the patient's back remained at the palladium test site. A biopsy showed epitheloid granulomas similar to those previously seen in the excised nodes.\nInductively coupled plasma mass spectrometry (ICP-MS) (ICP-quadrupole-MS, Varian 810-MS) detected palladium (105Pd) in all of our skin samples (thickness, 4 μm) and showed a 3-fold higher signal than that from skin samples of control patients. The content of 105Pd in each sample was semiquantitatively determined to be 0.6 ng of 105Pd per sample.","This 28-year-old woman has been experiencing swelling in her earlobes for several months after getting pierced. Tests revealed small, inflamed bumps on her ears, and a biopsy showed signs of a reaction to a foreign material. Patch testing identified nickel and palladium as the likely cause of the reaction. Further testing confirmed a high level of palladium in her skin, suggesting that the jewelry was the source of the problem."
426,"A 65-year-old healthy Japanese woman presented with a chief complaint of malaise and hematochezia. The physical examination revealed a 10-cm-diameter hard mass at the right lower quadrant of the abdomen without tenderness or cutaneous involvement. Laboratory findings demonstrated severe anemia and elevated tumor markers (hemoglobin, 2.9 mg/dl; carcinoembryonic antigen, 10.8 ng/ml; carbohydrate antigen 19-9, 21.6 U/ml). A colonoscopy showed a circumferential neoplastic lesion at the ascending colon which did not allow the scope to pass through (Fig. a). The biopsy reported a moderately differentiated tubular adenocarcinoma (Fig. b). RAS mutation was not detected. A CT showed an 8.6-cm-diameter tumor at the ascending colon which seemed to infiltrate the abdominal wall, small intestine, and retroperitoneum (Fig. a). Regional lymphadenopathies and ascites were also observed, but apparent distant metastases were not. Based on these findings, we made a clinical diagnosis of locally advanced ascending colon cancer stages T4b, N2a, M0, and IIIC according to the TNM classification []. Considering the possible extensive invasion to the surrounding structures, we recommended initial neoadjuvant chemotherapy followed by radical resection of the tumor.\nThe patient underwent 4 cycles of modified FOLFOX6 with panitumumab, and the tumor shrank only slightly to 6.9 cm in diameter (Fig. b). An additional 4 cycles of the same regimen were administered but the tumor actually enlarged to 10 cm in diameter (Fig. c), and thus, the disease was determined to be a progressive disease according to the Response Evaluation Criteria in Solid Tumors []. At this point, we felt more chemotherapies and/or surgeries were not recommended because of the evidence of disease progression and because the patient’s condition was quite frail. Instead, we recommended radiotherapy of 60 Gy in 30 fractions (Fig. ). The patient tolerated the treatments well, and no serious adverse effects were reported. After the radiotherapy, the tumor shrank to 6.6 cm in diameter with intratumor liquefactive degeneration (Fig. d). One month after the radiotherapy, she underwent right hemicolectomy with D3 lymphadenectomy. Metastatic lesions of the liver or peritoneum were not observed. The right ovarian vessels and ileum at 10 cm proximal from the ileocecal valve were infiltrated by the tumor and resected concomitantly. The adjacent abdominal wall was intact. The transverse colon and ileum at 30 cm proximal from the ileocecal valve were cut and anastomosed. The macroscopic exam of the resected specimen showed a 9.0 × 7.0-cm circumferential tumor with a 4.5 × 3.5-cm ulcer at the ascending colon, which extensively penetrated the colon serosa and infiltrated the ileum and the ovarian vessels (Fig. a). Histopathologically, the primary tumor of the ascending colon and enlarged regional lymph nodes consisted in its totality of granuloma-like or fibrous tissues and no residual cancer cells were found (Fig. b). Pathological findings revealed complete response, and the final findings were ypT0, ypN0 (0/15), and ypStage0. Neoadjuvant treatment effect was grade 0 according to American Joint Committee on Cancer System []. The postoperative course was uneventful. The patient remains alive without any additional therapies after 24 months for follow-up, with no signs of recurrence.","A 65-year-old woman came to the hospital because she was feeling unwell and had blood in her stool. Tests revealed she had a large, growing tumor in her colon that was spreading to nearby tissues and lymph nodes. After trying chemotherapy, the tumor didn't respond and actually grew larger, so doctors decided to focus on radiation therapy instead. Following radiation, the tumor shrank significantly, and she underwent surgery to remove it completely, with no signs of cancer remaining after 24 months of follow-up."
427,"A 62-year-old man with chronic hepatitis C was referred by his physician to our hospital for surgical treatment of hepatocellular carcinoma. Imaging findings on enhanced computed tomography (CT) and dynamic magnetic resonance imaging (MRI) revealed two tumors located in segments 5 and 8, respectively (Figs. and ). Although the alpha-fetoprotein level was within the reference range, the serum level of prothrombin induced by the absence of vitamin K or antagonist-II was high (530 mAU/ml). After a preoperative evaluation based on a three-dimensional (3D) imaging study, anterior segmentectomy was performed. Each branch of the hepatic artery, portal vein, and biliary duct for the anterior segment were ligated together as the Glissonean bundle (so-called, fully simultaneous transection of the Glissonean pedicle [FSTG]) (Fig. ). Perihilar FSTG involved a transfixation suture by using an absorbable thread. The tumor in segment 8 was in contact with the middle hepatic vein. However, this tumor was well-encapsulated, and the tumor and vein were easily dissectable. The patient’s postoperative course was uneventful, and he was discharged on postoperative day 14.\nThree months later, dynamic MRI was performed to check for intrahepatic recurrence, and no imaging findings of recurrence were observed. However, an arterioportal fistula and portal vein aneurysm were incidentally detected (Fig. ). Layers of old and subacute hematomas were clearly observed, and these layers surrounded the aneurysm. Surprisingly, the patient did not have subtle symptoms and showed no episodes of pain, ascites, liver dysfunction, or other abnormalities. We suspected a pseudoaneurysm at that time. Although a perfect angiographic evaluation could not be ensured, IVR was needed to avoid sudden rupture and possible death. Therefore, we decided to attempt IVR after evaluation of the vessels on dynamic CT, and transcatheter arterial embolization was proposed thereafter.\nFirst, angiography via the celiac artery was performed. Arteriography was subsequently used to create a portogram via this APF, and portography clearly revealed hepatofugal flow of the portal vein. Portography also showed that the stump of the anterior portal vein had developed a portal vein aneurysm (PVA) with a diameter of 40 mm (Fig. a). Selective catheterization of the common hepatic artery was then performed. This arteriography clearly demonstrated a fistula between the hepatic artery and portal vein (i.e., APF) at the stump of the anterior branches (Fig. b). Based on these angiography findings, we definitively diagnosed PVA due to an APF, not a pseudoaneurysm.\nNext, an adequate length of APF to perform embolic therapy was confirmed to avoid any occlusion and disturbance at the bifurcation of the right hepatic artery (Fig. b). Selective embolization of the anterior hepatic artery was then accomplished by placing several titanium coils in the whole length of the stump of the anterior hepatic artery. Finally, the flow of blood through the APF was drastically reduced (Fig. c). Arteriography via the superior mesenteric artery showed a remarkable restoration of portal venous flow, and hepatopetal portal flow was clearly observed (Fig. d).\nComplete closure of the APF could be estimated by additional expansion of the metallic coils over time after IVR. Dynamic CT and 3D images 3 days after embolization clearly demonstrated perfect interruption of the APF and disappearance of the PVA (Fig. ).\nImaging studies and serum biomarkers showed no evidence of recurrence. At the time of this study, the patient was good in health and had been reintegrated into society.","This 62-year-old man with hepatitis C was admitted to the hospital for surgery to remove two tumors in his liver. During the surgery, doctors carefully disconnected the blood vessels around the tumors. After the surgery, they found a problem with a connection between the hepatic artery and the portal vein, which they fixed with a procedure called transcatheter arterial embolization. The patient’s recovery was smooth, and follow-up scans showed no signs of the tumors returning, and the blood vessel problem was successfully treated."
428,"Case 13 concerns a 25-year-old man who was found dead in his flat. The autopsy revealed that the immediate cause of death was acute cardiac failure in the mechanism of arrhythmias due to chronic focal lesions in the myocardium. Moreover, according to the forensic pathologist, considering the pathomechanism of his death, the acute cardiac failure does not contradict the possibility of an unknown substance being co-responsible for the intoxication. This is suggested by the presence of gastric contents in his respiratory tract as a result of vomiting. Therefore, this case may be considered death resulting from a mixed causes of myocardium lesions with 4-CMC and amphetamine intoxication, as the concentrations determined in his blood (394 and 2200 ng/mL, respectively) are relatively high, and the detected concentration of amphetamine can be fatal to a nonaddict [].","A 25-year-old man died unexpectedly at his home. The autopsy showed his heart suddenly stopped working due to damage to his heart muscle. It’s possible that an unknown substance, along with amphetamines and 4-CMC, contributed to his death, as these drugs were found in his system at high levels. The autopsy revealed he had been vomiting, which caused these substances to enter his lungs. Further investigation is needed to determine the exact cause of his death."
429,"Case 14 concerns a 38-year-old man who was found dead in his flat. During autopsy, blood and a fragment of abdominal wall containing a pill were collected for toxicological tests. Analyses showed that the pill contained disulfiram, although it was not detected in the blood. Ethyl alcohol was not detected either. The autopsy did not reveal any bodily injuries. However, signs of sudden death were found, including recent passive congestion of internal organs, presence of liquid blood in the heart and great vessels, and intensive livor mortis. Moreover, the forensic pathologist indicated the action of a psychoactive substance on the body probably as the primary cause of his death. Therefore, it is highly probable that the death resulted from an overdose of 4-CMC only, because only nordazepam at therapeutic concentration was additionally determined.","A 38-year-old man was found dead in his apartment. Autopsy revealed a pill containing disulfiram in his stomach, but the drug wasn't found in his blood. The medical examiner believes he likely died from an overdose of a different drug, 4-CMC, possibly combined with a small amount of nordazepam. There were signs of a sudden death, including congestion in his organs and discoloration of his skin."
430,"A 65-year-old man was admitted to our department for a routine CABG due to left main coronary artery disease. The patient’s medical history included smoking, family history of early coronary artery disease, hypertension, diabetes, hyperlipidaemia, percutaneous transluminal coronary angioplasty to the left descending artery (LAD) and to the right coronary artery (RCA) 12 years earlier, and myocardial infarction 11 years earlier due to in-stent stenosis. In his past medical history, there was an unclear history of sensory or motor impairment after coccyx cyst surgery.\nAll laboratory data were within normal limits except for the erythrocyte sedimentation rate (521st, 1 132nd) and a C-reactive protein (CRP) > 2 mg/l. Echocardiographic findings were left ventricular ejection fraction (LVEF) of 45% and mild left ventricular hypertrophy. Coronary artery CT-angiography was performed and stenosis of three coronary arteries was established.\nThe induction of anaesthesia was performed with Dormicum 5 mg, Propofol 150 mg, Esmeron 60 mg and Sevoflurane. The patient underwent triple coronary artery bypass grafting as follows: left internal mammary to left anterior descending artery (LIMA–LAD), a saphenous vein graft to the first obtuse marginalis (SVG–OM1) and another saphenous vein graft to the right coronary artery (SVG–RCA). During surgery his vital signs were stable and the arterial blood gasses (ABGs) were within normal limits.\nAfter surgery the patient was moved to the cardiac ICU while intubated and unconscious, with a blood pressure of 110/60 mmHg, heart rate of 77 beats/min and normal sinus rhythm, central venous pressure of 8 cm H2O and peripheral capillary oxygen saturation of 100%. After admission to the ICU, his primary vital signs were normal. The patient was successfully weaned and extubated on the same day. The post-surgery drugs were: enoxaparin 40 mg daily, furosemide 20 mg daily, metoprolol 100 mg twice daily, clopidogrel 75 mg daily, atorvastatin 20 mg daily and acetylsalicylic acid 100 mg daily.\nOn the first postoperative day, laboratory findings in the ICU were: haemoglobin 10 g/dl, haematocrit 30.5%, platelets = 242 000 cells/l, white blood cell count = 9 100 cells/l, prothrombin time = 15.3 sec, activated partial thromboplastin time = 32 sec, INR = 1.47, sodium = 139 mEq/l, potassium = 4.9 mmol/l, blood urea nitrogen (BUN) = 17 mg/dl, creatinine = 0.94 mg/dl, creatine phosphokinase (CK) = 847 U/l, CK-MB = 58 U/l. He was moved to the cardiac surgery department. During this first postoperative day, the patient was stable, awake and oriented. No signs of haemodynamic instability or cardiac dysrhythmias were seen.\nThe second day after CABG, the overall condition of the patient was good but he had difficulty moving his lower limbs. Neurological consultation was done and the cranial nerves were found to be intact, cerebellar tests and sensory examinations were normal, muscle strength of the lower limbs was 3/5 and symmetric and plantar reflexes were double flexor.\nOn the third postoperative day, the overall condition of the patient was good but he still had difficulty moving the lower limbs. Progressive paraparesis developed and the muscle strength and deep tendon reflexes (DTRs) began to decrease gradually. Paraparesis progressed and muscle strength decreased from 4/5 to 3/5 and then to 2/5. In the evening, severe weakness of the lower and upper limbs developed, absence of DTRs, no plantar reflexes, and muscle strength was 1/5 on the left and 0/5 on the right side. That night the patient presented with respiratory failure; he was intubated and moved to the ICU.\nOn the fourth day, the patient was haemodynamically stable and he was transferred to the radiology laboratory in order to undergo magnetic resonance imaging (MRI). During the MRI examination, the patient experienced an episode of ventricular fibrillation and cardiac arrest. He was resuscitated after 20 minutes of cardiopulmonary resuscitation and two defibrillations. He was in haemodynamic instability and received high doses of dobutamine, norepinephrine and adrenaline.\nThe laboratory findings were: creatinine = 3.0 mg/dl, urea 111 mg/dl, aspartate transaminase (AST) 1 000 U/l, alanine transaminase (ALT) 6182, LDH 9 119 U/l, CK 3 915 U/l, CK-MB 315 U/l, troponin 10 000 ng/ml. The echocardiogram findings were left atrium 39 mm, telo diastolic volume of the left ventricle 50 mm, the left ventricle showed diffuse hypokinesis and akinesis, with a LVEF of 25%, and pulmonary artery systolic pressure was 40–45 mmHg.\nThe MRI report showed at the level of the fifth and sixth cervical vertebrae that there were posterior osteophytes and circular degeneration of the annulus fibrosis with high-grade stenosis and compressive phenomena in the spinal cord. From the second to the sixth cervical vertebrae, there was a pathological zone and oedema due to myelopathy and ischaemia ().\nOn the sixth day after surgery, the patient was better and was haemodynamically supported with low-dose norepinephrine. However he presented manifestations of post-cardiac arrest brain injury such as coma, seizures and myoclonus. He died 10 days after surgery due to septic shock.","This 65-year-old man had heart surgery to fix a blocked artery in his heart. He had a history of smoking, heart problems, and diabetes, and had previously had some artery work done. During the surgery, his heart function was closely monitored, and he was able to be weaned off a breathing machine and go home. However, after the surgery, he developed weakness in his legs and difficulty breathing, which led to a cardiac arrest and ultimately his death."
431,"A 53-year-old sub-Saharan African man with poorly controlled hypertension was referred to the cardiac intensive care unit (CICU) by his cardiologist for the management of a sudden-onset, severe and intractable retrosternal chest pain of approximately 50 hours’ duration. The pain was tearing in character, radiating to the back and lumbar regions, non-positional and associated with shortness of breath and headache. 50 hours’ duration. The pain was tearing in character, radiating to the back and lumbar regions, non-positional and associated with shortness of breath and headache.\nThe electrocardiogram (ECG), done three hours after the onset of pain, showed sinus rhythm and non-specific repolarisation changes (flattened or inverted T waves in leads I, aVL and V3–V6). Although ECG changes were suggestive of left ventricular strain, the presence of chest pain and a mildly raised troponin level (0.11 μg/ml) favoured myocardial infarction, and the patient was started on low-molecular weight heparin (LMWH) at a therapeutic dose, aspirin and nitrates.\nPersistence of the pain after initial therapy prompted referral to our centre. On examination, he was anxious, dyspnoeic (NYHA functional class III with a respiratory rate of 28 breaths/ min) and diaphoretic. His temperature was 36.9°C, heart rate was 79 beats/min, and blood pressure was 187/73 mmHg in the right arm and 145/56 mmHg in the left arm. Physical examination showed a systolic murmur (grade 3/6) in the aortic area, which radiated to the left carotid, but there were no signs of heart failure. The neurological examination was unremarkable.\nChest X-ray () showed enlargement of the mediastinum with cuffing of the aortic knob. The ECG () at our unit showed a normal sinus rhythm, normal QRS axis with sub-epicardial ischaemia in the inferior and apico-lateral leads. Echocardiography () showed a dilated left atrium, good left ventricular systolic function (ejection fraction 72%), and severe aortic insufficiency with dilatation of the aortic root and ascending aorta (44 mm).\nContrast-enhanced CT (CECT) angiogram of the thorax () showed dissection of the aorta from the ascending aorta to the iliac arteries, including the coeliac trunk and left renal artery, and causing splenic infarction. Doppler ultrasound of the carotid arteries did not show extension to the carotid arteries. These observations led to a working diagnosis of Standford type A acute aortic dissection. shows biological investigations done at presentation and throughout hospitalisation.\nThe patient was placed on high-flow oxygen at 5 l/min, nicardipine in an electric syringe titrated to a maximum of 10 mg/h, bisoprolol 5 mg/12 h, analgesics and compressive stockings. The LMWH was stopped. On day five of hospitalisation, he developed superficial thrombophlebitis on the left forearm (along the peripheral intravenous line). By day six of hospitalisation, blood pressure and heart rate targets (< 120/80 mmHg and < 60 beats/min, respectively) were achieved.\nOn day 10 of hospitalisation, the patient developed a temperature of 39.1°C and sudden dyspnoea at rest. Physical examination showed a heart rate of 119 beats/min, blood pressure of 124/76 mmHg and oxygen saturation of 98%. Chest examination revealed crepitation in both lung bases, more marked on the right. We decided on a presumptive diagnosis of severe pneumonia. A repeat chest X-ray () showed bilateral interstitial heterogeneous opacities.\nThe C-reactive protein (CRP) level was 310.43 mg/l with leucocytosis of 17.7 × 106 cells/l (). Blood samples were collected for culture, and antibiotics (amoxicillin–clavulanic acid 1 g eight hourly and clarithromycin 1 000 mg 12 hourly) were introduced. Blood culture results (which returned after the patient’s demise) were positive for Klebsiella pneumonia. About three hours later he had persistent dyspnoea and hypoxaemia (SpO2 ≤ 65% and PaO2 ≤ 60 mmHg). He was intubated and during the process sustained a cardiac arrest. The patient later died on day 12 of hospitalisation following cardiopulmonary arrest despite life support.","This 53-year-old man was rushed to the hospital with severe chest pain that didn't go away, along with shortness of breath and a headache. Tests revealed a tear in his aorta, a serious condition called aortic dissection, and he was also suffering from pneumonia. Despite treatment with medications and supportive care, he unfortunately experienced a cardiac arrest and passed away."
432,"An 18-year-old female was admitted to Peking Union Medical College Hospital in November 2016 with the chief complaints of abnormal limb movements and slurred speech for two years, which worsened 20 days ago. Initially, her movements were slower, her hands were clumsy, and she could not speak clearly. In the past 20 days, she gradually developed dysdipsia, unsteady gait, dyskinesia, significantly increased involuntary movements of limbs and fell > 4 times. Cranial magnetic resonance imaging (MRI) showed abnormal signals in bilateral basal ganglia and thalamus. Electroencephalography (EEG) demonstrated diffused 4–6 Hz theta waves. Slit-lamp examination showed Kayser–Fleischer (KF) ring in both eyes. Her serum ceruloplasmin concentration was 0.033 g/L (Normal range: 0.2–0.6). The symptoms progressively worsened, and she had a fever, with temperature between 37.5 and 38.0 °C, without chills, cough or diarrhea. The patient had difficulty in opening her mouth, could only speak one word at a time, and had occasional torsion spasm at the time of admission.\nThe patient had xerostomia, keratoconjunctivitis sicca, frequent oral ulcers, with no significant weight loss. There was no history of other diseases, but her mother recalled that she talked less, had behavioral changes, abnormal gait, involuntary smile and involuntary movements of all limbs since five years. The parents and older sister did not have similar symptoms. Physical examination revealed that the patient had normal comprehension, with low-grade fever, hepatomegaly, splenomegaly, dystonia, lack of coordination, slight tremor, dysarthria, dysphagia and right side Babinski sign positive.\nAfter admission, routine tests revealed decreased white blood cell count of 2.87*109/L (Normal range: 4–10). Liver function test showed slightly elevated transaminase level and normal bilirubin level. Albumin level was decreased to 32 g/L (Normal range: > 35). Renal parameters were marginally elevated with proteinuria (1.12 g/24 h) and hematuria (++). Lumbar puncture showed elevated intracranial pressure, normal white blood cell count of 2/ul, elevated protein of 0.69 g/L and elevated immunoglobulin G (IgG) of 58.8 mg/L (Normal range: 0–40). Anti-AQP-4 (Anti-aquaporin 4) IgG and myelin basic protein were negative. MRI revealed symmetric abnormal signals with low signal in T1-weighted image, and high signals in T2-weighted and FLAIR images of bilateral basal ganglia thalamus, midbrain, and pons (Fig. ). Computed tomography (CT) scans revealed diffused lesions in the liver, uneven density, and hepatosplenomegaly. The patient, her parents and her sister underwent genotype test for WD, which showed that the patient had a compound heterozygous mutation, while her family members did not.\nOther laboratory findings included elevated ESR, C-reactive protein, IgG, IgM and hypocomplementemia. The titers of antinuclear antibody (ANA) (S1:640), anti-SSA antibody (+++) and anti-rRNP antibody (+++) were remarkably increased, while anticardiolipin antibodies (ACL), Lupus anticoagulants (LA), and anti-β2-glycoprotein-1 (anti-β2GP1) antibodies were all positive. Stomatological and ophthalmological evaluations provided objective evidence of salivary gland (salivary flow rate and parotid sialography) and ocular (Schirmer’s test and ocular dye score) involvement. Salivary gland biopsy showed typical histopathology of Sjögren syndrome. Magnetic resonance angiography (MRA) showed normal arteries, and ultrasound examinations of arteries and veins of bilateral legs, bilateral subclavian, supra-mesenteric, inferior-mesenteric, bilateral renal found no thrombotic evidence.\nBased on all the findings, the final diagnosis for this patient was WD, SLE, secondary Sjögren syndrome with anti-phospholipid (aPL) antibodies. Therefore, we started therapy with iv sodium dimercaptopropane sulfonate (DMPS), full dose zinc sulfate for WD, and methylprednisolone (80 mg iv for 7 days, then 40 mg po for 3 weeks), and hydroxychloroquine po for SLE, respectively. Since has three kinds of aPL antibodies were positive, the patient was also treated with anticoagulant therapy (low molecular weight heparin, and then aspirin po). One month later, her neurological symptoms and laboratory tests showed improvement. WBC count, liver function test including transaminase level, bilirubin level and serum albumin level, urine test, ESR, CRP, IgG and complement levels were all normal. Repeat lumbar puncture showed normal parameters. The titer of ANA had declined (S1:160), and aPL (ACL, LA, anti-β2GP1) antibodies were negative. The steroid dosage was gradually tapered and the patient was given oral dimercaptosuccinate (DMSA), zinc sulfate, hydroxychloroquine and aspirin for maintenance.\nThe patient was followed-up every three months at the outpatient clinic. Six months after discharge, her symptoms recurred, and she developed hyponatremia, hematuria and proteinuria, when the steroid was reduced to 2 mg/day. Hence, methylprednisolone dosage was increased to 16 mg/day, while the other treatment remained unchanged. Symptoms and abnormal laboratory findings were relieved in the next follow-up.","This 18-year-old woman was admitted because she’s been experiencing unusual movements, difficulty speaking, and a loss of coordination for about two years. Tests revealed she has a rare genetic condition called WD, and also has an autoimmune disease called SLE, which is causing problems with her glands that make saliva and tears. She also has antibodies that can cause blood clots, so she’s taking medication to prevent them. After starting treatment, her symptoms improved, but they have come back recently, so her doctors are adjusting her medication to manage her condition."
433,"A 30-year-old gravida 2 para 0 presented at 31 + 3 weeks gestation with sudden onset, unprovoked, epigastric and left sided pleuritic chest pain. This was associated with nausea, vomiting and shortness of breath. Her bowels had opened that day and she was passing flatus. She denied any uterine tightenings, urinary symptoms or vaginal loss and reported normal foetal movements.\nThe patient was an otherwise well South Asian woman with good social supports and no significant medical, surgical or family history. She did however, have a similar presentation at 13 weeks gestation and was diagnosed with left lower lobe pneumonia and a possible empyema on chest x-ray. (Fig. ) Bronchoscopy and washings at this time were negative and she was managed conservatively with intravenous antibiotics. Her pregnancy then progressed uneventfully.\nOn presentation, her observations were unremarkable with oxygen saturations at 100% on room air, a respiratory rate of 20 and a normal cardiotocograph (CTG). She was, however, in significant distress secondary to pain, despite opiate analgesia. Respiratory examination revealed decreased breath sounds on the left hand side and abdominal palpation showed left upper quadrant and epigastric tenderness with normal bowel sounds and no signs of peritonism. Routine biochemical investigations including a full blood count, biochemistry and lactate were unremarkable. A chest x-ray, however, revealed evidence of a raised or ruptured left hemi-diaphragm with bowel visible in the chest. (Fig. ) A subsequent CT chest confirmed the diagnosis of a large left diaphragmatic defect with stomach, small and large bowel, and spleen in the chest cavity. (Fig. ) There was no evidence of a gastric volvulus or bowel ischemia. On retrospective review of her previous chest x-ray at 13 weeks gestation, what was originally presumed to be an empyema likely represented a small diaphragmatic hernia. (Fig. ) On further questioning, the patient reported that she was asymptomatic prior to pregnancy and had no prior chest or abdominal imaging for comparison.\nThe patient received a course of steroids for foetal lung maturation and was transferred to our tertiary centre for consideration of urgent delivery and repair of the diaphragmatic defect. On arrival, the patient was found to be haemodynamically stable and her pain was now better controlled with regular doses of opiate analgesia. Given no immediate evidence of bowel obstruction, visceral ischaemia, respiratory compromise or concerns for foetal wellbeing were present, a decision was made, jointly by the surgical and obstetric teams, to conservatively manage the patient. Delivery and repair were planned ideally for after 34 weeks gestation, or in the event of maternal or foetal deterioration. Due to her inability to tolerate sufficient oral intake, a nasogastric tube was inserted and the patient commenced on nasogastric feeds on day five of admission with dietician input. In order to meet nutritional requirements of pregnancy, feeds were titrated from 10 ml/hr. with the aim to achieve 60 ml/hr. However, the patient was unable to tolerate the required feed volume, experiencing nausea, pain and increased nasogastric aspirates. Due to the inability to meet nutritional requirements and the possibility of a partial intestinal obstruction, a decision was subsequently made for an earlier delivery at 32 + 3 weeks gestation.\nWe performed a lower uterine segment caesarean section followed by a left thoracotomy on day 7 of admission. The caesarean section was uncomplicated and a liveborn female infant weighing 1731 g was delivered. The thoracotomy identified a likely Bochdalek hernia involving stomach, small bowel, colon, appendix, spleen and omentum. The contents were reduced and the defect was repaired with four figure of eight Prolene sutures. The patient made an uneventful recovery and was discharged on day nine post-operatively. The neonate was admitted to the special care nursery due to issues of prematurity, specifically, mild respiratory distress, difficulty establishing feeds and jaundice.","This woman, who is 31 weeks pregnant, experienced sudden, severe chest pain and shortness of breath. It turned out she had a large hole in her diaphragm, causing part of her stomach and intestines to move into her chest. Previously, she had a similar issue at 13 weeks, which was thought to be pneumonia, but it was actually a smaller diaphragm defect. Doctors determined the defect was getting bigger and, because she couldn't keep enough food down, they decided to deliver her baby early at 32 weeks. The baby was born healthy, and the diaphragm was repaired, and the mother is now recovering well."
434,"The patient was a 69-year-old man with no history of urinary abnormalities or renal dysfunction. When he was 68, he underwent his first health checkup in several years and was found to have occult blood in his urine, proteinuria, and renal dysfunction. Urinalysis at the first examination showed urine protein of 0.49 g/gCr, urine red blood cells of 30–49/high-power field, and pathological granular casts, for which we decided to perform further studies including a renal biopsy. The patient had a history of untreated dyslipidemia. His family history was unremarkable. The patient was not taking any regular medication at the time of the first examination. Physical findings at the first examination were unremarkable. His blood pressure was normal (112/66 mmHg). There was no edema, lymph node involvement, splenomegaly, purpura, or bone pain. Table shows the results of urinary and blood analyses on admission for the purposes of the renal biopsy (dipstick test for occult blood 2+, urine protein 2+, and urine protein-to-creatinine ratio 0.30 g/g on a spot measurement). The number of dysmorphic red blood cells was 20–29 per high-power field. There were no abnormalities in complete blood count or the blood coagulation system. Serum urea nitrogen was 14.9 mg/dL, serum creatinine was 1.19 mg/dL, and estimated glomerular filtration rate by creatinine was 47.6 mL/min/1.73 m2. Serum cystatin C level was 1.73 mg/L and estimated glomerular filtration rate by cystatin was 37.1 mL/min/1.73 m2. Immunoglobulin levels were normal. Autoantibodies were negative. Serum and urine monoclonal immunoglobulin (immunofixation electrophoresis) were positive. The serum levels of the IgG κ and λ chains were 31.40 mg/dL and 33.60 mg/dL, respectively. The κ/λ ratio was 0.935. Serum cryoglobulin was negative.\nWith light microscopy, 54 glomeruli were observed, and two glomeruli showed global sclerosis. The remaining glomeruli were enlarged and showed lobular accentuation. In addition to mesangial cell proliferation, all glomeruli showed prominent endocapillary hypercellularity (Fig. –). Neutrophils and eosinophils showed marked infiltration. There was no hyaline thrombus, crescent formation, and double contour of the capillary walls. Interstitial fibrosis and tubular atrophy was mild. Direct fast scarlet staining was negative. Immunofluorescence was positive for C3 (1+) and C1q (2+) along the glomerular capillaries. However immunoglobulin G (IgG) was negative. The clone we used to originally test for IgG was produced by Medical & Biological Laboratories Co., Ltd. (Lot No. 104AG; Aich, Japan). All immunoglobulins were negative (Fig. ). Electron microscopy revealed marked endocapillary hypercellularity. There was infiltration of polymorphonuclear leukocytes and monocytes, occluding glomerular capillary lumens. In the subendothelial space, there were many tubular structures in parallel arrays. With higher magnification, the microtubules had a hollow core and the diameter was approximately 40 nm (Fig. ). Most of the deposits contained microtubular structures.\nBased on the characteristic electron microscopy findings, ITG was suspected; however, negative staining for immunoglobulins was an unusual finding for ITG. IgG detection by paraffin-immunofluorescence following pronase pretreatment was strongly positive within the capillary spaces of a glomerulus (Fig. ). Immunofluorescence examination of frozen sections using a different clone of anti-IgG antibody (55,144; MP Biomedicals, Tokyo, Japan) showed positive staining in capillary walls. Immunofluorescence for C4d was positive along glomerular capillaries (Fig. ). Collectively, the patient was diagnosed with ITG with false-negative IgG staining.\nAdditional laboratory data revealed that serum and urine were positive for monoclonal immunoglobulins. In a bone marrow biopsy specimen, the proportion of plasma cells was 1.6%, excluding plasma cell myeloma. Chromosomal aberrations were not found. 18F-fluoro-deoxy-glucose positron emission tomography demonstrated no significant uptake.","69-year-old man was found to have some protein in his urine and kidney problems during a routine checkup. Further tests, including a kidney biopsy, revealed that he has a condition called IgA nephropathy (ITG) with a slightly unusual pattern. The biopsy showed inflammation and scarring in the kidneys, and additional tests confirmed the diagnosis. The patient will need ongoing monitoring and management to protect his kidney function."
435,"A 32-year-old man was admitted to our hospital with appetite loss. He had a history of traumatic transverse cervical spinal cord injury at the C5 level due to suicide attempt at the age of 18. As a result of cervical spinal cord injury, he was paralyzed in the lower body. Contrast-enhanced computed tomography (CT) revealed a late-onset traumatic diaphragmatic hernia with strangulated ileum (Fig. ). The small intestine, transverse colon, and omentum were displaced into the left thoracic cavity, and some portions of these organs showed a decrease in blood flow. Left lung collapse and a compressed right lung with mediastinal shift were evident. The patient underwent emergency surgery. After replacing the incarcerated organs to their original positions, scattered areas of necrosis were identified in the small intestine, transverse colon, and omentum (Fig. ). By using interrupted sutures with non-absorbable 1–0 monofilament, the diaphragmatic orifice was closed. Wedge resection with primary closure was performed for the colonic necrosis in two places. Partial resection, 45 cm long, with end-to-end anastomosis was performed for the small intestine. The necrotic omentum was removed (Fig. ). In addition, a gastrostomy tube was placed since delayed initiation of oral intake was expected. The patient developed severe septic shock postoperatively. Treatment-resistant critical hypotension with non-compensatory tachycardia developed, likely due to parasympathetic nervous system damage related to the cervical spinal cord injury. On postoperative days (POD) 3 and 6, cardiac arrest occurred. Fortunately, he was rescued by cardiopulmonary resuscitation with administration of large doses of vasopressin and catecholamine. However, peripheral vasoconstriction, increased intra-abdominal pressure, and ischemia of the gastrointestinal tract developed, which resulted in colonic anastomotic leakage with diffuse peritonitis, abdominal wound dehiscence, and collapse of gastrostomy on POD 6 (Fig. ). The patient was unable to undergo surgical repair because of his poor general condition with continuing severe septic and neurogenic shock. Therefore, he underwent AVS through the open abdominal wound and it was the first procedure at the intensive care unit. The procedure of AVS was as follows: 1. the open wound and peritoneal cavity were rinsed with normal saline and necrotic and/or contaminated tissues were debrided (Fig. ); 2. wound dressing materials (DUOACTIVE® ConvaTec, New Jersey, USA) for protecting healthy skin around the open wound were patched along the abdominal wound in piecemeal fashion so as to adjust dressing materials to the complicated shape of the wound (Fig. ); 3. two drainage tubes with multiple side holes, up to 30 cm from the tip, were placed in the abdominal cavity through the open abdomen and the enteric contents were suctioned through the drainage tubes using a Continuous Suction Unit MERA Sacuum (Senko Medical Instrument Manufacturing CO, Tokyo, Japan) set to 50–75 mmHg continuous negative pressure; and 4) the entire wound was filled with saline-moistened gauzes and covered with polyurethane drape (Fig. ). The colonic anastomotic leakage showed gradual healing over the course of 2 months, followed by contraction and closure of wound dehiscence (Fig. ). Because the gastric fistula remained, a gastrostomy balloon catheter was placed through the gastric fistula. The patient resumed oral intake on POD 112 and left the hospital on POD 190 with the gastrostomy balloon catheter and without incisional hernia.","This 32-year-old man was admitted to the hospital after experiencing a significant loss of appetite. He has a history of a spinal cord injury from a suicide attempt, which left him paralyzed from the waist down. During his hospital stay, he developed a serious problem with his abdomen, including a collapsed lung and a strangulated intestine, requiring emergency surgery to repair the damage. Unfortunately, he then went into septic shock and cardiac arrest, but was successfully resuscitated. After further complications, a temporary device was placed to drain fluid from his abdomen, and he eventually recovered and was able to resume eating."
436,"A three-year-old girl was referred to the Reference Centre of Rare Diseases in Paris. Her medical history was noncontributory. According to her mother, she complained with pain while eating, moderate sensitivity during tooth brushing and above all poor aesthetic aspect of her teeth. Intraoral examination revealed a hypoplastic AIH with yellow teeth and rough surfaces (Fig. ). Brown extrinsic discoloration was seen in the hypoplastic area. Enamel was reduced in thickness and severely hypoplastic, giving the idea of a false microdontia with multiple diastemas. Molars were the most affected teeth showing reduced crown height. In addition, anterior open bite was noted without thumb sucking. Treatment was planned following 3 objectives at this age:Pain prevention and treatment Protection of dental tissue integrity in order to maintain occlusal function and limit dental biofilm retention Restoration of smile aesthetics.\nOn primary molars, the choice of treatment was stainless steel crowns (3 M™ ESPE™) because the occlusal morphology was lost (Fig. ). This way, vertical dimension was slightly increased and maintained. The incisors and canines were isolated with a rubber dam and direct dental composite restorations were placed (Herculite, Kerr [, ] with ER2 adhesives Optibond SL). Teeth were not prepared; we etched with 35% Phosphatidic acid for 30 s, rinsed for 30 s with air and water. Then teeth were air dried, adhesive was applied with an applicator tip, excesses were removed with air before polymerization for 45 s. Affected enamel was not removed but bonding was directly applied to it. As enamel surface appeared rough, a flow composite (Tetric Evoflow, Ivoclar) was applied and served as intermediate material. Its higher fluidity and wettability would allow penetrating enamel roughness (Fig. ). Because tooth morphology of anterior teeth was not severely altered, “Odus” molds were not useful to offer a correct restoration. Composite resins were applied in one layer. Finishing and polishing were achieved with abrasive discs (Sof-lex/3 M ESPE). Patient follow-ups were done 6 months and 1 year after treatment. Composite sealing and oral hygiene were controlled.","A three-year-old girl was seen for concerns about her teeth, including pain when eating and difficulty brushing. The dentist found that her teeth were unusually small and thin, with a discolored appearance. To help with the pain and protect her teeth, the dentist planned to place stainless steel crowns on her molars and composite fillings on her other teeth. The goal of the treatment is to make her teeth stronger, reduce pain, and improve the appearance of her smile."
437,"A 16-year-old girl was referred by an orthodontist to the Reference Centre of Rare Diseases in Paris. Orthodontic treatment was performed with classical bracket technique in order to close anterior open bite (Fig. -). At the end of the treatment, the patient requested full mouth rehabilitation. She complained first of all about aesthetics but she also reported difficulties and painful chewing. Intraoral examination revealed hypomineralized AI associated with some hypoplasia. A little open bite remained after orthodontic treatment. Teeth were small with diastemas that were not closed as requested by the practitioner. In this occlusal context dental rehabilitation may be done without teeth reduction. Treatment was discussed according to several objectives taking into account the patient’s age:Functional restoration Aesthetic restoration Lasting treatment Minimally invasive treatment\nMaster impression of the two arches was recorded with a silicone material and working cast was mounted onto a semi-adjustable articulator using a centric relation record. Composite veneers were applied on incisors and composite full crowns on all other teeth (Fig. ). Nanohybrid indirect composite (Premise Indirect System, Kerr) was used with dentin and enamel shades mimicking the clinical shade (A3 shade was used cervically, A2 in the core and A1 in the incisal edge). Each layer was polymerised. Rigorous polishing was done in order to obtain shiny surfaces (Tool kit, Kulzer). The restoration was bonded using dual cured composite resin (Variolink Esthetic, Ivoclar™ Vivadent™) taking care to separate each proximal contact with metal matrix. Carefully polishing was made especially at the gingival border with a Touati bur. The patient was very satisfied with the aesthetic appearance. She did not report any trouble with mastication. She was followed every 6 months. Oral hygiene and integrity of the restoration were scrupulously monitored. Direct composite was applied 3 years later, on the cervical part of the crown because gingival maturation occurred. She had only difficulty to control calculus deposition on the lingual part of mandibular incisors. Five years later, the restorations were still satisfactory (Fig. ).","This 16-year-old girl came to the dentist because she wanted to improve the look and function of her teeth after orthodontic treatment. She had an open bite and some small teeth with gaps, which made it difficult to chew and affect her smile. The dentist used special composite materials to create new crowns and veneers to close the gaps and improve the appearance of her teeth. The new restorations look great and she can chew comfortably, and she will be checked regularly to make sure they stay healthy."
438,"In November 2012, a 72-year old man was diagnosed with wet AMD in his left eye, based on fundus examination and optical coherence tomography (OCT), which was requested for the onset of metamorphopsia. At that time, he was treated with combination of angiotensin converting enzyme inhibitor plus thiazide diuretic for a 20-year history of well controlled hypertension.\nHis best-corrected visual acuity in the right and left eyes was 10/10 and 8/10, respectively. On slit-lamp examination, both anterior chambers showed clear aqueous humor and no inflammatory reaction. Dilated fundus examination revealed a subretinal whitish mass and adjacent subretinal hemorrhage. OCT confirmed the presence of a subretinal lesion and intraretinal edema. After obtaining informed consent, the patient was monthly treated with intravitreal administration of 0.5 mg ranibizumab for three months, without any complication and with complete retinal hemorrhage and edema resolution and increased visual acuity of left eye (10/10). Thereafter, the patient underwent routine follow-up visits, on a 2-month basis, including fundus examination and OCT which did not document any abnormal finding. In May 2014, a reduction of visual acuity (from 10/10 to 7/10) was registered. The patient was periodically followed-up but not treated with anti-VEGF drugs as there was no sign of neovascularization. In May 2015, visual acuity further reduced to 3/10 and both fundus examination and OCT revealed a reactivation of the neovascular membrane, edema and pigment epithelial detachment. For this reason, the patient was again treated with intravitreal injections of ranibizumab (0.5 mg), firstly on a monthly basis and thereafter using treat and extend approach, with overall six injections till the end of February 2016, when visual acuity increased to 6/10. At the follow-up visit in May 2016, the neovascular membrane appeared inactive and the visual acuity was stable at 6/10, so the ophthalmologist decided for a pro re nata approach (i.e., as needed).\nIn the same period, the patient referred to the Movement Disorders Clinic due to intermittent tremor on the left hand, started around February 2016. He did not complain non-motor symptoms.\nNeurological examination disclosed resting tremor on the left hand, mild bradykinesia of left lower limb, and mild rigidity of head and trunk. His motor Unified Parkinson’s Disease Rating Scale (UPDRS) was 11/108.\nThe patient had no family history of PD or other neurodegenerative diseases nor had he been ever exposed to pesticides. Magnetic Resonance Imaging of the brain showed rare small subcortical white matters hyperintensities on T2 (mainly periventricular and frontal) and some bilateral hypointensities in T1 in the striatum, more prominent on the right, compatible with small ischemic lesions.\nSingle Photon Emission Computerized Tomography (SPECT) of the Dopamine Transporter (DAT) with 123I-ioflupane documented a significant and clear low uptake of DAT, mostly in the right striatum (Figure ). A diagnosis of clinically established PD was made based on new criteria of the Movement Disorders Society ().\nA treatment with levodopa/carbidopa (300 mg/daily) was started at the beginning of 2017, due to worsening of tremor and bradykinesia leading to gait impairment and fatigue (motor UPDRS = 15/108). At follow-up examination in May 2017, response to levodopa was demonstrated by improvement of motor symptoms (motor UPDRS = 6/108), particularly of gait. Up to November 2017 other two injections of ranibizumab were intravitreally administered with visual acuity equal to 3/10 and the patient was in stable treatment with levodopa/carbidopa (300 mg/daily).","This patient has had a long-standing problem with wet age-related macular degeneration in his left eye, which was initially treated with injections to reduce swelling and bleeding. After a period of improvement, the condition flared up again, requiring further injections to stabilize his vision. In addition to his eye problems, he was recently diagnosed with Parkinson’s disease, which is causing tremors and stiffness in his hands and legs. He is now being treated with medication to manage his Parkinson’s symptoms, and his vision is being monitored to prevent further decline."
439,"A 35-year-old male presented to the Emergency Department of Civil Hospital Karachi with a 10-day history of high-grade fever, shortness of breath, palpitations, and joint pain. The patient had poor appetite and fatigue. He denied any history of trauma, allergies, any other medical conditions, or weight loss. A review of the cardiopulmonary, gastrointestinal, and genitourinary systems was unremarkable. He did not smoke or use alcohol, and there had been no changes in his daily routine. However, he was an intravenous drug abuser. The patient had no history of any major surgery. His history demonstrated an ostium secundum of 22.18 mm with left to right shunt, which was diagnosed previously by transoesophageal echocardiography (Figure ).\nOn physical examination, he was found to be dehydrated but oriented to time, place, and person. His blood pressure was 100/70 mm Hg and his heart rate was 100 beats/minute. The respiratory rate was 20 breaths/minute and his temperature was 102°F. His fever was associated with chills, rigors, and sweating with multiple joint pains. On cardiovascular examination, no murmur was auscultated. The lungs were clear on auscultation. The abdomen was non-tender; hepatomegaly or lymphadenopathy was not detected. However, his spleen was enlarged 2 cm below the costal margin. Motor and sensory examination of all four limbs were normal. Past and family history of the patient was insignificant.\nThe patient's workup was initiated. Laboratory findings included complete blood cell count (CBC) showing Hb level = 12.6g/dl (normal range: 13.0 - 16.5 gm/dL) and total leukocyte count (TLC) = 15,000/μL (normal range: 4 - 11 x 103/μL). Malarial parasite and dengue tests were insignificant. Other tests including serology for human immunodeficiency virus (HIV) and hepatitis showed normal findings. Due to his persistent high-grade fever, IE was suspected in the patient. Therefore, echocardiography and blood culture were performed.\nEchocardiography did not show any vegetation. However, two blood samples drawn from two different sites (12 hours apart for culture) were found to be positive for methicillin-resistant Staphylococcus aureus (MRSA). The patient was treated with intravenous (IV) vancomycin, 1 gm once daily, and IV gentamicin, 80 mg twice a day, for a period of six weeks for S. aureus. The fever eventually subsided, and subsequently, a surgery for the closure of ASD was planned and executed successfully. The patient was discharged from the hospital after he recovered well from surgery. He was further referred to the rehabilitation centre for drug abuse treatment. The patient visits on a monthly basis for follow–up and continues to do well.","This 35-year-old man went to the hospital with a 10-day illness including a high fever, shortness of breath, and joint pain. He admitted to using intravenous drugs, and tests revealed a hole in his heart that was causing a shunt. Blood cultures showed he had an infection with a type of bacteria called MRSA, which was treated with antibiotics. After the infection cleared, he had surgery to close the hole in his heart and is now recovering well and receiving support for his drug use."
440,"Our patient is a 39-year-old male who presented with metastatic stage IVa cT2cN2M1a rectal adenocarcinoma located 12 cm from the anal verge with metastases to the liver. He underwent neoadjuvant chemotherapy with seven cycles of capecitabine-oxaliplatin, followed by long-course chemoradiation. This course consisted of intensity modulated radiation therapy (IMRT) at an outside center, 45 Gy in 25 fractions to the pelvis with a subsequent pelvic cone down of 5.4 Gy in three fractions and an additional 3.6 Gy in two fractions to the gross rectal tumor, given concurrently with capecitabine and trastuzumab. He subsequently underwent laparoscopic-assisted low anterior resection with colorectal pelvic anastomosis and diverting loop ileostomy. Pathology from this revealed ypT4N1b rectal adenocarcinoma with lymphovascular invasion. Throughout this period, his liver lesions were successfully treated with several courses of microwave ablation and chemoembolization. Restaging positron emission tomography-computed tomography (PET-CT) scan five months later demonstrated a new single site of FDG-avidity within a 1.1 cm left pelvic sidewall lymph node with no other sites of disease. He was referred for curative intent radiation therapy to this lymph node to treat his only active site of disease.\nThis patient had already exceeded the ideal dose constraints for bowel tolerance utilized by our institution and as outlined in RTOG 0822 and RTOG 0529 [,]. Specifically, his prior radiation treatment included a maximum point dose to the small bowel of 54 Gy, with V (50 Gy) of 16 cc, V (45 Gy) of 105 cc, V (40 Gy) of 198 cc, and V (35 Gy) of 329 cc; the latter two volumes are nearly double the ideal volume receiving 35 and 40 Gy. Large bowel had also exceeded ideal constraints, with V (45 Gy) of 41 cc. However, in the context of this young patient with oligorecurrent disease, we aimed to offer this patient curative-intent treatment.\nThe patient was thus treated with MR-guided SBRT, 35 Gy in five fractions delivered every other day to the left pelvic sidewall lymph node plus a 3 mm planning target volume expansion (Figure , panel A). Cumulative EQD2 (using α/β = 3) maximum point dose to the small and large bowel was 71.5 and 84.5 Gy, respectively.\nThe location of the recurrence in relation to the prior irradiated field is demonstrated in Figure .\nThe simulation and daily setup pre-treatment MRI scans were obtained on the ViewRay system (ViewRay, Inc., Oakwood Village, OH) incorporating a 0.35 T MRI. The same imaging protocol using a True Fast Imaging with Steady State Free Precession (TRUFI) sequence was performed to acquire the 3D MRI images with 1.5 mm thickness and a field of view of 50 x 45 x 43 cm without contrast injection. The total acquisition time was 172 seconds.\nAfter image acquisition each day, a physician assessed the target and relation to surrounding anatomy. In the case of movement of critical structures close to the target, the physician had the option to create an adaptive plan that minimized the dose to critical structures. In this case, no adaptive planning was required as the position of the bowel relative to the target did not move significantly. Treatment was delivered using IMRT on the ViewRay system with three Cobalt-60 sources spaced at 120 degrees apart. During treatment delivery, cine MRI imaging at a rate of four frames per second was performed through a sagittal plane to track the intra-fractional motion of the target.\nThe patient experienced no acute toxicity. One month after completion of SBRT, the patient underwent a successful ileostomy reversal, with excellent progression toward return of normal bowel function. Repeat MRI at two months post-SBRT demonstrated no evidence of disease.","A 39-year-old man was diagnosed with stage IV rectal cancer that had spread to his liver. He received chemotherapy and radiation therapy to shrink the tumor, followed by surgery to remove the remaining cancer. Despite this treatment, a small area of cancer had returned, and he was treated with focused radiation therapy to eliminate it. The treatment was carefully planned to minimize damage to his nearby organs, and he is now recovering well with his bowel function returning to normal."
441,"A 67-year-old woman was admitted to hospital for an evaluation of worsening right upper abdominal tenderness associated with episodes of nausea and vomiting for the last month. She described the pain as mild to moderate, continuous, radiating to the back, unrelated to eating, and without alleviating or exacerbating factors. She denied fevers, jaundice, or issues with bowel movements and urination. She admitted to a 10 kg weight loss over the last two months. Her medical history was significant for sinus tachycardia with good medical control on metoprolol. She admitted smoking and drinking on social occasions. Her family history was unrevealing.\nAt initial evaluation, her vital signs were within normal limits with a body temperature of 98°F, a pulse rate of 61 beats per minute, a respiratory frequency of 12 breaths per minute, and a blood pressure of 130/85. The abdominal exam revealed a palpable, poorly defined, mildly to moderately tender tumor-like firmness in the right upper quadrant (RUQ) without rebound tenderness. The remainder of her physical examination was unremarkable. The laboratory evaluation revealed a white blood cell (WBC) count of 10.2×109/L, hemoglobin 135 g/L, glucose 6.1 mmol/L, creatinine 0.8 mg/dL, alanine aminotransferase (ALAT) 9 U/L, aspartate aminotransferase (ASAT) 18 U/L, total bilirubin 6.8 mmol/L (direct bilirubin 3.6 mmol/L), and an international normalized ratio of 0.94. Tumor marker cancer antigen 19-9 (CA 19-9) was 14.4 U/mL (normal limit: < 34 U/mL) and carcinoembryonic antigen (CEA) was 0.6 ng/mL (normal limit: < 5 ng/mL).\nAbdominal ultrasonography (US) and computed tomography (CT) of the chest, abdomen, and pelvis revealed a large, ill-defined, heterogeneous mass completely replacing the gallbladder body and fundus with an extensive involvement of the adjacent liver segments, the duodenum, the head of the pancreas, and the hepatic flexure of the colon (Figures -). There were several, enlarged, loco-regional lymph nodes with no other lesions suspicious of distant metastatic disease. There was no intra- or extrahepatic biliary tree dilatation confirmed by magnetic resonance cholangiography.\nAdvanced stage IIIb-IVa CG was the main differential diagnosis and the possibility of acute or chronic cholecystitis with diffuse mass-forming XG was lower on the list. A detailed discussion of the possible intraoperative scenarios and the extent of the surgical resection with the risks, benefits, and prognosis was carried out, and informed consent was obtained. Surgical exploration confirmed the preoperative imaging findings and revealed a mass occupying the entire subhepatic space adherent to the adjacent liver segments, omentum, hepatoduodenal ligament, the second portion of the duodenum, and proximal one-third of the transverse colon. No intra-abdominal collections, abscesses or lesions suspicious of metastatic malignant disease were seen. Intra-operative frozen-section biopsies were not performed. Despite the extensive fibrosis and adhesions, we were able to define and dissect through soft tissue planes and safely separate the involved structures to perform a resection of the entire mass with adjacent liver segments and loco-regional lymph nodes for proper staging and prognosis.\nSurgical pathology revealed an 8x11 cm XG tumor with diffuse, marked thickening of the gallbladder wall (up to 1.5 cm) and lumen filled with spongiform purulent matter. Histology showed deep, ruptured Rokitansky-Aschoff sinuses penetrating the muscle layer with areas of tumor-resembling adenomyosis (Figure ). There were multiple foci of crowding of foamy macrophages and xanthoma cells (Figure ) alongside foreign body granulomas consisting of cholesterol (Figure ) and tiny bile lakes (Figure ). There were mild to moderate reactive inflammatory changes of the adjacent hepatic tissue with lymphocytic infiltration of portal tracts and fatty degeneration. No atypical cells or malignant cells were observed and the enlarged periportal lymph nodes showed a nonspecific inflammatory reaction.\nThe postoperative course was uneventful, and the patient was discharged home on day seven after surgery. Six months after the operation, the patient remains asymptomatic and in good health.","This 67-year-old woman was admitted to the hospital because of pain in her upper right abdomen, nausea, and a recent weight loss. Tests revealed a large, concerning mass in her abdomen that was replacing her gallbladder and affecting nearby organs. Imaging showed the mass was connected to the liver, duodenum, and colon, and it was causing inflammation. After surgery to remove the mass, the patient is doing well and remains healthy six months later."
442,"Patient 1 was a 13-month-old female who sustained a flame injury from a house fire. She had 73% TBSA third-degree injuries. Her right knee had an open joint injury that was treated with four skin grafting procedures prior to SEF placement. SEF was placed 46 days after the injury and was kept on for three weeks. K-wires and a 140-mm bar were used as a micro-SEF system. The patient required one additional skin grafting procedure to her right knee after SEF was in place. She had an eight-month follow-up after the injury and her PT/OT notes reported ROM within normal limits (WNL) and a 5/5 motor strength. She did have continued joint instability after SEF that required eight weeks of knee immobilizer use. However, no long-term complications requiring additional surgeries were recorded.","This 13-month-old girl was burned in a house fire, sustaining significant injuries to her skin. She needed multiple skin grafts to repair the damage, and a special type of cast called a SEF was used to support her injured knee. After three weeks, the SEF was removed, and she needed one more skin graft to fully heal her knee. She made a good recovery with physical and occupational therapy, and her knee strength and movement are now normal."
443,"Patient 2 was a 10-year-8-month-old male who sustained a flame injury after a motor vehicle accident (MVA). He had 62.5% TBSA third-degree injuries. His right elbow had an open joint injury that was treated with seven skin grafting procedures prior to SEF placement. SEF was placed 55 days after the injury and was kept on for six weeks. Shantz pins and bars were placed as the external fixator system; this was locked in full extension. The patient required no additional skin grafting procedures to his right elbow after SEF placement. He had a 10-month follow-up after the injury and his PT/OT notes documented a final ROM of 30–110° in the extension and flexion arc, supination of 30° and pronation WNL, and a 5/5 motor strength. No other complications were recorded for this patient.","This 10-year-old boy was injured in a car accident and suffered serious burns to his body. He needed multiple skin grafts to repair the damage to his right elbow, which had an open joint injury. A special brace was placed on his arm to help it heal, and it remained in place for six weeks. After a year of physical therapy, his arm regained full movement and strength, and he was able to return to normal activities."
444,"Patient 3 was a 4-year-4-month-old male who sustained a flame injury from a house fire. He had 41.5% TBSA third-degree injuries. His right elbow had an open joint injury that was treated with two skin grafting procedures prior to SEF placement. SEF was placed 43 days after the injury and was kept on for four weeks. Shantz pins and bars were placed using the Synthes medium external fixator system; this was locked in full extension. The patient required no additional skin grafting procedures to his right elbow after SEF placement. He developed an elbow contracture that was treated with an antecubital soft tissue plastic surgery contracture release. Prior to his surgical release, he had an ROM of 0–25° in the extension and flexion arc, supination of 0°, and pronation 0°. He had a 13-month follow-up after the injury and his PT/OT notes reported a final ROM of 0°–119° in the extension and flexion arc, supination of 69° and pronation 80°, and a 5/5 motor strength. His only complication was the elbow contracture.","This 4-year-old boy was burned by flames in a house fire, resulting in significant burns to his body. He underwent several skin grafts and a special brace to help his right elbow heal properly. After the brace was removed, he developed an elbow contracture, which was treated with surgery to release the muscles and tendons. Following the surgery, he made significant progress in regaining full movement in his arm, and his physical and occupational therapists reported excellent strength and range of motion."
445,"Patient 4 was a 17-year-9-month-old male who sustained a flame and electrical injury after an MVA involving a power line. He had 30% TBSA third-degree injuries. His right elbow had an open joint injury that was treated with two skin grafting procedures prior to SEF placement. Other unknown procedures were performed in Mexico before transfer to our institution. SEF was placed 23 days after the injury and was kept on for 7.5 weeks. Four-millimeter Shantz pins and bars from the Synthes small external fixator set were placed; the fixator was secured in full extension. The patient had an SEF failure likely due to under sizing. The fixator was revised with a Synthes large external fixator system eight days after the initial one. The patient required three additional skin grafting procedures to his right elbow after SEF placement. He had a 12-month follow-up after the injury and his PT/OT notes reported a final ROM of 0°–110° in the extension and flexion arc, supination of 30°, and pronation 70°. He did have some continued weakness with elbow flexion at 4/5, extension 2/5, and pronation/supination 1/5 motor strength. His complications were SEF failure and weakness.","This 17-year-old male was seriously injured in a car accident involving a power line, resulting in significant burns and an open injury to his elbow. Initially, doctors used an external fixator to stabilize his elbow and promote healing with skin grafts. However, the initial fixator didn't hold properly, so it was replaced with a larger one. Despite multiple skin grafts and physical therapy, he experienced some ongoing weakness in his elbow, but his range of motion has improved significantly."
446,Patient 5 was a 7-year-4-month-old-male who sustained an electrical burn involving a power line. He had 32% TBSA third-degree injuries. His left knee had an open joint injury that was treated with three skin grafting procedures prior to SEF placement. SEF was placed 24 days after the injury and was kept on for 8.5 weeks. Shantz pins and bars were placed as the external fixator; this was locked in full extension. The patient required two additional skin grafting procedures to his left knee after SEF placement. He also required a bony prominence resection while in SEF. He had no PT/OT notes or follow-up recorded. He had no significant complications.,"This 7-year-old boy was seriously injured when he came into contact with a power line, resulting in severe burns covering a large portion of his body. He needed multiple skin grafts to repair the burns and an open joint injury to his knee. To help stabilize his knee, doctors used an external fixator with pins and bars, which he wore for several weeks. After the fixator was removed, he needed additional skin grafts to fully heal his knee, and a small bone piece was removed. Overall, he recovered well with no major problems."
447,"Patient 6 was an 11-year-6-month-old male who sustained a flame burn from an MVA. He had 25% TBSA third-degree injuries. His left knee had an open joint injury that was treated with three skin grafting procedures prior to SEF placement. SEF was placed 74 days after injury and was kept on for nine weeks. Shantz pins and bars were placed as the external fixator system; this was locked in full extension. He required no additional skin grafting procedures to his left knee after SEF placement. The patient had no ROM measurements recorded because the left knee was immobilized in full extension for a prolonged period of time. Complications were extensive. The patient ended up with significant damage to the medial collateral ligament and the medial femoral epiphysis from the injury developed a spontaneous knee fusion. Because of the growth plate disturbances, he developed a varus deformity and leg length discrepancy of 4.0 cm which caused persistent pain. He ended up requiring an osteotomy and Ilizarov placement for alignment correction and lengthening.","This 11-year-old boy suffered a serious burn injury from a car accident, resulting in significant damage to his skin. He underwent multiple skin grafts to repair the burn, and a special brace was used to stabilize his injured knee. Unfortunately, the injury caused damage to his knee joint, leading to a fusion of the joint and a noticeable leg deformity. To correct the leg deformity, he needed additional surgery, including an osteotomy and the use of an Ilizarov brace to realign his leg."
448,Patient 7 was an 8-year-5-month-old male who sustained a flame burn from an MVA. He had 79% TBSA third-degree injuries. His right knee had an open joint injury that was treated with six skin grafting procedures prior to SEF placement. SEF was placed 61 days after the injury and was kept on for five weeks. An SEF was placed using the Smith Nephew Richards system with 5.0-mm Shantz pins and bars. The patient required no additional skin grafting procedures to his left knee after SEF placement. He had a 10-month follow-up after the injury and his PT/OT notes recorded a final ROM of 0°–45° in the extension and flexion arc. His last recorded motor strength was 3+/5. His complication was residual knee stiffness and weakness.,"This 8-year-old boy was burned by a car accident, resulting in significant burns to his leg. He needed multiple skin grafts to repair the damage, and a special brace called an SEF was placed on his knee to help it heal properly. The brace stayed in place for five weeks and successfully prevented further skin grafts on his knee. After a year of physical therapy, his knee regained some movement, but he still has some stiffness and weakness."
449,"Patient 8 was a 16-year-6-month-old male who sustained an electrical burn from contact with a power line. He had 48% TBSA third-degree injuries. His right elbow had an open joint injury that was treated with six skin grafting procedures prior to SEF placement. SEF was placed 53 days after the injury and was kept on for six weeks. An SEF was placed using the Smith Nephew Richards system with 5.0-mm Shantz pins and rods. The patient required no additional skin grafting procedures to his left knee after SEF placement. However, he did require two additional surgeries after the SEF was removed because of complications, including one surgery that incorporated an ulnar nerve transposition, heterotopic ossification removal, and contracture release with arthrofibrosis excision. The second surgery was a flap to cover soft tissue deficit that occurred after the elbow release. The patient had a 13-month follow-up after the injury and his PT/OT notes reported a final ROM of 20°–90° in the extension and flexion arc, supination and pronation were not tested, and a 4-/5 motor strength. Prior to his elbow contracture release, he had an ROM of 0°–30° in the extension and flexion arc, supination of 25° and pronation 85°, and motor strength was not tested. Complications included heterotopic ossification, cubital tunnel syndrome, and elbow stiffness.","This 16-year-old boy was seriously injured when he came into contact with a power line, resulting in significant burns to his body. He needed multiple skin grafts to repair his injuries, and a special brace (SEF) was placed on his elbow to help it heal. Despite complications like stiffness and nerve problems, he underwent several surgeries to address these issues and improve his range of motion. After a long recovery period, he regained some movement in his elbow, but still has some limitations in strength and movement."
450,"Patient 9 was a 2-month-old female who sustained a flame burn from a house fire. She had 49.5% TBSA third-degree injuries and 3% TBSA second-degree injuries, 53% total. Her left elbow had an open joint injury that was treated with one skin grafting procedure prior to SEF placement. SEF was placed 74 days after the injury and was kept on for 8.5 weeks. Three-millimeter Shantz pins and rods were placed as an external fixator. The patient required no additional skin grafting procedures to her left elbow after SEF placement. However, she did require an SEF revision because the initial fixator was pulled out. She was lost to follow-up owing to a major natural disaster and transfer to a different hospital. The patient did develop some elbow stiffness and contracture at the time of SEF removal; however, her final ROM and strength are unknown.","This 2-month-old girl was burned in a house fire, sustaining significant burns to her skin. She needed skin grafts and an external fixator to stabilize her left elbow, which had an open wound. After several weeks with the fixator, it needed to be replaced due to being pulled out. Unfortunately, she was unable to continue her care due to a major disaster and was transferred to another hospital. Despite the treatment, she experienced some stiffness in her elbow."
451,"A 64-year-old female was admitted with progressively worsening dyspnea and cough of one-month duration. These symptoms were associated with an unintentional eight-pound weight loss. She was seen two weeks earlier by her primary physician and started on levofloxacin for what was thought to be pneumonia. Initial antibiotic therapy failed to improve symptoms and patient eventually required supplemental oxygen. A CT scan of the chest showed innumerable cystic lesions with diffuse ground glass opacities in both lungs and a lesion in the liver (Figure , Figure ).\nThe patient underwent extensive workup for lung and liver disease including viral, bacterial, and fungal infection workups, human immunodeficiency virus testing, hepatitis panel, expanded connective tissue disease workup, and comprehensive interstitial lung disease markers. Cancer markers revealed an elevated cancer antigen 19-9. An abdominal ultrasound revealed innumerable cystic lesions throughout the liver; the majority were simple in appearance. It also showed intrahepatic and extrahepatic biliary and pancreatic duct dilatation. Liver biopsy was suggestive of a benign simple cyst wall and acute inflammation of hepatocytes. These findings raised a differential diagnosis including bile duct obstruction, mass effect, adjacent abscess, or adverse drug/toxin effect. Eventually, due to the unusual findings on CT scan and the failure of liver biopsy to provide a diagnosis, a lung biopsy was performed. Lung tissue was obtained by videoscopic-assisted fluoroscopic surgery to the right chest with wedge resection. Pathological examination showed metastatic with moderately differentiated adenocarcinoma in the right upper lobe, middle lobe, and lower lobe.","This 64-year-old woman was admitted to the hospital because she was having increasing trouble breathing and a persistent cough for about a month. She had also lost eight pounds without trying and was initially treated with antibiotics, but it didn't help. A scan of her lungs and liver showed unusual growths, and further tests revealed elevated cancer markers. Finally, a lung biopsy confirmed that she has lung cancer that has spread to her liver."
452,"An 87-year-old man was conveyed to the emergency department after being found to be drowsy by his helper. He was watching television before he was found slumped in a chair, staring into space with saliva drooling. There was no history of recent fever, headache, fall, or trauma, and no recent hospitalization. Past medical history was significant for hypertension, stage 5 chronic kidney disease, a cerebrovascular accident, pacemaker insertion for sick sinus syndrome, a transurethral resection of the prostate for benign prostatic hyperplasia and pulmonary tuberculosis. His long-term medications were aspirin, omeprazole, amlodipine, and furosemide.\nOn examination, his Glasgow coma scale was 7 (M4E2V1), and his pupils were equal and reactive. His vital signs were stable, with a temperature of 36.7 degree Celsius, pulse rate of 66 beats per minute, respiratory rate of 18 breaths per minute, oxygen saturation of 99% on room air, and blood pressure of 144/84 mmHg. He had a new onset right hemiparesis and normal reflexes. There was no evidence of any head injury or cerebrospinal fluid otorrhea or rhinorrhea. Stat capillary blood glucose was 8.7 mmol/L. Infective markers were normal, white blood count was 5.6 x103/µL, C-reactive protein was 0.3 mg/L, urea was 17.7 mmol/L (stable), sodium was 137 mmol/L, potassium was 5.1 mmol/L, creatinine was 331 µmol/L (stable), and corrected calcium was 2.29 mmol/L. The liver function test was normal. Electrocardiogram (ECG) did not show any evidence of acute myocardial ischemia. An urgent CT brain revealed air bubbles within the dural venous sinuses (bilateral cavernous, superior sagittal, straight, and left sigmoid sinuses) (Figure ). There was no definite evidence of ischemic changes on CT brain. Pneumocephalus was initially thought to be the cause of his drowsiness and right hemiparesis. The neurosurgery team suggested conservative management of the pneumocephalus.\nA facial bone CT was ordered to rule out trauma, as it is the commonest cause of pneumocephalus. The CT scan, done two days later, demonstrated a minimally displaced fracture of the lateral wall of the right maxillary sinus. Of note, there was an acute left middle cerebral artery territory infarct with hemorrhagic conversion, a mass effect on the left ventricle, and a resultant midline shift (Figure ). The pneumocephalus had resolved.\nIn view of a poor premorbid state, the severity of the stroke, and a lapse in the diagnosis of stroke, the patient was conservatively managed. He was started on statins; antiplatelets were held off in view of significant hemorrhagic conversion causing the midline shift. He underwent physiotherapy but remained bed-ridden.\nA repeat CT brain one month later showed improvement in hemorrhagic transformation and a resolution of midline shift. He was then started on dual antiplatelet therapy. Despite the medical management, he demised one month after the initial presentation.","This 87-year-old man was found unresponsive at home, and doctors discovered he had a stroke with bleeding in his brain. The cause of the stroke was air bubbles in his brain sinuses, which were related to a sinus fracture. Despite treatment, his condition worsened, and he sadly passed away after a month. Doctors focused on managing his symptoms and provided supportive care during his illness."
453,"A 23-year-old woman presented with left lower quadrant abdominal pain and mildly elevated creatinine of 1.21 mg/dl (baseline 0.8-1 mg/dl) that was identified on routine blood draw at a one-month follow-up urology appointment. She had no urinary symptoms at that time and the review of systems was otherwise unremarkable. The physical examination was unremarkable without any focal abnormalities. Urinalysis and pregnancy testing were negative. Computed tomography abdomen revealed hydronephrosis of the left kidney as well as bilateral ureteral thickening (Figures -). One month prior, she had a right ureter stent placed after she presented with acute kidney injury, and the CT abdomen at that time showed evidence of right hydronephrosis and bilateral proximal ureteral thickening. In addition, biopsies of ureter specimens found normal urothelium with signs of chronic inflammation during that initial hospitalization. Further workup was negative, including chest x-ray, sexually transmitted infection testing, complement activity levels, and other autoimmune markers. During this admission, a stent was placed in the left ureter and the right ureter stent was replaced. Repeat biopsies of both the right and left ureters showed findings of benign epithelial growth and chronic inflammation that were previously noted the month prior. Per the operative report, the region of the ureter thickening had the gross appearance of ureteritis cystica. However, upon further questioning, the patient had a history of only one urinary tract infection, no nephrolithiasis, and no other risk factors for this presentation. The patient was discharged home symptom-free and proceeded with close urological follow-up.","This 23-year-old woman came to the hospital because of pain in her lower left side of her abdomen. Blood tests showed a slightly elevated kidney level, and a CT scan revealed swelling in her left kidney and the tubes that carry urine from her kidneys. She had a similar problem in her right kidney a month earlier, and biopsies of the ureters showed chronic inflammation. After placing stents to help drain the urine, the swelling decreased, and she was discharged home with a plan for continued urological care."
454,"A two-week-old female was brought in with an initial complaint of multiple masses that failed to regress since birth. She was born from a nonconsanguineous union and the mother’s pregnancy was uneventful. The baby was delivered vaginally at 37 weeks of gestation and multiples masses were found over the body. The treating physician advised follow-ups on discharge. However, the parents decided to seek more medical help for the child and she was admitted for more investigations.\nOn physical exam, the child was alert and active. She was not jaundiced and no pallor was noted on the extremities. Her vitals were all within the normal range and the birth weight and changes in weight corresponded properly. Two masses measuring 26 mm by 19 mm and 19 mm by 17 mm were observed on the forehead and the scalp (Figures -). One smaller mass was seen on the abdomen, measuring 11 mm by 10 mm (Figure ). All three masses were red and non-hemorrhagic. The parents reported that the size of the masses did not change since birth. No similar family history was found and both parents were healthy. Any hepatosplenomegaly was not observed on palpation nor were any other cutaneous lesions detected. She did not present with any other systemic abnormalities. An ultrasound was performed, and it revealed a normal liver, spleen, and kidneys with no masses or lesions. A Doppler examination also showed a fast-flow vascular lesion, as reported by many other articles []. The full blood count showed normal levels of white blood cells, platelets, hematocrit, and red blood cells.\nThe diagnosis of congenital hemangioma was confirmed and the family was properly educated about the condition as well as possible complications. A wait and watch approach was adopted and the child was scheduled for regular visits at three months. The family was asked to properly monitor the size of the masses and informed that appropriate surgical treatment would be provided if the masses fail to regress or if they continue to grow.","A two-week-old baby was brought to the hospital because she had several unusual red masses that hadn't gone away since birth. The baby’s mother had a normal pregnancy, and the baby was born healthy, but the masses were discovered. Tests, including an ultrasound, showed that the masses are likely congenital hemangiomas, which are common and usually grow and then shrink on their own. The doctors will continue to monitor the masses and schedule regular check-ups, and surgery will be considered if they don’t disappear or get bigger."
455,"A 19-year-old female suffered a motor vehicle accident causing her to be ejected from the car. Complete spinal precautions were followed at the scene and her neck was immobilized with a rigid collar. The patient then was transported to our emergency room. She was complaining of neck pain. Her vital signs and neurological examination were normal. She was found to have an associated left comminuted femur fracture. CT of the cervical spine showed concomitant atlanto-occipital and atlanto-axial dissociation (Figure ). MRI of the cervical spine confirmed the diagnosis with total ligamentous disruption at the craniovertebral junction (CVJ) and distraction of the atlanto-axial joints bilaterally (Figures -). While the CCI was normal (1.2 mm), the BDI was 19 mm, which is diagnostic of AOD.\nThe patient was immobilized with a crown-halo vest and a posterior occipitocervical fusion was performed urgently (Figure ). The patient then was discharged on a rigid collar and followed up in the clinic. During her one year appointment, the patient was neurologically normal, reported no neck pain, and denied any dysphagia.","This 19-year-old woman was seriously injured in a car accident and was carefully treated at the scene. She arrived at the hospital with neck pain and a broken leg, and tests revealed significant damage to the bones and ligaments in her neck. Doctors performed an urgent surgery to stabilize her neck and fused the bones together. After a year of follow-up, she is now neurologically normal and has no neck pain."
456,"A 71-year-old woman with a history of a right hip intra-capsular fracture in 2006 presented to the emergency department after a fall at her home. In 2006 she had a DHS (dynamic hip screw) as a result of right hip intra-capsular fracture. She developed osteonecrosis which led to a total hip replacement in 2010. Her medical history was relevant for hypertension and had a left mastectomy 25 years ago due to breast cancer. Prior to this episode she denied any trouble with this hip since her surgery in 2010.\nHer radiographs demonstrate a Vancouver B3 peri-prosthetic fracture (Figures -), with lateral extrusion of the highly polished double taper stem through the cement mantle and through lateral wall of the proximal femur. Two treatment options were considered;\ni) A femoral component revision with an allograft and\nii) A proximal femoral replacement.\nThe former option is often preferred for low-demand patients with extensive medical co-morbidities, while the latter- while representing a larger surgical insult for the patient- facilitates immediate weight bearing and early rehabilitation.\nThe patient underwent a both component revision arthroplasty procedure (Figure ); using an the multiple fracture lines already present through the proximal femur in lieu of an extended trochanteric osteotomy, the prosthesis and cement were removed from the proximal femur, and a modular endoprosthesis (LPS ® DePuy Limb Preservation System (Warsaw, IN, USA) proximal femoral replacement) inserted. The acetabular component was also revised. While representing an addition extra step and a slightly increased magnitude of the surgical insult, it allows use of a larger head, and the optimsation of any version issues to reduce the risk of post-operative dislocation. An additional trochanteric claw plate was used to re-attach the bone of the proximal femur to the prosthesis, thus ensuring good abductor function. The patient tolerated the surgery without incident. She was able to commence immediate full weight-bearing, protected with a Zimmer frame, on postoperative day one. At her six-week postoperative evaluation, she was ambulating independently, though had continued with the use of her walking frame for ‘balance and confidence’.","This 71-year-old woman fell at home and was found to have a fracture in her hip replacement from a previous surgery in Because of this fracture, doctors had to remove the old hip replacement and replace it with a new one. They chose a more complex procedure to ensure stability and allow her to start walking as soon as possible. She is now recovering well and is able to walk independently with the help of a walker."
457,"A 59-year-old left-handed female with a history of hypertension, steatohepatitis, hypothyroidism, and obstructive sleep apnea (OSA) was brought to the emergency department (ED) with progressive altered mental status, abulia, and inability to care for herself. The family reported inadequate dietary intake and increasing forgetfulness in the last week. Her history was significant for laparoscopic Roux-en-Y gastric bypass bariatric surgery one month prior. The post-operative course was uneventful, and she was discharged home with normal mental status on nightly continuous positive airway pressure (CPAP). Three days later she was brought to the ED in a lethargic state after falling out of bed in the setting of CPAP noncompliance. She was admitted to the surgical intensive care unit for acute hypoxic respiratory failure and was intubated. Computed tomography (CT) chest only showed small bilateral pleural effusions. She was eventually extubated and placed on a regimen of CPAP when asleep and transferred to the regular nursing floor. Despite adequate oxygenation, the patient remained arousable but disoriented with decreased attention span. Cranial nerve, motor and sensory examinations were normal. Magnetic resonance imaging (MRI) brain showed nonspecific white matter disease of the centrum semiovale (Figure ).\nLumbar puncture revealed an elevated myelin basic protein. Her vitamin D-25 and methylmalonic acid levels were low. Her thyroid function workup was consistent with hypothyroidism. The rest of her metabolic workup was unremarkable. She was eventually discharged to a skilled nursing facility (SNF) with neurology follow-up.","This 59-year-old woman was brought to the hospital because she was confused and unable to care for herself. She had recently had weight loss surgery and wasn’t using her CPAP machine regularly, which led to a breathing problem. Tests showed some changes in her brain and low levels of certain vitamins, and she was treated with medication to address these issues. After a period of recovery, she was discharged to a nursing home for continued care and follow-up."
458,"A 71-year-old female with a history of in situ ovarian adenocarcinoma status post appendectomy and right-sided hemicolectomy developed generalized tonic-clonic seizure activity. An initial seizure was noted while undergoing an endobronchial biopsy procedure for evaluation of a perihilar mass. Pathology was consistent with a benign reactive lymph node. During the procedure, she developed mottled discoloration of her skin, spreading from her abdomen to both shoulders. Bag ventilation was started, and she was transferred to the surgical intensive care unit (ICU) and intubated. CT head revealed multiple air emboli (Figure ).\nContinuous electroencephalography (CEEG) monitoring revealed frequent periodic lateralized epileptiform discharges. Her anti-epileptic medication was rapidly escalated to pentobarbital in addition to levetiracetam, lacosamide, and phenytoin. Her Glasgow Coma Scale was 3 (E:1;V:1;M:1). Neurological examination was significant for sluggish but reactive bilateral pupils and areflexic quadriplegia. She was transferred to the neurological ICU at that time. Her neurological examination remained same thereafter. On day 14 of her admission, EEG showed changes consistent with bilateral cortical dysfunction in bifrontal regions indicating severe diffuse encephalopathy. No seizure activity was noted on EEG. Cerebrospinal fluid (CSF) analysis revealed an elevated myelin basic protein but with undetected white blood cells (WBCs), red blood cells (RBCs), and negative cytology. MRI brain scan the following day showed progressive diffuse white matter changes in a watershed distribution and centrum semiovale (Figure ).\nShe was weaned from her antiepileptic regimen to levetiracetam monotherapy. She slowly improved, but required tracheostomy and percutaneous gastrostomy tube. She was eventually transferred to an acute rehabilitation facility.","This 71-year-old woman experienced a sudden seizure during a medical procedure. After being stabilized, doctors discovered she had air bubbles in her blood, which caused her to become very ill. Tests showed significant damage to her brain, and she required intensive care and support. Over time, she began to improve, but needed a tracheostomy and feeding tube before being transferred to a rehabilitation facility."
459,"An 87-year-old Caucasian male with a past medical history of benign prostatic hyperplasia and irritable bowel syndrome presented to the hospital with urinary incontinence, diarrhea, abdominal pain, hypotension and altered mental status. A diagnosis of septic shock secondary to urinary tract infection was made on arrival based on symptomology and initial investigation. The patient had a history of lower abdominal pain for last two weeks. He visited his primary care physician and underwent a computed tomography (CT) scan of the abdomen and pelvis which showed hypertrophy of the prostate and bilateral hydronephrosis. The patient had progression of symptoms leading to hospitalization. On arrival to the hospital, the patient was hemodynamically stable but quickly decompensated. Vitals showed a blood pressure of 88/55 mmHg, heart rate of 143 beats per minute, respiratory rate of 20 breaths per minute, and temperature of 96.3°F. Initial pertinent laboratory findings included acute kidney injury with serum creatinine of 12 mg/dL (from a baseline of 1.2 mg/dL) and blood urea nitrogen (BUN) of 161 mg/dL. Labs demonstrated an anion gap metabolic acidosis secondary to lactic acidosis. Urinalysis showed evidence of infection, and blood and urine samples sent for cultures. Physical exam at arrival was significant for a minor distress, diaphoresis, enlarged and tender prostate, abdominal distension without tenderness to palpation, guarding, rebound tenderness, or abnormal dermatological findings. The white blood cell values from the day of surgery until discharge are detailed in Table .\nThe patient received empiric intravenous antibiotics and fluid resuscitation in the emergency department along with placement of a urinary catheter to relieve urinary obstruction. Urinary catheter placement revealed gross hematuria, but hematuria resolved by the next day. Blood and urine cultures were positive for E. coli and initial antibiotics were deescalated to ceftriaxone, to which the organism was sensitive.\nNo acute cardiopulmonary changes were visualized on chest X-ray taken three days prior (Figure ).\nOn day five of his admission, the patient experienced increased abdominal pain, constipation, and subjective fevers. Vitals showed a temperature of 97.2°F, a blood pressure of 111/70 mmHg, a pulse of 87 beats per minute, respiratory rate of 20 respirations per minute, and oxygen saturation of 97%. Abdominal exam was significant for hypoactive bowel sounds, mild distension, guarding, tympany on percussion, and diffuse tenderness to palpation. An upright chest X-ray was ordered. Upright chest X-ray revealed free intraperitoneal air (Figure ).\nAn abdominal X-ray showed a nonobstructive bowel gas pattern without signs of dilated loops of bowel or air-fluid levels. The surgical team was emergently consulted for evaluation of pneumoperitoneum, and an urgent exploratory laparoscopy was recommended without further evaluation or imaging. The patient underwent emergent exploratory laparoscopy that was converted to laparotomy, as there was no evidence of a perforation visualized on laparoscopy. Meticulous examination of the abdominal cavity revealed no evidence of an intestinal or genitourinary tract perforation or any other surgical causes, peritonitis, free fluid, or abscess formation. Post-operative X-ray, completed on post-operation day one, demonstrated resolution of the pneumoperitoneum (Figure ).","An 87-year-old man was admitted to the hospital because he was feeling unwell with symptoms like urinary problems, diarrhea, abdominal pain, and confusion. Doctors discovered he had a serious infection in his urinary tract that led to septic shock, causing his blood pressure to drop and his kidneys to struggle. He received antibiotics, fluids, and a urinary catheter to help with the infection and his symptoms improved. However, he developed a serious complication with free air in his abdomen, requiring an emergency surgery to find and address the issue. After surgery, his condition stabilized, and he was able to recover and return home."
460,"History and physical examination\nA 75-year-old female with a past medical history of fibromyalgia and Sjogren’s syndrome presented to the emergency department with severe hypertension. The patient was not taking any medication at home.\nOn physical examination, the patient was in slight distress. The vitals were as follows: blood pressure of 182/91 mmHg, heart rate of 72 beats per minute, respiratory rate of 15 breaths per minute, and oxygen saturation of 93% on room air. The patient had 2+ pitting edema of lower extremities. The rest of the physical examination was unremarkable.\nHospital course\nThe blood pressure of the patient improved after giving hydralazine and clonidine. Nephrology was consulted for evaluation of increased creatinine (3.8 mg/dl) with blood urea nitrogen of 29 mg/dl. Review of the past medical record in the hospital showed her creatinine level of 2.5 mg/dl, a year ago. Further workup revealed 24-hour proteinuria of 3.89 grams. Urine dipstick showed 3+ protein, 5-10 red blood cells and 10-15 white blood cells per high power field.\nConsidering the past medical history of Sjogren’s syndrome, the patient was suspected to have acute interstitial nephritis or possible immune-complex-mediated disease. Her C3 and C4 complement levels were low. Serum immunofixation did not reveal any monoclonal immunoglobulin. Serologies for antinuclear antibody was positive (titer, 1:640), negative for anti-double-stranded DNA, hepatitis B and C, and antineutrophilic cytoplasmic antibodies.\nOn light microscopy (LM), three glomeruli were present for evaluation, two of which were globally sclerotic and one showed segmental scarring. The segmental lesion had an accompanying fibrous reaction, suggestive of a possible healed/fibrous crescent. The glomeruli also featured noticeable mesangial expansion, which was negative for Silver methenamine and positive for Periodic acid-Schiff (PAS) staining. Congo-red stain was also negative. No capillary wall deposits were seen on special stains and no active necrotizing or crescent lesions were present. Moderate interstitial fibrosis was present in the interstitium. Furthermore, moderate intimal fibrosis was seen in vessels, with no thrombosis or vasculitis.\nImmunofluorescent (IF) showed diffuse global 3+ smudgy mesangial; the capillary wall was positivity noted with immunoglobulin G (IgG) (Figure ). Glomerular staining showed 1+ IgM, C1q, 2+ C3, 1+ staining with both kappa and lambda light chains (Figure ). IgG and albumin stained protein reabsorption granules in the tubular cytoplasm were noted. No significant staining was seen with fibrinogen.\nEM confirmed the presence of extensive electron dense deposits in the expanded mesangial regions and throughout the thickened glomerular basement membranes (Figures -). These deposits had a distinct fibrillary substructure and they did not show any transmembranous spicule formation. No extra-glomerular deposits were seen.","A 75-year-old woman with a history of fibromyalgia and Sjogren’s syndrome came to the hospital with dangerously high blood pressure. Tests revealed that her kidneys were not functioning properly, with increased protein in her urine and signs of inflammation. Doctors suspect she may have developed acute interstitial nephritis, possibly due to an immune reaction. Further examination showed evidence of scarring in her kidneys, and electron microscopy revealed deposits in the kidney tissue. She is being closely monitored, and her blood pressure is being managed with medication, while doctors investigate the cause of her kidney problems."
461,"A 49-year-old woman with a past medical history of hypertension, preeclampsia, anxiety, and bipolar disorder on buspirone, presented to the emergency department with triage complaint of “multiple complaints”. Her history of present illness revealed a persistent headache that initially started one week prior to arrival. The patient recalled onset in the evening associated with nausea and vomiting. She went to an urgent care the next day and received medications, after which she felt better for about two days. When her symptoms recurred, she went to an outside hospital where she had a computed tomography scan of the head and lumbar puncture, both of which were negative. The patient was admitted for an elevated troponin level and received a cardiac catheterization without intervention, findings significant for 60% blockage of a single vessel. The patient presented to us one day post discharge due to persistent headache. She described the headache as similar to her prior preeclampsia headache, feeling ""like a grip around"" her entire head.\nHer initial vital signs were as follows: blood pressure 172/92 mmHg, pulse 81, respiratory rate 18, and oxygen saturation 99% on room air. A physical exam revealed a woman in pain but nontoxic appearing. Her heart and lung sounds were normal. Her neurological exam was unremarkable with no focal numbness, weakness, or abnormalities with coordination, gait, or cranial nerves. The chest plain film, initial lab, and electrocardiogram results were normal.\nThe patient received one liter intravenous fluids, metoclopramide intravenous, and ketorolac intravenous with minimal improvement and still appeared uncomfortable on multiple reassessments. The patient was placed in the observation unit and a magnetic resonance imaging (MRI) of the brain was ordered.\nThe magnetic resonance imaging (MRI) of the brain showed three punctate regions of focal restricted diffusion in the left middle frontal gyrus, right parietal lobe, and left temporal lobe that appeared consistent with acute infarcts. The image is provided below (Figure ).\nNeurology was consulted and the patient was admitted to the stroke service. Her stroke workup, which included computed tomography angiography of the head and neck, transthoracic echocardiogram, and a transesophageal echocardiogram, was unremarkable. The differential at that point was narrowed down to vasculitis versus RCVS as the cause of the severe headache associated with acute infarction. Neurosurgery was consulted and an angiogram was performed, showing evidence of RCVS in the middle cerebral artery, M1 segment, and distal middle cerebral artery territories as well as the right posterior cerebral artery and distal anterior cerebral artery. Dual antiplatelet therapy with aspirin and clopidogrel was initiated and the patient was discharged to follow up with the stroke clinic and her primary care provider.","This 49-year-old woman came to the hospital because of a persistent headache that felt like a tight band around her head. She had similar headaches in the past during pregnancy, and tests showed she had suffered a stroke affecting parts of her brain. After several tests, doctors found a blockage in a major artery in her brain, which led to the headaches and other symptoms. She was treated with medication and blood thinners, and is now being followed up to monitor her recovery."
462,"A 71-year-old man was referred to our hospital from his primary physician because of suspected LC. Computed tomography (CT) revealed a primary tumor in the right middle lobe and metastases in the lymph nodes (hilum of the right lung, bifurcation of the trachea, and left side of the neck), brain, both adrenal glands, and bones (Fig. ). Pathological examination of a needle biopsy of the left cervical lymph node clearly revealed adenocarcinoma. Immunohistological findings showed positive staining of cytokeratin 7 and thyroid transcription factor 1 (TTF-1) and negative staining of cytokeratin 20. Therefore, we definitively diagnosed pulmonary adenocarcinoma and multiple metastases. His LC was categorized as stage IVB (T2a N3 M1c) according to the TNM classification [].\nThis patient received chemotherapy with carboplatin, paclitaxel, and bevacizumab. One month later, he presented with right lower quadrant pain when he visited our hospital to receive his scheduled chemotherapy. His serum level of C-reactive protein was clearly increased at 11.67 mg/dL, although his white blood cell count was within the normal range. Enhanced CT findings showed an enlarged appendix and fluid collection near the distal appendix (Fig. ). A diagnosis of AA was made, and laparoscopic appendectomy was promptly performed. A swollen appendix and pus collection were clearly observed during surgery (Fig. ). Laparoscopic survey of the abdominal cavity revealed no additional metastases (e.g., appendiceal tumor or peritoneal dissemination).\nHistological analysis by hematoxylin eosin staining revealed invasive adenocarcinoma in the appendix that infiltrated the mucosal, submucosal, and muscular layers. Positive immunostaining of TTF-1 indicated that the appendiceal metastasis was from pulmonary adenocarcinoma, not a primary appendiceal malignancy (Fig. ).\nThe postoperative course was uneventful, and the patient was discharged on postoperative day 7. The patient’s pulmonary internist resumed continuous chemotherapy after surgery.","A 71-year-old man was diagnosed with lung cancer that had spread to other parts of his body, including his lymph nodes, brain, and adrenal glands. After experiencing abdominal pain, tests revealed an infection in his appendix that was also caused by the same type of lung cancer. Surgeons removed the infected appendix, and examination of the appendix showed it was also cancerous. The patient is now continuing chemotherapy and recovering well at home."
463,"Our patient was a 19-year-old primigravida Greek Pomak woman who was recently diagnosed as having hereditary AT deficiency. She had been previously referred for thrombophilia testing, due to a history of two first trimester pregnancy losses. She had no history of deep vein thrombosis (VTE), but her mother had suffered from postpartum VTE at a young age. Her basic screening for thrombophilia was normal: protein C, free protein S, AT, activated protein C (APC) resistance, lupus anticoagulant, FV Leiden, factor II (FII) G20210A mutation, fasting serum homocysteine, anticardiolipin antibodies, anti-beta-2 glycoprotein 1 (anti-b2 GP1) antibodies; however, she showed an AT activity of 51% (normal range 70–120%; chromogenic Liquid Antithrombin; Instrumentation Laboratory, Milano, Italy). Her mother and two out of three of her siblings were also found to have AT deficiency, so a diagnosis of hereditary heterozygous AT deficiency was established. AT antigen testing was not available so we cannot classify the disorder as type I or II deficiency.\nThree months after diagnosis she was pregnant again. We decided to manage her with adjusted dose of low molecular weight heparin (LMWH) throughout pregnancy due to the high incidence of fetomaternal complications in this disorder and our patient’s history of miscarriages. She was monitored monthly with d-dimers, AT activity, and anti-Xa measurements (liquid anti-Xa, one-stage chromogenic assay with no exogenous AT; Instrumentation Laboratory, Milano, Italy). After titrating tinzaparin dose, using chromogenic anti-Xa activity, she continued with a daily dose of 14,000 IU applied subcutaneously. With this dose the peak anti-Xa activity ranged between 0.46 and 0.79 IU/ml during the first 6 months of pregnancy, which was in great discordance with her body weight of 50 kg. This was attributed to the well-known heparin resistance phenomenon in patients with AT deficiency. During the last trimester anti-Xa activity dropped and ranged between 0.23 and 0.45 IU/ml. An attempt to raise the heparin dose did not result in significant increase in anti-Xa, but further decreased the AT levels; so we resumed the 14,000 IU dose. Throughout pregnancy d-dimers were low (93–317 μg/L) and AT was 33–35% until the 28th week, rising to 46–57% thereafter. Her pregnancy was uneventful. A cesarean section was scheduled at the 39th week due to breech presentation of the fetus. The last tinzaparin dose was given 24 hours before surgery. Prior to delivery, AT activity was 54%. In order to overcome the risk of thrombosis, 3 hours before delivery she received Kybernin P (human AT III concentrate; CSL Behring) prophylactically at a dose of 3000 IU intravenously administered, calculated according to current recommendations as follows: concentrate dose = (120% − current AT(%)) × body weight (kg) divided by 1.4. She proceeded to have general anesthesia and received tinzaparin subcutaneously 8 hours later at the conventional dose of 4500 IU. She delivered a healthy, 2610 g weight, small for gestational age male baby, who was also tested a year later and was found to have normal AT levels. There was no increased bleeding during and after caesarean section. Her AT level 2 hours after infusion was 112% and trough level the next day was 65%. Functional AT levels were measured daily prior to each dose of AT and levels were maintained between 60 and 100% by using approximately 66% of the initial AT dose or 2000 IU. We planned to administer AT for 6 days, according to various literature data, but in total she received AT for 4 days because she developed an allergic reaction after the fifth dose, so AT was discontinued and tinzaparin increased at the prior dose of 14,000 IU/day. She was discharged 6 days after delivery without complications and tinzaparin 4500 IU daily was continued for 6 weeks postpartum.","This woman was diagnosed with a rare condition called hereditary AT deficiency, which affects her blood clotting ability, and it runs in her family. Because of this, and a history of miscarriages, she was carefully monitored during her second pregnancy with adjusted doses of low-molecular-weight heparin. To ensure she didn't develop blood clots, she received a special medication called Kybernin P before and after her C-section. Overall, her pregnancy was successful, and she delivered a healthy baby, with careful monitoring of her blood clotting levels throughout."
464,"A 56-year-old Chinese Han female presented to Shanghai Dermatology Hospital in 2016 with symptoms of erythematous plaques and pain over her left upper limb for 2 days and foreign object sensation in the throat when swallowing for 3 days. The patient had a 6-year history of leprosy. She was diagnosed with BB in 2011 and received multidrug therapy (MDT) (600 mg of rifampin and 300 mg of clofazimine monthly; 100 mg of dapsone and 50 mg of clofazimine daily) for 1 year, resulting in a clinical cure in 2012.\nT1LR were initially considered, followed by treatment with 20 mg/day of methylprednisolone given orally. Two days later, the patient’s symptoms were aggravated, with neck muscle tension and difficulty in opening her mouth, and the erythematous plaques had spread over most of her left upper limb.\nOn physical examination, the patient had a normal blood pressure and pulse with a temperature of 37.8 °C, but she displayed shortness of breath. Her facial expressions included a wry smile and trismus, with the corners of her mouth pulling outward and upward, and she had difficulty speaking. The patient’s abdominal muscles were too stiff for palpation of the patient’s liver and spleen. Persistent stiffness was found in the neck and four limbs, together with opisthotonus and occasional paroxysmal spasms. Anesthetic erythematous plaques were observed over her left upper limb (Fig. ). The bilateral ulnar nerves and right common peroneal nerve were thickened and exhibited tenderness. The patient exhibited right foot drop, atrophy of the extensor of the right lower leg, and an ulcer on the right foot. Laboratory examinations showed a white cell count of 12 000/mm3 (reference value: 3690–9160/mm3) and neutrophils 81% (reference value: 50–70%). Her liver and renal function tests were normal. Slit skin smears showed the presence of acid-fast bacilli ranging from negative to a score of 1+ at 6 different sites. According to her medical history and clinical symptoms, she was diagnosed with tetanus and BT accompanying T1LR.\nThis patient was given the following: 100 000 IU tetanus antitoxin in a 500-ml 5% glucose-saline intravenous infusion daily; 200 000 U penicillin in an intramuscular injection four times a day; and 200 mg of hydrocortisone in a 250-ml 5% glucose intravenous infusion daily. Additionally, 10 mg/day of diazepam and 50 mg/day of phenergan were given by intramuscular injection. The patient was hospitalized in a dark, quiet room to reduce light stimulation and prevent spasms. In addition, a dental pad was placed in the oral cavity to prevent the patient from biting her tongue. The ulcer on her right foot was debrided every day, followed by rinsing with 3% hydrogen peroxide and injection of 20 000 IU tetanus antitoxin around the wound. The paroxysmal spasticity and intensity of the spasms started to decrease after 5 days. Twelve days later, she was discharged from the hospital in stable condition. She was followed up in our outpatient department and treated with MDT and 40 mg/day of prednisone orally with taper to prevent T1LR for 4 months; the erythematous plaques and neuropathic pain eventually subsided.","A 56-year-old woman with a history of leprosy was admitted to the hospital after experiencing painful skin rashes on her arm and difficulty swallowing. She was also struggling with muscle stiffness, spasms, and difficulty speaking. Tests revealed she had tetanus and a related infection, along with a worsening of her leprosy. She received treatment with antibiotics, tetanus antitoxin, and medications to manage her symptoms, and her condition gradually improved over several weeks."
465,"A 39-year-old Italian man presented to our department suffering from headache and nausea over the past months. CT and MRI revealed an ill-defined, 39 × 37 × 29 mm (anterior-posterior×transverse×cranial-caudal) tumor. On CT, the lesion presented as slightly hypointense with poor contrast enhancement. On MRI, a hyperintensity on fluid-attenuated inversion recovery (FLAIR) sequence and on T2-weighted imaging was detected. On T1-weighted imaging, the lesion showed a hypointensity. The lesion showed poor contrast enhancement of the right cerebellar hemisphere without an obstructive hydrocephalus on T1-weighted images with contrast enhancement (Fig. ).\nOur patient did not suffer from any other comorbidities; he had not undergone any surgeries. He did not use medication. He had never consumed alcohol, smoked tobacco, or used other drugs. He is married, has two children, and works as a cook in a family owned restaurant. Similar cases were not reported in his family; no relatives had suffered from a tumor in the past. Neurological examinations at admission showed no sensorimotor deficits, no cranial nerve deficits, normal response of his reflexes, and normal standing and walking abilities without any unstableness. Blood pressure, pulse, temperature, and laboratory findings (that is, complete blood count, liver function, renal function, and C-reactive protein) were within normal range.\nSurgery was indicated and written consent was obtained. Surgery was performed under general anesthesia with our patient in a semi-sitting position. Monitoring was done with somatosensory and muscle-evoked potentials. A right-sided suboccipital craniotomy was performed. On intraoperative examination, we observed a glassy gray-black tumor that was not well demarcated from the surrounding tissue. Piecemeal tumor removal was performed by microsurgical technique using the Sonoca 300 (Söring GmBH, Quickborn, Germany).\nA postoperative CT scan revealed a regular finding without hydrocephalus and hemorrhage. Our patient was observed in our neurosurgical intensive care unit for one night and was transferred to a general ward the following day without neurological deficiency. Postoperative MRI, which was performed 48 hours after surgery, showed no residual tumor. His postoperative course was uneventful. He received no adjuvant treatment and there has been no evidence of tumor recurrence over a period of 15 months (Fig. ). A neurological examination at last follow-up, 15 months after surgery, revealed no neurological deficits. The preoperative nausea and headache he experienced had stopped.\nOn histopathological examination, hematoxylin and eosin-stained paraffin sections showed predominantly small to moderately cellular tumor growing compactly, sometimes diffuse, infiltrating the surrounding cerebellar tissue. Tumor cells contained mainly light eosinophilic, sometimes clear, cytoplasm and round to oval nuclei and smaller nucleoli. Some tumor cells showed an astrocytic differentiation. Furthermore, around 10% of the tumor area comprised focal lipidized cells (Fig. ). No significant mitotic activity, < 1 mitosis/20 high-power field (HPF), and no necrosis were observed. In immunohistochemical analysis NeuN (Fig. ) was detected in 80% and synaptophysin (Fig. ) was detected in 30% of the non-lipomatous cells. Tumor cells were negative for neurofilament (Fig. ; surrounding CNS tissue stained positive) and chromogranin A. Glial fibrillary acid protein (GFAP) was observed in 20% of the tumor cells (Fig. ). Ki-67/MIB-1 proliferation index (Fig. ), as determined by nuclear MIB1 monoclonal antibody staining, was around 2% (Fig. ).","This 39-year-old man came to the hospital because of headaches and nausea that he had been experiencing for months. Doctors found a small tumor in his right cerebellum through CT and MRI scans. The tumor was carefully removed during surgery, and follow-up scans have shown no sign of it returning. He is now recovering well and his headaches and nausea have stopped."
466,"A 60-year-old white unmarried man with chronic schizophrenia fell to the floor and was unable to get up or walk. When examined he had an asymmetrical smile and apparent paresis of his left leg. He was hospitalized with a tentative diagnosis of stroke.\nFrom his relatives we learned that he had grown up in a village on the Norwegian coastline as the fourth of five siblings. He did not excel at school, and started at an early age to work in the local fishing industry. He held the job until at the age of 30 he moved to another part of the country. There he worked as a custodian at a hotel. At age 37 he went back to his home village to live close to his compassionate family of origin. He was then employed as an assistant custodian (supported employment) in the local fishing industry until he was 56-years old. He was treated for psychotic symptoms on-and-off from his mid-twenties. He was not diagnosed as having schizophrenia until he was 40-years old. Since then he received out-patient psychiatric treatment until the present illness occurred. At the age of 55, diabetes mellitus type 2 was diagnosed. Osteoporosis was diagnosed 2–3 months prior to the present illness.\nThis was the first time he had been hospitalized. His family members said that he had had swallowing problems, difficulties with speech, and unsteady gait for the last 4–5 years. This information was corroborated by our patient’s general practitioner. He had deteriorated physically over the last 3–4 months with increased fatigue. He had developed general inertia and was easily exhausted after a short period of physical labor. He had developed hypersomnia, with 10–12 hours of sleep per night, a weight loss of 4–5 kg, and an unsteady gait. To descend the stairs he preferred to sit on his buttocks and slide down the staircase one step at a time until reaching the lower floor. He had been a heavy tobacco smoker for several decades. His alcohol use was modest.\nHis main psychiatric symptoms before being hospitalized were social withdrawal and delusions about several small persons, the size of dolls, attached to his body. Furthermore, he had auditory and visual hallucinations. He was very reluctant to talk about the contents of the, probably imperative, auditory hallucinations. Antipsychotic medication, risperidone tablets, was first started in 1997. A year later the medication was switched to olanzapine tablets. The dosage varied between 7.5 and 15 mg per day without any objective or subjective side effects. There had been no unambiguous extrapyramidal side effects.\nOn physical examination, he was alert and orientated, but in some pain in his left hip and knee. He was afebrile with a body temperature (ear) of 37.4 °C, blood pressure was 136/83 mmHg, he had a regular pulse rate at 82 per minute, and oxygen saturation (SaO2) was 97%. Auscultation of his carotid arteries revealed no bruits. His heart rhythm was regular without any pulse deficit. There were no heart murmurs. A lung examination was suboptimal as inspiration was weak. It was possible that some crackles could be heard bilaterally at the base of his lungs. A neurological examination revealed impaired tongue wiggling when tested for quick side-to-side movements, dysarthria, symmetrically reduced muscle force (5−/4+) in his upper extremities, reduced force in his left leg (not quantified), and asymmetrical plantar reflexes (downward movement on the right side, indifferent on the left). His regular medication before admission was olanzapine tablets 12.5 mg/day (7.5 mg + 5 mg), metformin tablets 500 mg three times a day, calcium/cholecalciferol 500 mg/400 IU tablets two times a day, and paracetamol 500 mg two times a day. His complete blood count was normal: hemoglobin (Hgb) was 14.0 g/dL, hematocrit was 0.44, his white blood cell count was 8.2 × 109/L, his platelet count was 275 × 109/L, his neutrophil count was 5.6 × 109/L, his lymphocyte count was 1.5 × 109/L, his monocyte count was 0.7 × 109/L, his eosinophil count was 0.4 × 109/L, and his basophil count was < 0.1 × 109/L. The only pathological tests from the chemistry panel were a low creatinine level of 59 μmol/L (reference range, 60–105), a high glucose level of 10.0 mmol/L (reference range, 4.0–6.0), a high glycated hemoglobin (HbA1c) level of 7.5% (reference range 4.3–6.1), a high alanine transaminase level of 94 U/L (reference range 10–70), and a high alkaline phosphatase level of 130 U/L (reference range 35–105). Urine, collected from a urine catheter on the day of admission, was delivered immediately to the microbiology laboratory in the same hospital building, and cultivated. There were > 100,000 bacteria per ml, identified as Staphylococcus epidermidis, probably representing contamination. There was no bacterial growth in a repeat urine test taken 3 days later.\nThe day after admission a left dislocated hip fracture was identified. This information, in combination with a normal cerebral magnetic resonance imaging (MRI) and disappearance of his facial asymmetry, caused the clinicians to reject the stroke hypothesis. His hip fracture was operated on the following day. Antibiotic medication (cefalotin 2 grams administered intravenously) was given twice: at the beginning and at the end of the surgery. Blood cultures with two sets, each consisting of one aerobic and one anaerobic bottle (Virtuo® blood culture, bioMérieux), were taken from his antecubital veins the day after admission. The cultures were brought to the microbiology laboratory immediately for further cultivation. No bacterial growth was seen.\nAlthough the hip surgery was technically successful, it was not possible to physically mobilize our patient. The 12th day after hospital admission, a psychiatrist was consulted as our patient suffered from clouding of consciousness, episodic agitation, and increased anxiety. Olanzapine tablets were increased from 12.5 mg to 15 mg per day. On day 15 he was transferred to an acute psychiatric ward as it was considered the appropriate place for further treatment. This was unsuccessful as he deteriorated physically. As a consequence, he was returned to the intensive care unit. He was diagnosed as having bilateral lung emboli and suspected sepsis. New blood cultures were taken. Cefotaxime administered intravenously, 1 g three times a day, was started on day 18. Two days later, the cefotaxime dosage was increased to 2 g three times a day. The blood cultures revealed no growth.\nUnfortunately, from now on a clinical downhill course followed. Our patient got aspiration pneumonia and was unable to swallow food or fluids. It was decided to stop further oral nutrition (fluids, food, pills) in an attempt to prevent further aspirations to his lungs. Instead, total parenteral nutrition was started. The tentative neurological diagnoses being discussed at this point were motor neuron disease, diabetic neuropathy, and extrapyramidal side effects of antipsychotics.\nOn the 20th day, a neurological examination found only slightly reduced muscle strength (grade 4–4+) for adduction and abduction of his shoulders bilaterally and a tendency to lead pipe rigidity in his wrist joints. No conclusive diagnosis was made. Three days later (day 23), a repeat neurological examination by another neurologist showed essentially the same clinical picture. The lead pipe rigidity in his upper extremities lessened significantly, almost to normal muscle tone, when our patient managed to relax. However, his wrist joints were strongly flexed and his hands tightly clenched to the bed rails bilaterally. Still, no conclusive neurological diagnosis was made. A videofluoroscopic swallow study and an assessment by a speech therapist were suggested but never performed because he did not regain the ability to cooperate.\nThe 23rd day was also the time for the second psychiatric consultation at the intensive care unit. Our patient was awake with a clear consciousness. He was oriented for time, place, and situation. Rapport was satisfactory. He was relaxed when engaged in a conversation or otherwise taken care of in his room; when left alone, he was stressed and obviously not at ease. He denied hallucinations. However, his dysarthric speech was a hindrance to an adequate psychiatric evaluation. All in all, there had been some improvement in his psychiatric state since the first psychiatric consultation on the 12th day. Haloperidol tablets, sporadically used as on demand medication to calm him, were discontinued. On the 24th day, metronidazole 500 mg administered intravenously was added to the treatment. Both antibiotics were continued through the 28th day, and then terminated.\nOn the 38th day the neurologist found that the electromyography (EMG) and nerve conduction studies showed changes consistent with a sensorimotor polyneuropathy affecting his lower extremities. There was no EMG pathology in his upper extremities. The EMG/neurography findings were not compatible with motor neuron disease or acute polyneuropathy. No causal explanation for his dysarthria and dysphagia was found.\nThe one symptom that he confirmed on all psychiatric consultations (that is, on day 12, 23, 31, 32, 35, and 42 after admission) was anxiety. This was a generalized anxiety with fluctuating intensity that responded satisfactorily to diazepam 2–2.5 mg intravenously administered 4–5 times a day. The anxiety stressed him much more when he was left alone in his room. Having a nurse or a family member nearby calmed him significantly. Apart from adding antibiotics for pneumonia, the regular medication was re-evaluated throughout the course. Antidiabetic treatment was switched from metformin tablets to insulin in order to improve his blood glucose level. The antipsychotic medication (olanzapine) was reduced to 10 mg per day as we suspected the drug to be a cause of his hypersomnia and fatigue. Despite a range of efforts from specialists in orthopedic surgery, hand surgery, anesthesiology, pulmonology, neurology, and psychiatry, our patient did not recover. He died 44 days after being admitted to hospital.\nThe focus of this case presentation, however, is on the unusual observation of his clenched hands. Thus, we have to step back. During the second psychiatric consultation on day 23 after admission, he was observed clutching his hands onto the side rails of the bed. With some assistance he managed to let go of the rails, but his hands were still tightly clenched. When asked if he could extend his four ulnar fingers he only managed a slight active extension of them, just enough to let the doctor inspect and palpate his palms. On the four later psychiatric consultations, he no longer held onto the side rails. Both hands from now on lay on the duvet with his wrists in palmar flexion, the left one more strongly than the right one. His four ulnar fingers were fully flexed giving the impression of clenched fists (Fig. ).\nDuring these later examinations, he was still unable to open his hands voluntarily. Neither could he extend his wrists. On testing for passive extension of the wrist joints, proximal and distal interphalangeal joints, and metacarpophalangeal joints of his four ulnar fingers only slight extension was allowed for. His thumbs, however, could be fully extended, although with some resistance.\nDuring the extension of his four ulnar fingers there was a resistance that increased proportionally to the force applied by the examiner, giving it an “elastic feel.” Furthermore, there was a non-pitting swelling on the dorsum of his left hand and lower arm. Passive extension of his fingers allowed for examinations of his palms. There was no visible or palpable sign of Dupuytren’s contracture on either side. Neither were there signs of traumas to the hands. However, he had small wounds in the left fossa cubiti caused by syringes and peripheral venous catheters associated with blood test and intravenous infusions. This could have been the culprit for the abovementioned swelling.\nThe neurological work-up revealed no plausible organic pathology.\nAttempts to treat the clenched hands were obsolete as our patient was unable to cooperate in any way. However, he accepted a palliative application of hand orthoses that counteracted the wrist flexion to some degree during the last week of his life. According to information from family members he had had normal function of his hands prior to this hospital stay. They had a theory that his holding his hands clutched on the side rails was his attempt to prevent falling or being pulled out of his bed. Our patient himself could not explain why his hands were clenched. He had no pain in his hands, but he confirmed having more or less continuous anxiety during all six psychiatric consultations. Every attempt at mobilization in order to get him out of bed failed as he resisted both verbally, by crying out, and physically.\nOn day 42, a junior doctor at the Department of Hand Surgery responded to a request to examine our patient. After discussing the case with her senior colleagues, the doctor could not conclude on any plausible organic disorder. She recommended putting some insulating material between fingertips and palms to prevent maceration and wounding. She also suggested a repeat neurological examination in case he improved.\nAn autopsy concluded that the cause of death was aspiration pneumonia. In addition, an old infarction was found in the pons and medulla oblongata. Furthermore, there were discrete thickenings of blood vessels and old, small perivascular infarcts consistent with lacunar state in the brain. His relatives had never observed or heard our patient report symptoms compatible with stroke or cerebral insults prior to the current illness course.","This 60-year-old man was admitted to the hospital after a fall and difficulty walking, and doctors initially suspected a stroke. He had a long history of schizophrenia and anxiety, and had tried many medications to manage his symptoms, but none were effective. During his stay, he developed pneumonia and a hip fracture, and his condition worsened despite treatment. Ultimately, he passed away from aspiration pneumonia, and an autopsy revealed signs of small strokes and thickening of blood vessels in his brain."
467,"A 42 year-old woman was referred to the Master of Endodontics of the University of Santiago de Compostela with a chief complaint of a subgingivally fractured permanent maxillary left central incisor as a result of a domestic accident. Her medical history was unremarkable. Clinical and radiographic examinations were conducted. Clinical examination revealed a heavily restored maxillary left central incisor that was tender to palpation (Fig. A,B) and periapical radiograph and a CBCT revealed an oblique crown-root fracture that extended approximately one-third of the root length (Fig. C,D). Radiographic findings showed periapical radiolucencies in the adjacent upper left lateral incisor and canine (Fig. C), both teeth remaining negative to cold testing. The diagnosis was a globulomaxillary cyst, both teeth were root canal treated (Fig. E) and the cyst enucleated (Fig. F).\nIn order to regain the lost biologic width, orthodontic extrusion of the fractured permanent maxillary central incisor was required to move the vestibular fracture line approximately 6 mm above the alveolar crest. For the orthodontic extrusion, brackets were attached from upper right first premolar to upper left first premolar. An extrusion of approximately 6 mm was obtained within 6 months (Fig. A) and the extruded tooth was retained for 6 months. Periodontal surgery was performed to recontour the altered gingival and osseous margins at the end of the retention period. The root canal retreatment was performed and a fiber post was placed using a dual-cure cement. The post core was built up with a composite and the tooth was prepared for a crown (Fig. A). During the time it took to obtain the permanent restoration, the tooth was restored with a temporary crown and an external full mouth tooth whitening was performed before determining the shade of the permanent crown. The ceramic crown was seated to the prepared tooth (Fig. B,C) and the upper right central incisor was restored using composite. Good aesthetics were achieved and the patient reported no problems after 4 years of treatment (Fig. D). Patient’s informed consent was obtained.","A 42-year-old woman came to the clinic because a tooth in her upper jaw had cracked due to an accident. X-rays showed the crack extended into the tooth’s root and caused a small cyst to form in nearby teeth. To fix the problem, the dentist carefully moved the cracked tooth forward, recontoured the gums, and treated the root canals. After four years, the tooth was crowned with a ceramic restoration, and the surrounding teeth were whitened, resulting in a beautiful and healthy smile."
468,"A 47-year-old caucasian female was referred to the Endodontic Clinic of Dental Faculty at the University of Strasbourg). Her medical history found no outstanding findings that would contribute to treatment problems. Clinical examination revealed that the tooth had a MOD composite restoration. This was tender on percussion, and hence the patient reported periodic episodes of spontaneous pain. The periapical radiographic examination, with an orthoradial projection, showed the presence of a single root with an unusual anatomic variation, suggesting a probable endodontic dilacerated anatomy.\nThe endodontic treatment was performed in a single session. After local anesthesia, a rubber dam was placed, and endodontic access was performed with a # C 801L 012 round diamond bur (NTI, Kahla, Germany). The lingual and buccal canal orifices were localized with a START X 1 (DentsplySirona Ballaigues, Switzerland) using the operating microscope (Leika M320).\nDuring all instrumentation steps an aqueous 6% NaOCl solution was used for irrigation.\nIn order to avoid the risk of procedural errors the strategy was was not to use initial manual scouting, but to remove immediately the coronal and middle interferences with initial rotary preflaring to then perform a manual apical scouting of last 2 mm of the root canal.\nAt first, an initial mechanical preflaring was performed with the OneG (Micromega Besançon, France) until just above the first root canal curvature, using an inward and outward movement, without any pressure, and then with TS1 (Micromega Besançon, France) short of 1mm in regarding to the portion of canal preflared with OneG, using an endodontic engine (300 rpm/2 Ncm).\nThanks to the initial preflaring a #10 stainless steel MMC-file (Micromega Besançon, France) scouted the canal up to working length + 0, 5 mm. Length determination was obtained using an electronic apex locator (Root ZX; J Morita Co, Kyoto, Japan).\nAt this point of therapy we took a radiograph to allow subsequent execution of the following steps:\nGlide path until to a full working length in both the buccal and palatal canals with a One G (300 rpm/5 Ncm) instrument.\nShaping canals in minimal invasive way was performed with TS1 (300 rpm/2 Ncm), 25/04, extending until to full length of the buccal canal, until to the merged point for the palatal canal in order to avoid an apical zip and hazardous stress with the endodontic instrument, especially dangerous when navigating second canal curvatures.\nApical gauging: the foramen was gauged introducing a 25/02 NiTi hand file, which was snug at its working length.\nAfter the shaping procedure, in order to assure a three-dimensionally cleaning of the root canal system, an aqueous 17% solution of EDTA flooded into the pulp chamber was activated using a manual-dynamic activation by of some gutta-percha points for 120 seconds in each canal. After rinsing with physiological saline, a solution 6% of NaOCl flooded into the pulp chamber was activated again using a manual-dynamic activation for 120 seconds in each canal.\nThe canal system was then dried using sterile paper points. After having applied a drop of Kerr EWT pulp canal sealer (Kerr, Romulus, MI) with a coated paper point at the entrance of each canal, both canals were filled with Thermafil 2. The final radiographs showed two well-obturated canals of this single rooted maxillary premolar (Fig. ).","A 47-year-old woman came to the dentist because she was experiencing occasional pain in a tooth with a composite filling. The dentist found an unusual shape to the tooth’s root and carefully cleaned and shaped the inside of the tooth using specialized instruments and solutions. The dentist used a combination of rotary and manual techniques to ensure the root canals were thoroughly cleaned and filled. Finally, the tooth was sealed with a special material to prevent future problems, and X-rays confirmed a successful treatment."
469,"A 58-year-old caucasian female was referred to the Endodontics Clinic of the University of Strasbourg Dental Faculty for retreatment of a left maxillary second premolar. Endodontic treatment was performed in two sessions. Findings of a clinical examination revealed that the tooth had a prosthetic metal crown, that was tender on percussion with episodes of spontaneous pain.\nThe periapical radiographic examination, with different angle-shots, showed the presence of only one root with an unusual anatomic variation, and a probable endodontic dilacerated anatomy.\nAfter local anesthesia and crown removal, the walls of the access cavity were reconstructed with SDR composite (DentsplySirona Ballaigues, Switzerland), thanks to the installation of an orthodontic band.\nA rubber dam was placed, the buccal and lingual canals were located with Start X1 using an operating microscope (Leika M320) and the previous perforation was visualized.\nDuring the first session the perforation was treated with biodentine.\nFor this tooth we decided to plane using 2 different shaping techniques in order to compare the two different techniques.\nDuring all instrumentation steps an aqueous 6% NaOCl solution was used for irrigation.\nThe lingual canal was treated with an usual technique:\nmanual scouting\nglide path\nshaping\nIn order to improve the access to the canal, a SX instrument (DentsplySirona Ballaigues, Switzerland),)(300 rpm/5 Ncm) from ProTaper Universal system was used.\nAfter the use of Sx opener a # 08 stainless steel K-file (DentsplySirona Ballaigues, Switzerland),) wasn’t able to go up to the WL, ), falling 8mm short of the WL.\nIn order to facilitate the apical scouting a # 10 K file was used short of 9 mm and # 15 K-file short of 10 mm.\nSo at the second wave # 08 K-file was 7mm short from the WL so again # 10 K file and # 15 K-file were used short of 8 and 9mm, respectively from the WL\nAfter having repeated the scouting sequence numerous times and extensive use of a pre-curved manual K file we ended the scouting step.\nA mechanical glide-path with Proglider DentsplySirona Ballaigues, Switzerland), at working length was performed using an endodontic engine (300 rpm/5 Ncm).\nRoot canal preparation was performed using ProTaper Next X1(DentsplySirona Ballaigues, Switzerland) until to WL and ProTaper Next X2 (operating at 300 rpm and torque of 5 N/cm) until to 2.5 mm shorter from the WL. Then manual 20/02 and 25/02 NiTi hand file (Dentsply/Maillefer) sliding down the glide path up to working length, the shaping procedure finished after 58’ 45’’.\nThe buccal canal was treated with a modern step down-technique without initial manual scouting:\nInitial preflaring above of second curve with in and out movement\nApical scouting\nGlide path\nShaping\nThe initial mechanical preflaring was performed at first with proglider (Dentsplysirona) until to above the first root canal curvature applying an in and out movement, using an endodontic engine (300 rpm/5 Ncm).\nThanks to the initial preflaring using a #10 stainless steel K-file (Dentsply Maillefer) we scouted the canal up to working length + 0. 5 mm. Length determination was taken using an electronic apex locator (Root ZX; J Morita Co, Kyoto, Japan).\nA mechanical glide-path with proglider at working length was performed using an endodontic engine (300 rpm/5 Ncm).\nRoot canal preparation was performed by preparing the root canals to working length with a ProTaper Next X1, a Pro Taper Next X2 (operating at 300 rpm and torque of 5 N/cm) 2.5 mm shorter from the WL. After manual 20/02 nd 25/02 NiTi hand file (Dentsply/Maillefer) sliding down the glide path up to working length. The shaping procedure was finished after 6’ 17’’.\nAfter the shaping procedure, in order to assure a three-dimensionally cleaning of the root canal system, an aqueous 17% solution of EDTA was flooded into the pulp chamber was then activated using a manual-dynamic activation by a gutta-percha point for 120 seconds in each canal. After rinsing with physiological saline, a solution 6% of NaOCl flooded into the pulp chamber was activated using a manual-dynamic activation for 120 seconds in each canal.\nThen the canal system was dried using sterile paper points. After having applied a drop of EWT pulp canal sealer (Kerr) with a coated paper point in the entrance of each canal, both sites were filled with Thermafil 25. The final radiographs showed two well-obturated canals, with some lateral canal, of this single rooted maxillary premolar (Fig. ).","You had a previous root canal treatment on a second molar that wasn't successful. We found an unusual shape to the root and a perforation during the procedure. We carefully repaired the perforation and completed the root canals using modern techniques and specialized instruments. We then used a combination of manual and powered files to thoroughly clean and shape the canals, ensuring they were properly filled. Finally, we sealed the canals to prevent future infection, and took X-rays to confirm the treatment was successful."
470,"An 8-year-old female spayed Dachshund weighing 7.45 kg was presented to the University of Tennessee Veterinary Medical Center with a large, firm, bilaterally symmetrical, painful protrusion in the perineal region that had appeared acutely 2 days before presentation. The dog had a one-month history of dysuria and pollakiuria that was suspected to be a urinary tract infection; when the dog did not respond to amoxicillin-clavulanic acid, a free catch urine sample was submitted for culture. A few colonies of a Staphylococcus organism were grown from the culture, and treatment was changed to marbofloxacin. The dog also had a 2-day history of tenesmus associated with the appearance of the perineal protrusion. The dog had been spayed 2 years prior to presentation after multiple types of unsuccessful breeding.\nPhysical exam abnormalities revealed an otherwise normal dog with an approximately 5 × 4 × 2 cm firm, painful protrusion on midline of her perineal region, ventral to the anus. A fluid-filled structure was noted ventral to the rectum and distal colon on digital rectal exam; no pelvic diaphragm weakness was palpable, making perineal hernia unlikely. Serum chemistry and complete blood count were unremarkable.\nThe dog was anesthetized for computed tomography (CT) and vaginoscopy. On abdominal and perineal CT (Figures –), a large, tubular, fluid-filled structure was noted, measuring 4 cm in diameter at its widest point and 16.3 cm long. The structure extended from the perineum cranially, blindly terminating at the level of L5. It occupied the majority of the pelvic canal and much of the caudal abdomen, compressing the colon and rectum dorsally and the urethra ventrally and displacing the bladder within the abdomen to the left of midline. There was also mild dilation of both renal pelves and proximal ureters, which could have been secondary to pyelonephritis or backpressure from the dilated urinary bladder. The structure was interpreted to be the vagina and uterine stump and, based on results of physical exam, blood work, and CT, a hydrocolpos secondary to imperforate hymen was suspected. The ratio of vestibulovaginal junction width to maximal vaginal width was 0.825, ruling out vestibulovaginal stenosis.\nOn digital vaginal exam, a large, thin-walled, fluctuant structure was palpably filling the vaginal lumen just cranial to the urethral meatus. Digital pressure was used against the wall of the structure to perforate it; approximately 200 mL of brown, mucoid fluid was immediately released from the vulva. A sample was collected for cytology and culture. Within a minute, the drainage had ceased, and vaginoscopy was performed, revealing remnants of an imperforate hymen just cranial to the external urethral orifice. No fluid remained in the distended vagina, and no other abnormalities were seen. The remainder of the hymen was removed endoscopically with grasping forceps and submitted for histopathology.\nOn cytology, the fluid was moderately cellular, primarily consisting of neutrophils with rare clusters of epithelial cells. No bacteria were seen on cytology, and no organisms were cultured from the fluid. Histologically, the tissue was composed of a dense band of fibrous connective tissue covered by a nonkeratinized stratified squamous epithelium. It contained a medium-sized blood vessel and several smaller blood vessels that were surrounded by rare neutrophils. Findings were consistent with the hymen of a nonpregnant mammal. After recovery, the dog's dysuria, pollakiuria, and perineal swelling resolved. The dog was discharged with instructions to complete the previously prescribed course of marbofloxacin. At follow-up 2 months later, the dog was clinically normal.","This 8-year-old Dachshund came to the vet with a painful swelling in her rear end and difficulty urinating. Tests showed she had a bacterial infection and a blockage in her vagina, likely due to an imperforate hymen (a missing opening). The vet performed a CT scan and vaginoscopy to confirm the diagnosis and successfully removed the blockage, relieving the swelling and urinary problems. After treatment, the dog was healthy and discharged home with instructions to finish her antibiotics."
471,"Case 1 was a 28 kg, 11-year-old, neutered male Border Collie that presented with malaise, inappetence, and external signs of internal bleeding. Ultrasonography demonstrated a large renal mass and evidence of metastatic spread to the lungs. A biopsy of the renal mass was performed, and histological examination revealed sarcoma of endothelial origin.\nUnder the care of a licensed veterinarian, this dog was entered into our research protocol employing high dose oral DHEA (60 mg/kg/day) and ubiquinone (0.1 mg/kg/day), in divided daily doses. A dramatic improvement in quality of life (appetite, playfulness) occurred within a few days of initiation of the protocol. The patient was reexamined at biweekly intervals by his veterinarian, and complete blood chemistry panels were routinely performed, without identification of significant abnormalities. By one month of treatment, repeat ultrasonography demonstrated no further growth of the renal mass, that is, stable disease. However, the dog developed a generalized inflammatory condition that involved the skin, eyes, and nasal passages. Skin lesions ranged from nonpruritic maculopapular rash to urticaria, erythema nodosum, and purpura. The oral mucosa was inflamed, and uveitis was prominent. There was also episodic fever and an apparent increase in the patient's arthritis. Because the dog's owner had previously fed him large amounts of vegetable matter rich in phytates, the inflammatory reaction was originally postulated to be due to zinc deficiency caused by phytate sequestering of this critical metal. Zinc deficiency is known to produce symptoms very similar to those observed in this case. However, zinc supplementation did not improve his condition, and alternative causes for the inflammatory reaction were sought.\nBased upon our earlier in vitro work [, ], we considered the possibility that inhibition of the mevalonate pathway might be the cause of the autoinflammatory reaction observed. This proved to be the case. Administration of oral, encapsulated geraniol (60/mg/kg/day) was followed by a rapid clearing (3.5 days) of all lesions. This dog survived 315 days from original diagnosis, with pulmonary metastasis being the cause of death.","This 11-year-old dog was diagnosed with a serious cancer in his kidney that had spread to his lungs. His owners started him on a special treatment plan with DHEA and ubiquinone, which dramatically improved his energy and appetite. However, the treatment then caused a widespread inflammatory reaction affecting his skin, eyes, and nose. After further investigation, it was discovered that the treatment was interfering with a key pathway in his body, and a new medication, geraniol, quickly resolved the inflammation, allowing the dog to live for several months longer."
472,"Case 2 is a four-and-one-half-year-old, 28.4 kg, intact female Doberman pinscher who presented with lameness in her rear right leg in November of 2013. At that time, she was diagnosed with decreased conscious proprioception in the right hind limb. The patient was subsequently referred to the Oregon State University College of Veterinary Medicine in October of 2015 after presenting with non-weight bearing lameness and muscle atrophy on the right hind limb. Radiology of the hips revealed periosteal reaction on the right ischiatic table with a mass-like effect in the adjacent musculature. Ultrasound of the musculature adjacent to the right ischium showed an intramuscular mass. A fine needle aspirate of the mass was performed and confirmed a soft tissue sarcoma. Amputation followed by traditional chemotherapy was recommended, which was declined by the owners of this dog. The patient's hepatorenal values were also noted to be consistently elevated, which would potentially have complicated traditional chemotherapy.\nThis dog was entered into our G6PD inhibition protocol in October of 2015 and was treated daily with 60 mg/kg/day DHEA and 0.1 mg/kg/day ubiquinone. During treatment, an acute inflammatory reaction involving the eyes was noted (). This was followed by multiple inflammatory cutaneous lesions on the legs, foot pads, and trunk (). These lesions closely resembled those observed in Case 1. Additionally, this dog had intermittent episodes of fever and an apparent increase in arthralgia. Upon administration of oral geraniol (60 mg/kg/day in divided doses for seven days), all symptoms completely subsided. Of note, posttreatment radiographs showed complete tumor regression (). As of May 2017, this patient is alive and well with no evidence of tumor recurrence or recurring inflammation. Incidentally, she became pregnant during high dose DHEA treatment, subsequently delivering five healthy puppies, demonstrating that G6PD inhibition is surprisingly nontoxic to the developing fetus.\nBesides the similarity in gross appearance of the skin lesions, histologically both cases showed a neutrophilic exudate, and numerous bacteria (cocci or coccobacilli) were visible in surface keratin and hair follicles. There was a diffuse infiltrate of plasma cells, lymphocytes, neutrophils, macrophages, and eosinophils and, in Case 1, clear hypertrophy of dermal sebaceous and apocrine glands. Additionally, both cases responded to geraniol with clearing of the autoinflammatory lesions. In subsequent studies, it was demonstrated that simultaneous administration of geraniol or other terpenes with DHEA could prevent the formation of such autoinflammatory lesions.","This dog was first diagnosed with a hind leg problem in 2013 and then again in 2015, leading to a biopsy that revealed a soft tissue sarcoma. After the owners declined traditional treatment, the dog was given a new treatment plan involving DHEA and ubiquinone, which surprisingly led to inflammation and skin lesions similar to a previous case. The dog responded completely to geraniol, and the tumor disappeared, and the inflammation resolved. Remarkably, the dog remained healthy and even became pregnant with five puppies while receiving this treatment."
473,"A six-year-old female boxer dog presented with a 13-month history of progressive and mildly pruritic skin lesions. The dog was current on vaccinations and deworming and was being fed on dry commercial food of high quality. Previous treatments included amoxicillin plus clavulanic acid (20 mg/Kg/12 h), cefalexin (25 mg/Kg/12 h) alone or in combination with prednisolone (0.5 mg/Kg/24 h for 1 week and then every other day) for a period of approximately 3 to 4 weeks each, but of no avail. The owner also reported that the dog had intermittently been experiencing nonambulatory lameness on the right front leg.\nPhysical examination of the dog upon admission revealed no abnormality. On dermatological examination, 9 ulcerated and nonulcerated skin nodules were observed, ranged from 1 to 7 cm in diameter, and distributed mainly over bony prominences of distal extremities and digits of the front legs (), left elbow, and right hock. Other skin lesions included footpad hyperkeratosis, crusting, depigmentation, and erosions ().\nAt that time the main differentials included infectious or sterile nodules and neoplasia.\nFine needle aspiration (FNA) cytology made from material obtained from nonulcerated skin nodules revealed pyogranulomatous inflammation and numerous mainly extracellular round-shaped organisms, ranging from 20 to 30 μm in diameter, most containing 2 spores of approximately 10 μm in diameter. A tentative diagnosis of systemic mycosis was made. Culture of FNA material in dermatophyte test medium (DTM) was performed at room temperature. Furthermore, skin biopsies were obtained from intact nodules (the owner refused footpad lesions biopsy) along with blood and urine samples for further laboratory workup and serology. Survey thoracic and abdominal radiographs were also taken but were unremarkable.\nHematology, serum biochemistry, and urinalysis did not display any abnormalities and serology (snap ELISA, IDEXX®) for all of Leishmania infantum, Ehrlichia canis, and Anaplasma phagocytophilum antibodies and Dirofilaria immitis antigen was negative. After 4 days of incubation, smooth, creamy, yeastlike colonies were grown on DTM. Light microscopy of lactophenol cotton blue slide preparations made of these colonies revealed round sporangia containing spherical sporangiospores similar to those of P. wickerhamii (). P. zopfii cells are oval or cylindrical in shape, producing sporangia of larger diameter (15–25 μm) containing up to 20 sporangiospores. In contrast, P. wickerhamii cells tend to be round, forming sporangia (7–13 μm) containing up to 50 spherical sporangiospores [].\nHistopathology revealed nodular-to-diffuse, pyogranulomatous dermatitis and panniculitis (with lymphocytes, plasma cells, macrophages, and neutrophils) with numerous elements exhibiting Prototheca spp. morphology; their cell wall stained vividly purple with periodic acid Schiff (PAS) stain () and most of microorganisms were extracellular, either single or more often in groups, with only a few seen to be phagocytosed.\nApproximately 1 mm3 of culture material was used for DNA isolation, by employing the QIAamp Mini Kit (QIAGEN, Hilden, Germany), and following the manufacturer's instructions. A portion of the 28S rRNA gene was amplified by using already published primers []. The band was excised from the gel and DNA was isolated using the DNA Isolation Spin-Kit Agarose (AppliChem, Darmstadt, Germany). The isolated DNA was subsequently sequenced with the PCR primers; PCR produced a ~350 bp band. As sequencing of the complete length of PCR product was not possible, a 77 bp sequence was obtained by employing the U2 primer. Beyond that fragment the double peaks were indicative of the presence of more than 1 strain. Similar sequences were searched in the GenBank with the aid of the Web interface of Blast software which returned 9 of these sequences that belonged to Prototheca wickerhamii strains; the higher similarity applied to GenBank number AB183198 sequence (). This result confirmed the diagnosis of cutaneous protothecosis due to Prototheca wickerhamii.\nAs no treatment guidelines are available, the patient was treated with oral fluconazole (10 mg/Kg twice a day), based on reported agents likely to be most useful against Prototheca species such as amphotericin B (AMB), fluconazole, itraconazole, and possibly terbinafine []. Although significant clinical improvement was witnessed in footpad lesions after one month on fluconazole, this treatment regimen did little to slow the progression of skin nodules, because Prototheca organisms were found on cytology. At that time fluconazole administration was withdrawn and AMB was administered twice weekly as a subcutaneous infusion using a protocol developed to treat canine cryptococcosis []. Specifically, 0.5 mg AMB/Kg/sc per dose was administered twice weekly in 500 mL of 0.45% NaCl/2.5% dextrose fluids. The dog was given concurrently itraconazole (5 mg/Kg/per os, once daily). Due to nephrotoxicity, AMB was withdrawn after 7 infusions and the patient is still being treated with itraconazole alone for about six months. However, although skin nodules have not been improved with this treatment regimen, footpads remain close to normal.","This dog has been dealing with skin problems for over a year, including itchy bumps and changes to their skin. After tests, the vet discovered a rare fungus called Prototheca wickerhamii was causing the infection. The fungus was identified through skin samples and DNA testing. The dog is currently being treated with itraconazole to fight the fungus, but the skin bumps haven't completely cleared up yet. The vet is monitoring the dog closely and adjusting the treatment plan."
474,"A satin 2-year-old female guinea pig weighing 560 g was admitted to a private practice with anorexia, low weight, previous history of cheek teeth overgrowth and coronal reduction of 3 cheek teeth in recent months. Ten months before this visit the guinea pig was attended to for left forelimb lameness and lumbar deformity; a radiologic study was declined by the owner, and the lameness responded to meloxicam (Metacam; Boehringer Ingelheim, Sant Cugat, Spain) (0.3 mg/kg body weight, per os q 12 hrs, 15 days). Diet was appropriate, including nutrients rich in vitamin C.\nA physical examination revealed total loss of mobility of the left carpal joint, malocclusion of the incisors, and cheek teeth overgrowth. The radiological study of the head showed mandibular deformation, a marked bone trabecular pattern, areas of sclerosis, and incisor malocclusion. Before sedation achieved with midazolam (Midazolam Normon; Laboratorios Normon, Tres Cantos, Spain) (0.5 mg/kg body weight, intramuscular) and butorphanol (Torbugesic; Zoetis, Alcobendas, Spain) (0.5 mg/kg body weight, intramuscular) urine was obtained by cystocentesis and blood was collected from the cranial vena cava. A hematological (Chemray 120, Rayto, Shenzhen, China) and biochemical (MS4 Vet, Melet Schloesing, Osny, France) panel, hormonal determinations, ionized calcium, and urinalysis were performed (). A whole body radiograph showed deformity, double cortical line, marked trabecular pattern, and loss of definition of the medullary cavity in virtually all of the long bones (). Left carpal synarthrosis and misalignment of the spine at the L5-L6 level were also observed on the radiographs.\nA remarkable improvement was observed in the guinea pig's health after incisor and cheek teeth coronal reduction. As long-term management coronal reduction was performed when considered necessary (2-3 times per year), the diet was closely monitored and annual analytical controls were performed (). Three years later, the guinea pig had a good quality of life according to the owner, analytical values were stable (), and radiographs showed hyperostosis, sclerosis, and partial remodeling of the previously affected bone ().","This 2-year-old guinea pig was brought in because she wasn't eating and was very underweight. Previously, she had a problem with her leg and spine, but it improved with medication. The vet found that her joints were stiff, her teeth were misaligned, and her bones were deformed. After dental work, the guinea pig’s health improved, and her bones gradually healed over time, leading to a good quality of life."
475,"A privately owned two-year-old female guinea pig was referred for a repeated loss of material from the uterus and progressive weight loss. Anamnestically, the animal never conceived despite several attempts at mating. The day of the appointment, the pet expelled a large amount of hemorrhagic material. During the visit, the patient was quiet, depressed, and moderately responsive to stimulation. At physical exam, the patient showed tachypnea, vocalization upon manipulation of the abdomen that was tense, and dilated abdomen with a palpable mass. Ultrasonographic investigation evidenced a large well-defined 2 × 2 cm mass in the anatomic area of the uterus. The guinea pig underwent emergency surgery upon sedation with an association of butorphanol 0.7 mg/kg (Dolorex 10 mg/ml, MSD), medetomidine 0.07 mg/kg (Sedator 1,0 mg/ml, ATI), and ketamine 7 mg/kg (Imalgene 1000 100 mg/ml, MERIAL) administrated intramuscularly. Anesthesia was maintained with isoflurane 3% with a not cuffed endotracheal tube. The procedure was monitored with a multiparameter monitor, including ECG (II derivation) CO2, O2, and no invasive pressure and temperature. During the anesthesia, intravenous fluid (NaCl 0.9%) was administered at the rate of 5 ml/kg/h with infusion pump via intravenous catheter 24 G (Terumo). Surgical examination of the abdomen evidenced an enlarged and congested ovary and uterus and signs of peritonitis, including intra-abdominal fluid. The two organs were excised and submitted for histopathology. The patient was discharged on antibiotic (enrofloxacin 5 mg/kg bid/po Baytril flavour sosp os 25 mg/ml Bayer) and nonsteroidal anti-inflammatory drugs (meloxicam 0.3 mg/kg/sid/po Metacam sosp os 1,5 mg/ml flac 10 ml Boheringer) and ranitidine (3 mg/kg/bid/po Zantadine sol os 30 mg/ml Ceva) []. Histopathologic analysis revealed characteristic features of endometriosis both in the uterus and in the ovary. In detail, adenomyosis was described in the uterus because of the presence of ectopic glandular tissue in the muscular wall of the uterus (). On the other hand, at the level of the ovary, cystic enlargements filled with fluid were seen macroscopically; histologically, these cystic structures presented the classic glandular epithelium of the endometrium with one layer of cuboidal or tall cells, thus representing classic cystic endometriosis of the ovary (). Immunohistochemical staining was performed, by using the ABC method and diaminobenzidine, by means of specific antibodies for estrogen and CD10 in order to confirm the ability of the glandular epithelium to secrete estrogen and the presence of a stromal reaction surrounding the ectopic endometrial tissue (Figures and ) [, ]. The histopathological and immunohistochemical features described are strongly suggestive of endometriosis.\nThe guinea pig recovered from the surgery and was rechecked on a monthly basis. The patient died of unrelated causes three months later. A necropsy was performed at that time and did not show any sign of endometriosis.","Our little guinea pig was brought in because she was losing weight and had a problem with her uterus. We found a large mass in her uterus and discovered she had endometriosis, a condition where tissue similar to the lining of a female’s uterus grows in other parts of her body. After surgery to remove the affected organs, she recovered well, but sadly, she passed away a few months later from an unrelated illness."
476,"A 3-day-old, 9.5 kg female alpaca cria was presented for progressive weakness and dyspnea of a few hours' duration. The cria had an uneventful birth, stood, nursed, and passed urine and feces normally, but was less active than normal. The cria nursed regularly but only for very short periods. On presentation, the patient was tachycardic (heart rate 148) and appeared to be dyspneic (respiratory rate 28) with cyanosis of the oral mucous membranes and had a capillary refill time of 3 seconds. Cardiac auscultation revealed no significant abnormalities (excluding the tachycardia) and pulse pressure was considered normal. During examination, the cria intermittently lowered its head, became ataxic, and collapsed. These episodes were precipitated by handling or when nursing. After a few moments the cria sat sternal and then stood, appearing normal. Behavior and neurologic exam between episodes were normal. Differential diagnosis included septicemia, pneumonia, choanal atresia, meningitis, persistent fetal circulation, and cardiac abnormalities. No significant abnormalities were noted on complete blood cell count and blood chemistry. Standard lateral thoracic radiographs (evaluated by a board certified veterinary radiologist) revealed slight loss of cranial cardiac waist, distention of the caudal vena cava, and a mild diffuse interstitial lung pattern () without significant evidence of pulmonary venous congestion or overcirculation. A dorsoventral or ventrodorsal view may have provided additional information regarding the heart, but it is not routinely obtained in an unsedated or unanesthetized cria []. An attempt was made to perform upper airway endoscopy to assess for suspected choanal atresia; however, the procedure was aborted as the cria became progressively more distressed. The cria was subsequently anesthetized and placed on 100% oxygen. An endoscopic examination of the nasal passages and distal trachea revealed no significant abnormalities. An arterial blood gas was performed while on oxygen supplementation which revealed a marked hypoxemia (PaO2 19 mmHg, PaCO2 29.6 mmHg, and SaO2% 31.7) making cardiac disease with right to left shunting of blood more likely. As such, with no evidence of respiratory diseases or septicemia as the cause of the clinical signs, a congenital cardiac malformation was highly suspected and a cardiac evaluation was performed.\nTwo-dimensional (2D) echocardiography, color flow, and spectral Doppler examinations were performed under general anesthesia with an ultrasound unit (Vivid 7, General Electric Medical System, Waukesha, WI, USA) equipped with 1.5–3.6, 2.2–5, and 4.4–10 MHz phased-array transducers. Two-dimensional images revealed severe dilation of the right atrium and ventricle. Thickening of the right ventricle free wall was also identified. No significant dilation of the left atrium or thickening of the left ventricle was noted. The interventricular septum (IVS) was flattened and there was paradoxical motion of the IVS. At the base of the IVS a large ventricular septal defect (VSD) was detected (). Additionally, a patent foramen ovale (PFO) was noted in the atrial septum () and a single large artery overriding the VSD was also identified. The right ventricular outflow tract, origins of the pulmonary arteries, and a patent ductus arteriosus could not be visualized during the echocardiographic examination. Systolic function appeared normal as estimated by 27% fractional shortening (normal 32.8 ± 7.6) []. Color flow Doppler evaluation revealed bidirectional but primarily right to left shunting across both the defect in the atrial septum and VSD. Mild regurgitation across mitral, tricuspid, and the valve of the single large artery was noted. A contrast study was performed by injecting agitated heparinized saline into the external jugular vein while viewing the heart from the right parasternal view. Presence of bubbles from the right heart crossing the VSD into the left heart and main artery during systole confirmed the presence of a right to left shunting VSD (; Supplementary Information: Video 1 and Video 2 in Supplementary Material available online at ). Differential diagnosis based on the echocardiographic findings included Tetralogy of Fallot, severe pulmonic stenosis or pulmonary atresia with a VSD, and common arterial trunk (CAT). Surgical implantation of a vascular shunt and open-heart surgical correction of the malformation was discussed with the owners but due to the poor prognosis and limited treatment options, the owner elected humane euthanasia and postmortem examination.\nOn macroscopic examination, there was evidence of both right sided (liver congestion and pleural, pericardial, and peritoneal effusion) and left sided (marked pulmonary edema) congestive heart failure. Examination of the heart revealed marked dilation of the right atrium, a small PFO, a large VSD, and a single large vessel overriding the IVS. The vessel had a mildly thickened quadricuspid valve and appeared to be the only outflow tract for both the right and left ventricles consistent with a CAT. A separate pulmonary artery originating from the right ventricular outflow tract could not be identified despite careful dissection. However, a single pulmonary trunk arose from the common trunk prior to the arch and branched to the right and left pulmonary arteries. A patent ductus arteriosus was not identified. Coronary artery structure appeared to be normal. Histological examination identified the presence of pulmonary edema and hepatic congestion consistent with left and right sided congestive heart failure. There was an incidental finding of a cerebellar pseudocyst. Based on the postmortem examination, a diagnosis of a type I CAT (according to Collett and Edwards' classification) and PFO was made.","The alpaca cria, a baby alpaca, was having trouble breathing and seemed weak. After some tests, it was found that he had a heart defect called a Congenital Arterial Trunk (CAT) which meant his heart wasn't pumping blood properly. The doctors found a hole in his heart and a large vessel that was supposed to be two separate arteries, causing blood to flow in the wrong direction. Because of this, the cria was very sick and eventually, the owners made the difficult decision to euthanize him to prevent further suffering."
477,"In July 2015, an 8-month-old female of Staffordshire Bull Terrier, housed in Milan province (northern Italy) and purchased from an Italian dog breeder, was taken to a veterinary clinic on account of her frequent and violent sneezing that lasts for two days. During anamnestic data collection, the owner reported that sneezing occurred after the dog had been taken for a walk in a rural area close to his house. At clinical examination the bitch also presented stertorous and reversal sneezing. Anamnesis, dog breed, and symptoms made clinicians suspect a nasal foreign body and/or a brachycephalic airway obstructive syndrome (BAOS). No antimicrobial or anti-inflammatory therapies were being administered to the dog. The bitch was then anesthetized for laryngoscopy, tracheoscopy, and anterior and posterior rhinoscopy. Laryngeal inspection revealed everted laryngeal saccules, whereas tracheoscopy did not show any remarkable alteration. Posterior rhinoscopy evidenced few small mucosal erosions (diameter < 2 mm) surrounded by mildly thickened and oedematous mucosae in the rhinopharynx; a small quantity of mucus-like material was also present. The anterior rhinoscopy highlighted two and three whitish fusiform organisms in the right and in the left nasal cavities, respectively; all the observed organisms appeared to be vital, presenting high mobility on the nasal mucosal surface. Attempts to catch them using endoscopic forceps failed and only after nasal lavage was one of them isolated and collected. Noticeably, following nasal lavage, the acute and violent sneezing improved considerably which might be due to removal of most of the observed organisms. The collected organism resembled a larva of Diptera and while waiting for further investigations after rhinoscopy the dog was also treated for three times every 7 days (days 0, 7, and 14) with subcutaneous administration of 300 μg/kg of ivermectin. After treatment, sneezing disappeared completely, and only moderate reversal sneezing, probably due to everted laryngeal saccules, remained present. The larva was sent to the Department of Veterinary Medicine of Milan for identification; it was studied under the light microscope and identified according to morphological keys [–]. The specimen was identified as a first instar larval stage (L1) of O. ovis L. (Diptera: Oestridae). The fusiform and dorsoventrally flattened L1, about 1.18 mm long and 0.44 mm wide, was divided into 11 segments (). On its surface, these segments presented trichoid cuticular sensilla (). Such structures are thermosensitive; they allow L1 to both locate and, in association with its quick mobility, rapidly reach the nasal cavities to find a suitable niche for its development. Ventral and lateral clusters of spines were also evident on the larva surface. They measured about 20 μm and 30 μm in length, respectively, and their distribution resembled the typical pattern described in Oestrus larvae. In subfamily Oestrinae, lateral and ventral spines can help a larva attach to and move on the host's mucosal surface without being expelled by its sneezing. The larva under investigation showed a distinctive cluster of spines on the terminal abdominal segment, though its bilobated shape was not perfectly preserved. Cranially, a pair of prominent, dark brown oral hooks, connected to the internal cephalopharyngeal skeleton, as well as defined antennal lobes, measuring about 18 × 22 μm could be noticed. Broad tracheal trunks, about 20 μm wide, ended between the tenth and eleventh body segments ().","This dog was experiencing frequent and violent sneezing, leading her owners to believe a foreign object was stuck in her nose. After examination, the vet discovered a small fly larva in her nasal passages, which was causing the sneezing. The larva was removed, and the dog was treated with medication, resulting in a complete resolution of the sneezing. The vet identified the larva as a type of fly and determined it was using its spines to attach to the dog's nasal lining."
478,"An approximately 3-year-old, intact female, 260 g red corn snake (Pantherophis guttatus) was evaluated by the Zoological Medicine Service, University of Florida, for a midbody swelling and a three-week history of anorexia. The snake was purchased from a breeder 2.5 years priorly and did not have any previous health concerns. The reptile was kept in a tank with aspen bedding, in a room kept at 25°C throughout the year. During winter, supplemental heating was offered in one end of the tank using a heat lamp. Temperature and humidity in the enclosure were not monitored. The snake was fed a frozen-thawed adult mouse once weekly. During the feedings, the snake was transferred to a different container without any bedding, where it was left with the food item until consumed. The owner reported that the snake normally defecated regularly, but that for the past three to four weeks the stools had been dryer, smaller, and less frequent. The snake remained active at home and displayed a normal behaviour. The owner also had a wild caught, apparently healthy grey ratsnake (Pantherophis spiloides) kept in a separate tank.\nDuring physical examination, the red corn snake was quiet, alert, and responsive, and appeared in good body condition. A soft, ventral, intracoelomic swelling (6 × 4 cm) was palpable approximately at midbody and elicited discomfort to the patient when manipulated. Ultrasonography (Philips iU22 ultrasound machine, Philips Medical Systems, Bothell, WA 98021, USA), through multifrequency linear transducers, revealed a cylindrical structure lateral to the caudal liver, coursing caudally and medially to reside in proximity to the stomach. At this location, the segment thought to represent the stomach was markedly and focally fluid dilated. Caudally, from this region of dilation, an additional cylindrical segment surrounded those described previously, resulting in a concentric ring appearance (). The central component of the concentric ring demonstrated blood flow, using color Doppler. Based on these findings, a gastrointestinal intussusception was suspected and surgery was recommended.\nThe snake was sedated with 0.1 mg/kg of body weight (BW) of hydromorphone (West-Ward, Eatontown, NJ 07724, USA) administered intramuscularly. Intubation with a 14 G catheter was possible following the sedation and the animal was maintained on 1-2% isoflurane (Piramal Healthcare Limited, Andhra Pradesh 502321, India) in a mixture of oxygen and nitrous oxide (1 L/min of each). The patient received positive intermittent manual ventilation at a rate of four breaths per minute. The patient was placed in left lateral recumbency, and the surgical site was prepped aseptically. A 10 cm incision was made two scale rows dorsal to the ventral scales. The incision followed along the edges of the scales, forming a scalloped pattern. The underlying muscle layers were incised at the end of the ribs. A Lonestar retractor (Jorgensen Labs Inc., Loveland, CO 80538, USA) was used to improve visualization of the coelomic cavity. After manipulation of the tissues, a gastrotomy was made laterally and spanned most of the length of the stomach and into the duodenum approximately 2 cm. The esophagus was observed to be intussuscepted into the stomach (), and then this combination was further invaginated into the duodenum () forming a second intussusception. A diagnosis of double compounded esophagogastric and gastroduodenal intussusception was made. The stomach was extracted from the duodenum and then the esophagus was extracted from the stomach. There were adhesions formed between the esophagus and stomach that were bluntly dissected in order to extract the esophagus from the stomach (). Grossly, the gastric longitudinal rugae appeared hypertrophied. Samples of gastric mucosa were placed into 10% buffered neutral formalin for histopathologic evaluation. The stomach was closed in two layers with 3-0 PDS (Ethicon LLC, Cincinnati, OH 45242, USA) with a Lembert pattern in the mucosa/submucosa layer, followed by a simple continuous pattern for the serosal muscularis layer. Due to the nature of the intussusception, the incision in the duodenum had a transverse as well as a longitudinal component. The transverse incision in the duodenum was closed with four simple interrupted sutures using 4-0 PDS (Ethicon LLC, Cincinnati, OH 45242, USA). The longitudinal component was closed with a simple continuous pattern using 4-0 PDS. A gastropexy via interrupted circumcostal sutures was performed with 3-0 PDS to prevent recurrence of the intussusceptions. The body wall was closed routinely. The muscle layer was closed with 3-0 PDS in a simple continuous manner. The integument was closed with nine horizontal mattress and one simple interrupted sutures to cause an eversion of the scales using 3-0 PDS.\nThe patient was discharged the following day. Treatments included famotidine (Mylan Institutional LLC, Rockford, IL 661103, USA) at 0.026 mg/kg BW SC q48h, ceftazidime (Hospira Worldwide Inc., Lake Forest, IL 60045, USA) at 22 mg/kg BW SC q72h, and meloxicam (Putney Inc., Portland, ME 04101, USA) at 0.1 mg/kg BW SC q48h. The snake was not fed for two weeks before reexamination. The owner provided a supplemental heat lamp in the tank to have a warm basking spot and a cooler temperate area. All bedding was removed from the enclosure and only clean newspaper or paper towels were used as a substrate.\nIn histologic sections of the stomach, epithelium on the gastric surface and in gastric glands was hyperplastic (). Dilation of the mucosal glands and fibrosis of the lamina propria were also evident. Myriad protozoa that were 2 μm in diameter, eosinophilic to basophilic, and periodic-acid-Schiff- (PAS-) positive and that had variably distinct 0.5–2 μm basophilic nuclei were closely associated with the apical epithelial surfaces as well as being free within the lumen (). Small numbers of heterophils, lymphocytes, and plasma cells were in the lamina propria. A diagnosis of chronic proliferative gastritis due to Cryptosporidium sp. was made based on these findings.\nTwo weeks postoperatively, the snake was recovering well. The surgical site was clean, well apposed, and free of any discharge. The ventral surface associated with the incision was mildly distended and the scales appeared slightly dull. The owner had no concerns at that time, and the snake maintained BW. All medications were discontinued and the snake was tube-fed 2.5 mL of carnivore care (Oxbow Animal Health, Murdoch, NE 68407, USA) slurry to help encourage normal gastrointestinal movements and to administer a small first meal. The owner was allowed to start refeeding the snake smaller prey items such as pinkies every five days for 1 month, and then increasing to one hopper every 5 days for another month, followed by adult mice once weekly as the preoperative husbandry practices.\nAt four weeks after surgery, the skin sutures were removed. A brief ultrasound exam was performed and did not show any obvious abnormalities around the stomach. The owner reported one abnormal bowel movement, described as diarrhea, that occurred four to five days after feeding the first pinkie mouse. The owner also reported that the snake had a normal shed two weeks priorly without any complications. The animal had lost 30 g (approximately 12% of BW) since initial presentation, but this was expected due to the current feeding schedule. A gastric wash was performed and submitted for polymerase chain reaction (PCR) to speciate the Cryptosporidium present in the stomach. However, there were no organisms in the sample, and the PCR came back negative.\nUnfortunately, at 15 months after surgery, the snake was found deceased in its enclosure. The owner reports that the snake would have intermittent episodes of regurgitation when it was fed adult mice but seemed to tolerate eating smaller food items without difficulties. The snake behaved normally, remained with a good appetite, and did not have other clinical signs until the day it was found dead. The cause of the regurgitations is unknown, but worsening of the chronic Cryptosporidium sp.-associated gastritis is suspected. The snake was not submitted for postmortem examination.","The veterinary team examined a 3-year-old corn snake that was having trouble eating and had a swelling in its belly. The snake’s enclosure wasn’t being monitored for temperature, and it was being fed large mice, which may have contributed to the problem. After surgery to remove a part of its stomach that was collapsed into another part of its digestive system, the snake recovered well. However, the snake died 15 months later, and the cause of its final regurgitations is unknown, but it is suspected that the ongoing inflammation from the Cryptosporidium infection may have played a role."
479,"An eleven-year-old female spayed Labrador Retriever was presented for evaluation of multinodular subcutaneous masses in the dorsal cervical area ().\nThree nonadjuvant vaccinations, DA2PP-Lepto, Rabies, and Bordetella, had been injected into this same area during a wellness examination three weeks earlier. The dog's previous vaccination history included routine boosters at standard intervals administered at variable sites since puppyhood. Fine needle aspiration cytology of the masses revealed a mesenchymal spindle cell proliferation with a high level of atypia and minimal numbers of inflammatory cells. A wide surgical excision of the entire site with 3 cm margins was performed the following week. The excised tissue contained a regionally extensive, expansile, and infiltrative spindle cell neoplasm surrounded by a pseudocapsule and mild to moderate chronic inflammation with multifocal lymphonodular aggregates. The center of the neoplasm had undergone extensive necrosis, and the myxomatous matrix was admixed with grey-brown globular material. The neoplastic cells were fusiform to spindloid, formed interlacing bundles, and had moderate amounts of darkly eosinophilic cytoplasm. The nuclei were ovoid and had a stippled chromatin pattern with numerous, variably sized prominent nucleoli. There was marked anisokaryosis and anisocytosis. Binucleated and multinucleated cells as well as karyomegaly were multifocally observed. The mitotic count was 20 in 10 high powered fields (HPF, FN22), and there were occasional bizarre mitotic figures ().\nA grade 3 soft tissue sarcoma was diagnosed based on the degree of necrosis, cellular atypia, and the high mitotic count. The neoplasm had narrow but completely excised surgical margins. The surgical wound healed without any complications; however, multiple subcutaneous nodules were identified at the excision site ten weeks later. Repeat fine needle aspiration cytology confirmed a recurrence of the sarcoma. Hematology, serum biochemistry, and urinalysis were unremarkable, and three-view thoracic radiographs did not identify metastatic disease to the lungs. A second, broad excision of the injection site with 3 cm margins was performed. The neoplasm appeared histologically similar to the previously excised grade 3 soft tissue sarcoma with an increased mitotic count of 30/10 HPF. The sarcoma had focally infiltrated the skeletal muscle. Excision was reported to be complete with narrow margins. Neoplastic cells were immunohistochemically positive for Vascular Endothelial Growth Factor receptor (VEGFr) (), Platelet Derived Growth Factor receptor (PDGRr) (), Stem Cell Factor (SCF), and Epithelial Growth Factor Receptor (EGFR) and negative for VEGF, PDGR, KIT, and p-53.\nBased on the expression of VEGFr and PDGFr, a response to targeted tyrosine kinase inhibitor therapy with toceranib, a small molecule inhibitor of VEGFr2 and PDGFrβ, was hypothesized. Toceranib was initially administered orally at a dose of 2.1 mg/kg and then increased to 2.8 mg/kg on a Monday-Wednesday-Friday schedule. No adverse effects were noted at these doses and no hypertension or proteinuria was detected. Repeat CBC and serum biochemistry profiles were normal throughout the course of therapy and thoracic radiographs did not show any evidence of metastatic disease. A small cluster of subcutaneous nodules was identified at the excision site fifty weeks after the first surgery and a third complete excision with 3 cm margins was performed. Histology confirmed a recurrence of the grade 3 soft tissue sarcoma and the toceranib was continued. At the time of submission of this manuscript, the dog remains in remission ninety-three weeks after initial diagnosis.","This Labrador Retriever was seen for growing lumps on her neck. After examining the lumps, the vet determined they were a type of aggressive cancer called a soft tissue sarcoma. The tumor was surgically removed, but unfortunately, it came back a few months later. To fight the cancer, the dog was started on a targeted medication that helped slow its growth, and she is currently in remission after multiple surgeries and treatments."
480,"An adult captive male bateleur eagle (Terathopius ecaudatus) estimated to be 32 years old and weighing 2.4 kg was presented for evaluation of left hindlimb lameness. The animal had been transferred from another institution 4 weeks prior to presentation with a history of osteoarthritis at the left intertarsal joint and an asymptomatic systolic heart murmur. Laxity and varus deviation of the distal left hindlimb at the intertarsal joint were first observed 2 years prior to presentation at the animal's previous institution. It had also been treated for several previous episodes of bilateral pododermatitis.\nSeveral days after transfer into a new enclosure, the animal began to exhibit intermittent non-weight-bearing left hindlimb lameness and was prescribed tramadol (5.5 mg/kg bodyweight (BW) orally twice daily) for pain management. The animal failed to sufficiently respond to this treatment over the course of 1 month and began spending increased time in a sternal position or on the ground of the enclosure. Under general anesthesia with isoflurane gas and oxygen, a physical examination with radiographs, complete blood count, and plasma biochemistry was performed to evaluate the lameness. Physical examination revealed firm periarticular enlargement, reduced range of motion, subluxation, and dynamic varus deviation at the left intertarsal joint (). No evidence of pododermatitis was observed at either foot. Orthogonal view radiographs of the pelvic limbs revealed muscle atrophy of the left hindlimb, moderate soft-tissue expansion around the left intertarsal joint with an uneven joint space that was widened at its lateral and narrowed at its medial aspects on the dorsoplantar radiographic view, and evidence of degenerative joint disease at this joint (). A complete blood count and plasma biochemistry were considered unremarkable when compared to species reference values []. The chronic degenerative joint disease and instability likely associated with previous joint ligament or tendon rupture was thought to be the underlying cause of the lameness. The animal was prescribed meloxicam (0.5 mg/kg BW orally once daily) and continued on tramadol. A visual evaluation performed 2 weeks later revealed continued intermittent lameness of the left hindlimb with reduced weight-bearing and frequent placement of the limb in an abnormal extended position. Gabapentin (3 mg/kg BW orally once daily) was prescribed for additional pain management; however, progressive lameness of the left hindlimb persisted despite these conservative management efforts. Reevaluation was performed 10 weeks later when the animal was observed to be non-weight-bearing on the left hindlimb after several traumatic collisions within its enclosure. The patient was anesthetized as previously described. Physical examination findings were consistent with the animal's exam performed 10 weeks earlier, although bruising was present at the left ventral pelvic region, likely due to the recent observed collisions. Stress radiographs of the distal limbs were performed, confirming subluxation at the left intertarsal joint (). Repeated complete blood count and plasma biochemistry revealed elevated creatine kinase (1175 U/L; Species 360 database reference values 133–795) consistent with muscle damage from soft-tissue trauma or capture and handling []. A support bandage was applied to the left intertarsal joint region, and the animal's prescriptions of tramadol, meloxicam, and gabapentin were continued. Based on physical examination findings, diagnostics, and the patient's failure to respond adequately to conservative management alone, surgical stabilization of the left intertarsal joint was scheduled for the following week.\nThe animal was induced under general anesthesia as previously described, intubated, and maintained on isoflurane gas and oxygen throughout the surgical procedure. The left intertarsal joint was aseptically prepared, and sterile adhesive drape (Ioban™, 3M, St. Paul, USA) was applied to the limb. A 2 cm incision was made over the lateral aspect of the left intertarsal joint, and the soft-tissues were bluntly dissected from the distal tibiotarsus and proximal tarsometatarsus. A 30 ga. needle was placed into the intertarsal joint space to confirm its location, and a 2 mm drill bit was used to make an intraosseous tunnel through both cortices of the distal tibiotarsus extending proximomedially from the distolateral aspect of the metaphysis (). A second intraosseous tunnel was produced at the proximal tarsometatarsus extending distomedially from the lateral aspect of the bone. Incisions were made over the medial aspects of the tibiotarsus and tarsometatarsus at both exit points of the intraosseous tunnels, and soft-tissues were bluntly dissected away from the underlying bone to allow application of the stabilization implants. A flexible suture passer was used to shuttle a single strand of braided suture material (#2 Arthrex FiberWire®, Arthrex, Inc., Naples, USA) from the lateral entry points of both intraosseous tunnels to the medial exit points at both bones (). Each end of the suture material was threaded through both holes of a 2-hole titanium suture button (Arthrex, Inc., Naples, FL, USA) and back through its respective intraosseous tunnel to exit at the lateral aspect of the intertarsal joint. The buttons were positioned against the bones while the braided suture material was tied with the joint in a neutral position. Range of motion and varus/valgus stability of the intertarsal joint were assessed prior to tying of the suture to ensure appropriate joint stability and range of motion. Closure of the soft-tissues and skin was performed using 3-0 polydioxanone suture in a simple interrupted pattern. Postoperative radiographs revealed that the titanium button at the tarsometatarsus was positioned craniolaterally to what was considered ideal; however, the joint appeared stable during manipulation. The animal received perioperative butorphanol (2 mg/kg BW intramuscularly), meloxicam (0.5 mg/kg BW intramuscularly), clindamycin (20 mg/kg BW intravenously), enrofloxacin (15 mg/kg BW subcutaneously diluted 1 : 10 in lactated ringer's solution), and intraoperative lactated ringer's solution (10 ml/kg/hr BW intravenously). The patient continued tramadol, meloxicam, and gabapentin and was also prescribed prophylactic clindamycin (20 mg/kg BW orally once daily) and enrofloxacin (20 mg/kg BW orally once daily) for 7 days.\nInitial observations made during the first 3 weeks of postoperative recovery revealed gradual initial improvement in weight-bearing with significant persistent lameness. At 5 weeks after surgery, the animal was observed spending the majority of its time in sternal recumbency with reluctance to stand, and a reevaluation with the consulting veterinary surgical specialist was scheduled for the following week. The animal was induced, intubated, and maintained under general anesthesia as previously described. Radiographs confirmed suboptimal positioning of the previously placed joint stabilization implants, and persistent subluxation of the left intertarsal joint was demonstrated in stress radiographic views (). The previous surgical stabilization was considered unsuccessful, and a second joint stabilization surgery was elected. The patient was aseptically prepared, consistent with the initial surgical procedure. Incisions were made at the medial and lateral aspects of the left intertarsal joint, and evaluation of the previously placed stabilization implants revealed that the distal titanium suture button had become unsecured from the intraosseous tunnel at the medial tarsometatarsus, leading to surgical stabilization failure and subsequent postoperative laxity at the joint. The previously placed implants were removed, and the surgical stabilization procedure was repeated similarly to what was described for the first surgical procedure. The intraosseous tunnel at the distal tibiotarsus was re-used, and the intraosseous tunnel at the proximal tarsometatarsus was evaluated, was determined to have widened, and was revised. A 1.143 mm diameter K-wire was passed from proximolateral to distomedial across the proximal tarsometatarsus. Suture material (#5 Arthrex FiberWire, Arthrex, Inc., Naples, USA) was threaded through the intraosseous bone tunnels and titanium buttons as described for the first surgery. The suture was tightened with the aid of a suture tensioner (Arthrex, Inc., Naples, FL., USA) to 5 kg, and the joint was cycled to ensure stability and range of motion. The tensioner was then removed and the suture was tied. A two-layer closure of the soft-tissues and skin was performed using 4-0 poliglecaprone suture in cruciate and simple continuous patterns. Lidocaine (1 mg/kg BW) was administered as an incisional block, postoperatively. The animal received perioperative butorphanol (0.5 mg/kg BW intramuscularly), meloxicam (0.5 mg/kg BW intramuscularly), and enrofloxacin (10 mg/kg BW subcutaneously) administered in lactated ringer's solution (40 ml/kg BW subcutaneously). Postoperative examination revealed only mild medial subluxation of the left intertarsal joint during flexion that was comparable to that observed at the contralateral limb. No significant medial subluxation was elicited with the joint in extension. Postoperative radiographs indicated appropriate positioning of the stabilization implants (). Prophylactic enrofloxacin (15 mg/kg BW orally once daily) and clindamycin (20 mg/kg BW orally once daily) were prescribed for 28 days. Meloxicam, tramadol, and gabapentin were continued as previously prescribed, and activity restriction with lowered perches was implemented for 6 weeks.\nPostsurgical visual and physical evaluations were performed periodically over the next 6 months. At 3 weeks after the second surgery, the bird was noticed spending less time in sternal recumbency than following the initial surgery, although the animal continued to have noticeable lameness at the left hindlimb. A postsurgical reevaluation performed under general anesthesia at 7 weeks revealed improved medial-lateral stability at the left intertarsal joint, although the animal continued to exhibit lameness at the left hindlimb. At 14 weeks following the second surgery, the animal was observed perching normally with good weight-bearing on both hindlimbs. Only slight intermittent favouring of the left hindlimb was observed during ambulation. Due to clinical improvement, the animal was weaned from gabapentin at 20 weeks without any increase in lameness or time spent in sternal recumbency but was maintained on tramadol and meloxicam for management of chronic pain related to the animal's underlying degenerative joint disease. At 6 months following the second surgery, the animal was using the limb well during perching and ambulation with only mild occasional favouring of the limb observed.\nPeriodic examinations, observations, and reports from animal caretakers indicated overall improvement in the animal's mobility and use of the limb for several years following the second surgical stabilization and ongoing treatment with pain medications. Only mild occasional lameness of the left hindlimb was observed during this time. Three years after the second surgical stabilization was performed, the animal developed a more pronounced lameness. Evaluation of the animal at this time revealed radiographic progression of degenerative joint disease at the left intertarsal joint, although no change in joint stability was noted. A complete blood count and plasma biochemistry were considered unremarkable when compared to species reference values []. The animal's medications were adjusted with meloxicam administered at 1 mg/kg BW orally twice daily and tramadol at 10 mg/kg BW orally twice daily. The animal responded positively to this change and was maintained on these medications to better manage the chronic pain associated with the condition.","This bateleur eagle had a problem with his left hind leg that got worse over time. Initially, he had arthritis in the joint, and it became unstable, causing him to limp. After several attempts to treat the problem with different medications, a second surgery was needed to stabilize the joint. Despite the surgery, the leg continued to cause some lameness, and the bird was given pain medication to manage the chronic pain. Over the next few years, the bird’s leg continued to show signs of arthritis, but the bird was able to continue to use the limb well and was maintained on pain medication."
481,"An eight-year-old, female intact Golden Retriever, weighing 27 kg, was referred for investigation of urinary and faecal incontinence, which acutely appeared following two months of chronic vaginal discharge. The latter partially responded to antibiotic treatment. After neurological consultation, the animal was scheduled for magnetic resonance imaging (MRI) of the lumbar-sacral region under general anesthesia.\nOn preanesthetic examination the dog appeared slightly nervous but in good physical condition (ASA II). The heart rate was 90 beats per minute (bpm), respiratory rate was 15 breaths per minute (brpm), pulse quality was good, mucous membranes were pink, and capillary refill time was less than 2 seconds. Thoracic and cardiac auscultation were unremarkable, as were the results of hematological and biochemical blood tests.\nAfter a mild sedation was achieved administering methadone intramuscularly (0.2 mg kg−1; Synastone, Auden Mckenzie Ltd., UK), anesthesia was induced intravenously 30 minutes later with fentanyl (3 μg kg−1; Sublimaze, Janssen-Cilag Ltd., UK) and propofol (2.5 mg kg−1; Rapinovet, Schering-plough Animal Health UK). The trachea was intubated with a cuffed tube; the cuff was inflated; intubation was unremarkable. Anesthesia was maintained with isoflurane (IsoFlo, Abbott Laboratoires, UK) in 100% oxygen, delivered through a circle system. Monitoring consisted of capnography, measurement of inspired and expired anesthetic gases and oxygen, measurement of noninvasive arterial blood pressure (Datex AS3, Helsinki, Finland), and esophageal stethoscope.\nShortly after commencement of isoflurane administration, a gasping breathing pattern was noted, with a respiratory rate of 40–50 brpm and jerk movements of the mouth and all four limbs. Heart rate was 140 bpm. Inadequate depth of anesthesia was considered the cause of the observed movements; thus ventilation was assisted manually (15 brpm) and the vaporizer setting was increased from 2% to 3% (oxygen 3 L min−1) in order to deepen the anesthetic plane. Further, a bolus of fentanyl (1 μg kg−1) was administered intravenously. At that time, end-expiratory carbon dioxide tension (PE′CO2) was 22 mmHg. Because the respiratory pattern did not change during the following 10 minutes, and suspecting an underlying pulmonary disease, MRI was cancelled while thoracic radiographs and an arterial blood gases analysis were performed. Radiographs were unremarkable and alkalemia due to primary respiratory alkalosis was apparent in the arterial blood gases analysis results (). The dog was allowed to recover from general anesthesia. Once the vaporizer was turned off and the breathing system flushed with oxygen, the dog's breathing pattern improved and became normal. The recovery from general anesthesia was uneventful. MRI was rescheduled for the following day.\nOn day 2, preanesthetic assessment was unremarkable and similar to that obtained the previous day. Dexmedetomidine (1.25 µg kg−1 Dexdomitor, Orion Pharma, Finland) and methadone (0.25 mg kg−1) were administered slowly intravenously. The resulting sedative effect was good with the animal relaxed in lateral recumbency. Anesthesia was induced with propofol (1.5 mg kg−1) and, after intubation of the trachea with a cuffed tube, maintained with isoflurane (vaporizer setting was 3%) in 100% oxygen at the flow of 3 L min−1, delivered through a circle system. Also in this occasion tracheal intubation was unremarkable.\nAs on day 1, the animal started gasping and jerking continuously as soon it was connected to the breathing system. At that point, isoflurane was immediately turned off and the breathing system flushed with pure oxygen. Anesthesia was then maintained with a constant rate infusion (CRI) of propofol (0.3 mg kg−1 min−1), after slow administration of a loading dose (0.5 mg kg−1). As the animal's breathing pattern did not improve, atracurium (0.2 mg kg−1, Tracrium Injection, GlaxoSmithKline, UK) was administered intravenously and intermittent positive pressure ventilation (Penlon Nuffield 200 ventilator) was started to maintain eucapnia (PE′CO2 35–45 mmHg). Respiratory rate was set to 15 breathes per minute, tidal volume was 300 mL, and peak inspiratory pressure was 12 cmH2O. The rest of the anesthetic time was uneventful, but it was necessary to top up atracurium every 15–20 minutes, because hiccups restarted as soon as neuromuscular function started returning.\nAn extensive invasive sacrococcygeal neoplasia was found on MRI. The owner decided to euthanize the dog but declined postmortem examination.","This Golden Retriever was brought in because she was having trouble with both her bladder and bowels, and also experiencing vaginal discharge. After some initial tests, doctors decided to do an MRI to get a better look at her spine and tail. During the first MRI attempt, the dog started to have difficulty breathing, so the procedure was stopped and other tests, like X-rays and blood tests, were done. The second MRI was scheduled for the next day, and the dog was given additional medications to help her relax. Finally, the MRI revealed a serious growth in her tail, and the owner made the difficult decision to euthanize her."
482,"A 45-day-old male Saanen kid was presented to the Veterinary Teaching Hospital, College of Veterinary Medicine, Sudan University of Science and Technology, with a history of an increasing swelling over prescrotal region.\nOn physical examination, the kid was found to be alert. The temperature, pulse, and respiratory rates were found within the normal range. A fluid-filled pocket (Figures and ) ventral to the penile urethra was seen and the urine was observed dripping from the prepuce and urethral process. Manual compression of the diverticulum showed subcutaneous urine leakage. Only a small amount of urine could be voided from the external urethral opening.\nNeedle centesis of the pocket revealed a presence of a fluid which was confirmed as urine on physical and chemical examination. Ultrasonographic examination was done on the right flank of the kid to visualize the kidneys. A real-time ultrasound scanner (Pie Medical Esaote, Aquila, Netherlands) equipped with switchable frequency (3.5–5) MHz curvilinear probe was used. Both kidneys were normal. The urinary bladder was also assessed and it was full of urine. Blood sample was taken for a complete blood count which revealed normal values. Urinalysis was also done and it was within the reference range. No other congenital anomalies, such as cryptorchidism or hermaphrodism, were identified.\nThe kid was referred to surgery. The animal was sedated using xylazine (Xylovet 20 mg/mL-Cp-Pharma) at a dose rate of 0.15 mg/kg. The site of operation was aseptically prepared with iodine (yamidine-povidone-iodine 10% USP). The site was locally infiltrated by lidocaine (Lignox 2%-Indoco) and finally draped for surgery.\nUrethral diverticulectomy was performed by elliptical skin incision around the dorsal border of the diverticulum after complete evacuation of urine using a 10 cc syringe. After incising the subcutaneous tissue, two hypospadiac urethral fistulae were identified. A small one () was found in the cranial part of the penis 1 cm ventrocaudal to the urethral process. The other large one () was found 5 cm caudal to the small one and cranial to the scrotum. Urethral catheterization was performed before closing the 1st urethral opening. The other opening could not be corrected due to catheterization failure.\nIncision on the urethral mucosa was extended through the opening and then sutured together to close the opening through simple interrupted sutures using polyglycolic acid, size 2/0; Huai'an Pingan Medical Instrument Co. Ltd., China.\nSubcutaneous tissues were sutured through simple continuous suture using absorbable surgical suture, Truglyde USP (size, 1, suture India PVT, Ltd). Finally, the skin was sutured by horizontal mattress using Ethilon polyamide, size 1, Ethicon Ltd. UK. After finishing operation, antibiotic injections were given to the animal (Penicillin-Penivet) for five days. The wound was dressed daily till the stitches were removed after 10 days. No mention was given to the large opening because of catheterization failure.","This 45-day-old male kid was brought to the vet because of a growing swelling around his penis. Examination revealed a fluid-filled pocket connected to his urethra, making it difficult for him to pee properly. Ultrasound showed his kidneys and bladder were normal, and a fluid sample confirmed it was urine. The vet performed surgery to remove the fluid pocket and repair the urethra, and the animal was given antibiotics and monitored closely."
483,"A 5-year-old female Cownose (Rhinoptera bonasus) was managed in Oceanarium, the large scale exhibition area of Aquaria in Kuala Lumpur (KLCC) with over 40 species of elasmobranchs and teleost fish. She is fed with marine chopped fish twice a day and Mazuri supplement (vitamins and minerals).\nCownose's caretaker noticed her left eye ball is protruding out from the orbit. He suspected she had crushed into the artificial coral when the male rays chased her for mating, and the period correlates with the mating season. The case was presented to University Veterinary Hospital (UVH), Universiti Putra Malaysia, on the same day that he complained of the eye trauma during field visit to Aquaria KLCC.\nPhysical examination was carried out. The Cownose was alert and responsive as she was actively swimming in the holding area. She was weighing 8 kg and had a Body Condition Score of 3 out of 5 and less than 5% dehydration. Temperature and pulse rate were not obtained, while the respiratory rate was 65 breaths per minutes. The left eye globe with hyphema was protruded from the orbital space and attached to the optic nerve (). The pupillary reflex could not be assessed due to hyphema. The left orbital space was exposed and periocular tissue tear was present around the left orbit (). Besides, multiple abrasions were observed at the cranial margin of the both pectoral fins, which was more severe on left fin.\nImmersion anaesthesia was opted. Seventeen ppm of isoeugenol (brand name: Aqui-s) was used as anaesthetic agent for both induction and maintenance of surgical plane. The anaesthetic bath was prepared by filling a tank with 475 L of artificial sea water from the holding area and added with 14.8 mL of Aqui-s. Ventilation rate was the only parameter that was used to monitor the anaesthesia depth of the Cownose. The normal ventilation rate was taken at rest before induction, which will be used as the baseline to monitor ventilation throughout the anaesthesia []. Ventilation rate of Cownose was taken by observing movement of spiracular flap and recorded every 5 minutes.\nThe Cownose was left in the anaesthetic bath for 10 minutes until its ventilation rate ceased to 40 breaths per minute and dropped in pectoral fin stroke activity (). Later she was transferred by using canvas hammock to surgical tank with wet towel at the bottom and covered with another wet towel on her dorsum to maintain skin moisture [] (). The maintenance of surgical plane and establishment of rebreathing system was done by flushing water with anaesthetics agent through the gills via right spiracle (respiratory opening caudal to eye) with a 20 mL syringe.\nThe Cownose was presented in ventral recumbency and assistants aided in restraining by gently exerting force at the dorsum as a part of tonic immobilization. Routine skin preparation around the left eye was done by using diluted chlorhexidine gluconate (dilution ratio 1 : 30) and diluted povidone iodine (dilution ratio 1 : 30) [].\nA ring block was done around the left eye by using 5 mL 20 mg/mL Lidocaine as local anaesthesia to reversibly desensitize the skin as well as analgesia (). Diluted gentamycin (5 mL, 100 mg/mL gentamycin + 5 mL sterile water) was prepared to flush the left orbital space and globe to minimize secondary infection since sterile surgical field is impossible to be established in the field setting (). The optic nerve was clamped by using 2 Rochester Pean forceps (). A surgeon knot was placed proximal to the first forceps with 2.0 PDS to ligate the blood vessel for haemostasis. Then the optic nerve was transected between the 2 forceps by using scalpel blade #20 (). The globe, the remaining conjunctivae fat, and extraocular muscle tissue were removed. The pedicle was checked for presence of bleeding before releasing it into the orbital space. The inner and outer muscular attachments were sutured with 2.0 PDS using interrupted suture pattern to close the orbital space (). Periorbital skin closure was done with 2.0 PDS in interrupted suture pattern ().\nThe enucleation procedure took 15 minutes to be completed. Fresh sea water without an anaesthetic agent was used to flush through the spiracle to perfuse the gills and accelerate recovery from anaesthesia. The recovery from anaesthesia was indicated by increasing ventilation rate to preinduction rate, which was 66 bpm in Cownose and when she started struggling actively by flapping pectoral fins at 25th minutes.\nPostoperatively, 5 mg/kg enrofloxacin was administered intramuscularly at the dorsolateral musculature close to the spine and terramycin ointment (active component: oxytetracycline hydrochloride) was applied twice a day topically on the suture site to prevent secondary infection of the suture site, orbital space, and optic sulcus []. Besides, the caretaker was instructed to apply povidone iodine (brand name: Betadine) on the abrasions of the pectoral fins twice a day until wound healed to prevent the proliferation of Fusarium sp., an environment saprophytic fungi [–].\nClient education consisted of isolating the Cownose from the school in holding area until surgical wound healed before releasing it to the large exhibition area, for ease of monitoring and for the prevention of possible infection at suture site as well as for administering daily treatment []. Besides, daily monitoring of suture site for break and possible signs of secondary infection such as discolouration of skin, fungal growth on the wound, inappetence, and loss of body condition is recommended by Mylniczenko and Penfold []. Next, putting of the mating pairs into separate tanks during mating season is necessary and a good practice. The mating process of rays is always aggressive as female rays always are being bitten and injured by the male rays, in which death can occur in severe case []. Others include reducing risk of crushing into decorations; isolation also reduces the stress of mating animals by avoiding being attacked by other fish during and after mating.\nAt day 7 after operation, we had a revisit trip to Aquaria KLCC for the follow-up. She was responding well to the treatment with good appetite and no clinical abnormalities were observed, and suture materials were still intact (). The last follow-up was on day 24 after operation and was done by phone; Cownose was having good appetite and the scars were returning to normal skin colour.","This Cownose ray was brought to the hospital after getting injured when it was chased by a male ray during mating season, resulting in a problem with its eye. The vet performed surgery to remove the damaged part of the eye, and the area was cleaned to prevent infection. The ray is now recovering well with medication and careful monitoring, and is expected to return to its normal life at the aquarium. The aquarium staff are taking extra precautions to protect the ray during mating season and prevent it from being injured again."
484,"A 9-year-old, 18.1 kg, spayed female, Blue Heeler-Cross dog was presented to the University of Wisconsin Veterinary Care (UWVC) for a draining cutaneous lesion on the right flank, intermittent fever, spinal pain, pelvic limb paresis, and muscle wasting, progressive over 12 months. A year prior to presentation the draining cutaneous tract was biopsied and cultured by the primary care veterinarian. Histopathology showed pyogranulomatous inflammation with severe vasculitis, and aerobic and anaerobic bacterial cultures were negative. Thoracic radiographs showed normal pulmonary parenchyma with no evidence of fungal granulomas. Urine was submitted for Blastomyces quantitative sandwich enzyme immunoassay (EIA; Miravista Diagnostics) and was negative. Pentoxifylline [22 mg/kg orally every 8 hr (Mylan Pharmaceuticals Inc., Morgantown, West Virginia, USA)] had been administered for 30 days to treat vasculitis, with minimal improvement in the draining tract. Anti-inflammatory prednisone [1 mg/kg orally every 24 hr (Roxane Laboratories Inc., Columbus, Ohio, USA)] resulted in substantial improvement in the fever and lesion drainage. As the prednisone dosage was tapered to 0.25 mg/kg orally every 48 hours, the fever returned and the draining tract worsened. Travel history included west Texas and northern California, but the dog had resided in Iowa and Wisconsin for the preceding four years.\nOn physical examination at UWVC, the dog was quiet, alert, responsive, and hydrated, with pink mucous membrane, a normal capillary refill time, and normal heart and respiratory rates. The rectal temperature was elevated at 104.1°F. The dog was paraparetic and was in pain upon thoracolumbar spinal palpation. An alopecic lesion with thickened dermis and a draining tract producing serosanguinous and mucopurulent fluid was present in the right flank. A fluctuant subcutaneous mass was palpable between ribs 12 and 13 in the left hypaxial area. There was no evidence of retinal abnormalities, mucocutaneous lesions, or long bone pain, and the dog was neurologically appropriate. The remainder of the physical examination was within normal limits.\nGiven the history and clinical presentation, a primary differential diagnosis for the thoracolumbar pain, fever, draining tract, and fluctuant subcutaneous mass was a migrating foreign body with possible secondary bacterial infection. Cytological examination of fluid from the left hypaxial fluctuant mass showed mixed inflammation, with nondegenerate neutrophils predominating and no microorganisms seen. An aerobic bacterial culture of fluid from the draining tract grew Staphylococcus pseudintermedius and Streptococcus dysgalactiae. A complete blood (cell) count (CBC) revealed mild normocytic normochromic anemia (hematocrit: 0.35 L/L; reference interval: 0.39 to 0.57 L/L), with a mild leukocytosis consisting of mature neutrophilia (neutrophils: 12.2 × 109/L, reference interval: 2.6 to 10.0 × 109/L). The serum biochemical panel was normal except for elevated globulins (49 g/L; reference interval: 22 to 35 g/L), consistent with an inflammatory response.\nA CT scan of the chest and abdomen was performed under general anesthesia and revealed pulmonary nodules with mildly enlarged sternal and cranial mediastinal lymph nodes. There was mild permeative to moth-eaten lysis of the cranioventral aspect of the vertebral body of T13, consistent with osteomyelitis. Multiple fluid pockets were present in the subcutaneous tissues, with the largest within the left hypaxial muscles at the level of T13 and in the subcutaneous tissues immediately lateral to the right pelvic inlet.\nThe dog was treated with amoxicillin/clavulanic acid [25 mg/kg PO q8 hr (Augmentin; GlaxoSmithKline, Philadelphia, Pennsylvania, USA)] to treat a bacterial infection suspected to be secondary to a migrating cutaneous foreign body that could no longer be detected. The owners were instructed to treat the dog for a total of 2 months and to monitor lameness, body temperature, and draining tracts.\nThe dog continued to have febrile episodes, trembling, and cutaneous drainage despite treatment with amoxicillin/clavulanic acid. The dog also developed right hind limb lameness and a new soft tissue swelling at the level of the right tarsus. The dog represented to UWVC for reevaluation 6 months after initial presentation. Physical exam findings were similar to initial presentation with the addition of a right tarsal swelling. A repeat CT scan of the chest and abdomen showed static pulmonary nodules and the development of pleural effusion. The vertebral bodies of T13 and L1 had progressive lysis consistent with progressive osteomyelitis. The previously noted fluid pocket within the left hypaxial muscle at the level of T13 had enlarged in size, but the caudal right fluid pockets had decreased in size.\nAn undetected persistent foreign body was suspected, and the left caudal thorax and abdomen were explored surgically. An elliptical incision was made around the fluctuant subcutaneous swelling over the cranial left flank near the 13th rib, and the surrounding subcutaneous tissue was dissected to isolate the abnormal tissue, which was excised and submitted for histopathology. Below the swollen tissue, multiple draining tracts were identified and followed communication with the thoracic cavity was documented, but no foreign body could be identified. The peritoneum, abdominal musculature, and subcutaneous tissue were closed and a Jackson-Pratt drain was placed at in the cranial left flank. The skin was closed using staples.\nHistopathology of the abnormal tissue showed marked locally extensive pyogranulomatous and lymphoplasmacytic dermatitis and cellulitis with draining tracts and intralesional fungal conidia. The fungal conidia were round structures approximately 30–40 micrometers in diameter with 2 micrometers of thick pale basophilic cell walls and heterogeneous amorphous pale amphophilic central material, consistent with immature Coccidioides immitis spherules (). These findings were consistent with a diagnosis of disseminated coccidioidomycosis with cutaneous, vertebral, and pulmonary involvement. To enable future clinical monitoring, serum was submitted for detection of Coccidioides antibodies by agar gel immunodiffusion (IDEXX Laboratories), and the serum antibody titer was 1 : 32.\nTreatment with fluconazole (Harris, Fort Myers, Florida, USA), 7.7 mg/kg PO, and q12 h was started along with terbinafine (Camber, Piscataway, New Jersey, USA), 27 mg/kg, PO, and q24 h. Anecdotally, terbinafine has been proposed to have synergistic effects when added to fluconazole in the treatment of Coccidioides []. Over the next 4 weeks, the previous dosage of prednisone was tapered and discontinued.\nThe patient's response to antifungal therapy was evaluated at the initiation of and throughout treatment using a modified Mycosis Study Group (MSG) score that is used in human patients (). The original MSG score takes into account clinical signs, radiographic imaging, and antibody titer and generates a composite score [–]. A modified MSG score that omits the radiographic component was used in this dog as has been described previously [, ], since the dog's lesions were best documented using a CT scan and repeated CT scans under anesthesia to monitor treatment response were not considered in the dog's best interest. Thoracic radiographs were considered to be an inaccurate substitution for a CT scan in this case, since the pulmonary nodules seen on CT were below the limit of detection on the baseline radiographs performed by the primary care veterinarian.\nDuring treatment with fluconazole and terbinafine, the dog developed inappetence, which resolved when terbinafine was discontinued after 4 weeks of treatment. After 3 months of fluconazole treatment, the dog's energy level had improved and the cutaneous lesion was no longer draining, although there was a fluctuant subcutaneous swelling at the site of the previous tract. The dog continued to have febrile episodes with trembling, but they were less frequent. The Coccidioides antibody test showed a rising titer (1 : 64) despite fluconazole treatment. Recheck chemistry panel was normal except for persistently elevated globulins (50 g/L; reference interval: 22 to 35 g/L) and mildly elevated serum alkaline phosphatase (270 U/L; reference interval: 20 to 157 U/L). As a result of the persistent fever, rising antibody titer, and a MSG score that was classified as unresponsive, rescue therapy with liposomal amphotericin B infusions and voriconazole was offered to the owner. The owner declined hospitalization for amphotericin B but elected to start voriconazole (Glenmark, Mahwah, New Jersey, USA), at 2.7 mg/kg, PO, q12 h, on an empty stomach. One week after starting voriconazole, a serum trough concentration was measured at 1.7 mcg/mL, which is within the therapeutic range targeted in humans with systemic mycoses (reference interval: 1.0–6.0 mcg/mL; the University of Wisconsin Health and Clinics Clinical Laboratories in Madison, Wisconsin) [, ].\nAfter 3 months of voriconazole treatment, the dog was consistently afebrile with no draining tracts, resolved paraparesis, and a normal energy level and appetite. The remaining clinical signs were pain upon thoracolumbar spinal palpation and a fluctuant swelling on the right flank cranial to the location of the draining tract. Repeat Coccidioides antibody titer at 6 months after diagnosis and 3 months after starting voriconazole was at 1 : 32. Chest radiographs showed normal pulmonary parenchyma with no nodules and the absence of pleural effusion. Recheck voriconazole serum trough concentration was submitted to the Fungus Testing Laboratory at the University of Texas Health Science Center in San Antonio, Texas, and was considered therapeutic at 2.01 mcg/mL (reference interval: 1.0 to 6.0 mcg/mL). A serum biochemical panel showed new hypoalbuminemia (16 g/L; reference interval: 27 to 39 g/L) and continued but stable hyperglobulinemia (46 g/L; reference interval: 24 to 40 g/L). Alkaline phosphatase had improved (191 U/L; reference interval: 5 to 160 U/L) from the previous biochemical panel. Because of the severity of the hypoalbuminemia, additional testing was performed; serum bile acids were within normal limits, (1.6 μmol/L preprandial; reference interval: 0 to 6.9 μmol/L; 6.2 μmol/L postprandial; reference interval: 0 to 14.9 μmol/L), urine was negative for proteinuria, and a baseline cortisol was only modestly decreased at 38.6 nmol/L (reference interval: 55.1 to 165.54 nmol/L). An ACTH stimulation test was not performed because the negative predictive value for hypoadrenocorticism remains high at the cut-point of a baseline cortisol ≥40 nmol/L, [] and azoles are known to suppress endogenous cortisol concentrations []. The dog was not clinically treated for hypoalbuminemia and FAST scan of the thorax and abdomen was negative for free fluid. Overall, the patient's modified MSG score was improved based on the amelioration of clinical signs and decreased Coccidioides antibody titer.\nAfter 6 months of voriconazole treatment, the fluctuant right flank swelling was enlarged and was turgid upon palpation. The voriconazole serum concentration was below therapeutic range (0.60 mcg/mL; reference interval: 1.0 to 6.0 mcg/mL). The voriconazole dosage was increased from 2.7 mg/kg to 4.1 mg/kg twice daily. Recheck serum voriconazole concentrations after one week of this higher dose were within the therapeutic range (1.25 mcg/mL; reference interval: 1.0 to 6.0 mcg/mL). Repeat Coccidioides antibody titer was stable at 1 : 32. Serum biochemical panel showed static to mildly improved albumin (19 g/L; reference interval: 27 to 39 g/L) and stable hyperglobulinemia (46 g/L; reference interval: 24 to 40 g/L). The ALP was mildly increased to twice the upper limit of the normal range (336 U/L; reference interval: 5 to 160 U/L), which was suggestive of cholestasis secondary to azole therapy. The bilirubin and ALT remained within normal limits. The patient's modified MSG score after increasing the voriconazole dose was stable based on continued resolution of the fever, resolved skin drainage, improved muscle mass, and stable Coccidioides antibody titer. At the time of writing, 13 months after diagnosis of disseminated coccidioidomycosis and 7 months into voriconazole therapy, the dog continues to feel well and remains free of fever or draining tracts, with normal energy and pelvic limb strength, with the exception of residual thoracolumbar pain and subcutaneous swelling in the right flank.","This dog had a long and complicated illness that started with a draining sore on his flank and led to several serious problems. Initially, doctors thought it might be a foreign body causing infection, but tests revealed a serious fungal infection called coccidioidomycosis. The infection spread to his spine and lungs, causing pain and weakness in his legs. Despite trying many different medications, the dog’s condition improved with continued treatment, including antifungal drugs and supportive care. Over the course of 13 months, the infection was brought under control, and the dog is now stable and doing well, although he still has some lingering pain and swelling."
485,"A 12-year-old, 12 kg, spayed, female terrier mix was noted by her owner to have excess tearing and a collection of blood in her left eye. The primary care veterinarian diagnosed anterior uveitis and hyphema (OS). Serum chemistry revealed a mildly elevated AST of 73 (15-66) and CPK of 1267 (59-895). Complete Blood Count (CBC) revealed 6 nucleated RBCs/100 WBCs, and T4 was normal. Her primary care veterinarian attributed the hyphema to trauma and prescribed oral carprofen tablets (25 mg BID) and NeoPolyDex (neomycin, polymyxin B, and dexamethasone 0.1%) ophthalmic suspension (TID). After several weeks without improvement, the owner sought consultation with a veterinary ophthalmologist. Examination revealed continued anterior uveitis and development of iris bombe in OS, as well as early immature cataract in the right eye (OD). The OS showed a fixed pupil with absent light reflexes due to posterior synechiation. There was a normal palpebral reflex but absent menace response and very diminished, but present dazzle reflex. The present hyphema in OS prevented visualization of the posterior segment. The OD showed trace flare consistent with anterior uveitis. OD also showed normal pupillary light reflexes as well as normal palpebral reflex, menace response, and dazzle reflex. Tonometry revealed 14 mmHg in both eyes. The presence of bilateral uveitis was indicative of a systemic condition. Doppler oscillometry during the first visit revealed a systemic blood pressure of 85 mmHg which ruled out hypertensive retinopathy. An infectious disease panel was obtained and submitted. Dorzolamide 2% ophthalmic solution (OS TID) and atropine 1% ophthalmic ointment (OD for 3 days) were added to the treatment regimen. Follow-up evaluation showed the hyphema in OS to be resolved as was the uveitis in OD with the above treatment. In addition, the intraocular pressures were 9 mmHg OD and 15 mmHg OS. The left eye continued to show iris bombe; therefore, an ocular ultrasound was performed which revealed a retinal detachment and a preretinal hyperechogenicity suspected to be vitreous hemorrhage, but no evidence of an intraocular tumor.\nInfectious disease fungal serology was negative for histoplasma, blastomyces, aspergillus, cryptococcus, and coccidiomycosis. Urine blastomyces antigen was negative. Ehrlichia canis titer was negative; Rocky Mountain spotted fever titer was 1 : 64 (normal < 1 : 64) which was suspected to be secondary to prior exposure as opposed to active infection. Toxoplasmosis ELISA IgM titer was negative but IgG titer was 1 : 256. CBC and chemistries were normal at this time with normal AST and CPK and no nucleated red blood cells. Clindamycin (150 mg po BID; 25 mg/kg/day) was prescribed for 30 days on an empirical basis to treat presumptive toxoplasmosis. A decision was eventually made to enucleate the OS as the eye was no longer visual (previously only demonstrating light perception), and the intraocular pressure had increased to 36 mmHg. Uveitis in the OD also became progressively worse necessitating topical treatment with NeoPolyDex ophthalmic suspension (TID), several topical nonsteroidal anti-inflammatories (diclofenac 0.1% ophthalmic solution, then flurbiprofen 0.03% ophthalmic solution, both TID), and atropine 1.0% ophthalmic ointment once weekly.\nThe OS was submitted for histopathologic evaluation and revealed the following findings: (1) mesenchymal malignant neoplasia suggestive of metastatic hemangiosarcoma, (2) severe hyphema and hemorrhage in the posterior chamber and vitreous, (3) iatrogenic lens capsule rupture, and (4) chronic glaucoma. The presence of atypical fusiform cells, carpeting the posterior aspect of the iris and ciliary body surface, dissecting the hemorrhage in the posterior chamber, and forming vascular channels, was deemed to be suggestive of metastatic hemangiosarcoma to the eye (). Pleomorphism was moderate with a few karyomegalic cells and mitotic figures. There was no significant inflammatory component that would be consistent with infectious uveitis. This diagnosis prompted a search for the primary neoplasm and further diagnostic work-up including thoracic radiographs and abdominal ultrasound. The ultrasound demonstrated an ill-defined, heterogeneous, partially cavitated mass measuring 3.4 cm in the spleen, distorting the normal contour. Thoracic radiographs were unremarkable. The patient underwent splenectomy and liver biopsy which showed a mass in the spleen measuring about 5 cm in diameter, still contained within the capsule. Intraoperative evaluation/inspection of the liver did not reveal gross abnormalities. There was no evidence of gross metastasis in the omentum or mesentery. The pathology report noted part of the splenic parenchyma to be effaced by a nonencapsulated, invasive, neoplastic growth with extensive tumoral necrosis and hemorrhage. The growth comprised erratic streams of neoplastic, endothelial-like, fusiform cells forming the linings of tortuous blood-filled channels and cavities supported by fibrous stroma (). There was mild anisocytosis, mild anisokaryosis, and a low mitotic count (1 mitotic figure per 10 consecutive high power fields, 400x). To further confirm the diagnosis of splenic hemangiosarcoma, immunohistochemical analysis using a monoclonal mouse anti-human CD 31 antibody (DakoCytomation, Denmark) was conducted on the splenic tumor tissue using an automated slide staining system (IntelliPATH, Biocare Medical, Pacheco, CA). Presence of CD 31 (platelet and endothelial cell adhesion molecule) staining confirmed the tumor to be of vascular origin. As can be seen in , the neoplastic cells were immunoreactive for CD31 antibody. The remaining splenic parenchyma was congested with extramedullary hematopoiesis. The liver biopsy showed hepatocellular hydropic degeneration.\nA decision was taken by the owner to use conventional adjuvant chemotherapy with doxorubicin monotherapy. Following the first cycle at a dose of 28 mg/m2, the dog experienced several episodes of vomiting. No further cycles of intravenous chemotherapy were administered. After consultation with several schools of veterinary medicine and a veterinary oncologist, the owner decided on a regimen of I'm-Yunity (polysaccharopeptide from the Coriolus versicolor mushroom, dose of 100 mg/kg/day po), metronomic chlorambucil 1.0 mg po QD (2.0 mg/m2), high doses (900 mg) po QD of omega-3 fatty acids, and Yunnan Baiyao 250 mg po BID for two weeks on, one week off. Initially, firocoxib 57 mg po QD was used in combination with chlorambucil but was discontinued after two weeks due to gastrointestinal toxicity. Over the next 24 months, the patient maintained a stable weight, demonstrating high energy level and excellent quality of life. There were no adverse effects of the treatment regimen and the patient was on this combination continuously for two years. Her vision continued to deteriorate in the right eye due to progressive cataract formation and continued uveitis despite the use of flurbiprofen, tacrolimus, and NeoPolyDex TID. Serial thoracic radiographs and abdominal ultrasound obtained every 3-4 months over 24 months showed no evidence of visible pulmonary, hepatic, or right atrial metastases. Over the two-year time frame, the patient developed polyuria and polydipsia, mild anemia of chronic disease (Hgb/Hct = 13/34 decreased from 16/50), progressive increase in alkaline phosphatase (from 221 to 727, normal range 20–150), and rising BUN/creatinine (from 20/0.8 to 47/1.5 mg/dl) (IRIS stage 2 renal disease). Otherwise, her laboratory parameters were normal. Several days after achieving 24-month survival, the patient developed new onset tonic seizures. Phenobarbital was started at a dose of 16 mg po BID, increasing to 32 mg po BID. However, seizure frequency continued to increase with one to two episodes daily over the course of the next 10 days. Therefore, a decision was made to euthanize the patient given that the most likely cause of the seizures was a brain tumor.","Summary: This dog was diagnosed with a serious eye problem called uveitis, which caused blood in the eye and blurred vision. After trying several treatments, the doctors determined that the eye needed to be removed to prevent further damage. Further investigation revealed that the dog had a cancerous growth in the spleen that had spread to the eye, and the tumor was not responding to treatment. Despite ongoing supportive care and medication, the dog’s condition continued to decline, and the owners made the difficult decision to euthanize the dog to prevent further suffering. Over the course of two years, the dog received a combination of medications and supplements to manage the cancer and its side effects, but ultimately, the disease progressed, leading to seizures and a poor quality of life."
486,"A 60-year-old man who presented with a recent history of impaired vision of right eye for about 3 months due to brain lesions. He was diagnosed with liver cancer and underwent surgery three months ago. The pathological result is hepatocellular carcinoma. His right eye showed impaired vision and the proton MRI revealed brain metastasis located in the right orbital part (Figure ).\nThe patient was not suitable for the surgical excision due to his poor physical conditions after liver tumor resection. CyberKnife radiosurgery was delivered at a dose of 22.5 Gy in three fractions (Figure -).\nBoth sodium and proton MRI (Siemens Magnetom 7T, Erlangen, Germany) were performed periodically at 7T by the time schedule of pre-treatment, 48 hours after SRS, with one week follow up and one month follow up. The scan parameters are listed in Table .\nThe sodium signal in the tumor is obviously higher than the opposite normal brain tissue within one month after SRS (E-H). However, the T2 magnetic resonance imaging reveals no obvious changes (A-D) (Figure ).\nThe quantified sodium signal intensity in MRI was shown in Table .\nThe time course of sodium signal intensity in the tumor showed a dramatic increase in the treated brain tumor compared to the pretreatment and SRS within 48 hours. And the signal intensity decreased at one week after SRS compared to 48 hours after SRS. However, the increased signal intensity was observed at one month. The TSC ratio of tumor to cerebrospinal fluid (CSF) is much more intuitive (Figure ).","A 60-year-old man recently diagnosed with liver cancer had a new problem: his vision in his right eye was getting worse. Brain scans revealed a tumor in his eye, which was linked to the liver cancer. To treat the brain tumor, he received a focused radiation treatment called CyberKnife. Follow-up scans showed a significant increase in the tumor’s signal, indicating the treatment was effective, and the doctors are continuing to monitor the situation."
487,"An 11-year-old boy reported to the department of oral and maxillofacial surgery, Bhimavaram, Andhra Pradesh, India, with a chief complaint of swelling over the left cheek since three months. The swelling was asymptomatic and gradually progressing. A detailed history from the attending parent revealed no significant medical history and no previous history of trauma in the concerned area. On an extraoral examination, there was a gross facial asymmetry on the left side of the face due to the presence of a swelling that extended superiorly from the infraorbital margin to the upper lip inferiorly, obliterating the nasolabial fold. No secondary changes were noticed over the skin. There was no sensory deficit in relation to the facial structures. On a thorough intraoral examination, dentition was mixed, with mild caries affecting the deciduous teeth, none involving the pulp, and revealed a bicortical swelling on the left side extending from the labial frenum medially till the distal aspect of the deciduous second molar, obliterating the labial and buccal vestibule (Figure )\nAn orthopantomogram (OPG) revealed a well-defined unilocular radiolucent lesion circumscribing the permanent teeth buds of the central, lateral incisor and canine in the second quadrant (Figure ). It measured around 3 cm in its greatest dimension.\nThe paranasal sinus (PNS) view revealed radiolucency extending superiorly till the infraorbital margin with the canine involved (Figure ). The canine was in Nolla’s stage 7 and the central and lateral incisors were in stage 8.\nAspiration of the cystic contents revealed a straw-colored fluid. Based on the clinical and radiographic findings, a provisional diagnosis of a dentigerous cyst was made (Figure ). A differential diagnosis of unicystic ameloblastoma and a cyst involving the maxillary antrum were considered. Based on the above diagnosis, the patient was planned for decompression of the lesion under general anesthesia. After obtaining a procedural and general anesthesia consent from the parents, a thorough pre-anesthetic evaluation was carried out and the obtained routine blood investigation parameters were within normal limits.\nUnder general anesthesia, the extraction of the deciduous central, lateral incisors and canine was done and an opening was created in the anterior wall of the cyst through which part of the lining was removed and sent for histopathological examination. To maintain the patency of the opening, a marsupialization catheter device customized from the tip of a suction catheter (Figure ) was inserted through the opening and was secured to the margins using 3-0 silk sutures (Figure ). Parents were instructed to irrigate the cystic cavity using normal saline twice a day for a period of one month, through the opening.\nThe histopathological report obtained confirmed the diagnosis of a dentigerous cyst. The patient was regularly recalled every week and was assessed for the maintenance of patency through the device. Radiographs were taken at regular intervals to aid in assessing the eruption of the impacted permanent teeth. Device removal was performed after three months (Figure ).\nAfter a period of three months, the radiographic examination revealed a gradual resolution of the radiolucency of the lesion and a spontaneous eruption of the central and lateral incisors, indicating osteogenesis (Figure ). So, during a subsequent visit, the patient was referred to the department of orthodontics for the assistive eruption of permanent teeth into the dental arch through traction.\nInitially, brackets were bonded on the central and lateral incisors and the canine and the flap was closed. Strap-up was done with 0.016 Niti wire. Later, leveling and alignment was done for about six months with 0.0018 Niti 17x25 Niti, 19x25 Niti, 19x25 SS (Figure and Figure ). After reaching 21x25 SS, wire traction was applied using ligature wire. After the eruption of the central and lateral incisors, an open coil spring was placed for the alignment of the canine after gaining space (Figure ). The canine was brought into occlusion and finishing and detailing were done. The entire treatment spanned five years for all the three impacted teeth to get aligned in the dental arch (Figure and Figure ).","An 11-year-old boy came to the doctor because he had a growing swelling on his left cheek that had been bothering him for three months. Doctors found that the swelling was caused by a cyst around his baby teeth, which was blocking the eruption of his permanent teeth. The swelling was removed, and a special tube was placed in the cyst to keep it open and allow the permanent teeth to grow through. After three months, the swelling disappeared, and the permanent teeth erupted, and the boy’s dentist then worked to align the teeth for a healthy smile."
488,"A 63-year-old man with a deep vein thrombosis diagnosed 5 days prior, presented with fever, tachycardia, and nausea/vomiting. The patient had a past medical history of cerebrovascular accident 9 years prior with residual left-sided weakness, epilepsy, hypertension, and hyperlipidemia. The patient denied smoking, alcohol, or drug use. The patient was transferred from an acute rehab center where a left lower extremity deep vein thrombosis had been found on ultrasound 5 days prior, and anticoagulation started. He was bridged from enoxaparin to oral warfarin. Initial laboratories were as follows: white blood cell count 12,600/mL\n3\n, hemoglobin 14.0 g/dL, platelet 302,000/mL\n3\n, total bilirubin 2.1 mg/dL, aspartate aminotransferase 68 IU/L, alanine aminotransferase 56 IU/L, prothrombin time 20.3 seconds, international normalized ratio (INR) 1.95, albumin 3.1, fibrin degradation products > 10 and < 40, blood urea nitrogen 14, and serum creatinine 0.83. Initial physical exam showed left lower lobe decreased breath sounds, and an unremarkable abdominal exam. At that time, the patient denied any pain, current nausea/vomiting, shortness of breath, constipation, or diarrhea. Chest X-ray suggested consolidation in the left lower lobe. The decision was made to admit the patient as he met the criteria for systemic inflammatory response syndrome, possibly due to a left lower lobe pneumonia versus pulmonary embolism. For further evaluation, a chest computed tomography (CT) scan was ordered. While the CT scan showed no evidence of pulmonary embolism or pneumonia, it did incidentally reveal a distended appearance to the gallbladder (\n). Ultrasound showed distended gallbladder with a heterogeneous mixture of intermediate and low echogenic material with no evidence of stones, which was concerning for possible pus or hemorrhage. Liver was of normal size and echogenicity. Patient was started on ceftazidime, clindamycin, and vancomycin empirically for systemic inflammatory response syndrome/sepsis. The patient was initially ruled out as a surgical candidate due to his elevated INR, and given his extensive deep vein thrombosis, it was felt that the risk of stopping anticoagulation outweighed the benefits and he was recommended for interventional radiology (IR)-guided cholecystostomy. Repeat physical exam on hospital day #2 revealed a firm, tender right upper quadrant of the abdomen, with all other quadrants being soft and nontender. The patient's INR spiked to 6.28 on hospital day #2 and his hemoglobin dropped to 9.8 g/dL. This elevation of his INR was attributed to his continued anticoagulation, which was subsequently discontinued. INR on hospital day #3 was 4.53, and 4.94 on hospital day #4, at which point vitamin K and fresh frozen plasma were administered. INR improved to 1.69 on hospital day #5. IR-guided cholecystostomy was attempted on hospital day #4. The decision to proceed with an IR-guided approach had been made earlier as it was felt he was at high risk for surgery and septic. However, there was an organized clot with no drainable material and subsequently no drain left in place. An inferior vena cava filter was placed on hospital day #8. The family and patient had also up to this point refused surgery due to his history of stroke, do not resuscitate/do not intubate status, and their perceived risk of surgery. However, they eventually agreed to have surgery. On hospital day #11, laparoscopic cholecystectomy was performed, and revealed dense adhesions surrounding the gallbladder including the omentum and bowel. These adhesions were taken down to reveal a very large distended gallbladder with areas of necrosis and perforation. The contents of the gallbladder contained approximately 1,000 cubic centimeters of old clot which had perforated near the infundibulum with clot extending out into Morison's pouch and the right upper quadrant causing dense adhesions to the liver. The gallbladder was freed from these adhesions and the clot was removed. The gallbladder was dissected and freed from surrounding adhesions. The cystic duct was secured with a “PDS Endoloop” made by Ethicon. During the course of the operation, patient experienced significant bleeding and required four units of packed red blood cells. The operation was converted to an open cholecystectomy to obtain hemostasis, and a Jackson-Pratt drain was left in place. Pathology of the gallbladder specimen showed extensive hemorrhage, acute inflammation, and necrosis. Pathology of a liver specimen showed moderate fibrosis, which was suggestive of cirrhosis. However, hepatitis serology was all negative, and ultrasound of the liver showed a liver of normal size and echogenicity. No other investigation showed signs of chronic liver disease. Patient had one episode of bleeding from his drain which required transfusion, but the remainder of his hospital course was otherwise uneventful. The patient was considered too high risk for further anticoagulation and was discharged with an inferior vena cava filter in place.","This 63-year-old man was admitted to the hospital after developing a fever, nausea, and shortness of breath while recovering from a blood clot in his leg. He has a history of stroke and other health problems, including kidney issues. Tests revealed a possible infection in his lungs and a severely inflamed gallbladder, which was causing complications. After a series of tests and consultations, he underwent surgery to remove his gallbladder, and a filter was placed in his leg to prevent future blood clots. He was then discharged home with ongoing monitoring."
489,"A 38-year-old man with schizophrenia presented after his second suicide attempt through an overdose with 48 tablets of burotizolam, 42 tablets of haloxazolam and 14 tablets of levomepromazine. The patient's childhood and adolescent development was normal. He was a good student and an active soccer player in high school. His social skills were standard, and he had no family history of mental illness. When he was 23 years old and a fourth year university student, he became convinced that he was being observed and he withdrew from social activities. His parents brought him to a psychiatric hospital, and he was diagnosed with schizophrenia according to DSM-IV-TR (). The prescribed medication worked well and he was able to graduate from university at 27 years old. After graduating, he worked part time in a convenience store or at a nursery for several years. He then started to work at a distribution business under a handicapped employment program. His father committed suicide 3 years before he first presented at our hospital and a friend also died from a sickness. Because his auditory hallucinations repeatedly told him that he was responsible for their deaths, he could not stop blaming himself for their passing, in spite of his mother and brother telling him that he was not responsible. He was pessimistic about his future partly because he was able to earn only a meager income. In order to increase his income, he started a second part-time job at a supermarket in addition to his distribution job. He slept less and felt the accumulation of fatigue. He started to stockpile sleeping medications and he eventually took 76 tablets of brotizolam and 30 tablets of eszopiclone. The next morning his mother found him unconscious and called an ambulance. His mother brought his empty medicine containers to the hospital. At his first presentation, his physical examinations and vital signs were normal. He appeared to be very sleepy, but he managed to speak. The emergency department doctor ordered a blood test, a chest x-ray, an electrocardiogram test, a urine toxicology test, and a computed tomography brain scan. All results were within normal range, except a positive result for benzodiazepine in his urine and a slightly elevated white blood cell count (10.92 × 103/μL). The emergency doctor enlisted a psychiatric doctor to evaluate his mental state. The patient claimed that his auditory hallucinations sounded like someone was booing in addition to radio sounds from a distance. He also claimed he was being tracked by the police. He admitted suicidal ideation and reported that he was sad because he could not die. Because his depressive symptoms occurred 4 weeks prior to his first admission, the authors carefully excluded the possibility of schizoaffective disorder and depressive disorders or bipolar disorder. However, the patient did not show manic symptom or markedly diminished interest, and his depressive thoughts seemed to ease shortly after his admission. Obviously, his mood episodes have been present for a minority of the total duration of the active and residual phases of illness; however, his memory changing delusion and auditory hallucination remains continuously. Furthermore, he showed negative symptom that he had withdrew from social activity except working. The authors diagnosed schizophrenia according to DSM 5 (). His decreased ability to discriminate between his thought and true memories as mentioned previously suggests the presence of disturbance of the self which also supports this diagnosis (). The authors prescribed risperidone 6 mg, brotizolam 0.25 mg, and eszopiclone 2 mg. Soon after the treatment started, he became calm and claimed his suicidal ideation disappeared. However, during the patient's second hospitalization, 6 months later, he admitted that he had lied. He wanted to go home quickly so he pretended to be healthy. He subsequently obtained a distribution job contract for the coming season by himself and he was supposed to be followed by a nearby clinic as a condition of his hospital discharge. He started his distribution job but he could not work regularly. Again, he wanted to earn more money so he started attending lectures to get a healthcare worker license. Consequently, his sleep time was reduced and he started to feel life was troublesome once again. He subsequently overdosed as mentioned previously. The next morning, his mother brought him to the emergency department again. She had no idea when he attempted to commit suicide but she last saw him the previous night at 10 p.m. His mother brought his empty medicine containers. His vital signs were normal, and he managed to speak. The emergency doctor conducted a blood test, a chest x-ray and a computed tomography brain scan. All the results were normal, except an elevated white blood cell count (12.16 × 103/μL), creatine kinase (429 U/L), and chloride (109 mmol/L). His mother brought with her more than 100 risperidone tablets. It became obvious that he had not taken his pills regularly. The authors thought his adherence worsened during his psychotic period and started a long acting injectable antipsychotic (LAI). Because the patient worked regularly, the authors choose an injection given once in a 4-week period. Furthermore, because several studies showed it made significant improvements in the quality of life (), the authors chose aripiprazole LAI at 400 mg. The authors also prescribed 20 mg of suvorexant per day and gradually discontinued brotizolam 0.25 mg and flunitrazepam 2 mg because the authors were concerned about a possible third suicide attempt while using benzodiazepine. Because both of the patient's admissions were associated with poor sleep, the authors examined the patient by polysomnography (PSG) and a multiple sleep latency test (MSLT) to exclude comorbid diseases such as sleep apnea syndrome or restless legs syndrome. As shown in Figure , he woke frequently during his sleep (25.6 times per hour on average as shown in Figure ) and he lived with excessive daytime sleepiness (he fell asleep within 2 min; on four out of five trials during the MSLT, as shown in Figure ). His Apnea-Hypopnea Index (AHI) was slightly elevated (5.1 times/hour), and respiratory events were not associated with significant desaturations (the minimum SpO2 was 95%). His BMI was 19.8. Malocclusion or tonsil swelling was not observed. Figure shows the patient's sleep log. The patient did not show sleep phase advance or delay. The patient's Pittsburgh Sleep Quality Index (PSQI) score was 13, while over 5 points on the PSQI represents insomnia (). Two months after his second admission, he was discharged while being prescribed suvorexant 20 mg, and chlorpromazine 25 mg per day in addition to aripiprazole LAI 400 mg per month. His Brief Psychiatric Rating Scale (BPRS) () dropped form 48 at admission to 42 at discharge. Six months after his second admission, the authors and the patient started CBT-i according to the CBT-i therapeutic manual (). The authors also referred to the four causes cited by Chiu et al. (): (a) beliefs that sleep problems cannot be changed; (b) trauma and adversity; (c) lifestyle choices and lack of motivation; (d) medication side effects and the 12 problems cited by Waite et al. (): (a) Poor sleep environment; (b) Lack of daytime activity; (c) Lack of evening activity; (d) Disrupted circadian rhythm; (e) Sleep as an escape from distressing experiences; (f) Fear of bed; (g) Nightmares; (h) Night-time awakenings; (i) Sleep disrupted by voices/paranoia; (j) Worry; (k) Neuroleptic medication side effects; and (l) Reducing hypnotics. Our CBT-i consisted of eight sessions with each session ranging from 30 to 45 min. The first two sessions were educational sessions that attempted to find disturbances such as a misunderstanding of sleep hygiene or an inadequate sleep environment. In the other six sessions the authors and the patient tried to find other targets to tackle. For instance, the patient tried eating a carbohydrate (banana) before sleep, stopped checking his watch, warmed his body before going to bed, turned off small lights in his room, changed his routine of taking a bath before eating dinner to prevent him from taking a nap after dinner, bought a blackout curtain and an air conditioner. He also tried to wake up early in order to exercise in the morning instead of doing in the middle of the night because he believed he can fall asleep soon after the exercise. The whole course of sleep and psychological tendencies are shown in Figure . The patient's BPRS dropped to 24 and his PSQI dropped to 8. His sleep time increased steadily however, at his sixth session, he claimed that he could not sleep at night and he felt a strong sense of sleepiness during the day. His mental health care team consisted of two physician groups; with one group treating his psychiatric symptoms and the other group (the authors) treating his sleep abnormality. The first physician group increased the patient's chlorpromazine from 25 to 37.5 mg. The authors, as the second physician group treating the patient's sleep abnormality, discussed reducing the patient's chlorpromazine with the first physician group because the authors believed that his sleep troubles were not caused by a difficulty in falling asleep but by the dosage of chlorpromazine being too high for the patient's current ability to fall asleep which was gradually being strengthened by CBT-i. At the seventh session, the authors encountered another misunderstanding of the patient in which the authors believed the patient's headaches were being caused by a lack of sleep, while the patient used chlorpromazine as a painkiller. The authors prescribed acetaminophen 400 mg as a painkiller, and stopped the administration of chlorpromazine. At the eighth session, the patient claimed that he had almost no trouble sleeping except when he forgets to take suvorexant.","This man has a history of mental health challenges, including schizophrenia, and has attempted suicide multiple times. He’s been struggling with severe hallucinations and difficulty sleeping, which led to him taking a dangerous amount of medication. After several hospital stays and trying different medications, doctors found that he was having trouble sleeping and experiencing daytime sleepiness. They discovered this through tests and realized he needed help with his sleep habits, so they started a program called CBT-i to teach him how to sleep better. With the help of therapy and medication adjustments, he’s been able to improve his sleep and overall mental health."
490,"In 2016, a 25-yr-old woman complained of fever and abdominal pain. She was living in a small village in Mianeh, East Azerbaijan Province, Iran ().\nThe latitude for Mianeh is: 37.426434 and the longitude is 47.724111. The patient declared a regular picking and eating local watercress in the rural area. She was admitted to a local hospital and was treated symptomatically. Despite treatment, fever, jaundice, right upper quadrant abdominal pain and loss of appetite continued for 6 months. Informed consent was taken from the patient.\nInitial laboratory findings were as follows: white blood cells 4.3×103 μL, platelets 245 ×103 μL, hemoglobin 12.3 g/dL, hematocrit 37.9%. Peripheral blood smear revealed eosinophilia as high as 36% of the white blood cells. Stool examination was negative for ova/cysts. Liver function tests showed elevated liver enzymes (aspartate transaminase 37 [normal < 31] U/L, alanine transaminase 63 [normal < 32] U/L, alkaline phosphatase 458 [normal 98–279] U/L and a normal total bilirubin.\nAbdominal ultrasonography showed splenomegaly (140 mm). A magnetic resonance cholangiopancreatography (MRCP) revealed an ill-defined lesion of 96×53 mm at 4th and 8th segments of liver with dilation of intrahepatic bile ducts inside the lesion and splenomegaly was seen. The initial pre-treatment CT imaging revealed a hepatosplenomegaly with regional lymphadenopathy and several subcapsular lesions in both liver lobes.\nIn the search for a potential malignancy, liver biopsy was performed. Section from liver tissue showed focal lobular necrosis with peripheral palisading spindle-shape epithelioid cells; surrounded by moderate inflammatory cells mainly eosinophils and plasma cells beside a few lymphocytes. Other parts of liver tissue showed mild infiltration of a few eosinophils, lymphocytes and few plasma cells without piecemeal necrosis or bile duct damage, which led to the suspicion of a parasitic infection (toxocariasis, capillariasis or strongyloidiasis).\nOn May 24, 2016, the patient was referred to the Department of Medical Parasitology and Mycology of Tehran University of Medical Sciences, Tehran, Iran. After reviewing the clinical / para-clinical findings and interviewing, fascioliasis was suspected. ELISA test was performed as previously described (). Antibodies specific to Fasciola ES antigens were detected in the serum sample. Furthermore, the formalin ether concentration technique showed the presence of F. gigantica eggs (135 μm-80 μm) in feces ().\nEgaten treatment was started at the dose of 10 mg/kg. The drug was given as a single oral dose. Three months after treatment, the patient was examined again, fecal examination was negative for F. gigantica eggs, and IgG titers decreased but remained positive during 9 subsequent months. Blood examinations revealed an improvement in the liver function tests with a decrease in blood eosinophil counts (5%)\nIn order to provide genetic diagnosis, the stool sample was washed twice with phosphate buffered saline (PBS) to remove the ethanol. The egg walls were mechanically disrupted using glass beads (0.45–0.52 mm diameter) and by freezing and heating (−70 °C for 5 min and 90 °C for 5 min). Total genomic DNA was extracted, using a commercial kit (QIAamp DNA Stool Mini Kit; Qiagen GmbH, Hilden, Germany) according to the manufacturer’s instructions. ITS-1 fragment (about 700 bp) was amplified by PCR using a set of 5’- ACCGGTGCTGAGAAGACG -3’ and 5’- CGACGTACGTGCAGTCCA -3’ as forward and reverse primers, respectively, following the protocol previously described (). The negative control was a reaction mixture of distilled, without the DNA template. . The PCR products were separated in 1.5% agarose gel using Simply Safe (Eurx, Cat. No. E4600-01). A phylogenetic tree was constructed, based on the ITS-1 gene sequences to show the relationships between the available sequences of F. hepatica, F. gigantica in the GenBank, and sequence of our sample (700 bp), using the MEGA 6 software () ().","This 25-year-old woman was admitted to the hospital after six months of persistent fever, abdominal pain, and jaundice. Initial tests revealed high levels of white blood cells and eosinophils, and imaging showed an unusual growth in her liver and enlarged spleen. A liver biopsy suggested a parasitic infection, and tests confirmed she had a liver infection caused by tapeworms. She was treated with medication, and follow-up tests showed the infection cleared, with a decrease in the number of parasites and improved liver function."
491,"A 61-year-old man underwent resection of a part of his tongue due to tongue cancer and was admitted to our hospital for hepatocellular carcinoma with about 5 cm diameter of tumor at the liver segment IV. Computed tomography (CT) showed that the tumor was enhanced during the arterial phase and washed out during the portal phase, and the tumor pressed the right anterior branch to the main branch of Glisson and the middle hepatic vein; hence, the diagnosis of hepatocellular carcinoma was made (Fig. ). The indocyanine green retention rate at 15 min was 21.8%. Child-Pugh score was A. The patient tested negative for hepatitis B surface antigen and hepatitis C virus antibody. He had a history of excessive consumption of alcohol, and alcoholic liver damage was considered as a possibility. Partial hepatectomy with segments IV + V and cholecystectomy with cystic duct-tube drainage were performed. Intraoperative findings indicated that the anterior branch of bile duct was exposed at the resected area, and some small bile ducts were ligated. On POD 1 following hepatectomy, bile leakage developed from the drain placed in the foramen of Winslow. CT showed fluid collection in the cavity between the liver and fistula to drain. Cholangiography via the endoscopic bile duct enhancement showed no communication between the common bile duct and abdominal cavity. Drip infusion cholangiography (DIC)-CT revealed the bile duct of the peripheral side. We maintained simple drainage to reduce the cavity. On POD 19, this patient had fever and CT revealed that the fluid collection has increased (Fig. ). Percutaneous drainage to the cavity near the liver cut surface was performed. Cholangiography via the c-tube did not show the anterior branch of the bile duct. Fistulogram from the drainage tube at the abdominal cavity showed the bile duct at segments V and VIII (Fig. ). We diagnosed the bile leakage from the isolated bile duct of segments V and VIII. Further management was needed to control the persistent biliary leak of 200–250 mL/day. Liver function was evaluated again. The indocyanine green retention rate at 15 min was 27.7%. LHL15 was 0.575. Liver volume of segments V and VIII was 260 mL, and remnant liver volume was 1272 mL, which were calculated using the 3D image analysis system (SYNAPSE VINCENT; Fuji Photo Film Co., Ltd.). Functional remnant liver volume (FRLV) was calculated based on the liver volume using gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid-enhanced MRI for 20 min []. FRLV after liver resection of segments V and VIII was 2176 mL. Even if the function becomes extinct by PTPE to segment V and VIII, remnant liver volume was sufficient. We considered that bile leakage could not be cured with either PTPE or bile duct ablation alone, because of the large volume of bile leakage more than 200 mL per day. The therapeutic strategy involved combination therapy of PTPE and bile duct ablation. First, percutaneous transhepatic cholangiography drainage (PTCD) tube was inserted to the bile duct of segment V. PTPE with coil embolization was performed to the part of the portal vein of segments V and VIII by puncturing the part of the portal vein of segment V. The liver volume of segments V and VIII was decreased from 260 to 123 mL after PTPE. After the PTPE, bile leakage decreased to about 50 mL/day. We confirmed that cholangiography via the PTCD tube showed the bile duct at segments V and VIII. For bile duct ablation, 1.2 mL pure ethanol was injected from the PTCD tube. Over 1.2 mL of ethanol leaked into the abdominal cavity. After ethanol injection, the PTCD tubes were clamped for 5 min. Then, another bile duct ablation with 2.0 mL of pure ethanol was performed 1 week after of the first procedure. After bile duct ablation, bile leakage has decreased from 50 to 10 mL/day gradually. The patient left the hospital, and he was rehospitalized and reinjected with 2.0 mL of pure ethanol for three times. The drainage tube and bile duct tube were removed on POD 139 (Fig. ). The clinical course is summarized in figure. Complications with combination treatment of PTPE and bile duct ablation were not noted.","A 61-year-old man was admitted to the hospital after surgery to remove part of his tongue due to cancer, and he was also diagnosed with liver cancer. Tests showed the liver cancer was pressing on important blood vessels, and a drainage tube was placed to manage a leak from the bile duct. Despite treatment, the leak continued, and further procedures were needed to stop the bleeding. Ultimately, a combination of treatments successfully controlled the leak, and the patient was discharged home."
492,"A previously healthy 48-year-old male was found to have multiple hepatic tumors and a giant tumor (67 × 53 mm) superior to the inferior vena cave by abdominal ultrasonography during a routine medical examination. He consulted a general physician for further evaluations and had been referred to us because plain CT confirmed multiple tumors, including a giant tumor, in the liver. A laboratory workup on admission showed that total bilirubin (0.6 mg/dL) and albumin (4.2 g/dL) concentrations were within their normal ranges, whereas aspartate aminotransferase (37 IU/L), alanine aminotransferase (70 IU/L), alkaline phosphatase (176 U/L), and gamma-glutamyl transpeptidase (170 IU/L) concentrations were mildly increased. Tumor markers, including alpha-fetoprotein (2.9 ng/ml), protein induced by vitamin K absence or antagonist-II (11 mAU/ml), and carcinoembryonic antigens 19-9 (4.0 IU/L), were within normal limits.\nAbdominal ultrasonography (US) revealed well-defined, hypo echoic masses in segment 1 (S1) (67 × 53 mm in diameter), S6 (13 mm), and S2 (9 mm) in the liver (Fig. –). Abdominal dynamic CT revealed a low-density 65-mm diameter mass with an irregular margin in plain, peripheral early ring enhancement in the arterial phase, and internal heterogeneous enhancement in the delayed phase (Fig. –). Gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid-enhanced magnetic response imaging (EOB-MRI) demonstrated a low-signal intensity mass on T1-weighted images. This mass contained several high-signal intensity areas on T2-weighted images. EOB-MRI also revealed a hypointense mass in the hepatobiliary phase (Fig. –). Furthermore, it showed higher intensity compared with the normal liver parenchyma on DWI with a high b value of 1000 (Fig. ). Its ADC value was 2.11 × 10−3 mm2/s (peripheral area) and 2.45 × 10−3 mm2/s (central area), and the ADC mean was 2.33 × 10−3 mm2/s. It was described as a high-intensity mass on the ADC map (Fig. ). There was no accumulation of [18F]-FDG on FDG-PET (Fig. ). Hepatic arteriography revealed hypervascular masses in S1 (65 mm in diameter), S6 (16 mm), and S2 (9 mm) of the liver, with no pooling (Fig. , ). In addition, gastroscopy and colonoscopy showed normal findings.\nOn the basis of these imaging findings, a preoperative diagnosis of multiple scirrhous hepatocellular carcinomas characterized by abundant fibrous stroma was made, and caudate lobectomy of the liver and partial hepatectomy of S2 and S6 were performed. The resected specimen contained a 55 × 56 mm yellowish white solid mass. The cut surfaces of the tumor were elastic and soft with smooth margins (Fig. ).\nHistopathological examination showed that the tumors were composed of various sized irregularly dilated vessels with some blood thrombi, inflammatory cell infiltration, fibrous and hyalinized sclerotic or myxomatous stroma (Fig. , ). On immunohistochemistry staining, these vascular endothelial cells were positive for CD34, suggesting sclerosing hemangioma (Fig. ). The patient tolerated the procedure well and was discharged on postoperative day14. The patient gave consent for publication of details of his case.\nHemangiomas are the commonest benign hepatic tumors, being found in up to 7% of autopsies in one series []. Hemangiomas have a predilection for women in a ratio of 5:1. They are characteristically discovered incidentally during abdominal imaging in individuals aged 40 to 50 years []. Hepatic sclerosing hemangioma, first reported by Shepherd et al. in 1983 [], is a rare type of hepatic hemangioma composed of abundant acellular hyalinized tissue in which small vessels are occasionally seen. Another study reported finding them in only two of 1000 autopsies []. Hepatic sclerosing hemangiomas are caused by degenerative changes such as thrombus formation, necrosis, and scar formation in hepatic cavernous hemangiomas; however, the mechanism(s) for these degenerative changes has not yet been determined []. Makhlouf and Ishak compared the findings in sclerosed hemangioma and sclerosing hemangioma []. They asserted that recent hemorrhages and hemosiderin deposits rich in mast cells are present in sclerosing hemangiomas, whereas fibrosis, increased elastic fibers, and dystrophic or psammomatous calcifications with decreased numbers of mast cells are characteristic of sclerosed hemangiomas [].\nThese pathological changes can result in the radiological features on CT and MRI being atypical of hemangioma; thus, these lesions can be diagnosed as malignant tumors such as intrahepatic cholangiocarcinoma, metastatic liver cancer, and hepatocellular carcinoma. We searched for “hepatic sclerosing hemangioma” in PubMed and ICHUSHI, a bibliographic database established in 1903 and being updated by the Japan Medical Abstracts Society, and identified 41 patients, including our patient, the characteristics of which we here summarize.\nConcerning imaging studies, Miyamoto et al. [] summarized the imaging findings in 41 hepatic sclerosing hemangiomas. Their average diameter was 41.8 mm, ranging from 10 to 145 mm. Abdominal US showed hyperechoic masses in 10 patients and hypoechoic masses in 14. Plain CT generally showed a low-density mass, whereas dynamic CT showed ring enhancement, resembling metastatic liver cancer or intrahepatic cholangiocarcinoma, in 31 of 40 (78%) reported cases. MRI showed low-intensity signals in 28 of 30 (93%) reported cases on T1-weighted images and high-intensity signals in 25 of 30 (83%) reported cases on T2-weighted images. FDG-PET showed no accumulation of [18F]-FDG in six patients who underwent this procedure []. The radiological features revealed by dynamic CT and MRI resembled those of hepatic malignancies. Thus, there were indeterminate imaging features, some imaging findings pointing to intrahepatic cholangiocarcinoma, metastatic liver cancer, or scirrhous hepatocellular carcinoma (ring enhancement, hyperintensity on T2 with hypointensity on T1 imaging). Generally, imaging findings of ring enhancement on dynamic CT is less typical of classical hepatocellular carcinoma than of metastatic liver cancer or intrahepatic cholangiocarcinoma. In recent years, there have been a small number of reports in which DWI and DWI-based ADC were successfully used to differentiate sclerosing hemangioma from liver malignancies. The ADC values for hepatic hemangiomas are reported to be higher than those of malignant liver tumors due to restricted water diffusion from high cellular density [, ]. In our case, the ADC mean was 2.33 × 10−3 mm2/s, and the mass showed higher than the normal liver parenchyma with a high b value of 1000 on DWI. It was described as a high intensity mass on an ADC map, indicating that possibility of the sclerosing hemangioma, used as a diagnostic aid to detect. FDG-PET showed no accumulation of [18F]-FDG, it suggested the possible benign character of this sclerosing hemangioma. However, it should be noticed that negative predictive value of FDG-PET for primary hepatocellular carcinoma is less than 50% with sensitivity over 80% []. Therefore, we selected hepatic resection as a first-line strategy for the management of tumors with unknown potential.\nHistopathologically, the cut surfaces of our patient’s resected tumors corresponded with cross-sectional CT images; the dynamic enhancement pattern is related to the vascular spaces component and the central areas of low density on the arterial and delayed phase to the sclerotic component, which on examination were yellowish-white with many hyalinized areas with poor vessels and fibrous changes [, ]. Additionally, our patient’s serum tumor markers, including AFP and PIVKA-2, were within normal limits. If these multiple tumors had been hepatocellular carcinomas, concentrations of these markers may have been high. Thus, it also suggests that the multiple tumors were not hepatocellular carcinomas.\nConfirmation of our provisional diagnosis would have required obtaining a liver biopsy. Although a fine needle biopsy can differentiate a sclerosing hemangioma from hepatocellular carcinoma, this procedure can potentially lead to rupture or seeding of hepatocellular carcinoma. Fine needle biopsies should only be performed to confirm inoperable hepatocellular carcinoma because seeding of tumor in the needle tract has been reported in 1–3% of cases []. We suspected that our patient’s lesions were scirrhous hepatocellular carcinomas because of the imaging features. In addition, it was considered that the lesion protruding into the intraperitoneal cavity rupture was at risk of rupture. The likelihood of malignancy and risk of rupture resulted in our decision to perform hepatic resection on our patient.","A 48-year-old man was found to have several tumors in his liver during a routine checkup. These tumors were identified through imaging tests like ultrasound and CT scans, which showed unusual patterns. The tumors caused some changes in his liver function tests, and further tests ruled out other possible causes like gallbladder problems. The imaging results initially resembled those of more serious conditions like cancer, but the patient’s tumor markers were normal. After careful examination, doctors determined that the tumors were likely benign sclerosing hemangiomas, a rare type of non-cancerous growth. They decided to remove the tumors through surgery to be sure. The surgery was successful, and the patient is now recovering well. The doctors believe that the surgery has addressed the issue and that he is in good health."
493,"A 52 year-old Chinese man presented with gross hematuria and had a left nephrectomy done in January 2014. Pathology revealed clear cell RCC of Furhman 2 grading, with invasion of the renal vein and peri-renal fat. There were synchronous solitary right lung and right hilar lymph node metastases. Having declined high-dose interleukin-2, he was started on pazopanib in March 2014, achieving partial response. In January 2015, pazopanib was stopped and surgery was attempted for the oligometastatic disease, but the right hilar node was found to be stuck down intra-operatively. External beam radiotherapy was administered post-operatively in February 2015 to the hilar node (55 Gy in 20 fractions). In May 2015, after 4 months off anti-angiogenic therapy, there was global progression of disease with the right hilar node enlarging and new metastases appearing in multiple sites (lungs, muscle, bones). Sunitinib was started (May to September 2015), and the patient went on to receive further lines of drug treatment with everolimus (October 2015 to February 2016) and axitinib (March to October 2016). He also had palliative surgery to the right radius (curettage and fixation in October 2015) and right proximal femur (curettage and bipolar hemiarthroplasty in November 2015). In November 2015, radiotherapy was also given to the right radius and femur post-operatively, to an enlarging and symptomatic scalp metastasis at the vertex, and to 4 brain metastases by gamma knife technique. Further courses of radiotherapy were given to a large lytic sacral metastasis (February to March 2016), several skin and subcutaneous tumors (May to June 2016), and the left knee (July to August 2016). In addition, subcutaneous denosumab was given as adjunctive treatment for bone metastases from October 2015 to September 2016.\nIn October 2016, computed tomography (CT) scan showed widespread metastases with interval progression in the skeletal muscles, liver, spleen, right kidney, right adrenal, pancreas, peritoneum, lungs and right hilar nodal mass. Apart from 3 new small cutaneous metastases, the patient did not have symptoms related to any specific organ site. He required the use of a walking aid after his previous hip surgery. Performance status was 2 by Eastern Cooperative Oncology Group score (ECOG). There were multiple enlarging metastases within the single right kidney, but renal function was appropriate for a post-nephrectomy setting. For example, an upper pole lesion now measured 6.5 × 4.3 cm compared to 2.5 × 2.4 cm in the scan 5 months prior. In view of the florid radiologic progression and previous multiple lines of anti-angiogenic treatment, he was offered immunotherapy. Axitinib was stopped and a single dose of nivolumab at 100 mg was given within the same day.\nThe patient developed acute renal failure 2 weeks later with oliguria, rising creatinine and hyperkalemia. Serum creatinine rose progressively from a pre-nivolumab level of 117 μmol/L to a high of 247 μmol/L in the following 5 weeks (Fig. ). Urine sediment was not active (1 white cell and 1 red cell per high power field each, and no casts). Serum phosphate and serum creatine kinase were normal. Other blood indices showed anemia, mild hypercalcemia (2.81 mmol/L), raised alkaline phosphatase (ALP) and lactate dehydrogenase (LDH). Ultrasound (US) of the remaining kidney showed infiltrative renal tumors. There was no hydronephrosis and renal perfusion was good. Except for renal parenchymal tumor infiltration and inflammatory edema, no other plausible causes of acute renal failure were identified. Renal biopsy was not performed as this was a single kidney. There was clinical deterioration with development of dyspnea, back pain, edema and drop in performance status to ECOG 3. At 4 weeks from nivolumab, the subject of hemodialysis was broached but the patient declined. He also expressed his preference not to have aggressive resuscitation or intensive care unit management, and was referred to the home hospice service. Although the recent 3 new skin metastases had resolved, a non-contrasted CT at 5 weeks post-nivolumab documented worsening tumor in multiple sites: skeletal muscles, liver, spleen, pancreas, peritoneal nodules, lungs, right hilar nodal mass and other soft tissue areas. The right kidney was larger, consistent with increasing intrarenal tumor or inflammation. He was at home on expectant management for the next 4 weeks. Several visits were made by the home hospice team. Palliative medications included oral tramadol and gabapentin, and he was prepared for death.\nAt week 10, the patient unexpectedly walked into the clinic, having felt better a week prior. There was clinical improvement in his general condition and he reported an increase in urine output. Serum creatinine had improved to 131 μmol/L (Fig. ), ALP was normal, and serum calcium had normalized without any anti-resorptive agent. There was severe anemia (hemoglobin 4.4 g/dL) and the LDH was raised at 1019 units/L (range 250–580). Chest radiography showed improvement in the right hilar and lung shadows. Red cell transfusion was administered. At week 11, non-contrasted CT scan showed improvement in tumor status in most of the involved sites including a decreased size of the right kidney. Blood and bone marrow investigations for the anemia were consistent with immune-mediated hemolysis and oral prednisolone was started at week 13. The patient continued to improve and a contrast CT at week 20 showed dramatic improvement in tumor status. In some sites, including the kidney, essentially complete remission was seen. Serum creatinine returned close to baseline (Fig. ). Prednisolone was tapered off to complete a 3 month course with hemoglobin stabilizing at 11.3 g/dL. At 6 months post-nivolumab, the patient was doing well without further immunotherapy.\nSerial CT and US images of the right kidney were analyzed (Fig. ). CT imaging showed marginal increase in kidney size from baseline to the 5 week post-nivolumab scan, and subsequent decrease at the 11 week scan when the renal function had recovered. There was no pre-nivolumab US scan, but the US scans done at 2 and 5 weeks post-nivolumab showed worsening of the renal tumor load (Fig. ). Onset of diffuse renal cortical swelling was also noted in the US at 5 weeks post-nivolumab, as demonstrated by the progressive compression and obscuration of renal medulla and sinus fat. The US changes are commensurate with the progressive worsening of renal function at these time points. A lower pole metastasis shown in the US at 2 weeks post-nivolumab was significantly larger than the corresponding lesion on the baseline contrast CT, despite the differences in imaging modality. A contrast CT at 4 months as well as an US at 6 months post-nivolumab showed decreased renal size and near complete resolution of the renal metastases.","This man has had a long and challenging battle with cancer. He was originally diagnosed with kidney cancer that had spread to his lungs and other parts of his body after a previous surgery. He received several different treatments over many years, including chemotherapy, radiation, and targeted drugs, but the cancer continued to grow. Despite these efforts, he developed acute kidney failure after starting a new immunotherapy drug. However, surprisingly, his condition improved significantly a few weeks later, and his kidney function returned to normal. Further scans showed a dramatic reduction in the cancer throughout his body, and he is now doing well with ongoing monitoring."
494,"A 30-year old woman presented with two weeks of left lower quadrant discomfort during her second month of pregnancy. Ultrasound revealed a mixed cystic and solid left adnexal mass measuring 8.6 × 6.7 × 8.3 cm. Left salpingo-oophorectomy was performed early in the second trimester. Grossly, the left ovary demonstrated an intact 7.5 cm complex cystic-solid mass, with nearly half involved by a friable, tan-yellow tumor with papillary excrescences, some of which were freely floating within the cyst cavity. Histologic sections demonstrated a struma ovarii with over 50% characterized by the classic features of a well-differentiated papillary thyroid carcinoma (Fig. ). The periphery of the tumor impinged on the ovarian capsule and vascular structures; however, no definitive evidence of invasion was seen on the histologic sections reviewed (Fig. -). Typical histology for an ovarian mature cystic teratoma (such as areas of squamous differentiation) were present in other areas of the tumor (Fig. ). The tumor expressed positive immunohistochemical staining for thyroglobulin (Tg) and thyroid transcription factor 1 (TTF1, Fig. ), but was negative for BRAF V600E. Serum Tg was 83.5 ng/mL (reference range for euthyroid status in nonpregnant adults 0–35 ng/mL) seven days after surgical resection of the ovarian mass. The elevated Tg level is reasonably expected given the proximity to surgery and Tg half-life of 65 h [] and the known increase in Tg levels during pregnancy [, ]. Thyroid function tests (TSH 1.66 mU/L) and thyroid ultrasound were normal. Levothyroxine (L-T4) was started to suppress thyroid stimulating hormone (TSH) to 0.1–0.5 mU/L although TSH ranged 0.86–1.41 mU/L during pregnancy. After delivery, Tg was 2.9 ng/mL (TSH 0.57 mU/L). With thyroglobulin levels in a reasonable range as above, the risk of additional studies during pregnancy in terms of radiation or hypothyroid exposure, unknown risk of metastatic disease, and unknown incremental benefit of intervention during pregnancy, the decision was made to delay further testing and therapy until after delivery. Two months after delivery, the patient underwent total thyroidectomy to permit evaluation for metastatic disease and monitoring for recurrence by Tg levels. The thyroid pathology was benign.\nSeveral months after delivery and cessation of lactation, the patient underwent L-T4 withdrawal and her Tg unexpectedly rose from 0.8 ng/mL (TSH 5.8 mU/L) to 113.7 ng/mL (TSH 46 mU/L). A concurrent diagnostic I-131 scan (1 mCi) with planar and SPECT/CT imaging revealed focal central neck activity consistent with thyroid remnant and the presence of iodine-avid skeletal metastatic disease involving the left ninth rib, bilateral proximal femurs, and sacrum (Fig. -). Following 320 mCi I-131 therapy based on blood dosimetry calculations, post-therapy scan revealed the additional finding of miliary pulmonary metastases (Fig. ). Due to high-quality SPECT/CT images, separate dedicated CT and bone scan were not necessary. She was subsequently treated with TSH suppression resulting in an undetectable Tg < 0.1 ng/mL (TSH 0.02 mU/L). In order to assess the interval response to I-131 treatment, follow-up evaluation after L-T4 withdrawal protocol was obtained nine months later, demonstrating a stimulated Tg of 0.2 ng/mL (TSH 36.4 mU/L) and negative diagnostic I-131 scan without focal abnormal radioiodine uptake in the neck or skeleton. As she was planning for a second pregnancy and her pulmonary disease was not visible on the prior diagnostic I-131 scan but became apparent only on the post-therapy scan, a 100 mCi I-131 therapeutic challenge was performed to eliminate any microscopic residual disease and more accurately ascertain the treatment response. The post-therapy I-131 scan demonstrated complete resolution of pulmonary metastatic disease; however, faint residual foci of activity were demonstrated in the left ninth rib, sacrum, and left proximal femur, consistent with a partial therapeutic response in the skeleton (Fig. ). Eight months later, Tg was < 0.1 ng/mL (TSH 0.02 mU/L). The patient continues to do well, being conservatively managed with active clinical and biochemical monitoring during L-T4 suppression therapy.","This patient was diagnosed with a cancerous growth on her ovary during her second trimester of pregnancy. To protect the baby’s health, the growth was surgically removed. The growth was identified as a type of thyroid cancer that had spread to her ovaries. After the surgery, doctors monitored her thyroid levels and waited until after the baby was born to further investigate the cancer. Later, the cancer spread to her bones and lungs, requiring radiation treatment. After treatment, the cancer was successfully eliminated, and she is now being closely monitored to ensure it doesn’t return."
495,"A 79-year-old woman was referred for evaluation of abnormal murmur. She presented with a 3-months history of worsening shortness of breath and orthopnea. Physical examination revealed a soft continuous murmur at the left upper chest with basal crackles in both lungs. Chest radiography showed mild cardiomegaly with mild pulmonary congestion. The ECG showed regular sinus rhythm without evidence of ischemia or prior myocardial infarction.\nTransthoracic echocardiography demonstrated a mildly dilated LV with markedly dilated left atrium. The LV ejection fraction was 60% with no wall motion abnormality. There was an unusual, tubular-like structure inside the interventricular septum with a turbulent, predominantly diastolic flow on color Doppler (Fig. , arrows; Additional file ). Transesophageal echocardiography revealed a markedly dilated RCA arising from the right aortic sinus (Fig. , arrow; Additional file ), while the origin of the left coronary artery (LCA) could not be demonstrated. There was a tortuous, abnormal vessel located adjacent to the main pulmonary artery (MPA) emptying into the posteromedial aspect of the MPA. There was an accelerated, continuous flow across the stenotic ostium (asterisk, Fig. , Additional file ). Moreover, there was severe MR from a restricted posterior leaflet of the mitral valve (MV) associated with ventricular remodeling in combination with mitral annular dilatation (Fig. , Additional file ).\nCoronary angiography with a single RCA injection revealed a markedly dilated RCA (Fig. ) providing multiple intercoronary collaterals of various sizes communicating with the left coronary system (Fig. ). The LCA later opacified the MPA through a stenotic ostium (Fig. , asterisk; Additional file ), establishing the diagnostic hallmark of ALCAPA syndrome. The calculated ratio of pulmonary-systemic blood flow was 1.4, confirming a significant left-to-right shunt. Coronary computed tomography angiography clearly identified the ALCAPA with a retropulmonary ostium (Fig. and , asterisks). Volume-rendered image depicted the course of the anomalous coronary arteries and its inter-coronary collateral pathways along the epicardial surface and where the LCA connected to the MPA (Fig. and , open arrow; Additional file ). Stress cardiovascular magnetic resonance perfusion imaging demonstrated no myocardial ischemia, suggesting adequate collateral circulation to the LV.","This 79-year-old woman came to the doctor because she’s been having trouble breathing and experiencing swelling in her legs. Tests showed that her heart is slightly enlarged, and there’s an unusual, twisted artery inside her heart that’s causing some problems with blood flow. This unusual artery is connected to the pulmonary artery and is causing a significant shunt, where blood flows from the lungs to the heart. The doctors have identified this as ALCAPA syndrome and are planning further treatment to address the issue and improve her breathing."
496,"Herein, we describe the case of a 73 year-old man in good health, who developed widely disseminated sebaceous carcinoma including metastases to brain, visceral organs, lymph nodes, and bone.\nHe initially presented in late October 2016 for removal of a rapidly growing nodule in the anterior abdominal wall. Two days later he developed confusion, urinary incontinence and progressive aphasia. Emergent magnetic resonance imaging (MRI) of the brain showed 4 enhancing gray-white matter junctional lesions, the two largest measured 3.8 × 3.3 cm in the right frontal lobe and 2.3 × 2.5 cm in the left frontal lobe. Two smaller enhancing lesions in the right parietal lobe measured 8 mm and 4 mm in diameter. In November 2016, he underwent craniotomy and resection of bilateral frontal lobe tumors, and he made a full neurologic recovery and went on to receive post-operative gamma knife radiosurgery to the resection cavities and the small parietal brain lesions (Fig. ).\nHis case was reviewed in melanoma tumor boards at the Masonic Cancer Clinic, University of Minnesota. Sections of tumor revealed sheets of epithelial cells with moderate eosinophilic cytoplasm and areas of tumor infiltrating lymphocytes (Fig. ). Cells exhibited nuclear pleomorphism and increased mitotic activity (Fig. ), desmoplastic stromal reaction and necrosis (Fig. ). Immunohistochemical staining was positive for cytokeratin AE1/AE3 and cytokeratin 7, and negative for S100, HMB45, Melan-A, CD45, calretinin, ERG, p40, TTF1, CDX2, and GATA3. The immunoprofile ruled out melanoma, mesothelioma, lymphoma, sarcoma with epithelioid features, and most visceral carcinomas. Microscopic examination revealed intracytoplasmic lipid vesicles (Fig. ), confirmed by diffuse membranous reactivity for adipophilin [, ] (Fig. and ). The findings supported a histopathologic diagnosis of sebaceous carcinoma. Importantly, additional tumor testing confirmed high expression of PD-L1 in 100% of tumor cells (Fig. ). Commercial genomic testing using next-generation sequencing (Foundation Medicine, Massachusetts, USA) confirmed the tumor was microsatellite stable and carried a mutational burden of 17 mutations/Mb. Table also shows various somatic mutations in genes for regulatory transcription factors, DNA repair proteins, growth factor receptors, and targetable MAPK signaling proteins. Several of the affected genes have also been described in cases of sebaceous carcinoma reported in the COSMIC () database [].\nInitial staging positron emission tomography-computed tomography (PET/CT) revealed evidence of widely disseminated disease involving lung and liver, muscle, bone, and multi-compartment bulky lymphadenopathy in chest and abdomen (Fig. ). Standard chemotherapy approaches using platinum-based chemotherapy were reviewed. However, the patient and family strongly favored a less toxic therapy, considering advanced age and quality of life concerns. Given the strong rationale for use of checkpoint inhibitors in several other tumor types, moderately high tumor mutational burden (17 muts/Mb), and strong PD-L1 expression the patient opted for anti-PD1 immunotherapy. He initiated off-label treatment with pembrolizumab (2 mg/kg, every 3 weeks) in December 2016.\nFollow up PET/ CT scan 3 months after the initiation of anti-PD1 therapy revealed remarkable improvement in lymph nodes, lung, and soft tissue, however, there were multiple new and enlarging hepatic and osseous metastases initially worrisome for progression(Fig. ). After multidisciplinary review, the findings were felt consistent with pseudo-progression and immunotherapy was continued. Restaging PET/ CT obtained after 6 months of treatment showed further significant improvements in all previously noted lesions (Fig. ), with residual FDG activity seen in small mediastinal and abdominal lymph nodes. To further characterize the patient’s innate and adaptive immune status at the time of his near complete response, a flow cytometry study of peripheral blood was performed. Lymphocyte subset analysis showed evidence of circulating CD45RA-CD27+ central memory (CM) and effector memory (EM) T cells, and a population of mature CD16 + CD57+ NK cells (Fig. ).\nHe continued on pembrolizumab, however, after 10 months of therapy he developed severe fatigue and orthostatic hypotension requiring hospitalization. Laboratory testing showed him to have secondary adrenal insufficiency with low (< 0.7 mcg/dL) serum cortisol and low (< 11 pg/mL) ACTH levels. He began 1 mg/kg prednisone with a prolonged steroid taper, and during high dose steroid therapy pembrolizumab dosing was interrupted. In December 2017, with 12 months of follow-up, restaging PET/CT was obtained and showed new FDG avid mediastinal and abdominal lymph nodes and a new hepatic and small bowel lesion (Fig. ). Biopsy of the liver lesion in January 2018 confirmed recurrent metastatic sebaceous carcinoma, and repeat immunohistochemical staining showed tumor cells continued to express high levels of PD-L1 (not shown). After discussion, the patient elected to restart immunotherapy, and he was continued on maintenance adrenal replacement therapy with hydrocortisone (10 mg/5 mg). Recent restaging studies demonstrate growth of the mediastinal metastases and a reduction in the size of the hepatic and small bowel metastases, suggestive of pseudoprogression and a durable ongoing response to pembrolizumab (Additional file ).","This man, age 73, was diagnosed with a rare and aggressive type of cancer called sebaceous carcinoma that had spread to his brain, organs, and bones. After initially removing a tumor from his abdomen, doctors discovered more tumors in his brain and treated them with radiation therapy. Despite this, the cancer continued to spread, and doctors determined that immunotherapy, specifically pembrolizumab, was a promising option. The patient responded well to the immunotherapy, with significant improvements in his condition, but unfortunately, the cancer later returned. He is currently receiving ongoing treatment and monitoring to manage the cancer's progression."
497,"A 70 years-old man with a dilated ischemic cardiomyopathy, triple coronary artery bypass grafting (CABG) in 1990 and chronic renal failure (baseline GFR: 45 ml/min/1.73 m2) underwent a coronary angiography for a Non-ST segment elevation myocardial infarction (NSTEMI). Baseline values of Hs-Troponin T and CK-MB were 497 ng/l and 211 U/l respectively. Native coronary circulation was completely occluded at the proximal segments. Grafts angiography showed a tandem calcified lesions of SVG on distal right coronary artery (RCA) (Fig. ) and an ostial stenosis of the SVG on first obtuse marginal branch (OM1). Left internal mammary artery on the mid left anterior descending artery was patent. Ad Hoc PCI of SVG on RCA was attempted. The proximal calcified stenosis has been crossed with a 1.5 x 12 mm balloon only with the support of Guidezilla™ guide extension catheter (5-in-6 Fr), however the non compliant (NC) balloon 2.5 x 15mm was unable to break the hard and calcified plaque (Fig. ). After several attempts, the procedure was interrupted with a suboptimal result (Fig. and ). An elective PCI of SVG on RCA with rotational atherectomy was performed (left radial approach, 6 French). Two runs with 1.25 mm burr (Fig. ) and 2 runs with 1.5 mm burr (Fig. ) were carried out. Then, the use of distal anchoring balloon warranted support and tracking, made as centring rail for the advance of the tip of the “mother-and-child” catheter into the SVG. During slow deflation of the balloon, the Guidezilla™ catheter (5-in-6 Fr) was advanced distal to the proximal stenosis to be stented, thus allowing a first drug eluting stent (DES) 3.5 × 48 mm to be placed on the mid-distal segment of the graft according to a LSD technique. The same technique was applied to deploy a second DES proximally to the first one (3.5 × 28 mm on proximal segment). During both rotational atherectomy and PCI, as well as at the final angiographic control, we did not observe any sign of embolization and the procedure was successfully completed with a final TIMI III flow without complications. A slight elevations of the cardiac biomarkers the day after the procedure (Hs-Troponin T 564 ng/l and CK-MB 308 U/l) was followed by a gradual reduction of the latters in the next 4 days and the patient was discharged in stable conditions.","A 70-year-old man with heart problems and kidney issues had a blockage in his arteries, leading to a heart attack. Doctors tried to open the blockage with balloons and special tools, but it was too difficult due to the hardened plaque. Ultimately, they successfully placed two stents to improve blood flow to his heart. Although his heart enzymes were slightly elevated after the procedure, he recovered well and was discharged from the hospital."
498,"A 9-month-old female presented with a 2-month history of abdominal distension and weight loss.\nThe patient underwent an uncomplicated birth following an unremarkable pregnancy at 39-weeks gestational age via elective caesarean section. The toddler attained normal developmental milestones aside from the inability to roll prone from supine. Initially at the 70th percentile at birth for weight, she presented at the 30th percentile with stable height and head circumference (50th percentile). The patient did not exhibit any clinical features of Beckwith Wiedemann Syndrome including omphalocele, macroglossia and macrosomia.\nThere is no consanguinity, no family history of recurrent malignancies, haematological or renal conditions.\nInitial limited ultrasonographic study of the abdomen demonstrated massively enlarged kidneys with loss of corticomedullary differentiation. The right kidney measured 13.1 cm and the left measured 15 cm (normal approximately 6 cm) []. Multiple ill-defined hypoechoic areas were seen randomly interspersed within the renal parenchyma bilaterally suggesting presence of nephrogenic rests and therefore nephroblastomatosis. It was not possible to rule out the presence of Wilms tumour within the nephrogenic rests on ultrasound. No evidence of hydronephrosis, hydroureter or free fluid was seen.\nComputed tomography (CT) of the abdomen and pelvis with IV and oral contrast demonstrated homogenous, diffuse, renal enlargement and loss of normal architecture with renal parenchyma replaced by homogenous low attenuating peripheral masses bilaterally (Fig. ). There were two ill-defined hypodense areas in the medial aspect of the left kidney concerning for malignancy. Residual normal renal parenchyma was present as areas of striate enhancement, hyperdense in comparison to the thick rind of peripheral nodules. Based on clinical presentation and CT findings, the patient was diagnosed with bilateral DHPLNB.\nGadolinium enhanced magnetic resonance imaging (MRI) of the abdomen performed after 6 weeks of chemotherapy demonstrated hypointense peripheral masses on T1/T2 images with thick septations suggestive of bilateral DHPLNB. A small heterogeneously enhancing lesion with multiple small linear and round cysts in the medial left kidney was characterized on T1 imaging, suspicious for a superimposed Wilms tumor (Fig. ). Diffusion weighted imaging was not conducted.\nNo metastases were seen on CT chest with contrast. Pathology after left partial nephrectomy confirmed Wilms tumor.\nCytogenetic analysis was conducted revealing a normal female karyotype (46XX). No other tumour markers or genetic testing was performed.\nBilateral DHPLNB complicated by a left renal mass (likely Wilms Tumour) Renal lymphoma (uncommon in infants and young children).\nThe patient was treated with COG protocol AREN 0534 EE-4A chemotherapy, including vincristine and dactinomycin for 20 weeks. Due to the presence of the superimposed left renal mass, a left partial nephrectomy and perihilar lymph node dissection was performed. Following the surgery, she also received left flank radiotherapy to a dose of 1080 cGy in 6 fractions.\nPathology was consistent with DHPLNB and resection of a Wilms tumor. Resection margins were positive. Nodal dissection was negative. Following surgical resection and chemotherapy, the patient was followed with MRI every 3 months for a year. Thereafter, the patient was followed with MRI every 6 months for 2 years. Currently, the patient is now on surveillance with ultrasounds every 3–4 months.","This 9-month-old girl has been growing slowly and has had a swollen belly, which led doctors to investigate further. Tests showed that her kidneys are much larger than they should be, and there are some unusual areas within them that could be cancerous. A CT scan revealed a Wilms tumor in her left kidney, and she underwent surgery to remove it, along with chemotherapy and radiation. Currently, she is being closely monitored with regular scans to ensure the cancer doesn't return."
499,"The patient was a 7-year-old Turkish girl born to non-consanguineous parents. She was being followed up since 3 years of age because of bilateral glaucoma and was prescribed medicines [50 mL of Sholl solution and anti-acidosis capsule (three times a day)] and eye drops (β-blocker and carbonic anhydrase inhibitor). Her mother had oligohydramnios; the patient had intrauterine growth retardation and was born prematurely. There was no family history of any inherited diseases, cataract or pRTA. Both her weight and height were below the 3rd percentile, and she demonstrated intellectual disability. However, the other physical examinations, including neurological signs, were unremarkable.\nThe laboratory tests were as follows: Na, 139 mmol/L; K, 3.1 mmol/L; Cl, 110 mmol/L; blood urea nitrogen, 12 mg/dL and creatinine, 0.9 mg/dL. Blood gas analysis revealed a pH of 7.22, HCO3− concentration of 11 mmol/L and PCO2 of 29 mmHg. In addition, urinalysis revealed a pH of 5, no protein and no glucose. The urinary excretion of amino acids was normal, and the urinary β2-microglobulin level was 110 μg/L (normal: < 240 μg/L). These investigations revealed that the patient had pRTA without Fanconi syndrome – generalized dysfunction of proximal tubule. Considering her short stature, the levels of thyroid hormones, IGF-I and IGFBP3 were normal. Furthermore, whereas the renal ultrasound revealed a 9-mm diameter parenchymal stone in the right kidney, brain MR imaging revealed no intracranial calcification. Neither of the parent showed these symptoms.\nAt the age of 9, our patient presented with micrognathia, fish-mouth, epicanthal folds, ptosis, low-set ears, a short neck with a low hairline, a broad shield-like chest, wide-spaced nipples, hypoplastic areolae, cubitus valgus and short fourth metacarpals, with other symptoms due to NBCe1A absence such as dental abnormalities, suggesting the coincidence of Turner’s syndrome. In addition, her weight and height were still below the 3rd percentile. Hormonal investigation data were as follows: FSH, 69.3 mIU/mL (normal: 4.5–20.0 mIU/mL); LH, 15.9 mIU/mL (3.5–14.0 mIU/mL) and oestradiol, < 5.0 pg/mL, suggesting hypergonadotropic hypogonadism. Her karyotype was 45, XO which confirmed the diagnosis of Turner’s syndrome.\nFrom 200 μL of peripheral blood samples obtained from the patient and her parents, we extracted DNA using the QIAamp DNA Blood Mini Kit (Qiagen Inc.) according to the manufacturer’s instructions. Similarly, we extracted RNA from 1 to 2 mL of the peripheral blood sample using the Isogen (Nippon Gene) or the QIAamp RNA Blood Mini Kit (Qiagen Inc.) according to the manufacturer’s instructions. Then, the complementary DNA (cDNA) of the patient was synthesised from the polyA(+) RNA of the peripheral white blood cells using the cDNA Synthesis Kit (Takara) as previously described [] or the RevertAid First Strand cDNA Synthesis Kit (Thermo Scientific) according to the manufacturer’s instructions.\nThe polymerase chain reaction (PCR) condition used was as follows: denaturation for 9 min at 95 °C, followed by 35 cycles of 95 °C for 1 min, 60 °C for 1 min and 72 °C for 1 min, with a final extension at 72 °C for 7 min. PCRs were performed using a thermal cycler PerkinElmer GeneAmp PCR System 2400 (PerkinElmer Japan, Applied Biosystems Division, Tokyo, Japan). The DNA sequence of each PCR product was determined using the Sanger sequencing method, with the primers shown in Table , in an ABI3100 sequence analyser (Life Technologies, Carlsbad, CA). In addition, AmpliTaq™ (Roche) and attached buffers were used for PCR. The primers in Table were used for the analyses of exons and splicing site sequences of SLC4A4.\nThe sequences of primers used for the detection of β-actin and fragments of SLC4A4 coding sequences were as follows: hACTB748F, 5’-ATTGGCAATGAGCGGTTC-3′, and hACTB979R, 5’-TCTTCATTGTGCTGGGTGC-3′; exon2-3bridgeF, 5’-GTTGGTGGAGATGATTGTTGAC-3′, and exon6-7bridgeR, 5’-GTCATGGAACACCTCATCAGAC-3′; exon5-6bridgeF, 5’-TGCCCACAAGGTTCTTGTTC-3′, and exon8-9bridgeR, 5’-ACCACAGAACCGTCCAGTTC-3′.\nThe quantitative RT-PCR (qRT-PCR) was performed according to its instructional manual, with TaqMan Gene Expression Master Mix (Applied Biosystems, Foster City, CA, USA), TaqMan Gene Expression Assays (Hs00186798_m1 for SLC4A4, Hs01060665_g1 for β-actin; all from Applied Biosystems) and sequence detection system (7500 Fast Real-time PCR System; Applied Biosystems). The expression level was quantified relative to the abundance of β-actin cDNA.\nThe sequencing analysis of the SLC4A4 gene (OMIM 603345, ENST00000340595.3, NM_003759.3) across each exon, including the adjacent intronic sequences of approximately 100 base pairs of the proband, revealed two heterozygous mutations as follows: (a) c.1076 + 3A > C, three bases after the end of exon 7 (Fig. and ) c.1772 − 2A > T, two bases before the beginning of exon 12 (Fig. ). In addition, we analysed the SLC4A4 genes of her parents and confirmed that her mother and father had heterozygous mutations c.1076 + 3A > and c.1772 − 2A > T, respectively. No other mutations in the SLC4A4 gene were detected in the genomes of the patient or her parents. Of note, both mutations are absent from the ExAC database (/).\nOwing to the locations of both mutations on the splice sites, we performed in silico assays to elucidate whether the splicing sites were altered in the proband.\nWe used the webtools ‘Splice Site Score Calculation’ () [, ], ‘NetGene2 Server’ (), ‘Human Splicing Finder Version 3.1’ () and ‘Berkeley Drosophila Genome Project Splice Site Prediction by Neural Network’ () for the in silico evaluation of these mutations. The ‘Splice Site Score Calculation’ demonstrated that the scores of the original sequences were 9.2 and 9.8, whereas the scores of the aberrant sequences were 2.5 and − 1.2, respectively (in order of c.1076 + 3A > C, c.1772 − 2A > T). Because the mean score of the 3′ splice site in constitutive exons was 7.9 and that of the 5′ splice site in constitutive exons was 8.1, the proband’s data suggested that the mutations could cause aberrant splicing (data not shown).\nIn contrast, ‘NetGene 2 Server’ suggested that there may be no splice donor site for the c.1076 + 3A > C mutation and that there may be an aberrant acceptor splice site in c.1772 − 2A > T (data not shown), whereas The ‘Human Splicing Finder Version 3.1’ suggested that in c.1772 − 2A > T the acceptor splice site is broken (data not shown). The ‘Berkeley Drosophila Genome Project Splice Site Prediction by Neural Network’ [] suggested that c.1076 + 3A > C mutation abolishes the original splice donor site and provides an alternative splice donor site (c.1076 + 197_198GT). It also suggested that c.1772 − 2A > T abolishes the original acceptor site and provides alternative acceptor sites (c.1772 − 29_ − 28AG, c.1772 − 37_ − 36AG, c.1772 − 168_ − 167AG).\nFurthermore, the estimated models of aberrant transcription according to previous literature [, ] suggested the appearance of nonsense codons in each allele of the patient’s genome (Fig. and ).\nWe tried to assess the sequence of the SLC4A4 cDNA because we obtained the cDNA of the proband from her mRNA. However, we did not detect the expression of SLC4A4 (Fig. ). Then, owing to the detection of the expression of β-actin in the proband’s cDNA (Fig. ), the absence of the SLC4A4 cDNA sequence suggested that SLC4A4 was either not expressed or expressed at extremely low levels in the proband.\nWe further tried to confirm the expression amount of the SLC4A4 compared to β-actin in the patient by the qRT-PCR. The relative expression ratio of SLC4A4 to β-actin in the proband was extremely low compared to that of the healthy human control (P < 0.01), mother (P < 0.05) and father (P < 0.1) (Fig. ).","This 7-year-old girl has a complex set of health issues, including glaucoma, kidney problems, and a genetic condition called Turner syndrome. She was born prematurely and has experienced growth delays and intellectual disability. Recent tests revealed a rare genetic mutation that affects how her body processes certain substances, leading to further health complications. Despite treatment, her condition remains challenging, and doctors are continuing to monitor her closely."
500,"The patient was a 57-year-old woman who lives in Chiang Klang District, Nan Province, northern Thailand. She is a gardener and has never been abroad, only travelling to Phitsanulok and Phijit, provinces near Nan in Thailand. The patient presented in May 2014 at Chiang Klang Hospital with a single skin nodule on her left cheek (1.0 × 1.5 cm), and also with crusting at the left angle of the mouth (Fig. ). No skin nodules in other sites of the body were observed. Two pieces of formalin-fixed skin biopsy from the cheek nodule (0.6 × 0.5 × 0.4 cm3 and 0.5 × 0.5 × 0.2 cm3) were sent to the Department of Pathology, Faculty of Medicine, Chiang Mai University for histopathological investigation. Histopathological analyses revealed epidermal ulceration with a heavy, chronic inflammation of the dermis (Fig. ) and numerous intracellular small round or oval-shaped bodies, with the appearance of amastigotes (1–2 μm in width and 2–4 μm in length) of Leishmania spp. (Fig. ). A week later, a fresh skin biopsy from the nodule (0.4 × 0.5 × 0.3 cm3) was collected and sent to Department of Parasitology, Faculty of Medicine, Chiang Mai University for confirmation of diagnosis by parasite culture and species identification. The skin biopsy sample was cultured in Schneider’s insect medium supplemented with 20% foetal bovine serum (FBS) and 50 International Units penicillin/ml, 50 μg/ml streptomycin at 25°C. Motile promastigotes were first observed on day 3 of the culture. Therefore, the patient was confirmed as diagnosed with cutaneous leishmaniasis. She was treated with oral amphotericin B at 1 mg/kg/day for 1 day and fluconazole at 200 mg/day for 45 days. The skin lesion had disappeared completely by six months after treatment (Fig. ). Pre-treatment laboratory investigation showed only mild anaemia with a haemoglobin concentration of 10.9 g/dl, white blood count of 7700 cells/mm3, and platelet count of 483,000/mm3. There was no hepatosplenomegaly or palpable lymph nodes. Liver function was not investigated, renal function was within normal limits and HIV serology was negative. The patient declined a bone marrow biopsy for evaluation of visceral leishmaniasis. She did not report any other underlying disease, routine drug use, or any other symptoms, and in general was in a good state of health.","This 57-year-old woman from northern Thailand was diagnosed with cutaneous leishmaniasis, a skin infection caused by a parasite, after finding a nodule on her cheek. Tests of skin biopsies confirmed the presence of the parasite, and she was treated with medication. The infection cleared up completely within six months, and her blood tests showed she was otherwise healthy. She lives in a rural area and has not traveled outside of Thailand."
501,"An 85-year-old Japanese man with a history of malignant melanoma of the nasal cavity presented to our department with severe thyrotoxicosis and poor blood glucose control. He had been treated for hypothyroidism secondary to Hashimoto’s disease and type 2 diabetes mellitus with insulin self-injection therapy before undergoing treatment of malignant melanoma. A surgical operation of his nasal cavity was done at first, followed by one of four planned cycles of nivolumab therapy. Subsequently, two courses of ipilimumab were given as standard therapy after nivolumab therapy. Two weeks later after receiving a dual course of ipilimumab as a third treatment, he presented with a fatigue, nausea, and sweating, which progressed to clinical and biochemical thyrotoxicosis. On admission to our hospital, he was febrile with a temperature of 38.0 °C, tachycardic, agitated, and acutely anxious but still conscious (restlessness). His Glasgow Coma Scale score was 14/15. His blood pressure had decreased to 70/50 mmHg.\nHis physical examination revealed a diffuse goiter without exophthalmoses. His abdomen was soft and non-tender and his skin was warm and wet. He was also diaphoretic with jugular venous distension and peripheral edema, and his chest was clinically clear. His medical history included hypothyroidism due to Hashimoto’s disease, diagnosed at 62 years of age and treated with thyroid hormone replacement, as well as type 2 diabetes mellitus treated by self-injection of insulin with a good glycemic control. He had both family histories of thyroid diseases and diabetes. An electrocardiogram showed marked tachycardia with atrial fibrillation, but a chest radiograph was normal.\nThe laboratory data are shown in Table . Laboratory investigation revealed the following thyroid function results: thyroid-stimulating hormone (TSH) below sensitivity, free triiodothyronine (FT3) 31.7 pg/ml, and free thyroxine (FT4) 3.43 ng/dl. Remarkably, his thyroglobulin was elevated to 48,000 IU/ml. TSH receptor antibody was negative, and a 99mTc-labeled thyroid scan revealed a markedly decreased uptake (Tc retention index-uptake ratio 0.0%; normal range 0.4–3.0). His blood glucose was markedly elevated; in which case, we needed to increase the amount of insulin to control his blood glucose. Further immunological investigation revealed normal serum levels of anti-glutamic acid decarboxylase (GAD) antibody, anti-insulinoma antigen 2 (IA-2) antibody, and insulin autoantibody (IAA).\nAccording to the diagnostic criteria of the Japan Thyroid Association for thyroid storm [], he was diagnosed as having thyroid storm 1 (TS1), definite thyroid storm, since he had thyrotoxicosis, a central nervous system symptom (restlessness), fever (38 °C), GI symptoms (nausea, vomiting), and tachycardia (135 beats per minute) in atrial fibrillation. Based on the diagnostic criteria of Burch and Wartofsky for thyroid storm, he scored 60; a score higher than 45 is suggestive of thyroid storm []. Graves’ disease was less likely as thyroid-stimulating immunoglobulin was within the normal range, and 99mTc-scintigraphy revealed a quite low uptake. These results indicated that his diagnosis of thyroid storm was due to destructive thyroiditis.\nHis clinical course is shown in Fig. . He was treated with an intravenously administered insulin infusion and intravenously administered fluid therapy. At first, the thyroid storm was treated with orally administered potassium iodide (50 mg every 6 hours) and a short-acting beta-adrenoreceptor blocker, landiolol hydrochloride, was used at 4–10 μg/kg per minute to control his heart rate. The potassium iodide was aborted when he was diagnosed as having distractive thyroiditis. Prednisolone was given at 0.5–0.7 mg/kg per day as a treatment for irAE and thyroid storm. Although previous reports suggested the optimum dosage of prednisolone to be 1–2 mg/kg per day [, ], we used a lower dose due to the coexisting and uncontrolled diabetes mellitus. By day 5, his tachycardia had resolved, and the landiolol hydrochloride was discontinued. On day 11, his thyroid function was found to have improved, and the amount of total insulin used to control his blood glucose was decreased. On day 25, he was found to have hypothyroidism, and so we restarted the replacement of thyroid hormone. He was discharged from our hospital on day 35 on daily maintenance insulin injection and levothyroxine sodium hydrate.","This 85-year-old man was admitted to the hospital after developing a sudden and severe thyroid problem, called thyroid storm, which caused his heart to race, his blood pressure to drop, and he became very anxious and feverish. He had a history of thyroid problems and diabetes, and his thyroid storm was likely caused by inflammation of his thyroid gland. He received treatment with insulin, fluids, and medications to control his symptoms, and his condition gradually improved. After recovering, he was started on thyroid hormone replacement therapy and continued to manage his diabetes."
502,"A 20-year-old female patient presented to our hospital with a history of severe and dull substernal chest pain and breathlessness after being hit by a baseball to her chest 2 h ago. Immediately after being hit, she had an episode of syncope briefly without convulsion. The symptoms lasted for half-an-hour and resolved spontaneously. Her vital signs showed blood pressure of 95/64 mmHg and pulse of 70 beats/minute. There was no sign of chest wall penetrating injury. Electrocardiogram (ECG) revealed sinus rhythm with QS complexes in leads V2 to V3, ST segment elevation and T-wave inversion in leads V2 to V5 (Fig. ). Laboratory tests showed cardiac Troponin I level of 19.03 ng/ml (normal range < 0.01 ng/ml). Chest computer tomography (CT) revealed no traumatic injury but demonstrated coronary artery calcifications (Fig. ). Her parents recalled a history of high fever lasting for several days at the age of 5-year-old. The patient was admitted to our hospital diagnosed of acute traumatic MI. Serum Troponin I was peaked to 20.3 ng/ml, creatine phosphokinase(CK) to 1237 U/L and CK-MB to 101 U/L 12 h after admission. Serum BNP level was normal at 85.9 pg/ml on admission. During hospitalization, serial ECG changes were consistent with an evolving MI. She had frequent ventricular premature beats on the Holter monitor. Trans-thoracic echocardiography revealed normal left ventricular size and mild anterior hypokinesis. Notably, multiple giant left anterior descending (LAD) aneurysms with diameters from 7.5 to 8.5 mm (Fig. ) and slow flow velocity were detected. To further assess CAA, we performed contrast enhanced coronary artery computer tomography angiography (CTA)with three-dimensional (3-D)reconstruction of coronary arteries. A ringed calcification in the proximal portion of LAD artery with multiple aneurysms, thrombi and occlusions were visualized (Fig. ). CTA also demonstrated multiple aneurysms with beads-on-string appearance in the LAD artery (Fig. ). Antinuclear antibody (ANA) was minimally positive (1:10) with unknown significance. Serum levels of C3 (0.864 g/L), C4 (0.912 g/L), CRP (< 1.0 mg/L), ASO (< 25 IU/ml), ESR (15 mm/h), Rheumatoid Factor (< 25 IU/ml) and ANCA were within normal ranges. Those results did not seem to support active vasculitis, rheumatic or immunologic diseases. Based on the typical images and her childhood fever history, we recognized that the patient’s underlying coronary structural abnormality was most likely originated from KD. Considering stable hemodynamic parameters and multiple giant aneurismal dilatations of LAD artery, we managed this young patient with a conservative strategy including dual anti-platelet treatment with aspirin and clopidogrel for at least 1 year, and titrations of captopril and metoprolol to prevent ventricular remodeling. At the 2-month follow-up, the patient remained asymptomatic. ECG showed QS complex only in V2 and V3 leads and other abnormalities were resolved (Fig. ). Echocardiography revealed left ventricular diastolic dimension of 48 mm, normal LAD artery velocity, and no ventricular dilatation or akinesis. She remained asymptomatic two year later.","A 20-year-old woman was brought to the hospital after being hit in the chest with a baseball, experiencing dizziness and chest pain. Tests showed a significant heart problem, including calcium buildup in her arteries and several large aneurysms in the main artery supplying the heart. Her heart function was also affected, and she experienced irregular heartbeats. Doctors treated her with medications to prevent further heart problems and will continue to monitor her closely."
503,"We present the case of a 33-year-old female who was an unrestrained driver in a MVC with major front-end damage where airbags were deployed. She arrived to Advocate Christ Medical Center (a high volume, academic, level 1 trauma center) unresponsive with a Glasgow Coma Scale (GCS) of 5 and was intubated for airway protection. Breath sounds were noted to be present bilaterally. She was tachycardic and hypotensive with a heart rate of 143 and blood pressure of 71/46. A focused assessment with sonography for trauma (FAST) demonstrated no evidence of intra-abdominal or pericardial fluid. A left sided hemothorax was present on chest x-ray, and a chest tube was placed with 1200 mL of bloody output initially (See Fig. ). At this point, the decision was made to take patient emergently to the operating room (OR) for exploration.\nA left anterolateral thoracotomy incision was made with the patient in a supine position. There was a significant amount of blood upon entry into the chest cavity, and there was no cardiac tamponade. Despite cross clamping the pulmonary hilum, the bleeding continued. While being resuscitated with the massive transfusion protocol, there was no end tidal CO2 noted. The pericardium was opened, heart delivered, and cardiac massage was started. A separate defect in the superior, right side of the pericardium was found as well as a 3 cm defect in the anterior right atrium (See Fig. ). Despite measures to control hemorrhage and resuscitate the patient, the patient did not survive. The blood loss was greater than 6 l.","This woman was involved in a serious car accident where she became unconscious and needed to be put on a breathing machine. She had a collapsed lung and was very low on blood pressure. After doctors placed a tube in her chest to drain the blood, they realized she needed emergency surgery to stop the bleeding. Unfortunately, despite all efforts, she did not survive."
504,"The second case is a 58-year-old male who was involved in a high-speed MVC. The patient had a prolonged extrication from his vehicle and was intubated after being found unresponsive. On arrival his GCS was 3 T. He was hypotensive with FAST negative for intra-abdominal blood, but a large left hemothorax was identified on the left upper quadrant view. A chest tube was placed with initially 900 mL of blood out followed by 200 mL per hour for 2 h. Based on the output and ongoing resuscitation requirements, the decision was made to take the patient to the OR.\nA left anterolateral thoracotomy incision was made with the patient in supine position. There was approximately 1 L of clotted blood within the chest cavity coming from an anterior pericardial laceration, about 15 cm in length. Through the defect there was brisk bleeding coming from a 1 cm laceration on the left atrial appendage (See Fig. ). The injury was debrided and repaired using a running 3–0 polypropylene suture over a Satinsky clamp. The pericardial defect was closed to prevent cardiac herniation. The patient recovered well and was eventually discharged home.","This 58-year-old man was seriously injured in a car accident and had to be put on a breathing machine. He was found unconscious and had a large blood collection in his chest, requiring a chest tube to drain it. During surgery, doctors discovered a tear in his heart and repaired it. He recovered well and was discharged home after a few days."
505,"A 42-year-old female was seen by a primary care physician, whose examination of the oral cavity revealed smooth bilaterally enlarged tonsils, with the right tonsil being slightly larger than the left. No surface abnormality was evident. She was prescribed two courses of amoxicillin but did not improve. She was then referred to our otorhinolaryngology department. Her medical history was unremarkable. We found no unexplained cervical lymphadenopathy, no significant systemic symptom, no malignancy, and no immunocompromise. All laboratory parameters were within normal limits. Magnetic resonance (MR) images of the palatine tonsils did not reveal any obvious mass lesion. Axial noncontrast T1 (600/8/2 [TR/TE/NEX]) and coronal STIR MR (5700/80/1) images of the palatine tonsils showed that the right tonsil was larger than the left. No mass or abnormal T2 prolongation (suggestive of a tumor) was evident. An axial T2-weighted image (4400/100/2) and a postgadolinium T1-weighted image with fat saturation (550/8/1) also failed to reveal any mass in the right tonsil, but on postcontrast MRI, the left tonsil showed mucosal crypts with linear enhancement, while on the right side the crypts were partly obliterated by a large mass and did not display enhancement (Fig. ).\nThe patient underwent complete surgical resection of the right tonsil because of a possible malignancy. Histological examination of the specimen revealed diffuse sheets of monomorphous plasmacytoid cells with abundant, eosinophilic finely granular cytoplasm, and eccentric nuclei. Occasional binucleated and pleomorphic cells with giant nuclei and prominent nucleoli were also observed, as were mitotic figures. No amyloid deposition was apparent. Immunohistochemically, the plasma cells were diffusely immunoreactive for the λ light chain (DAKO, Glostrup, Denmark), IgG (DAKO), and CD43 (DAKO); partially positive for epithelial membrane antigen (DAKO); but negative for the κ light chain (DAKO), IgA (DAKO), IgM (DAKO), and CD20 (DAKO). Additional immunohistochemical staining showed that the cells were positive for the plasma cell markers CD138, MUM-1, and CD56. Thus, we diagnosed a plasmacytoma (Fig. ).\nA metastatic workup was performed to search for multiple myeloma/light chain disease. Laboratory testing revealed the absence of anemia, and the serum calcium, albumin, total protein, and creatinine levels were normal. The urine lacked Bence-Jones protein. The bone survey was negative. Bone marrow biopsy revealed < 5% plasma cells, ruling out plasmacytoma of the bone. No monoclonal gammopathy was evident, precluding an immunofixation disorder. The kappa-to-lambda ratio was normal. No adjuvant treatment was indicated, and the patient remains clinically free of disease after 6 months of follow-up.","This 42-year-old woman was referred to an ear, nose, and throat specialist because her tonsils had enlarged and she wasn't getting better with antibiotics. After further tests, including MRIs, doctors discovered a large mass in her right tonsil that appeared to be cancerous. The mass was surgically removed, and the tissue analysis showed it was a rare type of cancer called a plasmacytoma. Thankfully, tests ruled out other related conditions, and she has been closely monitored for six months with no signs of recurrence."
506,"A 29-year-old male, with a known surgical history of LRYGB 9 years ago, presented to the emergency department complaining of sharp, severe abdominal pain in the right upper quadrant (RUQ). The pain began two months ago in an intermittent fashion that would occasionally radiate to his right shoulder and progressively became worse with time. The patient did not report any constipation, distension, or vomiting despite feeling nauseated during these attacks. The pain was not related to any movement or meals and it was moderately relieved by over the counter analgesia.\nTen years ago, the patient was diagnosed with obstructive sleep apnea (OSA), through a sleep study, due to morbid obesity. He then was advised to undergo LRYGB as a treatment. He does have any other medical problems. Family history is notable for diabetes mellitus and hypertension but no history of hyperlipidemia or obesity. His body mass index (BMI) prior to the surgery was 49.3 Kg/m2. He underwent a successful LRYGB with no complications or readmissions. After the surgery for a year and a half, he followed up with his primary surgeon. During that time, he lost 75 Kg, reaching a BMI of 26.9 Kg/m2. He then was able to maintain his weight following the operation, but failed to continue to follow up for the past 2 years. In those 2 years, he gained weight, raising his BMI to 29.9 Kg/m2, after which he modified his diet to reduce his BMI to 23.9 Kg/m2. This weight reduction was achieved prior to his presentation.\nOn physical examination, the patient was afebrile with normal vital signs. Examination of his abdomen revealed moderate tenderness over the epigastrium, central abdomen, and right hypochondrium with a positive Murphy’s sign. His laboratory investigations, including complete blood count, urine analysis, liver and renal function tests, were all within normal limits. An ultrasound examination revealed two gallstones and upper borderline diameter of common bile duct, raising the suspicion of biliary obstruction.\nCorrelating his investigation results with his symptoms, he was admitted and scheduled for a cholecystectomy. Formal laparoscopic cholecystectomy was carried out the next morning. During exploration, twisting of the common channel was observed, indicating an internal hernia, although the patient’s complaint did not suggest internal hernia in the differential diagnosis. The internal hernia was identified but reduction was not feasible through laparoscopy. Mini midline laparotomy was subsequently done for reduction of the hernia and more detailed exploration. The hernia was through the mesenteric defect between the alimentary limb and the biliopancreatic limbs. Both limbs of the anastomosis were identified and complete reduction of the internal hernia was done, in addition to suturing of the defect found in the mesentery. No other defects were found. The small intestine’s mesentery showed prominent whitish colored vessels. Whitish thick fluid was noticed, accumulating in the right paracolic gutter and in Morison’s pouch. All of the fluid was then aspirated, measuring a total of 752 mL. Analysis of the fluid showed a triglyceride level of 534 mg/dL and a Lactate Dehydrogenase (LDH) level of 512 U/L. A classic Blake drain 24 F was inserted into the pelvis. The patient passed an uneventful post-operative period. On Postoperative Day (POD) one, fluid collected from the drain was 440 ml, chylous in appearance, while on POD 2–4 the drainage was serous in appearance. The drain was removed on POD 4. The patient was discharged on POD 4 after being able to ambulate independently on his own and eat and drink with no complications.\nThe patient did not return back for his follow-up visits and when he was called by the team, he confirmed that he has no complains up to this day.","This 29-year-old man came to the hospital with severe abdominal pain that had been getting worse over the past two months. He had previously undergone surgery to improve his breathing and weight loss, but his weight has increased recently. Tests revealed gallstones and an internal hernia in his digestive system, which were successfully repaired during surgery. The fluid collected during surgery was chylous, indicating a problem with the absorption of fats, and the patient recovered well and was discharged home."
507,"A 34-year-old man with a 15-year history of Crohn’s Disease (CD) was admitted to our hospital due to abdominal pain, non-bloody diarrhea and weight loss. Physical examination demonstrated moderate abdominal tenderness with an abdominal mass in the right lower quadrant. Laboratory findings revealed a significantly elevated C-reactive protein (CRP 7.5 mg/dl). Colonoscopy with ulcerations localized at the Bauhin’s valve and histological examination of obtained mucosal biopsies were suggestive for active CD. As endoscopic intubation of the terminal ileum was not possible, MR enteroclysis was performed and indicative of a predominant inflammatory, short-segment stenosis of the terminal ileum. Given the acute disease flare and the stricturing phenotype, medical treatment was switched from prednisolone and azathioprine to the anti-tumor-necrosis-factor (TNF)-alpha antibody adalimumab. Twelve weeks after induction of adalimumab therapy, clinical remission was achieved and CRP level returned to normal. Another four months later, clinical remission was still maintained and laboratory inflammation markers remained low, but the patient presented in the clinic for Cranio-Maxillo Surgery due to severe pain in the mandibular area. Examination of the oral cavity detected ulcerative lesions of the buccal-side mucosa of the right mandible (Fig. ). To rule out malignancy, a biopsy of the oral lesions was obtained and revealed ulcerative stomatitis with noncaseating granulomas consistent with oral CD (Fig. ). Intensification of immunosuppressive therapy was initiated by shortening the adalimumab administration interval to weekly administration. A follow-up examination after 10 weeks confirmed complete healing of the oral CD lesion (Fig. ). During a follow-up period of 12 months, no signs of active CD became evident under continued therapy.","This 34-year-old man has been battling Crohn’s Disease for 15 years, which causes abdominal pain, diarrhea, and weight loss. Tests showed inflammation in his intestines and a narrowing of the last part of his small intestine. He started a new medication, adalimumab, which helped him achieve clinical remission. However, he recently developed sores in his mouth that were also caused by Crohn’s Disease, and his doctors adjusted his medication to treat this new problem. After several months of treatment, the sores healed, and he continues to manage his Crohn’s Disease effectively."
508,"An 11-year, 7-month-old girl visited the Kyungpook National Children’s Hospital because of a short stature. The patient had been born via vaginal delivery at a gestational age of 38 weeks; she weighed 2.6 kg at birth, and there were no perinatal problems. However, she had an incomplete cleft palate and craniosynostosis at birth. Hearing loss in both ears was detected at the age of 1 month by brainstem-evoked response audiometry during an initial screening test. An ‘inborn errors of metabolism’ workup performed after birth revealed congenital hypothyroidism, for which the patient received levothyroxine at the local hospital until she was 3 years old; normal thyroid function was confirmed after discontinuing the medication.\nWhen she was 1 year old, she was transferred to our hospital because of a developmental delay and for a repair of her incomplete cleft palate and craniosynostosis. Brain computed tomography revealed an auditory defect, and she began wearing hearing aids, as prescribed by an otolaryngologist. The patient underwent skull reconstruction and incomplete cleft palate repair at the Departments of Plastic Surgery and Neurosurgery, respectively. She was scheduled for follow-up at the Department of Pediatric Neurology due to her developmental language delay, but she was lost to follow-up.\nWhen the patient re-visited our hospital at the age of 11 years and 7 months, she had a height of 124.8 cm (standard deviation score [SDS]: − 3.6), weight of 46 kg (SDS: 0.65 kg), and body mass index (BMI) of 29.53 kg/m2 (Z score: 1.89). She had distinctive facial features, including abnormally long openings between the eyelids, arch-shaped eyebrows, a thin upper lip, and large ears (Fig. ). Moreover, she showed postnatal growth retardation and skeletal anomalies, including an incomplete cleft palate, craniosynostosis, and brachydactyly (Fig. ). These dysmorphic features and her developmental delay were considered suggestive of Kabuki syndrome. Her karyotype was 46,XX. Her bone age was 8 years and 10 months, which was considered delayed according to the Greulich and Pyle atlas []. Based on these characteristic clinical features, mutational analyses for the KMT2D and KDM6A genes were performed.\nThe CARE guidelines were followed in reporting this case, and the molecular analyses were approved by the Institutional Review Board of the Kyungpook National University Chilgok Hospital after obtaining informed consent from the patient’s parents. Genomic DNA was extracted from peripheral blood, and library preparations were performed with the TruSight One Sequencing Panel (Illumina, Inc., San Diego, CA, USA), which enriches a 12 Mb region spanning 4813 genes of clinical relevance. Large-scale parallel sequencing was performed with the Illumina NextSeq platform. Sequence reads were aligned with the hg19 reference sequence using the Burrow-Wheeler Aligner (version 0.7.12, MEM algorithm). Duplicate reads were removed using Picard Tools (version 1.96). Local realignment and base quality recalibration were performed with the Genome Analysis Toolkit (GATK, version 3.5), and variant calling was performed using the GATK HaplotypeCaller. Variants were annotated with the Variant Effect Predictor and AnnoVar. Common variants with minor allele frequencies (≥1%) were filtered out using public databases (1000 Genomes Project, Exome Variant Server, and Exome Aggregation Consortium). Population-specific common variants were further filtered using the Korean Reference Genome Database []. The patient had the nonsense mutation of c.8200C > T, p(Arg2734*) in exon 32 in the KMT2D gene. As neither parent had this mutation, as determined by Sanger sequencing, the patient had a de novo heterozygous mutation in the KMT2D gene (Additional file : Figure S1).\nWe then performed additional examinations to detect other anomalies associated with Kabuki syndrome. Two-dimensional echocardiography, abdominal ultrasonography, and brain magnetic resonance imaging that had been performed at 1 year of age, showed no abnormalities. Ophthalmological findings were also normal.\nThe patient exhibited several endocrine problems (transiently congenital hypothyroidism, CDP, obesity and GH deficiency); additional endocrine tests were performed upon admission to our hospital (at the age of 11 years and 7 months).\nThe patient had transiently congenital hypothyroidism. When she was 7 days old, her free thyroxine (T4) was 1.09 ng/dL (reference range [RR], 0.8–2.0 ng/dL), triiodothyronine (T3) was 1.3 ng/mL (RR, 0.2–2.0 ng/mL), and thyroid-stimulating hormone (TSH) was 22.8 mIU/L (RR, 0.7–13.1 mIU/L). She received levothyroxine until she was 3 years old, and normal thyroid function was confirmed after discontinuing the medication. Upon admission to our hospital, she was 11 years and 7 month old her free T4 was 1.6 ng/dL (RR, 0.8–1.8 ng/dL), T3 was 0.8 ng/mL (RR, 0.6–1.9 ng/mL), and TSH was 3.5 mIU/L (RR, 0.3–4.0 mIU/L), indicating normal thyroid function.\nRegarding puberty, the patient showed Tanner stage I–II of breast development; her basal luteinizing hormone level was < 0.07 mIU/mL (RR, 5–20 mIU/mL), basal follicle-stimulating hormone level was 6.19 mIU/mL (RR, 0.3–10 mIU/mL), and estradiol level was < 11.8 pg/mL (RR, 0–16 pg/mL), suggesting a CDP.\nShe was obese and had a buffalo hump. Her cortisol levels while awake at 8 AM, 5 PM, and midnight were 6.93 μg/dL (RR, 3–22 μg/dL), 12.46 μg/dL (RR, 3–22 μg/dL), and 14.87 μg/dL (RR, < 7.5 μg/dL), respectively. Her corresponding adrenocorticotropic hormone levels were 34.2 pg/mL, 40 pg/mL, and 42.8 pg/mL, respectively (RR for all, 0–60 pg/mL). Her urine-free cortisol was 28.9 μg/dL (RR, 4.3–176 μg/dL). Even though the midnight cortisol level was elevated, a low-dose dexamethasone suppression test showed a cortisol level of 0.68 μg/dL (RR, < 1.8 μg/dL). A lipid profile showed a total cholesterol level of 150 mg/dL (RR, 125–200 mg/dL), triglyceride level of 66 mg/dL (RR, < 200 mg/dL), and LDL-cholesterol level of 53 mg/dL (RR, < 130 mg/dL). Her aspartate aminotransferase and alanine aminotransferase levels were 24 U/L (RR, < 97 U/L) and 17 U/L (RR, < 41 U/L), respectively.\nA GH stimulation test (spanning 2 days) was performed because of her short stature and delayed bone age. On the first day, the patient was administered levodopa (500 mg); the samples for the GH assessment were acquired at 0, 30, 60, 90, and 120 min. On the next day, the patient fasted for 8 h in the morning and was intravenously administered 0.1 U/kg of rapid-acting insulin diluted in 5 mL of normal saline for over 1 min, after which the samples for GH assessment were acquired at 0, 15, 30, 60, 90, and 120 min. The maximum GH concentrations in both tests were below 5 μg/L (RR, > 7 μg/L); therefore, the patient was diagnosed with GH deficiency. Her insulin-like growth factor 1 (IGF-1) and insulin-like growth factor-binding protein 3 (IGFBP-3) levels were 130.5 ng/mL (RR, 99–537 ng/mL, SDS: − 1.67) and 3760 ng/mL (RR, 2400–8400 ng/mL, SDS: 0.91), respectively. The patient started GH replacement therapy (Eutropin 0.03 mg/kg/day) when she was 11 years and 7 months old and is currently scheduled for regular follow-ups for growth and puberty changes.","This 11-year-old girl was born with some health challenges, including a cleft palate and hearing loss. She was diagnosed with a growth hormone deficiency and a condition that affects puberty, which is why she was started on a growth hormone replacement medication. She also has a genetic mutation that is associated with Kabuki syndrome, a condition that can cause various physical features and developmental delays. Despite these challenges, she is currently stable and being monitored closely by her doctors."
509,"A 43-year-old man with a history of hypertension, hyperuricemia, android obesity and bilateral carpal tunnel, presented at his general practitioner with intermittent pain in right lumbar region.\nOn abdominal examination we palpates an epigastric mass, the rest of the physical examination was normal.\nA abdominal CT scan was realized and demonstrates the presence of a voluminous mass of 24 × 15 × 13 cm3, partially cystic, located in the epigastric region, between the left lobe of the liver, the stomach, the pancreas and the transverse colon. The differential diagnoses are: cystic lymphangioma, cystic mesothelioma, non-communicating GD and cystic GIST.\nA biology carried out at the same time an increased CEA level of 281 µg/L, and CA 19-9 being within normal limits.\nMRI gave a diagnosis of a non-communicating GD (Fig. ). The PETCT showed a moderately hypermetabolic uptake in the posterior wall of the mass, which could not differentiate a benign or malignant origin. Gastric endoscopy showed an extrinsic compression of the gastric body, with a normal mucosa.\nA decision for laparotomy surgery was made in view of the possible neoplastic degeneration and the difficulties caused by the volume of the mass. During the surgery, a GD of 30 cm of major axis, located along the entirety of the small curvature and under tension was highlighted (Fig. ). The incision of the cyst allowed 2 L of mucus to be evacueted. The lumen of the cyst did not communicate with that of the stomach. We performed a total gastrectomy with a roux-en-y loop oesophago-jejunostomy and a lymphadenectomy.\nThe histological examination described a normal stomach with a GDC without communication and adjacent to the little curvate. The size of the empty GDC was 21 cm × 10 × 9.5 cm (Fig. ). The sample of the GDC showed a mucus-secreting epithelium with some zones of ulceration. The wall was thick and had a fibrous and muscular aspect.\nNo evidence of dysplasia or metaplasia was found in the stomach and neither in the GDC.\nThe immune marking of pancytokeratine was normal and the KI67 proliferation was little elevated. The PAS coloration showed mucus but there was no signet ring cell. Overall, 18 sampled lymph nodes were free of malignancy.\nThe diagnosis was a total GDC with no malignant transformation.\nThe postoperative course was uneventful and, the patient was discharged on the 10-day postoperatively. At 6 months postoperatively the patient is currently well. The CEA is currently at 3,06 mg/L.","A 43-year-old man with a history of high blood pressure and weight issues came to his doctor with pain in his lower back. Doctors found a large, fluid-filled mass in his stomach that was pressing on nearby organs. After further tests, including surgery, they determined the mass was a type of benign gastric dilatation cyst (GDC) and removed it along with part of his stomach. The surgery was successful, and the patient is doing well six months later, with his blood tests returning to normal."
510,"A 24-year-old Caucasian male presented to our outpatient clinic with fever and pain in the right upper abdominal quadrant. He had a history of a recent upper respiratory tract infection, which was treated with oral amoxicillin. The patient also had a history of left adrenal gland resection, distal pancreatectomy, and splenectomy due to a large pheochromocytoma two years earlier. His past medical history was otherwise unremarkable. A clinical examination revealed a tenderness in the epigastric abdomen and a cervical lymphadenopathy. An ultrasound examination revealed a thickened gallbladder wall as a sign of acute cholecystitis without evidence of gallstones or sludge, as shown in Figure .\nDue to increasing leukocytosis, abdominal pain, and the history of a huge pheochromocytoma, computed tomography (CT) was performed. This confirmed an acalculous cholecystitis and showed a generalized lymphadenopathy. Laboratory values were significant for: white blood cell (WBC) count 23.940/µl (range: 4.500-9.500/µl), thrombocytes 426.000/µl (150.000-400.000), lactate dehydrogenase (LDH) 438 U/l (135-225), aspartate aminotransferase (AST) 116 U/l (10-50), alanine aminotransferase (ALT) 185 U/l (10-50), gamma-glutamyltransferase (GGT) 258 U/l (10-71), alkaline phosphatase (AP) 437 U/l (40-129), c reactive protein (CRP) 10.2 mg/l (< 5mg/l). Table summarizes the laboratory values throughout the hospital stay. Figure presents the course of the patients' liver enzymes.\nThe mononucleosis enzyme-linked immunosorbent assay (ELISA) showed elevated virus capsid antigen (VCA) immunoglobulin G (IgG) levels (39 U/l; range < 0 U/l) and normal VCA immunoglobulin M (IgM) levels. The heterophile antibody test for mononucleosis IgM antibodies was positive. We performed a cytomegalovirus (CMV) ELISA, which showed normal values for IgG and IgM. Epstein-Barr virus (EBV)-specific nuclear antigen (EBNA) antibodies were negative. A VCA IgM control test two weeks later showed elevated VCA IgM antibodies. The peripheral blood smear confirmed atypical lymphocytosis. Under symptomatic therapy, the patient’s state improved rapidly and he was discharged on the 17th day after admission in good general condition.","A 24-year-old man came to the clinic with a fever and pain in his upper right stomach area. Tests showed he had an infection of his gallbladder, likely due to a recent illness, and also showed swelling in his lymph nodes. His previous surgery to remove a large tumor in his adrenal gland may have contributed to his condition. Blood tests revealed signs of infection and inflammation, and tests confirmed he had a recent viral infection (mononucleosis). He improved quickly with treatment and was discharged home feeling better."
511,"A 47-year-old woman presented to the emergency department with concerns of left upper abdominal pain for one day. She reported that the moderate to severe abdominal pain had a gradual onset, was crampy, constant, and located in the left upper part of her abdomen radiating to the lower chest and back. The pain was associated with diaphoresis, nausea, and several instances of vomiting non-bloody, non-bilious liquid. On admission, her vitals were stable. The results of her complete blood counts, comprehensive metabolic panels, and hepatic function tests were within reference ranges. Her coagulation profile was at a subtherapeutic level. Her past medical history includes nonischemic cardiomyopathy with left ventricular thrombus and four previous cerebrovascular accidents with residual right leg numbness. Her condition was being managed with warfarin. A physical examination showed fine crackles within the left lower lobe and significant tenderness in the left upper abdomen. The patient reported that she had stopped taking warfarin two days prior to admission. Cardiac magnetic resonance imaging (MRI) showed septal and apical predominantly transmural infarct. A transthoracic echocardiogram showed a mildly dilated left ventricle with moderate to severe left ventricular dysfunction, an ejection fraction of 30% to 35% with distal anterior, anteroseptal, and apical akinesis with mild mitral regurgitation. Cardiac catheterization findings were unremarkable. A computed tomography (CT) scan of her abdomen showed mild cardiomegaly with previous infarction on the anterior-inferior wall of the left ventricle. We noted a 37.47 mm calcified thrombus in the left ventricular apex with peripheral calcifications (Figure ).\nWe also noted infarction in the spleen and right kidney with bilateral renal scarring (Figures -).\nThe patient was initially started on a heparin drip and later bridged to warfarin on the third day. She was discharged with complete resolution of her abdominal pain after seven days, and medication compliance was reinforced before discharge.","A 47-year-old woman came to the hospital because of a day of moderate to severe pain in her upper left abdomen, which spread to her chest and back. She had nausea and vomiting, and she was sweating. Tests showed a blood clotting problem and evidence of a previous heart attack and kidney problems. After receiving treatment and monitoring, her pain resolved, and she was discharged home with instructions to continue her medications."
512,"A 27-year-old African American male with a past medical history of asthma and allergic rhinitis presented with a three-week history fever of unknown origin. Associated symptoms included drenching night sweats, weight loss, fatigue, nausea, and diarrhea. On physical examination, he was found to be hemodynamically stable and febrile at 102°F. He was also found to have extensive left sided, posterior, cervical lymphadenopathy. Laboratory studies indicated a mild anemia with negative hepatitis panel, anti-nuclear antibody (ANA), human immunodeficiency virus (HIV1/HIV2), Lyme antibody, and infectious mononucleosis (IM) serology, non-reactive rapid plasma reagin (RPR), and a negative Babesiosis smear. Computed tomography (CT scan) of the chest and abdomen were unremarkable. Ultrasound (U/S) and CT imaging of the neck demonstrated extensive adenopathy throughout the left posterior cervical carotid chain (Figure ). U/S guided lymph node biopsy showed polymorphous appearance with areas of zonal necrosis and a subpopulation of medium-large atypical lymphoid cells suggesting an atypical lymphoproliferative disorder. Excision biopsy was performed to rule out malignancy and Hodgkin disease. Pathology showed histiocytic necrotizing lymphadenitis (Figure ). The histologic, immunohistochemical and flow cytometric findings were consistent with histiocytic necrotizing lymphadenitis. Immunohistochemical stains were performed to include BCL2, CD2, CD3, CD5, CD7, CD15, CD20, CD30, CD34, CD68, CD117, CD138, Epstein–Barr virus (EBV), herpes simplex virus (HSV), PAX5, Alk1, cytomegalovirus (CMV), Ki67, pankeratin, S100. Proliferating histiocytes marked with CD68 were negative for the listed T cell and B cell markers. Phenotypic analysis by flow cytometry revealed a mixed population of mature T and B lymphocytes with normal T cell:B cell ratio for lymph node. Monoclonal T cell or B cell populations were not detected and there was no immunophenotypic evidence of an acute leukemic process. The patient was managed with ibuprofen and fully recovered in five months’ time.","A 27-year-old man has been experiencing a fever and other symptoms like night sweats, weight loss, and fatigue for three weeks. Doctors found swollen lymph nodes in his neck and blood tests ruled out common infections like hepatitis, HIV, and Lyme disease. A biopsy of the swollen lymph nodes revealed a rare condition called histiocytic necrotizing lymphadenitis, which is not cancerous. The patient was treated with medication and has fully recovered after five months."
513,"We present a case of a 68-year-old Caucasian female known to have multiple comorbidities: seropositive rheumatoid arthritis, common variable immunodeficiency, hypertension, hypothyroidism, and osteoporosis. The patient was diagnosed with rheumatoid arthritis in 2013. She developed cutaneous vasculitis, confirmed by biopsy performed by her primary care physician in May, 2015. She was initially treated with mycophenolate, hydroxychloroquine, and prednisone with initial improvement. The prednisone was tapered off, but she then developed worsening of joint symptoms and synovitis. For this reason, she was switched to methotrexate. Six months later, leflunomide was substituted due to hair loss and inadequate disease control on methotrexate. It was several months later that her cutaneous lesions worsened again. The decision was made to start the patient on abatacept. Arrangements were made with required laboratory testing and screening prior to starting abatacept. On the following visit, the patient was given abatacept 10 mg/kg intravenous infusion with concurrent therapy of leflunomide, hydroxychloroquine, and prednisone. The patient was reevaluated after two weeks of infusion, with clearing of lesions several days after the first dose of abatacept. On her four-month follow-up visit, the patient continued to demonstrate a satisfactory response to this therapy. Prednisone was discontinued, and the patient was kept on abatacept, hydroxychloroquine, and leflunomide. The patient returned to office a month later without any recurrence of her cutaneous lesions.","This 68-year-old woman has several health problems, including rheumatoid arthritis and a weakened immune system. She initially improved with several medications, but her rheumatoid arthritis symptoms and skin rashes returned. To help manage her condition, her doctors switched her to a new combination of medications, including abatacept, leflunomide, and hydroxychloroquine. After a few weeks, the skin rash cleared up, and she has been doing well on this treatment plan for the past four months."
514,"The case of a 46-year-old male patient with a background of HHT and aortic valve replacement due to aortic insufficiency is presented. He denies having had psychiatrict treatment, consumed antipsychotic drugs, had recent molar extractions or a background of TMJ dislocation.\nOn the fifth day after a lung transplant, in the intensive therapy unit, the patient begins to experience symptoms of mandibular pain, trismus and extrapyramidalism symptoms in the form of oromandibular dystonia. In physical exam he presented signs of bilateral jaw subluxation with reuptake inhibition of the articular condyle–disc complex, contraction at the masticatory muscles level, fascies dolorosa, dystonia of the facial third with articular blocking and incapacity for bucal closure or lateral excursion and slight dysphagia. As background of the hospitalization, he had received, an hour before said event, 2.5 mg of haloperidol and 0.25 mg of risperidone (two doses) due to hyperactive delirium. It was decided to take a computed tomography (CT) of the craniofacial complex and brain without IV contrast (Fig. ), where the following was seen: dislocation of both mandibular condyles with anterior position to the temporal’s glenoid cavity, without evidence of associated bone injuries.\nAnalgesia and muscular relaxation of the patient was carried out. The Nelaton reduction maneuver and later placing of Barton bandaging was done following the technique, using an antiseptic. Posterior clinical and radiological reduction is verified through CT, evidencing a normal condylar position. The patient progresses without further subluxations during his hospitalization.","This 46-year-old man, who had previously undergone a lung transplant and aortic valve replacement, started experiencing jaw pain, stiffness, and involuntary muscle movements shortly after his transplant. Physical examination revealed problems with his jaw joint, and a CT scan showed the condyles (the ball-like part of the jaw joint) had shifted out of place. The symptoms were likely caused by the recent medications he received – haloperidol and risperidone – which can sometimes cause these side effects. After a special procedure to reposition his jaw, he stabilized and didn’t experience any further problems during his stay in the hospital."
515,"A 61-year-old male was referred to our burn center with burn injuries. The burn\npercentage was 20% Total Burn Surface Area (TBSA) and IIb-III degree, involving\nthorax, abdomen, and right arm. Patient was treated with split-thickness skin graft.\nWe discharged the patient after successful management with healed graft and nearly\nhealed donor wounds. 8 months later, the patient was referred to us from his primary\ncare physician for management of infected open wounds in donor sites. Physical\nexamination revealed infected area with hypergranulated tissue in donor sites, left\nupper limb and left lower limb (Figure 1 ). Wound cultures showed Staphylococcus\naureus, Escherichia coli and\nStaphylococcus epidermidis (Methicillin-resistent, MRSE)\nbacteria. Patient was a heavy smoker and suffered from multiple sclerosis. Up to our\nknowledge, neglect and inadequate wound care were responsible for the opening and\nsecondary infection of donor sites. One day after admission we performed a surgical\ndebridement and removed the hypertrophic granulation tissue. After the operation we\nstarted daily wound dressing with a non-adherent antimicrobial alginate dressing\n(Silvercell®) and irradiation with water-filtered infrared-A\n(wIRA). Water-filtered infrared-A (wIRA) protocols used at our department were\nradiations all wound areas three times daily with a 60 cm distance for 30 minutes\nwith a “Hydrosun 505®” model (Hydrosun Medizintechnik,\nMüllheim, Germany). The patient tolerated this application well without any\ncomplaints. After 5 weeks the patient was discharged with healed donor sites left\nthigh and left lower leg and nearly totally healed sites left forearm and left upper\narm (Figure 2 ).","61-year-old man was admitted to our burn center because of infections in the skin where skin grafts were taken. The infections were caused by bacteria and were worsened by his smoking and multiple sclerosis. We treated the infections with surgery to remove the damaged tissue and special dressings and light therapy. After five weeks, the wounds had healed, and he was discharged home."
516,"An 80-year-old man with known cervical canal stenosis due to ossification of the cervical posterior longitudinal ligament was transferred to an emergency room soon after a falling accident. The patient was alert and had complete tetraplegia, sensory deficits below the C4 cervical sensory level, and progressive restrictive respiratory failure that required assisted ventilation, and, eventually, a tracheostomy. Cervical spine magnetic resonance imaging showed cervical cord compression that was worse at the C3–C4 intervertebral level, and an intramedullary high signal at C3 and C4 vertebral levels, and the patient was managed conservatively. During the conventional weaning process of trials of intermittent spontaneous breathing in the ICU, an unexpected cardiac arrest made both the patient and medical staff reluctant to proceed with further weaning. Fifty-eight days after cervical cord injury, the patient was transferred to our hospital for further rehabilitation. He was fully conscious and received pressure-support mechanical ventilation with back-up rates of 16 breaths. He had total tetraplegia below shoulder girdle muscles with a preserved sensation from C2 to C3 on both sides, and a urinary obstruction. The limbs and trunk were stiff with contracture, and muscle stretch reflexes of the limbs were abolished. Neurological level of injury was C3 according to the ISNCSCI, with a total sensory score of four. There was no sensory sacral sparing, while the deep anal pressure was preserved, and the American Spinal Injury Association scale graded B. Chest roentgenogram and computerized tomography showed bilateral, widespread atelectasis. The patient occasionally showed signs of pulmonary infection. Intensive pulmonary care and rehabilitation, including physical support for expectoration, mechanically assisted removal of tracheal secretions (CoughAssist E70®, Philips Japan, Tokyo), ventilator muscle training, and management of the spasticity of the abdominal wall, resulted in the improvement of the roentgenological findings. His expiratory tidal volume (VT) measured 150 mL.\nAlthough the neurological status according to the ISNCSCI was unchanged, the patient was willing to be weaned from mechanical ventilation as his general condition improved. After informed consent was given, we attempted automated weaning from mechanical ventilation using IntelliVent®-ASV on and after 131 days of injury. IntelliVent®-ASV (Hamilton Medical AG, Switzerland) is a closed-loop ventilation mode that adjusts the pressure support in terms of the percentage of the ideal minute volume (%MV). The ideal MV (100%MV) is calculated from the patient’s height and gender. Based on Otis’ equation [], adaptive support ventilation will select the best VT–respiratory rate (RR) coupling for the optimal work of breathing. IntelliVent®-ASV has interrelated functions: an auto-adjustment for carbon dioxide (CO2) elimination, an auto-adjustment for oxygenation, an auto-weaning tool named Quick Wean (QW), and a spontaneous breathing trial (SBT). The setting of the target %MV is automated based on either the monitored end-tidal CO2 or monitored spontaneous breathing rate. The auto adjustment of fraction of inspiratory oxygen or positive end-expiratory pressure is based on the monitored oxygen saturation fraction of hemoglobin with a pulse oximeter (SpO2) []. The QW mode is an optional automated weaning, and progressively reduces the pressure support, monitors for readiness-to-wean criteria, and provides the option to automatically conduct a fully controlled SBT. In the QW mode, when the spontaneous rate is less than the upper limit of the predicted target range, %MV is automatically decreased gradually to the selected level. To facilitate the recovery of VT by means of the loading work on respiratory muscles, we set the level of support to 70%MV for the QW mode. When spontaneous breathing satisfied the pre-determined conditions of oxygenation and ventilation for 1 min, the SBT mode was used. We operated the SBT mode during the day from 9:00 a.m. to 6:00 p.m.\nIn the QW mode, the duration of 70%MV and SBT time (25%MV) increased gradually (Figs. and ). Twenty-two days after the introduction of the QW and SBT modes in association with intensive pulmonary rehabilitation, SBT was running almost fully during the daytime. At this time, the expiratory VT was increased to 350 mL from 150 mL upon admission to our hospital. Thereafter, we withdrew the pressure support ventilation during the nighttime 39 days after the introduction of the auto-weaning mode, and the patient’s weaning process was completed.","An 80-year-old man was brought to the hospital after a fall, resulting in paralysis from his neck down and needing a tracheostomy. After several attempts to wean him from a ventilator, he experienced a cardiac arrest, making further weaning difficult. Now, he’s receiving intensive care and rehabilitation to improve his breathing and manage muscle stiffness. Through a new automated ventilation technique, his breathing has improved, and he’s now able to be weaned from the ventilator, with plans to eventually breathe on his own."
517,"A fifty-six-year-old hypertensive male came in for consult due to an inability to abduct the right eye. History of the present illness started about thirty-four years prior when he first noted a limited abduction of the right eye. During the interim, he noted a gradually progressive limitation in all movements of the right eye, and a limitation in adduction of the left eye, associated with occasional diplopia. No consults were sought until a month prior when he noticed a sudden-onset right-sided facial weakness and dysarthria, with no hemiparesis or hemiplegia.\nThe patient came into the clinic with good visual acuity, good color vision, and no relative afferent pupillary defect, but with a right horizontal conjugate gaze palsy, a limitation of adduction of the right eye with associated nystagmus of the left eye on abduction, and a right peripheral facial nerve palsy demonstrated by a four-millimeter lagophthalmos, a shallow nasolabial fold, and an inability to wrinkle the forehead. The rest of the neurologic examination was unremarkable. Slit lamp examination was also essentially unremarkable except for immature cataracts. Indirect ophthalmoscopy showed evidence of hypertensive retinopathy on both eyes.\nFigure 1 shows the patient’s extraocular muscle movements in a composite diagram. There is a right horizontal gaze palsy and a limited adduction of the right eye. The sole extraocular muscle movement remaining is an abduction of the left eye.\nFigure 2 highlights the peripheral facial nerve palsy. The patient has a shallow nasolabial fold on the right, a four-millimeter lagophthalmos on the right, and weakness of the occipitofrontalis, corrugator supercilli and procerus muscles.\nOn magnetic resonance imaging, there is a hemorrhage with a complete hemosiderin ring at the level of the pons/ponto-medullary junction, associated with a well-defined lesion with a “popcorn ball” appearance resembling that of a cavernoma (Figure 3 , Figure 4 , and Figure 5 ).\nThe patient was co-managed with neurosurgery and neurology services for possible resection.","This 56-year-old man has been experiencing a gradual loss of movement in his right eye for over 30 years. Recently, he developed a sudden weakness on the right side of his face and difficulty speaking, along with a noticeable double vision. Examination revealed a complete loss of movement in his right eye and a facial nerve problem, causing drooping of the right eyelid. MRI scans showed a small blood clot in his brain that is causing these symptoms, and he is being managed by both neurology and neurosurgery to explore possible treatment options."
518,"A 21-year-old female presented with complaints of seeing a black spot in front of the right eye associated with distortion of the central vision of one week duration. There was no history of trauma. Best-corrected visual acuity (BCVA) in both eyes was 20/20. Anterior segment examination was essentially normal in both eyes. Fundus examination of the right eye revealed a yellowish-gray subretinal lesion with subretinal fluid supero-temporal to the fovea and pigment epithelial detachment (Figure 1 ). The lesion spared the fovea. Optic disc and retinal vasculature appeared normal and no vitreous cells were noted. Fundus examination of the left eye was normal.\nThe patient was a diagnosed case of factor VIII deficiency about a month back. Her coagulation profile and hemogram were normal. However, factor VIII – C level was 37.5% (Normal range 50–150%). Fundus fluorescein angiography (FFA) revealed a classic extrafoveal choroidal neovascular membrane (CNVM) (Figure 1 ). Focal laser photocoagulation to the CNVM was done after discussing the treatment options with the patient. The patient reported after 6 weeks with visual acuity in the right eye 20/30, N6. Clinical examination revealed a regressing extrafoveal neovascular component and an active juxtafoveal neovascular component. Clinical findings were confirmed on FFA and optical coherence tomography (OCT) (Figure 2 ). The patient was treated with photodynamic therapy (PDT) followed by intravitreal triamcinolone acetonide (IVTA) injection (4 mg/0.1 ml) 2 days later. After 6 weeks, visual acuity in the right eye was 20/30 and the intraocular pressure (IOP) was 32 mmHg. Topical Timolol maleate 0.5% twice a day was started. Since there was persistent hyperfluorescence related to the extrafoveal CNVM, thermal laser photocoagulation was done.\nWith treatment, IOP came down to 12 mmHg in 2 weeks and the same treatment was continued. On the next visit eight weeks later, visual acuity was 20/60, N10. Clinical examination revealed recurrence of the juxtafoveal choroidal neovascular membrane, which was confirmed on FFA and OCT. The patient was treated with four intravitreal injections of bevacizumab (1.25 mg/0.05 ml) at an interval of 4–6 weeks. The IOP was found to have normalized and hence topical antiglaucoma treatment was stopped.\nSix weeks after the fourth injection, visual acuity in the right eye improved to 20/30, N6. CNVM appeared scarred (Figure 3 ). However, there was persistent leakage on FFA. The patient was treated with a combination of PDT and intravitreal bevacizumab. After 7 weeks, visual acuity improved to 20/20. Clinically, CNVM appeared scarred. The patient was followed up at intervals of 2 weeks. Fourteen weeks later, the patient reported with an increase in distortion in the right eye. BCVA was 20/20 in the right eye. OCT showed subretinal fluid and FFA showed recurrent extrafoveal CNVM. The patient was treated with intravitreal bevacizumab (1.25 mg/0.05 ml). The patient was seen six weeks later when she reported resolution of symptoms; her visual acuity was 20/20. Clinical findings were confirmed on OCT and FFA (Figure 4 ). When seen last (i.e. 10 years post bevacizumab injection), BCVA was maintained at 20/20 and the fundus remained stable.","This 21-year-old woman came to the doctor because she was seeing a black spot in her vision and experiencing blurry vision. Tests revealed a small growth behind her eye that was causing these problems. She received laser treatment to remove the growth, and then another treatment to further reduce the risk of it returning. Over time, her vision improved, and she was able to maintain 20/20 vision."
519,"A 54-year-old woman with all limbs intact received BPA in advance of right thumb carpometacarpal arthroplasty with ligament reconstruction. Immediately after BPA onset, she felt her right forearm and hand resting across her chest when it was hanging over the side of the gurney. After surgery, her right hand felt “heavy” with the fingers stacked vertically on top of each other, as shown in Figure . She began experiencing right thumb pain 14–16 h after the operation had been completed. However, the sensation of immobility of her 2nd through 5th digits in the stacked position lasted for ~3.5 days after surgery and 69 h after the anesthesia wore off. During this time, although the patient described the phantom sensations as being uncomfortable, she experienced no pain in the fingers. No nerve conduction studies were performed.\nThis study was carried out in accordance with the recommendations of the University of Tennessee Health Science Center. The procedure discussed in this report was not part of a research study but rather routine clinical care. The subject gave written informed consent for publication of her clinical details in accordance with the Declaration of Helsinki.","This 54-year-old woman had surgery on her right thumb to repair a joint. Right after the surgery, she reported feeling like her arm and hand were resting on her chest, and her hand felt heavy and stiff. For a few days afterward, she experienced phantom sensations of her fingers being stacked on top of each other, although she didn't feel any pain. The unusual sensations gradually disappeared after about three and a half days."
520,"A 70-year-old man presented to the emergency department (ED) after falling twice at home. While standing after eating a light meal, he had two separate episodes of brief loss of consciousness. On the second fall, the patient had hit his right shoulder, cheek, and foot, prompting the visit. The patient had received a colonoscopy earlier in the day for routine cancer screening. He had followed proper protocol regarding his bowel prep and had not had any immediate complications related to the procedure or anesthesia. Since the colonoscopy, he had continuous bouts of cramping abdominal pain. He had also felt dizzy throughout this period but attributed it to dehydration related to his bowel prep. The patient denied striking his head, chest pain, shortness of breath, nausea, vomiting, or blood in his stools. His past medical history was significant for coronary artery disease, hyperlipidemia, and hypertension. The patient took his benazepril, aspirin, and atorvastatin on the day prior to colonoscopy.\nOn physical exam, the patient was afebrile with a heart rate of 87 and blood pressure of 130/78. The patient's head, neck, chest, and neurological exams were entirely normal. His abdominal exam was remarkable for tenderness in the right and left lower quadrant with some voluntary guarding, but no masses or rebound was appreciated. His orthopedic exam demonstrated tenderness to his right acromioclavicular joint and ecchymosis of his right 5th metatarsal with no deformity and normal range of motion at all joints.\nOn laboratory evaluation, the patient's hemoglobin was 12.4 g/dL, with normal platelets and chemistry studies. Head computed tomography (CT) and shoulder and chest radiography were normal. Foot radiography demonstrated a nondisplaced 5th metatarsal fracture. CT of the abdomen and pelvis demonstrated moderate hemoperitoneum with splenic laceration (Figures , , and ).\nThe patient was admitted to the intensive care unit and underwent serial abdominal exams and every four-hour hemoglobin measurements. His hemoglobin fell to 8 g/dL at 48 hours after admission but subsequently stabilized. The patient was observed in the hospital and ultimately discharged home on hospital day 3 in good condition.","This 70-year-old man went to the hospital after falling twice at home, which led to a suspected broken foot and a significant injury to his shoulder and cheek. He had recently undergone a colonoscopy and was experiencing abdominal pain and dizziness. Tests revealed a tear in his spleen and a fracture in his foot, causing bleeding in his abdomen. He was treated in the intensive care unit and his condition stabilized, and he was discharged home after three days."
521,"A 36-year-old woman (height, 147 cm; weight, 50 kg) with CIPA was scheduled for revision of left total hip arthroplasty. She was diagnosed as having CIPA because of recurrent episodes of unexplained fever, anhidrosis, burns, and bone fractures after birth. She had previously undergone 7 operations for spinal deformity and 1 operation of total hip arthroplasty in both the left and right sides. Although lack of general diaphoresis and thermal nociception were observed, the patient performed body surface cooling at her own discretion when she felt she was at a risk of hyperthermia, and her body temperature was kept approximately 36°C. No signs of mental retardation or orthostatic hypotension were observed. No abnormality was detected on chest radiographs and electrocardiograms. Blood biochemistry revealed no abnormality except mild anemia indicated by a hemoglobin level of 10.6 g/dl.\nNo premedication was administered. After the patient was brought into the operating room, routine monitoring and measurement of the bispectral index (BIS) were started. Body temperature was measured at 3 different sites (urinary bladder, esophagus, and precordial skin) and controlled by a hot-air-type heater. Propofol was administered at an effect-site concentration of 4 μg/ml by target-controlled infusion. After muscle relaxation had been achieved by administration of 50 mg of rocuronium, the trachea was intubated. Immediately after endotracheal intubation, systolic blood pressure increased from 130 to 145 mmHg, and heart rate increased from 60 to 95 beats per minute (bpm). Two minutes later, systolic blood pressure had decreased to 125 mmHg. Propofol was continuously infused intravenously at a target concentration of 2 to 4 μg/ml () and BIS levels were maintained between 40 and 60. After an arterial catheter had been placed, her position was changed from the supine to right lateral position. Surgery was then started.\nSince no circulatory change associated with pain occurred during surgery, opioids were not administered. Regarding hemodynamics, when 600 ml of blood was rapidly lost within 20 minutes, blood pressure decreased from 113/66 to 93/55 mmHg and heart rate increased from 55 to 70 bpm ( a). Similarly, when 850 ml of blood was lost within 30 minutes, systolic blood pressure decreased from 108/65 to 95/60 mmHg and heart rate increased from 66 to 74 bpm ( b). Administration of 0.1 mg of phenylephrine increased blood pressure from 87/55 to 117/76 mmHg and decreased heart rate from 70 to 65 bpm ( c).\nThe operative time was 6 hours and 49 minutes, and the duration of anesthesia was 8 hours and 41 minutes. The volume of blood loss was 3350 ml. Blood transfusion was performed with 1600 ml of preoperatively donated autologous blood, 900 ml of salvaged blood, and 720 ml of fresh frozen plasma. Intraoperative body temperature was controlled and kept between 36.0°C and 36.9°C at all 3 measurement points. After surgery had been completed, the patient was returned to the supine position and she was extubated. Since she did not complain of any pain after the surgery, no analgesic was administered. She was discharged at 6 weeks after the operation.\nBlood samples were collected 3 times: before anesthesia induction, after the start of surgery, and at the end of surgery. The levels of catecholamine fractions and cortisol were measured. Norepinephrine levels were below the normal range at all time points, and the levels of epinephrine and cortisol were within the normal ranges at all time points ().","This 36-year-old woman, who has a history of unexplained fevers and other health issues, is scheduled for a revision of her left hip replacement. During the surgery, doctors carefully monitored her blood pressure and heart rate, and used medications and fluids to maintain stability as she lost a significant amount of blood. The surgery lasted six and a half hours, and she received a large amount of her own blood to help with recovery. She was successfully discharged six weeks later, and her body temperature was carefully controlled throughout the procedure."
522,"The first patient, who was a man in his late seventies with an unremarkable previous medical history, presented with isovascular nodule accompanied by slow venous wash-out on abdominal dynamic CT in the left lobe of liver. The laboratory data, including the blood cell count, chemistry, and tumor marker levels, were within the normal limits, with the exception of mildly elevated CRP (0.39 mg/dL) and decreased hemoglobin (8.9 g/dL) levels. Neither infection of HBV nor infection of HCV was noted. Based on the clinical findings, the initial diagnosis by the clinicians was most likely HCC, and, thus, left partial hepatectomy was performed. On gross examination, the cut surface of hepatic nodule () showed a poorly demarcated peripheral nodule in lobulated margins, measuring 32 x 21 mm in diameter, which appeared clearly whitish in color. The background of this liver showed no remarkable change (). A microscopic examination of the tumor demonstrated an unencapsulated, ill-defined, and expansive nodule (). This cancerous nodule showed a solid proliferation of atypical epithelial cells, arranged predominantly in solid nests (), trabeculae, and/or ill-defined, fused tubule-like structures (). On a high-power view, these atypical cells were small-to-medium-sized and relatively uniform, having enlarged hyperchromatic nuclei and scant cytoplasm without any evidence of intracytoplasmic mucin (). Intriguingly, prominent fibrous stroma was not evident in this tumor. Immunohistochemistry revealed that the abovementioned carcinoma cells were specifically positive for not only CK7 (cholangiocytes marker) but also CK8/CK18 (markers for both hepatocytes and cholangiocytes) and CK19/CD56 (potential stem cells markers), whereas they were negative for Hepatocyte (hepatocytes marker) [, ]. Based on all of these features, the final diagnosis was primary CHC-INT. To date, this patient has been followed up for 1 year since surgery, and he remains well without any sign of recurrence.","A man in his late seventies was admitted after finding a growth in his liver. Tests showed slightly elevated inflammation and a lower than normal red blood cell count. Doctors initially suspected liver cancer and performed surgery to remove the growth. The examination of the removed tissue revealed a type of liver cancer, but the patient is currently doing well and being monitored closely for any signs of recurrence."
523,"The second patient, who was also a man in his early seventies with more than 25-year follow-up for HCV-positive chronic hepatitis and recurrent HCC, presented with mildly hypervascular and ring-enhanced nodule accompanied by venous wash-out on abdominal dynamic CT in the S5 of the remnant liver. The laboratory data were mostly within the normal limits, with the exception of mildly elevated AST (37 IU/L), total bilirubin (1.8 mg/dL), and CEA (5.55 ng/mL) levels. No infection of HBV was observed. Based on the clinical findings, the initial diagnosis by the clinicians was recurrent HCC, and, thus, partial S5 hepatectomy was performed. On gross examination, the cut surface of hepatic nodule () showed a poorly demarcated portal nodule in lobulated margins with central necrosis, measuring 35 x 26 mm in diameter, which appeared clearly whitish in color, accompanied by not only gross but also histopathological portal vein permeation (). A microscopic examination of the tumor showed an unencapsulated, ill-defined, and expansive/infiltrative nodule, displaying a solid proliferation of atypical epithelial cells, arranged predominantly in solid nests, trabeculae, and/or irregular and fused tubule-like structures, aggressively involving the portal vein with focal perineural invasion (). On a high-power view, these atypical cells were very similar to those of the abovementioned first case. Prominent fibrous stroma was not seen either. The background of this liver showed mild chronic hepatitis (F1/A1) and steatosis. Immunohistochemistry showed that those carcinoma cells were specifically positive for not only CK7 () but also CK18 () and c-kit (potential stem cells marker) [, ], whereas they were negative for CK8, Hepatocyte, and CD56. Based on all of these features, the final diagnosis was primary CHC-INT as well. The recurrence of CHC-INT in the remnant liver occurred 1 year and 3 months after this surgery, but he remains not worse with follow-up for the postoperative 2 years.","This 70s-year-old man with a history of chronic hepatitis and previous liver cancer was seen because of a new, concerning spot found on a scan of his liver. His blood tests showed some mild abnormalities, and doctors suspected a recurrence of the liver cancer. A surgery to remove the spot was performed, and the tissue examined revealed it was a type of liver cancer. The final diagnosis was primary CHC-INT, and he is being closely monitored with follow-up appointments."
524,"A 62-year-old male presented with refractory sacral coccygeal pain. The patient had past medical history of type 2 diabetes mellitus (treated with linagliptin/metformin), nontreated high blood pressure, right-sided hemiparesis following meningitis in childhood, nephrolithiasis, and smoking history. The pelvic computed tomography (CT) revealed a 9x7.5x9 cm bulky mass in the sacrum with locally increased soft tissue density, causing extensive lytic lesions of the sacred vertebrae and extending to the left iliac bone, suggestive of chordoma. The patient underwent total sacrectomy with partial excision and reconstruction of the left iliac bone. The anatomopathological examination revealed sacrococcygeal involvement by a thyroid carcinoma, as verified by immunohistochemical staining for thyroglobulin and TTF-1, predominantly papillary (follicular variant), however with foci of nondifferentiated (insular) carcinoma (Figures and ). Thyroid ultrasonography showed a solid nodule of 20 mm in the right lobe and two solid hypoechogenic nodules of 11 and 9 mm in the left lobe, the smallest with coarse calcifications. No lymphadenopathies were found. 18F-fluorodeoxyglucose positron-emission tomography (18F-FDG-PET) revealed a hypermetabolic focus in the left lobe of the thyroid, consistent with the suspected malignant neoplasia, and uptake of the radiopharmaceutical drug in the fifth lumbar vertebra and pelvic bones, consistent with secondary involvement (). Consequently, the patient underwent total thyroidectomy. Histological examination revealed only follicular and oxyphilic variants of multifocal papillary carcinoma (at least six foci) and none of insular carcinoma, with dimensions ranging from 2 to 15 mm, without signs of hematogenic, lymphatic, or perineural permeation, as well as no signs of invasion of the capsule or extrathyroidal extension, with resection margins uninvolved by tumour (pT1b[m]NxM1R0) (). Radioactive iodine therapy (RAI) was then performed. Posttherapy scintigraphy showed hyperfixation in the remnants of the sacrum and lower lumbar spine, bilateral iliac bone, and anterior cervical region (). TSH-stimulated thyroglobulin was 24490 ng/mL. Follow-up magnetic resonance imaging (MRI) revealed persistence and progression of the pelvic lesion. At this time the patient was unable to walk, had a chronic indwelling bladder catheter, suffered from fecal incontinence, and presented with uncontrolled refractory chronic pain.","62-year-old man came to the hospital with severe pain in his sacrum (tailbone). Tests revealed a large, cancerous mass in his sacrum that was spreading to his spine and hip bone. After surgery to remove the mass, doctors found that it was a type of thyroid cancer that had spread from the thyroid gland. Further tests showed the cancer had spread to other areas, and he will need ongoing treatment, including radioactive iodine therapy, to manage the cancer."
525,"A 64-year-old male presented with pain in the left iliac region for 6 months. His past medical history was significant for gastric peptic ulcer disease (submitted to partial gastrectomy and chronically treated with lansoprazole), nephrolithiasis, and hyperuricemia. At admission a poorly defined mass on the left posterior iliac crest was palpable. The patient underwent a pelvic CT, which revealed a 9 cm lytic lesion of left iliac bone with soft tissue involvement (). A biopsy was performed and the histological examination and immunohistochemical staining for thyroglobulin and TTF-1 showed iliac involvement of a well-differentiated thyroid carcinoma (Figures and ). Thyroid ultrasonography disclosed a poorly defined 8 mm nodule in the left inferior lobe, heterogeneous and partially calcified, and a 4 mm hypoechoic nodule in the right lobe, without associated lymphadenopathies. The patient was submitted to total thyroidectomy and anatomopathological examination revealed a 1.1 cm papillary carcinoma, of follicular variant, with capsular invasion and limited extrathyroidal extension (ETE). Consequently, RAI therapy was performed. Postablative 131 iodine scintigraphy and 18F-FDG-PET () did not reveal further lesions. TSH-stimulated thyroglobulin was 185051 ng/mL. The patient is currently waiting for a hemipelvectomy.","64-year-old man came to the hospital because of pain in his hip for six months. Tests revealed a tumor in his hip bone, which was determined to be a type of thyroid cancer. Further tests showed small nodules in his thyroid gland, and a complete thyroidectomy was performed to remove the cancer. The patient is now waiting for surgery to remove the affected part of his hip."
526,"A 57-year-old Japanese man was admitted to our hospital with a chief complaint of back pain and fever for one month. A computed tomography (CT) scan showed an aneurysm of the infrarenal aorta, with a diameter of 34 mm, and inflammation of the surrounding adipose tissue, nodular lesions of the bilateral lungs, and left maxillary sinusitis (). He was referred to our hospital for further evaluation and treatment.\nAt the time of admission, the patient was 168 cm tall and weighed 56.6 kg. His blood pressure was 98/69 mmHg, pulse was 84 beats per minute, and body temperature was 39.6°C. Serum creatinine was 0.66 mg/mL, and urinalysis showed 1+ occult blood; urinary sediment contained 20 red blood cells per high-power field. Serum analysis showed leukocytosis (10,000/μL) and an elevated C-reactive protein level (29.5 mg/dL). The anti-PR3-ANCA level was 187 IU/mL, and the anti-MPO-ANCA level was normal. The abdominal aortic aneurysm was suspected to be infected, and we began administering antibiotics. The patient's general medical condition failed to improve.\nThe clinical findings of left maxillary sinusitis, multiple nodular lesions in the lungs, fever, and positive anti-PR3-ANCA were clinically suspicious for GPA. Video-associated thoracoscopic lung biopsy was performed. The biopsy specimens demonstrated granulation tissue with necrosis and multinucleated giant cells (). Most infiltrating cells were neutrophils. The ratio (%) of IgG4 to total IgG-positive cells was 33%, and there were 60 IgG4+ plasma cells per HPF in the lung.\nThe patient was diagnosed with GPA. He was treated with an intravenous semipulse dose of methylprednisolone, followed by oral prednisolone 1 mg/kg (55 mg) per day and intravenous administration of cyclophosphamide (700 mg/body once per month). All of the patient's symptoms and CT findings rapidly improved (), and the PR3-ANCA level promptly decreased to the normal range. The diameter of the aneurysm changed from 34 mm to 21 mm after treatment. On tapering doses of steroids, the patient is currently in remission, and the inflammation and PR3-ANCA elevation have completely resolved. There has been no disease recurrence for 4 years after initiation of therapy.","This 57-year-old man was admitted to the hospital because of back pain and a fever that lasted for a month. A scan revealed a problem with his aorta (a major blood vessel), inflammation in his lungs, and a sinus infection. Tests showed he had a high level of a specific antibody (anti-PR3-ANCA) that suggested a condition called GPA. After a lung biopsy, he was diagnosed with GPA and started on medication to control the inflammation, which quickly improved his symptoms and the size of the aortic aneurysm."
527,"A 55-year-old male presented to the emergency department 3 weeks after right frontal nodule resection of his multifocal glioblastoma multiforme. The tumor was also in the corpus callosum and the right pontine area. The patient was known to have high ICP (confirmed by magnetic resonance imaging) due to the mass effect of the tumor for which a debulking surgery was done. Recovery from surgery went well, and the patient had a residual left-sided weakness and was able to ambulate by using a walker at discharge. At presentation to the emergency department, the patient had 2 episodes of unprovoked syncope with nausea, somnolence, and worsening of his left-sided weakness. The patient's past medical history was insignificant except for controlled hypertension and epilepsy. The patient's home medications were dexamethasone, levetiracetam, hydromorphone, perindopril, amlodipine, and pantoprazole. While in the emergency room, the patient was noted to have a temporary pause on the cardiac monitor which was symptomatic. The patient's vital signs on admission were a Glasgow Coma Scale of 15, blood pressure of 132/86, heart rate of 61, and respiratory rate of 18 (regular), afebrile, and an oxygen saturation at 94% on 2 L/min of oxygen. When the oxygen was removed, the patient's respiratory rate decreased to 12, oxygen saturation dropped to 89%, and the patient became nonalert. An EKG was done which showed multiple intermittent sinus pauses of durations of 4–6 seconds. The blood work and urine analysis were all within the normal range. A diagnosis of SSS was made, and the patient was admitted to the CCU for pacemaker implantation. While admitted to the CCU, his EKG showed intermittent sinus pauses with the longest having a duration of around 3.6 seconds. A pacemaker was inserted. A 24-hour monitoring post procedure was uneventful, and the patient's telemetry showed a normal sinus rhythm in the 80s to 90s with no paced beats. The patient was then discharged home to be followed up at the pacemaker clinic. During the follow-up, the patient's hemiparesis did not recover and was still using the walker to ambulate. A computed tomography (CT) scan done later showed rapid progression of all lesions, except the one in the pons, with vasogenic edema. There was a significant mass effect with midline shift and uncal herniation.","This 55-year-old man had surgery to remove a brain tumor that was causing dangerously high pressure in his brain. He has been recovering well, but recently experienced episodes of dizziness and weakness. During a hospital visit, he had a pause in his heart rhythm, which led to him becoming confused and short of breath. After being evaluated, he was diagnosed with a slow heart rhythm and a pacemaker was implanted to help regulate his heartbeat. Despite the pacemaker, his weakness from the tumor hasn't improved, and his tumor has unfortunately grown more quickly."
528,"A 66-year-old phototype 6 woman from North-Africa was admitted to our department due to an indolent ulcerovegetative mass that developed on the anterior face of the right thigh. The lesion spontaneously exhibited greyish discharge. The patient anamnesis revealed that she had sustained a hot-water burn to her leg at the age of 11 years. She stated no definitive treatment by skin grafting and had only been treated conservatively following injury. The burned right thigh healed progressively, leaving a large scar on the skin surface. There had been no particular issues concerning the lesion until 2 months previously, at which point the patient noticed a progressive mass in the scar area but had not taken any steps to have it cured. Upon physical examination, the mass was 4 x 2 cm and suggested at diagnosis of Marjolin's ulcer (Figures and ). Histopathologic features of the surgical specimen were characterized by predominant chondroid matrix with marked cellularity, high grade atypia, and high mitotic activity. Foci of bone and osteoid formation were also seen (Figures and ). A diagnosis of cutaneous extraosseous osteosarcoma was suggested after excluding an origin in bone or other primary tumor sites by computed tomography. Indeed, clinical examination and extensive total body radiologic workup revealed absence of bone lesions in any body site. Unfortunately, the patient rejected the therapeutic proposal of definitive large surgical excision and we failed to obtain any notice regarding her further evolution.","66-year-old woman with a history of a childhood burn developed a concerning growth on her thigh. The growth, now diagnosed as cutaneous osteosarcoma (a rare type of cancer), was found in the area of an old scar. Tests showed the growth contained unusual bone-like tissue and was growing rapidly. The patient declined surgery, and we are monitoring her condition, but unfortunately, we cannot provide further treatment options at this time."
529,"A 48-year-old woman, gravida 1, para 1, visited the internal medicine department at another hospital with a complaint of abdominal fullness and weight loss of 10kg during the last year. A huge abdominal mass was palpated, and she was referred to the gynecology department to search for a tumor of uterine origin. She was premenopausal and had no significant past medical history. Physical findings revealed a large elastic hard mass extending from the xiphoid to the pubic bone. The magnetic resonance imaging (MRI) examination revealed a huge tumor on the uterine corpus, and a number of dilated vessels were observed between the tumor and the myometrium. Therefore, the tumor was suspected to derive from the uterus. The tumor showed an uneven signal on T2-weighted sagittal section (), and the enhanced MRI study showed that the tumor edge but not the center was enhanced (). As such, necrosis was suspected to have occurred in the center of the tumor. Uterine sarcoma was primarily suspected due to the large size, degeneration, and necrosis on MRI imaging. Computed tomography (CT) examination showed no lymph node swelling or distant metastasis. Preoperative laboratory testing revealed anemia (hemoglobin level, 5.6g/dl). We transfused 18 units of RCC before surgery. CT examination and ultrasonography on lower extremities indicated an absence of thrombosis. Preoperative serum levels of CEA, CA 19-9, CA 125, and LDH were within normal limits. A biopsy of the endometrium was not collected as the sounding examination of the endometrium was unsuccessful due to a deviated uterine cervix. At this point, preoperatively, we suspected the tumor was a leiomyosarcoma or leiomyoma with degeneration.\nThe patient underwent laparotomy, where we identified a huge tumor occupying a space from the pelvis to the diaphragm. The tumor surface was smooth and hard with many dilated veins (). A massive tumor with a diameter of 30 cm was observed arising from the posterior uterine wall with a smooth contour and invaded the retroperitoneal cavity under the mesentery. The tumor was firmly adhered to both the mesentery and right ovary. There were no findings of extra-uterine dissemination. The intraoperative frozen section report for the uterine tumor was of degenerated myoma with no findings indicating malignancy. A total abdominal hysterectomy (TAH) and right salpingo-oophorectomy (RSO) were performed. The operation duration and blood loss were approximately 216 minutes and 1000 ml, respectively. The excised specimen weighed 7600 g.\nMacroscopic findings of the tumor revealed a well-circumscribed tumor showing extensive continuity with the posterior wall of the uterus, measuring 28 × 23 cm (). On the sliced surface of the tumor, an obvious heterogeneous pattern was recognized within the mixture of a whitish homogeneous area, suggesting benign uterine fibroids, and a vulnerable area, due to bleeding and necrosis ().\nFor the intraoperative frozen section, we examined three areas, namely, a white homogenous part, a necrotic part, and a cystic part, of which all were findings of a leiomyoma. In the permanent histological examination, 10 additional sections were collected from the tumor. The basic histological findings of all the sections were the same. The tumor was comprised of spindle-shaped cells, homologous to smooth muscle cells, which were arranged in bundles with areas of hyalinization, consistent with a degenerated leiomyoma. The tumor was mostly comprised of degenerated uterine leiomyoma. However, enlarged blood vessels were observed within an area of approximately 2 cm × several mm, and proliferation of atypical cells showing a fine meshwork microvascular structure was observed in the blood vessel cavity (). These atypical cells consisted of various contours, such as cubic, polygonal, and short spindle shape. The nucleus was circular with a high degree of vacuolar enlargement and pleomorphism. Abnormal mitotic figures were also interspersed (). A tumor derived from a blood vessel was thus considered, and malignancy was suggested by the presence of nuclear atypia and abnormal mitosis.\nImmunohistochemical analysis revealed the atypical tumor cells to be positive for ERG, CD31, and AE1/3 (Figures and ), partially positive for Factor VIII, and negative for α-SMA, desmin, H-caldesmon, EMA, CD34, and D2-40. From the above, the atypical tumor cells were of epithelial origin and the final diagnosis was epithelioid angiosarcoma arising in a degenerated uterine leiomyoma.\nThe efficacy of postoperative adjuvant therapy for angiosarcoma has not been demonstrated and there is currently no established chemotherapy regimen. In this case, because the atypical tumor was observed in the blood vessel cavity, we thought it could have been spread hematogenously throughout the body. Hence, we selected adjuvant chemotherapy rather than adjuvant radiotherapy. Six courses of combination adjuvant chemotherapy with paclitaxel (150mg/m2), epirubicin (50mg/m2), and carboplatin (area under the curve = 4) were administered in the present case, following referral to previous reported cases. No recurrence has been observed 10 months after the primary surgery.","The patient came to the hospital because she had abdominal fullness and lost 10 pounds over the past year. Doctors found a large tumor on her uterus and suspected it might be a type of cancer. After further tests, including MRI scans, they determined the tumor was actually a degenerated leiomyoma (a type of benign uterine fibroid) that had developed into an epithelioid angiosarcoma, a rare type of cancer that grows in blood vessels. The patient underwent surgery to remove the tumor and her uterus and ovaries, and is now receiving chemotherapy to treat the cancer."
530,"A 60-year-old woman came to our attention for abdominal pain, distension, and weight loss (about 6 kg in two months). Abdominal examination revealed a bulky mass occupying the right abdominal quadrants. She was submitted to laboratory routine tests without evidence of chronic liver disease and no alterations of serum tumor markers. Computed tomography (CT) scan confirmed the presence of an expansive mass (15 × 12 × 23 cm), poorly separable from the surrounding liver parenchyma, with heterogeneous contrast enhancement. The lesion was partially cystic with thickened walls and an intralesional solid component ().\nAfter multidisciplinary discussion with radiologist and oncologist, indication to surgery was given and the patient underwent laparotomy. At exploration, a well-defined, partially cystic tumor was found originating from the fifth and sixth hepatic segments and adherent to—but not infiltrating—the right colon and omentum. En bloc resection of the mass and a S5-S6 liver bisegmentectomy were performed. The postoperative period was uneventful, and the patient was discharged on sixth postoperative day.\nThe surgical specimen consisted of a lobulated yellow-reddish neoplasm sized 33 × 19 × 11 cm, with gelatinous cystic and hemorrhagic areas on cut surface. A fibrous discontinuous pseudocapsule separated the tumor from the adjacent compressed liver parenchyma. Microscopically, the tumor was composed of stellate or spindle shaped cells with bizarre morphology and ill-defined outlines, loosely arranged in an abundant myxoid matrix (). Scattered tumor cells with marked nuclear abnormalities and hyperchromasia, as well as multinucleated giant cells, were present. Atypical mitotic figures were easily found. Characteristically, tumor cells showed multiple, different-sized, eosinophilic, PAS-positive globules in the cytoplasm. Immunostainings revealed CD10, CD68, and vimentin expression in tumor cells, with focal and weak expression of wide spectrum cytokeratins (AE1/AE3) (). These features were sufficient for the diagnosis of UESL.\nAfter the definitive histological diagnosis and multidisciplinary evaluation, the oncologist proposed an adjuvant therapy of six cycles with vincristine, actinomycin D, and cyclophosphamide, which was accepted by the patient. 30 months after surgery, she is alive without signs of recurrence.","60-year-old woman was seen for abdominal pain, bloating, and weight loss. Tests revealed a large, concerning mass in her liver. Surgery was performed to remove the mass, which was found to be attached to her colon. The tumor was successfully removed, and she is now doing well and is being monitored closely."
531,"A 14-year-old male with DMD has been on daily oral steroid since 9 years of age. He weighed 53 kg (47th percentile) and was 147 cm tall (less than 3rd percentile). He became nonambulatory at 12 years of age. Forced vital capacity (FVC) was 2.37 L or 80% predicted. A polysomnogram was completed and revealed moderate obstructive sleep apnea. His echocardiogram (ECHO) was normal with cardiac magnetic resonance imaging (MRI) demonstrating normal left ventricular ejection fraction (LVEF). He participated in many school activities including wheelchair soccer.\nHe presented to the emergency department with confusion, tachycardia, tachypnea, and fever up to 39.2 degrees Celsius a few hours following soccer practice. During practice, he had been transferred from his wheelchair and his right leg had brushed the ground causing him to have to be placed on the ground until more help was obtained to put him back in his wheelchair. Brain computed tomography (CT) was negative for any acute intracranial process contributing to his current state. Chest computed tomography (CT) scan was negative for pulmonary embolism but showed patchy diffuse nodular airspace opacities seen scattered throughout both lung fields (). He had progressive acute hypoxic respiratory failure requiring mechanical ventilation. Shortly after intubation, he suffered cardiac arrest requiring 3 minutes of cardiopulmonary resuscitation (CPR) before return of spontaneous circulation. ECHOs were consistent with increased pulmonary vascular resistance including moderate right ventricle dysfunction and elevated pulmonary arterial pressure. Duplex ultrasound evaluation of lower extremities was without evidence of deep venous thrombosis bilaterally. X-rays of lower extremities revealed cortical step off at the proximal left femoral neck and nondisplaced fracture at the distal right tibial metaphysis and fibular metaphysis (). Ophthalmologic exam revealed Purtscher-like retinopathy. The patient was treated with 48 hours of antibiotics that were discontinued with negative blood, urine, and mini bronchoalveolar lavage (BAL) culture. The patient was treated with mechanical ventilation and right ventricular afterload reduction with milrinone and inhaled nitric oxide for pulmonary hypertension. These therapies were able to be weaned off, and the patient was able to be extubated on day five of admission. He was later transferred to the pediatric floor and later discharged home without any residual effects from the entire event.","This 14-year-old boy with a genetic condition (DMD) has been taking steroids since he was young and has had difficulty walking since age He recently went to the hospital after a fall during soccer practice, which caused him to become confused, have a fever, and struggle to breathe. Tests showed he had lung problems and his heart was working harder than usual, and he experienced a cardiac arrest. After receiving treatment and antibiotics, he was able to breathe on his own again and was discharged home without any lasting issues."
532,"An 11-year-old male with DMD has been on daily oral steroid since 9 years of age. He weighed 30 kg (5.6th percentile) and he was 120 cm tall (less than 3rd percentile). He is able to ambulate without assistance. FVC was 1.5 L or 96% predicted. His ECHO was normal with cardiac MRI demonstrating normal LVEF.\nHe sustained a nondisplaced Salter-Harris type II fracture of the distal left femur () from a fall that required surgical fixation and was able to be discharged home on the day of operation. He presented to the emergency department two days after his operation with seizure, tachycardia, tachypnea, and fever up to 38.6 degrees Celsius. He had progressive acute hypoxic respiratory failure requiring mechanical ventilation. Shortly after intubation, he developed pulmonary hemorrhage and anemia remedied with fresh frozen plasma, ventilator adjustments, and blood transfusion. A fast spin MRI of his head was negative. CT scan of his chest demonstrated bilateral patchy infiltrates; no pulmonary embolism was identified (). ECHO revealed mildly elevated tricuspid regurgitation velocities representing increased right ventricular and pulmonary artery pressures. He was placed on broad spectrum antibiotics for 72 hours but blood, urine, and mini BAL remained negative. Mechanical ventilation was able to be weaned and the patient was extubated on day five of admission. He was later transferred to the pediatric floor and later discharged home without any residual effects from the entire event.","This 11-year-old boy with a genetic condition (DMD) has been taking steroids for several years to help manage his condition. He recently broke his leg and had surgery to fix it, but then he became very sick with a fever, rapid breathing, and seizures. Doctors needed to put him on a breathing machine and give him blood transfusions to help him breathe and fight the infection. After several days in the hospital, he recovered and was able to go home without any lasting problems."
533,"Twin 1, a three-year-old Caucasian boy, was initially referred by his family physician with photophobia and frequent blinking of the left eye to Royal North Shore Hospital. He had varicella-zoster virus (VZV) keratitis of the left eye with positive VZV on Polymerase Chain Reaction (PCR) and was treated with intravenous acyclovir and topical acyclovir. His symptoms and follow-up examinations showed resolution.\nOne year from this presentation, best-corrected visual acuity (BCVA) in the right was 20/80 and could not be obtained in the left due to objection to occlusion. Examination under anaesthesia (EUA) revealed corneal neovascularization bilaterally (Figures -). There was blepharitis and conjunctivitis bilaterally, with a stromal infiltrate and overlying epithelial defect in the right eye. He was investigated for causes of interstitial keratitis and corneal neovascularization. Blood tests, and corneal and conjunctival swabs were unremarkable (). Given the results and EUA findings the diagnosis was staphylococcal hypersensitivity/chronic blepharokeratoconjunctivitis (BKC) secondary to meibomianitis, with corneal neovascularization and scarring [, ]. He was prescribed oral erythromycin (160mg, twice a day), chloramphenicol ointment (1%, three times a day) for both eyes, and topical fluorometholone (0.1%, twice a day) for the left eye.\nReview two weeks later showed progressive corneal neovascularization and infiltrate. Due to the progression despite conventional treatment and to aid compliance with the treatment, the decision was made for subconjunctival triamcinolone acetonide and bevacizumab, with regular EUA for treatment and monitoring of progress and side effects. Two weeks later, he received subconjunctival triamcinolone (4mg/0.1mL × 0.55mL) to the inferior conjunctiva and subconjunctival bevacizumab (2.5mg/0.1mL × 0.05mL) to the superior conjunctiva of the right eye. Extensive lid hygiene including 5% povidone-iodine lid scrubs and Meibomian gland expression was performed, and this was repeated at subsequent EUA. Intraocular pressures (IOP) by iCare® tonometer (iCare Finland) were 14mmHg in each eye. He continued on the same medication regimen and SteriLid (TheraTears®) two to three times per week.\nSix weeks after the injections, the mother of Twin 1 reported significant improvement in his symptoms. At EUA eight weeks after the injections, the right cornea looked markedly improved (Figures -). IOPs were 12mmHg in the right and 6mmHg in the left eye. Given that there had been dramatic improvement with no significant side effects observed, he was continued on the same regimen and the left eye was injected with the same dose of triamcinolone and bevacizumab.\nAt EUA 11 weeks after the left eye injections, there was quiescent interstitial keratitis. Adjunctive diathermy was applied to the patent superior and inferior feeder vessels in the left eye. Further subconjunctival triamcinolone (4mg/0.1mL × 0.5mL) was injected inferiorly in each eye. Binocular BCVA (due to objection to occlusion) was 20/63.\nAt EUA 5 months after the further bilateral triamcinolone injections, there was central corneal scarring and suppressed blood vessels bilaterally (Figures -). Diathermy was applied at the limbus to further feeder vessels of each eye. IOP was 16mmHg in the right and 19mmHg in the left eye. Binocular BCVA (due to objection to occlusion) 24 months after presentation was 20/63.","This young boy initially had an eye infection caused by chickenpox that led to some scarring. A year later, he developed more significant problems with his eyes, including new blood vessels and inflammation, which made it difficult to get a clear reading. After trying several treatments, doctors found that injections into his eyes helped to reduce the inflammation and improve his vision. Over time, his eyes continued to heal, and his vision improved, although some scarring remained."
534,"A 59-year-old man presented to the urgent care clinic at the School of Dentistry complaining of an upper lip mass for one-year duration. The mass started out as a small bump and had grown steadily since then. 3 months prior to his presentation, a draining parulis developed on the mass. The patient had no history of systemic symptoms such as fever, chills, weight loss, or fatigue. He was aware of a dark-colored “dead tooth” for several decades in the area of concern but denied any previous history of swelling in the area. The patient had recently moved to the United States from Nigeria and had previously been without access to adequate dental care. The patient said that a doctor in Nigeria told him that the lesion was likely cancerous.\nOn exam, there was a large, painless, fibrous, exophytic mass in the anterior maxillary labial vestibule (Figures and ). The base of the mass approximated the apex of tooth #8. A yellow purulent material was observed draining from the parulis (). Tooth #8 was discolored and was confirmed to be nonvital on pulp testing. There was a significant gap between teeth #7 and 8. Tooth #8 was displaced medially and was extruded relative to the adjacent dentition.\nA periapical radiograph revealed a large unilocular radiolucency associated with the apex of tooth #8 (). Cone-beam computed tomography again demonstrated a large cystic-appearing defect in the anterior maxilla with perforation of the buccal and palatal cortices . The lesion extended to the nasal floor on the ipsilateral side.\nThe patient was referred to the oral surgery department for excisional biopsy. After tooth #8 was removed, an incision was made around the base of the stalk that connected the mass to the labial and alveolar mucosa. Sharp dissection was used to free the mass, and the specimen was sent for histopathologic analysis. The mass communicated with a cystic lesion of the maxilla. The cyst was enucleated with a curette and also sent for pathology. Perforation of the cyst through the buccal and palatal cortices was noted during the procedure. Slight undermining of the wound margins allowed for closure with resorbable sutures.\nAt the patient's one-week follow-up (), he was doing very well. He reported minimal pain, no neurosensory disturbances, and no systemic or local symptoms of infection. He and his family were very relieved to learn that the lesion was benign. He was happy with his appearance after having the mass removed.","A 59-year-old man came to the urgent care clinic because he had a growing lump on his upper lip for a year. The lump had a draining area, and he had a dark tooth in the same area for many years, but he hadn't had any swelling before. Tests showed the lump was a benign cyst connected to a damaged tooth, and it was removed with a biopsy. The patient is now doing well and relieved to have the lump removed and his appearance restored."
535,"A 15-year-old female with a past medical history of obesity presented to the emergency department with a marked 90-degree flexion at the thoracolumbar region. She was able to walk only by using a walker in an unusual manner; she placed her axillae on the hand grips. The patient described constant back pain almost daily for which she took ibuprofen with moderate relief. These symptoms began three years ago following a sexual assault and have progressed significantly. As per the patient's mother, the only time the patient's spine straightened out was during deep sleep. She had no neurologic complaints, specifically denying any sensory or motor deficits, fatigue, ptosis, or any complaints consistent with any form of endocrinopathy. Her family history included siblings with epilepsy, ADHD, and mood disorders.\nThe patient denied any substance abuse, including use of alcohol or cigarettes, as well as being sexually active. She reported no history of mood or psychotic symptoms and no suicide attempts.\nDespite the pain, however, the patient was not concerned with her condition. The patient herself was not interested in physical therapy or additional diagnostic testing or counseling despite her mother's encouragement. She refused a gynecological examination due to anxiety following having been a victim of sexual assault; it was unclear the extent of counseling, if any, she received following the assault.\nThe patient was a seventh grader at a local school where she participated in extracurricular activities. She reported being well accepted by her peers. She stated having a good social support network including friends and family. Her parents were separated and she lived with her mother and brother.\nPhysical exam revealed an obese (BMI 39.2) Caucasian female, leaning forward with the spine nearly ninety degrees at the waist. Extremities showed normal strength, sensation, and range of motion. The patient was unwilling to attempt spinal extension. Tenderness to palpation of the paraspinal muscles was present. The rest of the physical examination was unremarkable.\nUpon Pediatric Neurology consultation, no evidence of neuromuscular disease was found. Plain radiography of the cervical, thoracic, and lumbar spine was limited due to her deformity, but no abnormalities were noted. Magnetic resonance imaging of the same was unrevealing. Psychiatric consultation was obtained; they concluded that the patient's symptoms were likely manifestations of Conversion Disorder, following exclusion of organic causes.\nWhile she is currently receiving social services support and physical therapy; she declines psychiatric outpatient care at this time.","This 15-year-old girl has been experiencing severe back pain for three years, which has caused her spine to bend almost completely to the side. The pain started after a difficult experience, and it’s affecting her ability to walk and stand up straight. Although she’s been trying to manage the pain with ibuprofen, she’s resistant to further testing or treatment, and she’s not interested in physical therapy or counseling. Doctors believe her symptoms are related to a psychological issue, and she is receiving support from social services, but she is not seeking psychiatric care at this time."
536,"A 28-year-old female with insignificant past medico surgical history presented with one day of acute onset pain in the periumbilical region that later migrated and confined to the RIF. She had associated intermittent fever, nausea, and loss of appetite. She did not have any urinary symptoms, bowel irregularities, or gynecological complaints. Abdominal examination was performed by two senior surgeons at two different occasions; the same day had findings of guarding and rebound tenderness at RIF. Hematological tests showed polymorphonuclear leukocytosis with left shift. Biochemical tests and urinalysis were normal. Urinary pregnancy test was negative. Abdominal radiographs were unremarkable. USG could not visualize appendix and was inconclusive except for probe tenderness in RIF. CT scan of the abdomen could not be done due to unavailability. A clinical diagnosis of acute appendicitis was made assigning an Alvarado score of 9/10. Laparotomy was performed using the Lanz incision in RIF. Intraoperatively appendix was found to be normal without evidence of inflammation or infection in RIF. In view of symptoms and signs, a possibility of other pathology was thought. Walking the bowel proximally up to 3 feet (1 m) did not show a Meckel's diverticulum or any other small bowel lesions. There were no obvious mesenteric lymph nodal enlargement and pelvic organs looked pristine. Approaching closure, just when the medial edge of the incision was retracted superomedially, a hemorrhagic lesion seemed to appear little deeper in the mid abdomen. Therefore, the incision was extended transversely from the medial edge to explore further. Entire bowel was explored and this revealed an ulcerated lesion measuring 7 × 5 cm arising from the antimesenteric border of the ileum 8 feet (2.5 m) from ICJ with localized interloop hemoperitoneum and inflammatory exudates as shown in . Resection of ileal segment containing the lesion was performed followed by restoration of bowel continuity and peritoneal toileting. The lesion was subsequently reported to be an ulcerated malignant ileal GIST.\nHistopathologically, gross examination confirmed the operative findings, and the cut section revealed a nodular lesion protruding out of the serosal surface measuring 7 × 5 cm along with 2 lymph nodes each measuring 2 × 1 cm.\nMicroscopically, the growth from the ileum had villous lining epithelium with focal ulceration. The submucosal region had a circumscribed nodule with proliferation of loosely cohesive spindle cells; some of which were arranged in vague storiform pattern and others in long fascicles. There were areas with epitheloid cells forming small anastomosing nests and cords. The areas in between these showed skenoid fibers along with focal areas of hemorrhage, infarction, and congestion as shown in . The mitotic figures were seen (8/50 high-power field). The lymph nodes were microscopically identified to be reactive, and the resected margins of the ileum were free of tumor.\nBased on tumor size and mitotic activity, possibility of a malignant GIST was suggested along with immunohistochemical analysis (CD117 and CD34) for further confirmation. The patient had an uneventful recovery and was discharged on the 8th postoperative day. She was advised to review a week later at the outpatients but failed to report. All possible contacts were used to trace her, but she remained inaccessible and lost to follow-up.","A 28-year-old woman came to the hospital with a sudden, sharp pain in her abdomen that moved to her right side. She also had a fever, nausea, and lost her appetite. Doctors initially suspected appendicitis, but after exploring her abdomen, they found a large ulcerated growth in the small intestine that turned out to be a type of cancer called an ileal GIST. The growth was removed, and the area was carefully cleaned, but the patient was unable to be contacted for follow-up. She had a successful surgery and recovered well, but doctors are concerned about her lack of contact and recommend she seek medical attention as soon as possible."
537,"A 54-year-old, obese, Caucasian female, a current smoker, presented to the clinic with complaints of intermittent vaginal bleeding for approximately one week and that she “felt something” in her vaginal area. Her past medical history includes diabetes mellitus, hypertension, and an abdominal hysterectomy 30 years ago due to heavy menstrual bleeding of benign etiology. Upon examination, a pedunculated mass was found on the vaginal wall at approximately 7 o'clock. The mass, measuring 2.0 × 1.5 × 1.3 cm, was subsequently excised.\nHistologic examination revealed a clear cell carcinoma. Immunohistochemical stains were positive for CD10, PAX-8, and carbonic anhydrase 9/IX (CA-IX) and negative for CK7. A diagnosis of metastatic CCRCC was made (). Radiological studies then revealed a left renal mass, bilateral adrenal masses, and enlarged retroperitoneal lymph nodes. A subsequent left radical nephrectomy and adrenalectomy was performed. On gross examination, a unifocal tumor that measured 14.7 cm in its greatest dimension was identified. Pathologic examination confirmed the renal origin of the vaginal carcinoma. The nephrectomy diagnosis was established as CCRCC with rhabdoid differentiation and multinucleated giant tumor cells. The World Health Organization (WHO)/International Society of Urologic Pathologists (ISUP) grade was 4. The tumor invaded into the perinephric fat tissue, renal sinus, and major branches of the renal vein. Tumor necrosis and lymphovascular invasion were identified. All margins, including Gerota's fascia, ureteral and vascular, were free of malignancy. The left adrenal gland was involved with metastatic CCRCC. The final pathological stage was pT3a pNx pM1.\nMultiple pulmonary nodules, measuring up to 4 mm, and a right adrenal nodule were observed on imaging. The patient is now receiving adjuvant targeted therapy (Sunitinib 50 mg). The lung nodules are no longer seen, and the right adrenal nodule had decreased more than 30% in size. Interestingly, her vaginal mass recurred, increased in size, and changed in shape since her last resection. She, thus, underwent a partial excision of the new lesion on the vaginal wall. The pathologic examination revealed a CCRCC, with extensive rhabdoid differentiation this time ().","Here’s a 3-5 sentence summary of the doctor’s note for the patient: This woman came to the clinic because she was experiencing vaginal bleeding and a feeling of something unusual in her area. During an examination, a small growth was found on her vaginal wall and removed. Tests revealed that the growth was a type of cancer that had spread to her kidneys, adrenal glands, and lungs. She underwent surgery to remove the kidney and adrenal gland, and is now receiving targeted therapy to help fight the cancer. Unfortunately, the cancer has now returned in her vagina, and she is undergoing further treatment."
538,"A sixteen-year-old boy nonswimmer was hospitalized after drowning in cold river water. Based on data given by the passing eyewitness, the teenager jumped into the river to retrieve a sheep he was tending but immediately disappeared from the water surface. This youth of the same age promptly dived into the river, found the victim's body on the bottom at a depth of 2 metres, raised him, and swam to the shore, which was about 3 metres away. Based on the rescuer's statement, the drowning boy was unconscious, was not breathing, had blue lips and nail pates, and had very cold skin. Due to knowledge about First Aid acquired in high school, the rescuer started measures for basic life support (BLS (Basic Life Support)), freed the airway, performed several mouth to mouth breaths, and started chest compression. After 2-3 minutes the victim vomited, threw up water, coughed, and started breathing. In the next few minutes he regained consciousness. In the meantime, members of his family had been called and they brought the patient to our institution in a private vehicle.\nAt arrival he was somnolent, oriented, anxious, and pale, with tachypnea (number of respirations 30/min), with hypothermic-body temperature 35.1°C, shivering (whole body), and with the subjective sensation of cold. He was admitted to the JIT and the following was monitored: ECG TA, pulse, body temperature, and pulse oximetry. A nasogastric tube and a urinary catheter were placed. Auscultation of both lungs revealed weakened breathing sounds, especially in the basal and middle parts, with many early and late inspirium cracklings and low tone whistling. Cardiac action was arrhythmic accompanied by tachycardia up to 120/min, BP (blood pressure) 100/55 mmHg. There were no signs of neurological deficit or lateralization, GCS (Glasgow Coma Scale) 13/15.\nUsing transcutaneous pulse oximetry at admission we measured the following: O2 saturation, which was 80% (during the course of treatment it normalized); pH 7.32; O2 54.8 mm Hg; CO2 55 mm Hg; BE—1.60 mmol/L; C-reacting protein 96 mg/L; Er 5.11 × 1012/L; Hb 136 g/L; Le 14.3 × 109/L; glycemia 4.0 mmol/L. Chest radiography showed spotty shadows in the middle and inferior lobes with free costodiaphragmatic recess sinuses bilaterally.\nThe ECG at arrival indicated an irregular rhythm, HR (heart rate) 120/min with short-term atrial fibrillation that later corrected spontaneously into normal sinus rhythm.\nImmediately upon admission we continued reanimation. The patient was warmed up with thermal blankets. Oxygen therapy was applied using an oxygen mask with reservoir without rebreathing, at a flow rate of 8 l/min, decreasing to 6 l/min in order to maintain SpO2 ≥ 94%. Parenteral antibiotic therapy (III generation cephalosporin, aminoglycosides, and metronidazole) and parenteral rehydration were administered.\nOn the fifth day after admission the patient showed complete recovery, including withdrawal of the chest radiographic symptoms and no pathological signs were visible on physical examination of the lungs.\nHowever, during each attempt to get up, we registered atrial fibrillation or individual SVES (supraventricular extrasystole) and VES (ventricular extrasystole) triplets. On echocardiographic examination the structure of the left ventricular myocardium was hyperechogenic; echocardiography was performed with slightly restricted LVEF\nDuring halter ECG examination we registered multiple strings of VES triplets, frequency 150 per minute. An appropriate dose of Presolol was included in the therapy, with advice of strict inaction and avoidance of any kind of physical exertion.\nTwo months later the control echocardiography revealed that heart function had improved further, FS 0.34%; EF > 45%.","This 16-year-old boy was rescued after drowning in a river and brought to the hospital. He was unconscious and struggling to breathe when he was found, but thanks to quick action, he was revived with basic life support and oxygen. He was then treated for hypothermia and other complications, including a lung infection and an irregular heartbeat. After several days of care and monitoring, he has made a full recovery and is now being closely watched for any recurrence of heart rhythm problems."
539,"A 27-year-old male was referred to our hospital by a secondary care center due to a sudden onset of visual loss in his right eye, which occurred while weight-lifting in sports gym 4 days previously. The clinic referred the patient with fundus photographs which were screened 4 days before () and after the Nd: YAG laser hyaloidotomy treatment. Snellen acuity was 10/10 in both eyes. There was no afferent papillary defect and anterior segment examination was normal. The intraocular pressures were measured as 14 mmHg in both eyes.\nDilated fundus examinations revealed a normal macula in left eye. But there was a retinal pigment epithelium (RPE) alteration at the margin of the inferior temporal arterial vascular arcade in the right eye and resolved preretinal and subretinal hemorrhages were seen in the macula (). A diagnosis of Valsalva retinopathy was made based on the history and the treatment photographs of Nd:YAG laser hyaloidotomy (). The patient was also screened with fundus fluorescein angiography for any other vascular pathologies ().\nAt 1st month of examination all hemorrhages were resolved but RPE alterations were still at the margin of the inferior temporal arterial vascular arcade in the right eye (). The patient was screened with (OCTA) (OCTA; Avanti, Optovue RTVue XR). The OCTA images revealed 2 lesions. On en face OCT angiogram of OCTA full thickness retinal hole formation and ellipsoid zone damage at the superior and inferior margin of the inferior temporal arterial vascular arcade were seen (). Superficial vascular plexus was also damaged at that region. The projection of the evacuation of blood from subhyaloid space and the full thickness retinal hole formation were same ().","This 27-year-old man experienced sudden vision loss in one eye while weightlifting. After an initial examination, doctors found bleeding in the back of his eye, which they identified as Valsalva retinopathy, likely caused by pressure during the weightlifting incident. Although the bleeding has mostly cleared up, there is still some damage to the retina. Further tests revealed a hole in the retina and damage to the blood vessels, and the patient will need to continue monitoring to ensure the damage doesn't worsen."
540,"A 5-year-old boy was hospitalized with a 3-month history of intermittent fevers, fatigue, migratory joint pain, and weight loss. Three months ago, the patient was diagnosed with group A Streptococcus pharyngitis and was prescribed a 10-day course of amoxicillin. Two weeks later, he started having pain in his knees, elbows, shoulders, and neck. The patient was diagnosed with influenza and completed a course of oseltamivir. His joint pain and intermittent fever persisted. He began to develop malaise, weakness, and difficulty waking and was noted to have cough. During the next month, the patient was evaluated twice in different emergency departments with the same complaints. Evaluation at the emergency department revealed a mildly elevated C-reactive protein and erythrocyte sedimentation rate. Chest X-ray findings were consistent with pneumonia, and he was prescribed amoxicillin/clavulanic acid. Blood and urine cultures were negative. Three weeks later, the patient presented to his pediatrician with complaints of bilateral ear pain. His fevers, joint pain, difficulty walking, and fatigue persisted. During physical examination, the pediatrician noted a new systolic murmur. It prompted his hospitalization for further evaluation and management.\nOn examination, the patient appeared unwell, but nontoxic. His temperature was 96.8°F (36.0°C), heart rate was 123 beats/min, respiratory rate was 23/min, blood pressure was 109/65 mm Hg, and oxygen saturation was 98% on room air. His growth was plotted on the 25th percentile for weight and height. He had a V/VI holosystolic murmur with thrill at the mitral area with radiation to the axilla and interscapular area. The boy reported pain during active and passive motion of both knees and the neck. His left knee was slightly swollen. Examination of the skin revealed presence of small (1 centimeter in diameter) painless, firm nodules over extensor surfaces of ankles and elbows bilaterally, consistent with subcutaneous nodules.\nFurther review of the patient's history of present illness reveled noncompliance with previously prescribed treatment. Parents reported that the boy refused to take antibiotics. Laboratory evaluation showed the white blood cell count of 9.9 × 103 μ/L (9.9 × 109/L), platelet count of 495 × 103/µL (495 × 109/L), hemoglobin level of 11.2 g/dL (112 g/L), C-reactive protein level of 84.3 mg/L (802 nmol/L), and erythrocyte sedimentation rate of 93 mm/hr. Antistreptolysin O titers were 4,133 IU/ml (reference range, <150 IU/ml). Results of urinalysis were normal. Electrocardiography showed normal sinus rhythm with signs of left ventricular hypertrophy with volume overload with increased R wave voltage above 98th percentile for age in leads V5 and V6 and Q wave in lead V6 above 98th percentile for age (). Echocardiographic findings included moderate left atrial enlargement, severe mitral valve regurgitation (), and aortic valve regurgitation. The PR interval was within a normal limit of 0.120 seconds for 5-6-year-old children.\nBased on the history of preceding group A Streptococcus infection, clinical symptoms, elevated acute phase reactants, and echocardiographic findings, the patient was diagnosed with acute rheumatic fever. This patient received intramuscular penicillin G benzathine and was started on glucocorticoids for severe carditis. He also was prescribed aspirin for symptomatic management of arthritis with an initial dose at 75 mg/kg per day in divided doses every 6 hours during two days of hospitalization. The dose of aspirin at discharge was decreased to 50 mg/kg per day in divided doses every 8 hours. The patient was discharged with recommendations to continue secondary prophylaxis with penicillin G benzathine every 4 weeks for the next 10 years.","This 5-year-old boy has been having a long period of illness with fever, joint pain, and fatigue for the past three months. He initially had a strep throat infection, but his symptoms didn’t go away and he developed other problems like pneumonia and swelling in his joints. During his hospital stay, doctors found a heart murmur and noticed some bumps on his skin, leading to a diagnosis of acute rheumatic fever, which can damage the heart. He is now receiving medication to prevent future problems and will need to continue taking antibiotics regularly for many years."
541,"An 81-year-old man presented with abdominal discomfort and distention. His medical history was remarkable for hypertension and type 2 diabetes mellitus. He and his family denied alcohol abuse. Physical examination revealed hepatomegaly without jaundice, ascites, or hepatic encephalopathy. Laboratory data included platelet count 101,000/mm3, total bilirubin 0.9 mg/dl, AST 33 IU/L, ALT IU/L, and PT-INR 1.15. The serum AFP and PIVKA-II levels were 1081.0 ng/ml and 43 mAU/ml. Serologic tests for hepatitis B and C virus were negative. The diagnosis of nonalcoholic steatohepatitis with Child-Pugh A liver cirrhosis was made and four HCC lesions were found in segments II, VI, and VIII on the imaging. The largest one was located near the liver capsule in segment II measuring 6.5 cm in diameter. The other three lesions were less than 2 cm with one in segment VI and two in segment VIII. Segmental TACE with epirubicin and iodized oil was performed and he was discharged uneventfully.\nHe was doing well until he developed abdominal pain 15 days after TACE which was gradually getting worse emergency transport to the hospital. His temperature was 37.7°C, blood pressure 102/41 mmHg, and pulse rate 79/minute. On physical examination, the abdomen was distended and hard to palpation without rebound tenderness. His hemoglobin was 12.6 g/dl. A contrast enhanced computed tomography (CT) scan of the abdomen showed iodized oil and intraperitoneal free air with a rupture of the HCC in segment II into the peritoneal cavity adjacent to the gastric wall (). Extravasation of contrast medium was not seen. Emergent upper gastrointestinal endoscopy confirmed no gastric mucosal lesions or a site of perforation. Without evidence of septic shock or hemorrhage, surgical drainage and transcatheter arterial embolization (TAE) are considered less effective. He was treated nonoperatively with piperacillin and tazobactam. Although an abdominal abscess formed, he was discharged on hospital day 18 with continued antimicrobial therapy.\nFive weeks after undergoing TACE, he was readmitted with hematochezia and hemorrhagic shock. The hemoglobin level was dropped to 6.6 g/dl. A contrast enhanced CT scan demonstrated gastric extraluminal compression by an HCC lesion with extravasation of contrast medium into the stomach (). Emergent upper gastrointestinal endoscopy showed a submucosal tumor with central ulceration located on the anterior wall of the gastric body, corresponding to extraluminal compression by a HCC (). The hemorrhage from the ulcer was successfully controlled by hypertonic saline-epinephrine injection. Another submucosal tumor was found in the gastric fundus without ulcer formation (). The patient's condition stabilized and he was discharged for palliative care and died six weeks after undergoing TACE.","This 81-year-old man was admitted to the hospital with worsening abdominal pain and a distended abdomen. Tests revealed he had severe liver damage (cirrhosis) with several cancerous tumors in his liver. After a procedure to treat the tumors, he experienced a rupture of one of the tumors, leading to bleeding and a drop in his blood count. Despite treatment, he unfortunately passed away six weeks later."
542,"A previously healthy 16-year-old female visitor from Japan presented to an adult emergency room (ER) with altered mental status and emesis. She was found lying on a bathroom floor in her hotel. There was a history of polyuria and polydipsia for 2 weeks and 8 to 10 kg weight loss during the previous month. Her vital signs upon arrival to the pediatric intensive care unit (PICU) were temperature of 98.1° Fahrenheit, heart rate 110/min, respiratory rate 26/min, blood pressure 140/81 mm of Hg, and 100% oxygen saturations without supplemental oxygen. Her capillary refill time was 4-5 seconds. Her weight was 57 kg. Her Glasgow Coma Scale (GCS) was 13 in the ER, which improved to 15 upon arrival to PICU. Skin rash with infected lesions was noted in her groin. The rest of her physical examination was unremarkable.\nWorkup in the ER showed hyperglycemia of 472 mg/dL, metabolic acidosis (pH 6.75, pCO2 18.4, pO2 149, HCO3 2.5, base deficit 32.6, anion gap 23.5), ketosis (beta-hydroxybutyrate 11.41), glucosuria, and ketonuria, which were consistent with diabetic ketoacidosis. Her white cell counts were 22.3 k/L, hemoglobin 15.8 g/dL, and hematocrit 47%. The rest of her workup was unremarkable.\nThe patient received fluid resuscitation with 30 mL/kg of 0.9% normal saline (NS) and 50 mEq of sodium bicarbonate in the ER. Continuous insulin infusion was started at 0.1 unit/kg/hr. After that she was started on intravenous fluids containing 0.45% saline and 75 mEq/L of sodium bicarbonate. This was administered at 150 mL/hr, which was 1.25 times the usual daily maintenance requirement for her weight. A consultation with our PICU was obtained at this stage. No further bicarbonate boluses were given. Her fluids were changed to isotonic fluid with potassium phosphate and potassium chloride and without any bicarbonate. It was administered at the rate of 1.5 times maintenance of daily requirement for weight. She was transferred to the PICU at this stage, where she continued to exhibit severe metabolic acidosis with pH 6.97, pCO2 26.7, HCO3 6.1, and base deficit of 24.5. The DKA management was continued with close monitoring and serial laboratory evaluations.\nAfter few hours of her arrival in the PICU, the patient became disoriented and confused. She was given one time 5 ml/kg of 3% hypertonic saline (HS) due to concern for cerebral edema and she responded well. Four hours later she developed lethargy followed by apnea. She was given additional doses of 3% HS bolus (5 mL/kg) and 0.8 g/kg of 20% mannitol. The hyperosmolar therapy was effective but only transiently; the patient eventually required intubation and mechanical ventilation for recurrent apnea.\nCT head obtained at that time showed a thin, right parietal subdural hemorrhage without any evidence of edema or mass effect. Of note, CT scan was obtained after the initiation of hyperosmolar therapy.\nHyperosmolar therapy was continued afterwards with 3% HS and 20% mannitol. It was guided with close monitoring of renal function including serum sodium and serum osmolarity. The doses and other parameters were maintained per standard of care [, ].\nThe patient's serum sodium was 135 upon arrival in the ER and 143 at around 11 hrs before the initiation of hyperosmolar therapy and reached to 160 at 71 hrs when hyperosmolar therapy was discontinued. It was normalized to 143 at 131 hrs of admission. Her osmolarity was 298, 305, 335, and 299 at those timings (sodium in mmol/L and osmolarity in msom/kg). The rate of glucose reduction was < 50 mg/dl/hr.\nMRI brain obtained at 60 hrs of admission showed mild edema of the cortex and sulci and diffuse edema of the pons and midbrain with restricted diffusion in the pons consistent with central pontine myelinolysis also known as osmotic demyelination syndrome. There was no significant change in the subdural hemorrhage (Figures –). MR angiogram showed no evidence of vessel abnormality.\nThe patient required continued mechanical ventilation and further management in the PICU. She became more responsive on day 5, and her support was gradually weaned until extubation on day 7. At that time she exhibited only mild residual dysmetria, trivial ataxia, mild left sided weakness, and neglect. All deficits subsequently improved.\nHer course was complicated by an isolated focal seizure that responded to standard antiepileptic medication. She also developed bilateral upper extremity deep venous thromboses (DVT). Hematology workup showed no underlying hypercoagulability. Thus the DVT was considered as another complication of DKA [–].\nHer overall hospital length of stay was 14 days, and by the date of discharge she was able to ambulate with a walker and speak and eat without difficulty.","A 16-year-old girl from Japan was found unconscious in a hotel bathroom and brought to the hospital. She had been feeling very thirsty and peeing a lot for two weeks, and had lost a significant amount of weight. Tests showed she had dangerously high blood sugar and a life-threatening condition called diabetic ketoacidosis. Doctors treated her with fluids, insulin, and medications to correct her blood sugar and acid levels. She also developed a small bleed on her brain and needed extra treatment for that. After several days in the PICU, she started to improve and was able to walk and talk normally. During her stay, she experienced a seizure and developed blood clots in her arms, but she continued to recover and was eventually discharged home with a plan for continued follow-up care."
543,"A previously healthy 29-year-old female was being transferred to the “Sismanoglio” General Hospital of Athens, Greece, due to pulmonary embolism (PE) 24 hours following a caesarian section. She had already received a bolus of intravenous 5000 E Heparin. The patient was hemodynamically unstable (blood pressure= 90/40 mmHg, heart rate= 140 beats/min, saturation= 85%, and breathing rate= 30 breaths/min) and afebrile (37 C). During the transportation, she suddenly went into cardiopulmonary arrest with pulseless electrical activity. CPR was immediately instituted according to advanced life support guidelines. Manual chest compressions and artificial ventilation were performed. The CPR was effective and the patient was then transferred to the Intensive Care Unit (ICU). About 3 hours later, she continued being hemodynamically unstable, her hematocrit had dropped 10 points (from 33% at ICU admission to 23% at that point), and she had a distended abdomen. She was transfused with two blood units and an emergency Diagnostic Peritoneal Lavage (DPL) was positive for intra-abdominal bleeding. At that point she was urgently taken into the operating theatre. An exploratory laparotomy was decided upon. About two liters of blood was removed from the abdomen. The intraoperative finding was an approximately 5 cm liver laceration at the left lobe, near the attachment point of the left coronary ligament. Liver suturing was performed and the intra-abdominal bleeding was successfully controlled. Unfortunately the patient passed away 3 hours after the operation. Cause of death was the massive pulmonary embolism.","This 29-year-old woman was admitted to the hospital after a sudden pulmonary embolism following a cesarean section. She became very ill, with dangerously low blood pressure and difficulty breathing, and went into cardiac arrest. Doctors immediately started CPR, which successfully revived her, and she was transferred to the ICU. However, she remained unstable and had significant internal bleeding, requiring a blood transfusion and emergency surgery to repair a laceration in her liver. Sadly, despite the surgery, she passed away due to the pulmonary embolism."
544,"A previously healthy 62-year-old female was transferred with an ambulance to the emergency department due to drowning at the sea. CPR had been instituted in situ by an experienced lifeguard. At presentation the patient was hemodynamically unstable (blood pressure= 100/55 mmHg, heart rate= 135 beats/min, and breaths= 25 per min) and afebrile (36.7 C). Her laboratory investigation showed hematocrit (Hct)= 23% and hemoglobin (Hgb)= 7.3 g/dL. Fluid resuscitation was performed and she was also transfused with 2 blood units, but she continued being hemodynamically unstable. After the transfusion she had Hct= 19% and Hgb= 6.4 g/dL. The abdominal ultrasonography revealed free intra-abdominal fluid. Bedsides, chest X-ray was negative for tympanic abdomen or pneumoperitoneum. At that point an exploratory laparotomy was decided upon. The site of bleeding was found to be vessels originating from the lesser curvature of the stomach, which were successfully ligated. The patient had an uneventful recovery and was discharged on the 12th postoperative day. Eight years after the event, she continues her everyday activities, without any limitations.","This 62-year-old woman was brought to the hospital after being rescued from the sea following a drowning incident. She was very sick when she arrived, with low blood pressure and a rapid heart rate, and needed immediate treatment with fluids and blood transfusions. During surgery, doctors found bleeding from blood vessels near her stomach and successfully stopped the bleeding. She recovered well and was discharged after 12 days, and she has been healthy and active since then."
545,"A 49-year-old Japanese woman underwent an MRI examination in the referring hospital for an assessment of the orthopedic disease of her right hip joint, and the MRI revealed a tumor in her pelvis. She was referred to our hospital, where an MRI examination again showed tumor mobility (Fig. ), and a tumor derived from the intestinal tract was suspected.\nIn the contrast CT image (Fig. ), a mass approx. 45 mm in size was found on the right side of the pelvic floor, and in the blood vessel construction image, the tumor was nourished by vessels from the splenic artery to the greater omentum. In 18F-fluoro-2-deoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT), no abnormal accumulation was observed (Fig. ). The results of complete blood cell counts and biochemical tests were all normal. Cancer antigen 19-9 (CA19-9) showed a mild elevation at 37.8 U/mL (normal range < 37.0 U/mL), and carcinogenic embryonic antigen (CEA) was normal at 0.8 ng/mL (normal range < 5.0 ng/mL). Based on all of these results, the preoperative diagnosis was a suspected omental primary tumor or accessory spleen.\nThere was a danger of torsion irrespective of the presence or absence of malignancy, and the patient desires surgery to remove the tumor; the surgery was conducted concurrently with the diagnosis and treatment. The surgery was a single-incision laparoscopic-assisted greater omental tumor resection. A longitudinal incision was made approx. 4 cm around the umbilicus. A multichannel port (x-Gate®, Sumitomo Bakelite, Tokyo) was inserted in the wound. Observation of the abdominal cavity revealed a red and solid tumor mass of approx. 4 cm. Adhesion with the surrounding tissue was not observed. The greater omentum and the tumor were guided out of the body through the gate hole (Fig. ), and the tumor was excised together with the greater omentum. The tumor, a light brown tinged solid mass with a smooth border, was 5.2 × 3.8 × 3.5 cm in size with a well-defined border (Fig. ).\nHistopathologically, the tumor involved large and small blood vessels (Fig. ), and tumor cells with eosinophilic cytoplasm were increased in number, in sheet form (Fig. ). In immunohistochemical staining, the tumor cells were found to be positive for HMB45 (Fig. ), Melan-A (Fig. ), and α-SMA (Fig. ), but negative for CD34, Desmin, c-kit, and s-100.\nBased on the above results, the tumor was diagnosed as an omental PEComa. There has been no recurrence at 16 months after the surgery.","A 49-year-old woman was referred to our hospital after an MRI showed a tumor in her pelvis. The tumor appeared to be connected to her digestive system and was removed through a minimally invasive surgery. The surgery successfully removed the tumor and a portion of her greater omentum. Pathology revealed the tumor was a rare type called omental PEComa, and she has been doing well with no signs of recurrence after 16 months."
546,"A 26-year-old primigravida at 39-week gestation by date of last menstrual period, presented to the Muhimbili National Referral Hospital, Tanzania, in active labor. She had no significant past medical history and an uncomplicated antenatal course. Shortly after midnight she had a precipitous vaginal delivery. After delivery, the woman began hemorrhaging profusely. Her uterus was vigorously massaged, ten international units (IU) of intravenous oxytocin were administered, and the placenta manually removed. Despite the interventions, the patient continued to bleed and lost consciousness. On pelvic examination, the uterus was well contracted, bleeding superior to her cervix was minimal, and second degree bilateral vaginal sulcal lacerations were identified. Fluid resuscitation was initiated, and the patient was emergently taken to the operating theatre by the in-house Obstetrician and Gynecology resident for examination under anesthesia and repair of her vaginal lacerations. Despite attempts at suture repair, hemorrhage from the laceration sites continued. The senior Obstetrician and Gynecology consultant was also unsuccessful in gaining control of the hemorrhage and therefore placed an ESM-UBT device into her vagina. The ESM-UBT device was inflated with 300 cc of water and secured with vaginal packing. Hemorrhage ceased and antibiotics, intravenous fluids, and blood products were administered. The ESM-UBT device was removed after 48 hours, no further repair was necessary, and bleeding did not recur. The woman was discharged home on postpartum day two and at her six-week postpartum visit had fully recovered.","This woman was in labor at 39 weeks pregnant when she experienced a rapid and heavy vaginal delivery. Despite efforts to stop the bleeding, she became unconscious and continued to hemorrhage. Doctors quickly repaired her vaginal tears and then used a special device to help control the bleeding. After the device was removed, the bleeding stopped, and she made a full recovery and was discharged home on day two after delivery."
547,"A 22-year-old gravida two para one woman, six-week pregnant by dating from her last menstrual period, presented to a private maternal health facility for termination of her pregnancy via dilation and suction curettage. Upon attempted cervical dilation the woman began hemorrhaging profusely. She was referred by ambulance to the Mahatma Gandhi Institute for Medical Sciences where she presented extremely pale, weak, and in shock with vital signs including a blood pressure of 80/30 mm/Hg and heart rate of 145 bpm. Outside imaging that was brought with the patient, when reviewed at the referral facility, clearly identified a cervical pregnancy. The patient was emergently taken to the operating theatre where resuscitation was initiated, and a rapid pelvic exam was performed. Examination revealed severe bleeding and a swollen cervix ballooned with clots. An ESM-UBT device was intentionally placed into the cervix, inflated with 200 cc of water which formed a dumbbell, filling the cervix and ballooning into the lower uterine segment and vagina. Vaginal packing was used to secure the uterine balloon's position. The woman was transferred to the intensive care unit where she received isotonic fluids and two units of blood. The ESM-UBT device was removed after 24 hours without any resumption of bleeding. There were no complications on six-week follow-up.","This 22-year-old woman, who was six weeks pregnant, came to the hospital to have her pregnancy terminated. During the procedure, she began to bleed heavily and became very weak. It was discovered that she was experiencing a cervical pregnancy, where the fertilized egg had implanted outside the uterus. Doctors successfully stopped the bleeding by placing a special device in her cervix to prevent further blood loss, and she is now stable and recovering well."
548,"A 27-year-old gravida three para two woman, 23-week pregnant by date from her last menstrual period, presented to the Government Medical College Nagpur National Hospital, India, with vaginal bleeding. She had no significant past medical history. On physical examination, her fundal height was consistent with a 32-week uterus, there was moderate vaginal bleeding, and her cervix was closed. An ultrasound examination was remarkable for absence of a fetus and a solid collection of echoes with numerous anechoic spaces consistent with a molar pregnancy. The patient was taken to the operating theatre for cervical dilation and evacuation under general anesthesia. Following evacuation, the uterus was atonic and hemorrhage ensued with an estimated blood loss of greater than two liters. Bimanual uterine massage was performed, the bladder was drained, fluid resuscitation was initiated, and oxytocin (20 IU) and ergometrine (0.4 mg) were administered intravenously. Despite these interventions, profuse bleeding continued, and her vital signs deteriorated to a blood pressure of 84/60 and heart rate of 120. An ESM-UBT device was inserted into the uterus, inflated with 300 cc of water, and hemorrhage was immediately arrested. Her condition stabilized, and she was transfused blood. The ESM-UBT device was removed after 24 hours with no bleeding recurrence and the woman was discharged home two days thereafter. Pathology results confirmed a complete molar pregnancy. There were no complications on subsequent two- and six-week follow-up visits and she remains within their system for clinical monitoring throughout the year.","This 27-year-old woman, who was 23 weeks pregnant, came to the hospital because of vaginal bleeding. An ultrasound showed she was carrying a molar pregnancy, which is a rare and abnormal pregnancy where the tissue doesn't develop properly. She underwent a procedure to remove the molar pregnancy, but unfortunately, she started to bleed heavily afterward. With the help of special equipment and blood transfusions, her bleeding was stopped, and she was able to go home and continue her care."
549,"A 67-year-old Caucasian female patient presented to the hospital with one day history of hematemesis and melena. She had two episodes of bright red emesis and four episodes of dark colored stools the day she presented, followed by a brief syncopal episode lasting for 10-15 seconds. She also complained of epigastric pain. She was diagnosed with polycystic liver disease and portal hypertension one year ago when she presented similarly with hematemesis and melena. At that time, she had an esophagogastroduodenoscopy (EGD) which showed esophageal varices and underwent banding. She denied any history of alcohol use, NSAID ingestion, or peptic ulcer disease. She was gravida 3, para 3, and had tubal ligation following her last pregnancy. She denied use of oral contraceptive pills or hormonal replacement therapy in the past. Her family history was significant for cystic liver disease and chronic liver failure in mother and maternal aunt.\nAt the time of presentation, she was afebrile (temperature of 98.7°F), tachycardic (heart rate of 110 beats/min), tachypneic (respiratory rate of 18/min), and hypotensive (blood pressure of 97/60 mmHg). Cardiopulmonary examination was normal. Abdominal examination showed mildly distended abdomen with epigastric and right upper quadrant tenderness, and bowel sounds were normal. Extremities showed bilateral 1+ pitting edema. Initial laboratory work showed hemoglobin of 7.7 g/dl, hematocrit of 24%, white blood count of 10.8 cells/mm3, and platelet count of 162 cells/mm3. Metabolic panel showed nonanion gap metabolic acidosis with serum bicarbonate of 17 mEq/L, normal serum BUN, and creatinine of 23 mg/dl and 0.57 mg/dl, respectively. Liver panel showed total bilirubin of 2.2 mg/dl, direct bilirubin of 0.4 mg/dl, albumin of 2.5 g/dl, alkaline phosphatase of 51U/L, AST of 22 U/L, and ALT of 16 U/L. Gamma-glutamyltransferase was measured to be 27 U/L and prothrombin time (INR) was measured to be 1.46.\nShe was aggressively resuscitated with fluids, pressors, and packed red blood cell (PRBC) transfusion. Octreotide and proton pump inhibitor infusions were started. Patient was electively intubated for hemodynamic instability and altered mental status. An EGD was done which showed four columns of large esophageal varices with red wale sign showing stigmata of high risk for bleeding and type 1 gastroesophageal varices (). Four bands were successfully applied starting from cardia up to midesophagus. A computed tomography (CT) of abdomen showed multiple cysts in the liver with evidence of portal hypertension in form of splenomegaly, ascites, and esophageal varices (). Liver surface was smooth and no thrombus was identified in hepatic veins and inferior vena cava. Bilateral kidneys were normal with no evidence of renal cysts (). Vital signs and hemoglobin were monitored closely. Patient was successfully extubated next day and pressors were weaned off. Patient did not have any further episode of hematemesis but continued to have melena. She slowly dropped her hemoglobin requiring transfusions with PRBC. An EGD was repeated which reveled 3 more columns of esophageal varices with intact bands from previous EGD. Four more bands were placed on remaining varices. Following repeat EGD patient's hemoglobin remained stable for rest of her hospital stay. During her hospital stay, she also underwent therapeutic paracentesis with removal of 330 ml of peritoneal fluid for worsening ascites (). Peritoneal fluid analysis showed serum ascites albumin gradient (SAAG) of more than 1.1 g/dl, normal white cell count, negative gram stain, and cultures. She was started on propranolol, furosemide, and spironolactone. She was discharged home with recommendations to follow up in outpatient clinic with a plan of repeat EGD in 3-4 weeks.","67-year-old woman came to the hospital because she was vomiting blood and had dark-colored stools. She had a similar episode a year ago and was treated with banding for esophageal varices, which are enlarged veins in her esophagus. During her current visit, she was low blood pressure and had a syncopal episode, so she was given fluids, medications, and eventually intubated to help her breathe. After receiving treatment and having additional bands placed on her varices, she was able to be removed from the breathing tube and discharged home with follow-up appointments."
550,"A 59-year-old Asian male presented to the gastroenterology office for a follow-up of a previously found duodenal lesion. He had an EGD at an outside facility 2 years earlier for evaluation of abdominal pain which showed a 1.2 cm duodenal polypoid lesion in the second portion of the duodenum. Pathology of the polyp revealed heterotopic gastric mucosa with benign lobules of gastric glands. He also was found to have chronically active H. pylori gastritis with numerous Helicobacter organisms and atrophic gastritis with patchy areas of intestinal metaplasia. At that time, he was instructed to follow up for a repeat endoscopy for surveillance in two years. As recommended, two years later he presented to our office for surveillance of his duodenal lesion. Due to his history of atrophic gastritis and a large duodenal lesion, we performed an upper endoscopy that showed atrophic gastritis, duodenitis, and a single 10 mm polyp in the second portion of the duodenum. The duodenal polyp was biopsied. Pathology revealed polypoid gastric metaplastic mucosa with focal epithelial atypia including nuclear enlargement, stratification, nucleoli, and few mitoses. The patient was referred for endoscopic ultrasound to further characterize the lesion and complete resection. Several weeks later he underwent an endoscopic ultrasound that revealed a 13-mm pedunculated and sessile polyp on the lateral wall opposite to the major papilla in the second portion of the duodenum (). The polyp was removed en bloc using a hot snare following a 4-mL saline lift. Two endoclips were placed for hemostasis. Pathology revealed a pyloric gland adenoma (Figures , , and ) with predominantly low grade dysplasia (Figures and ) and some small areas of focal high grade dysplasia. The slides were read by the in-house pathologist and were also reviewed by a GI expert pathologist at the University of Michigan. The patient was recommended to follow up for surveillance upper endoscopy in 6 months due to the areas of high grade dysplasia found on pathology. He was lost to follow-up for over one year. Fifteen months after the PGA was removed, the patient did follow-up for a surveillance upper endoscopy. A duodenal scar was found at the site of the PGA and a biopsy was taken from the site. There was no evidence of residual pyloric gland adenoma. The patient was then recommended to follow up for surveillance endoscopy in 5 years in accordance with the ASGE guidelines for surveillance of gastric adenomatous polyps.","This 59-year-old man came in for a check-up of a previous duodenal polyp. During the examination, doctors found that he had significant changes in his stomach lining, including inflammation and some abnormal cells. A biopsy of the polyp revealed it was a pyloric gland adenoma with low-grade dysplasia. The polyp was removed, but further testing showed some areas of high-grade dysplasia, requiring close monitoring with follow-up endoscopies."
551,"The patient is a 56-year-old man with a past medical history significant for poorly controlled insulin-dependent type 2 diabetes mellitus, hypertension, hypothyroidism, and meatal stenosis with incomplete bladder emptying who presented the University Medical Center emergency room with a seven-day history of perineal pain and fever. One week prior to presentation, he was seen in the emergency room with the same symptoms and was placed on a 14-day course of ciprofloxacin for a suspected urinary tract infection. Also, the patient was admitted to the medical intensive care unit 10 months previously for severe sepsis secondary to Candida glabrata urinary tract infection with candidemia. The patient denied penile trauma, gross hematuria, and dysuria. Upon presentation, he was hemodynamically stable with a blood pressure of 145/77 mmHg, and he had mild tachycardia with a heart rate of 110 beats per minute and a fever of 101.4 F. On physical exam, the patient had mild tenderness to palpation at the penoscrotal junction with induration, no crepitus was palpated, and there were no skin changes. His laboratory values were significant for an elevated white blood cell count (WBC) of 20,000/μL, a creatinine level of 1.4 mg/dL, and a hemoglobin A1c level of 9.4%, and all other values were within normal limits. On computerized tomography (CT) scan, emphysematous changes were noted with in the ventral portion of the penile shaft with air at the bulb of the corpus spongiosum (Figures and ).\nThe patient had a postvoid residual of 320 ml; thus, a transurethral Foley catheter was placed with withdrawal of 700 ml of clear yellow urine which was sent for culture. The patient was begun on intravenous (IV) meropenem, fluconazole, and daptomycin. Repeat CT scan on hospital day 1 was largely unchanged, and the patient remained febrile to 102.3 F. On hospital day 2, meropenem and daptomycin were discontinued, and the patient was switched to piperacillin/tazobactam based on recommendations from the Infectious Disease team. A pelvic magnetic resonance imaging (MRI) with and without gadolinium was obtained at this time which showed reduced blood flow to the corpus spongiosum compared to the corpus cavernosum on T1-weighted imaging with gadolinium ().\nThe patient was then taken to the operating room on hospital day 3 due to worsening penile pain and induration, a persistently elevated white blood cell and continued fever. On cystourethroscopy, the urethral mucosa appeared dusky from the anterior to the membranous urethra. A longitudinal incision was made in the perineum, the urethra was palpated and noted to be indurated and firm, and thus the bulbospongiosus muscle was split and a longitudinal incision was made in Buck's fascia with expulsion of approximately 10 ml of purulent fluid which was sent for culture and gram stain. The remaining necrotic spongiosal tissue was debrided with blunt dissection, a penrose drain was placed within the defect, and the skin was closed loosely. On postoperative day 2, his white blood count decreased to 13,000/μL and he remained afebrile for 48 hours. The patient's intraoperative wound culture was found to be positive, and using automated Matrix Assisted Laser Desorption Ionization Time of Flight (MALDI-TOF) mass spectrometry, the organism Candida glabrata was identified by our microbiology laboratory. The wound culture result was identical to his urine culture. The patient was then switched to micafungin based on the Infectious Disease team's recommendations. He was subsequently discharged to home on postoperative day 14 on a 14-day course of oral voriconazole with the transurethral Foley in place.","A 56-year-old man was admitted to the hospital with a painful and infected area on his penis, a problem he’d experienced before. Tests revealed a serious infection with bacteria and fungus, and a previous urinary tract infection. Surgeons performed a procedure to clear the infection and remove damaged tissue, and the patient’s fever and white blood cell count gradually returned to normal. He was discharged home with oral medication and a catheter to drain any remaining urine."
552,"The following case is of a 66-year-old man with a complicated cardiac history, who for some time has been experiencing limiting angina despite being on maximally titrated medical therapy of aspirin, prasugrel, carvedilol, ranolazine, isosorbidemononitrate (sustained release), atorvastatin, lisinopril, nitroglycerin patch, and as needed sublingual nitroglycerin. His cardiac history includes extensive coronary artery disease (CAD). \nStatus post coronary artery bypass graft 15 years ago (CABG: reverse saphenous vein graft to first and second obtuse marginal arteries in a sequential fashion and reverse saphenous vein graft to posterior descending artery and posterolateral artery in a sequential fashion) Status post redo CABG 2 years ago (CABG: left internal mammary artery [LIMA] to the left anterior descending artery [LAD]) Status post multiple stents\nHe also has a history of hypertension, diabetes (on metformin), dyslipidemia, and prior bilateral carotid endarterectomy. He was evaluated by cardiothoracic surgery 8 months prior, and they recommended against reoperation.\nAbout 4 months prior, he underwent another coronary angiography. This angiogram showed a relatively long 95% stenosis in the mid-LAD. The stenosis extended from the mid-LAD to a bit passed the LIMA anastomosis, with retrograde flow into the LIMA (). Some tenting of the LAD at the anastomotic site was also noted. Several predilations were performed at 8 and 10 atmospheres with no significant improvement to the stenosis. A 2.25 × 28 mm Synergy drug-eluting stent (Boston Scientific) was then deployed at 12 atmospheres and postdilated with 2.25 × 20 mm noncompliant balloon at 14 atmospheres. The stent was successfully deployed in the mid-LAD with no residual stenosis. A slight kink in the distal one-third of the stent at the LIMA insertion point was noted (). further demonstrates the movement of the intact stent during diastole and systole. The patient's symptoms temporarily improved.\nA short time after, he presented again with rest pain. His vital signs were stable. Physical examinations, including cardiac exams, were unimpressive. Electrocardiogram (EKG) showed normal sinus rhythm without significant ST/T changes. Two troponin levels drawn 12-hour apart were both normal. Repeat coronary angiography was performed. It revealed a mid-LAD stent fracture with 70% in-stent restenosis (fractional flow reserve of 0.78) (). Coronary intervention was performed and a Xience Alpine DES was deployed inside the fractured Synergy DES with TIMI III flow and no residual stenosis (). The patient tolerated the procedure and was discharged in stable condition.","66-year-old man with a history of heart disease and multiple surgeries was evaluated for ongoing chest pain. After several tests, doctors found a crack in the stent in his main artery and a narrowing of the artery, which was treated with a new stent. Despite the new stent, the artery continued to narrow, so another procedure was performed to repair the damaged stent. The patient is now stable and discharged home."
553,"A 26-year-old-woman, with history of labial herpes and asthmatic bronchitis, presented with bilateral retroocular pain, odynophagia, fever, vaginal discomfort and vulvar ulcers. The ulcers continued to progress despite treatment with nonsteroidal anti-inflammatory drugs and valacyclovir, prescribed at the primary healthcare site. Two days later, after initial consultation at our emergency room, she was admitted immediately at the Vulvar Clinic of our institution, with increasing vulvar pain, without other symptoms. Physical examination of the vulva showed extended vulvar oedema and kissing pattern ulcers on labia minora and majora, vagina and cervix (). Inguinal lymph nodes were also bilaterally swollen. The patient denied the use of other medications and sexual activity in more than 6 months. First blood tests only showed C-reactive protein 12.35 mg/dL (normal: <0.5 mg/dL). Serologies for herpes virus 1 and 2, Ebstein-Barr virus (EBV; IgG+), cytomegalovirus, mycoplasma pneumoniae, parvovirus B19, toxoplasmosis, rubella, hepatitis, human immunodeficiency virus, and syphilis (using the Venereal Disease Research Laboratory test) were negative. After this, a multidisciplinary approach was performed. When directly asked, she complained about mild eye dryness and she often felt a discomfort of dry mouth. There was no familial history of autoimmune diseases, but her father had some episodes of oral aphthosis. Immunological examination was positive for rheumatoid factor (RF – 22.3 UI; normal: <15 UI), anti-nuclear antibodies [ANA (speckled, titer 1:320)] and antibodies to SSA/Ro (SSA 3+/ Ro52KD 3+)—initial screening step of ANA by indirect immunofluorescence on HEp-2 cells (Euroimmun ®, Germany); autoantibodies confirmation assay by line immunoblot (ANA profile 3 - Euroimmun ®, Germany)—antibodies detected on strips were evaluated semiquantitatively (negative, 1+, 2++, and 3+++). Anti-SSB/La, anti-RNP, anti-Sm, anti-dsDNA, antineutrophil cytoplasmic, anticardiolipin, and anti-beta(2)-glycoprotein1 antibodies were negative. Serum C3 level was 1.79 g/L (normal: 0.9-1.8 g/L) and C4 level was 0.31 g/L (0.1-0.4 g/L). Immunoglobulins (IgG, IgM, IgA) were measured and a high IgG level was found (20.50 g /L; normal: 7-16 g/L). Erythrocyte sedimentation rate was also high (45 mm/h; normal: <16 mm/h). Lupus anticoagulant and HLA-B27 were both negative. Ophthalmological evaluation was refused by the patient. Labial salivary gland (LSG) biopsy revealed focal lymphocytic sialadenitis (FLS), with a focus score (FS) =1 (per 4 mm2) obtained by four LSGs (3-5 mm). No other histopathological features were reported. The patient was diagnosed as having pSS on the basis of dry eyes and dry mouth, positive anti-SSA/Ro antibody, and typical histopathologic abnormalities on LSG biopsy. Prednisolone 20 mg/day was prescribed and vulvar healing appeared within 2 weeks with partial loss of left labia ().","This 26-year-old woman presented with worsening pain, sores, and swelling in her vulva, despite previous treatment. Tests revealed she had an autoimmune condition called Sjögren’s syndrome, characterized by dry eyes and dry mouth, along with inflammation of her salivary glands. Blood tests showed elevated levels of certain antibodies and immune system markers, confirming the diagnosis. She was started on medication to manage her symptoms, and her vulvar sores began to heal."
554,"A 25-year-old gentleman presented to our clinic with a complaint of recurrent pain and swelling on his right cheek of three-month duration. He visited a general practitioner each time, and the condition was resolved with analgesic and antibiotics. However, his symptoms got worse and he attended our Oral Surgery Clinic for consultation.\nThe patient is a fit and healthy young man with no relevant medical history and no known history of allergy. Past surgical history revealed that he had underwent bimaxillary orthognathic surgery one and half year earlier in a local hospital. Although the postoperative period was uneventful, the surgical team informed him that there was a dislodged orthodontic appliance in his right cheek that must have occurred during the operation. The team explained to the patient that this accident was realized later on the next day after the surgery when the molar tube from the right maxillary second molar was found missing, and its presence was confirmed high up in the right maxillary-zygomatic buttress area shown in the postoperative X-ray image taken on the next day following the surgery. A series of further postoperative radiographs confirmed its location, lying outside the right maxillary antrum. Due to the pronounced postoperative facial oedema at that time, no attempt was made to remove the appliance. The absence of sign and symptoms during further follow-up sessions confirmed the decision to leave it in-situ with continuous clinical observation.\nOn examination, there was no extraoral swelling noted. The mandible and maxilla seemed firm indicating good healing following previous mandibular saggital split and maxillary Le Fort I osteotomy sites and a stable class I dental occlusion. Intraorally, there was a sinus with slight pus discharge on the upper right buccal sulcus region adjacent to the upper right first premolar. All teeth in that quadrant were firm and vital. Tenderness was elicited upon palpation on the upper right vestibular region. We suspected the sinus track may originate from the dislodged appliance embedded in the cheek soft tissue. A periapical view was then taken with gutta-percha inserted into the sinus for foreign body localization purpose. The radiograph revealed the gutta-percha pointed towards the site of titanium plate and screws placed used for rigid fixation, and with the molar orthodontic tube appliance in its vicinity (). A cone beam CT was performed to provide a 3D detailed location of the appliance (Figures and ) and confirmed it to be located outside the maxillary antrum.\nThe presence of the molar orthodontic tube foreign body reaction was suspected as the most probable cause of the recurrent right cheek pain and swelling associated with an intraoral discharging sinus. Exploration of the site was performed through the sulcular incision under general anesthesia. The dislodged molar tube was identified lying on the zygomatic bone just beneath the raised flap. It was removed by dividing some surrounding fibrous tissue strands. Just below it, one titanium straight bone plate with four screws used for fixing the previous Le Fort I osteotomy site was inspected and found to be rigidly embedded in normal bone. However, a decision was made to remove them based on the fact that they are present in an infected area. (). The Le Fort I osteotomy site showed good healing with new bone formation. Patient had an uneventful recovery thereafter, and the orthognathic surgical team who attended him previously was informed of his progress.","A 25-year-old man has been experiencing recurring pain and swelling in his right cheek for three months. It turns out a small piece of a missing orthodontic appliance from a previous jaw surgery was dislodged and became trapped in his cheek tissue. The trapped appliance was causing a sinus infection and inflammation, leading to the pain and swelling. The appliance was successfully removed, and the sinus infection cleared up, resulting in a full recovery."
555,"A 9-year-old girl was admitted due to repeated loss of consciousness, concomitant with a pale face, palpitations, and convulsions, which had persisted for 2 years and had been aggravated during the previous 2 months. These symptoms occurred automatically. The patient denied experiencing any sweating, nausea, vomiting, trembling, or an obvious sense of hunger before meals. The patient was previously misdiagnosed with epilepsy in another hospital, but no abnormal findings were detected on a 24-h electroencephalogram at our hospital. Her abdominal perfusion CT showed a highly perfused nodule within the pancreatic tail; A magnetic resonance scan confirmed the location of this nodule and indicated that its size was 11.6 × 13.2 mm (Fig. -). Additionally, 68Ga-exendin 4 PET-CT showed a region in the pancreatic tail with abnormally high metabolism and overexpression of the glucagon-like peptide-1 receptor (Fig. -). Lab testing showed a low fasting blood glucose (BG) of 2.2 mmol/L (reference range: 3.9–6.1 mmol/L), a high proinsulin level of 4455.9 pg/mL (reference range: 30–180 ng/mL), a normal C-peptide level of 2.56 ng/mL (reference range: 0.8–4.2 ng/mL), a serum insulin level of 15.35 μIU/mL (reference range: 5.2–17.2 μIU/mL), and a gastrin level of 92.6 pg/mL (reference range: < 100 pg/mL). These results confirmed a diagnosis of insulinoma. Imaging examination showed no abnormalities indicative of parathyroid adenoma or malignancy in the pituitary or adrenal glands. Lab testing showed normal levels of parathyroid hormone (PTH), blood calcium, phosphate, follicle-stimulating hormone (FSH), growth hormone (GH), prolactin (PRL), adrenocorticotropic hormone (ACTH), 24-h urinary free cortisol (24 hUFC), and serum cortisol. Her luteinizing hormone (LH) level was 0.24 IU/L (reference range: 2.12–10 IU/L during the follicular phase), which was considered related to her age.\nPreoperative preparation: To avoid recurrent symptoms and to maintain her fasting BG at a tolerably low level, the patient was given regular snacks before bedtime. BG can be controlled at a level between 50 and 60 mg/dL preoperatively.\nSurgical procedure: The patient underwent minimally invasive insulinoma enucleation surgery under the Da Vinci robot-assisted system with intraoperative ultrasound (IOUS) connected. The patient was put in a head-low, feet-high and left-lateral position. The robotic system was positioned at the head of the patient, while the assistant surgeon stood between the patient’s legs. Abdominal exploration via laparoscopy was conducted, and no obvious abnormalities were found. The robotic lens and operating arms were docked. The gastrocolic ligament was dissected with an ultrasonically activated scalpel. The head of the pancreas was exposed by grasping the colon downward and lifting the stomach. Towards the tail of the pancreas, we separated and exposed the spleen. The surgeon then controlled the ultrasound probe, exploring the tumor from the pancreatic tail to the head and the uncinated process with the assistance of a prograsp clamp. A quasi-circular, hypoechoic lesion was found at the end of the pancreas with a diameter of approximately 10 mm and a clear boundary. We marked the normal pancreatic tissue around the lesion with an electrotome, and while dividing the pancreas sequentially, suction was used continually to visualize the tumor capsule. Precise positioning was achieved using IOUS, and the tumor was completely resected along the capsule (Fig. -). A peritoneal drainage tube was placed. The surgery went well, lasting 65 min (skin to skin), and the volume of intraoperative bleeding was 5 mL. Intraoperative BG is documented in Table .\nAfter surgery, the patient was given liquid diet on POD2. The drain was clean and was removed on POD4, and the patient gradually resumed her normal diet. She was discharged to home on POD6. During the following 1.5 years, the patient had no recurrence of the disease. No postoperative complication occurred, such as pancreatic fistula or pancreatic function deficiency.\nPathological examination showed that the tumor was a pancreatic neuroendocrine tumor (Grade 2 with a Ki-67 index of 4%) (Fig. -). This tumor was positive for CgA, Syn, and AE1/AE3 (Fig. -). Insulin staining was partially positive (Fig. ), while gastrin, glucagon, and somatostatin staining were negative (Fig. -).\nSanger-directed sequencing for the MEN1 gene mutation was performed on a peripheral blood sample, revealing a homozygous pathogenic mutation of c247_250delCTGT (p.Ile85Serfs*33) (Fig. ). This point mutation was also detected in the frozen tissue of the patient.","This 9-year-old girl has been experiencing fainting spells, along with other symptoms, for two years. After tests, doctors discovered she has a small tumor in her pancreas that was causing her blood sugar to drop too low. Imaging showed the tumor was located in the tail of the pancreas and was actively producing insulin. She underwent successful surgery to remove the tumor, and her symptoms have completely disappeared."
556,"A 41-year-old woman presented to our hospital for further evaluation and management of persistent liver dysfunction, painless xanthochromia, and skin itching for 3 months.\nOn admission, a physical examination showed severe icteric sclera and skin without abdominal tenderness or positive shifting dullness. Liver function showed severe hepatic injury and obstructive jaundice (Table ). US showed a 5.0 × 5.3 cm, well-circumscribed anechoic lesion with many separations, increased through-transmission of sound, and no evidence of mural nodularity. Epigastric enhanced CT showed a cystic mass with sharply defined margins and smooth, thin, non-reinforced walls in the left medial liver lobe, and obstruction of the biliary tract. The contents of the mass were homogeneous, with water-density lesions, suggesting that it was a cyst. MRI showed a homogeneously hypointense lesion on T1-weighted imaging and this was homogeneously hyperintense on T2-weighted imaging. US, CT, and magnetic resonance cholangiopancreatography (MRCP) showed a slightly ectatic right hepatic duct, a greatly ectatic left hepatic duct and common bile duct, an enlarged gallbladder, and a normal distal end of the choledochus (Fig. ). Possible reasons for biliary obstruction were bile duct tumor, common bile duct stones, or compression of the common bile duct by a hepatic cyst.\nEndoscopic retrograde cholangiography (ERC) was performed to define the cause of obstruction. This procedure showed that the right hepatic duct was ectatic, but the left hepatic duct and common bile duct were not observed. There were no stone, but a large lump was observed in the common bile duct, which suggested suspicious bile duct tumor. Endoscopic sphincterotomy, endoscopic retrograde biliary drainage(ERBD), and endoscopic nasobiliary draingage(ENBD) were successfully performed to drain bile for severe hepatic injury and jaundice, rather than laparotomy, because of coagulation disorders (Fig. ). After these procedures, the icteric sclera and skin gradually faded, liver function was obviously improved, and coagulation disorders were close to normal levels (Table ).\nFour weeks later, an open operation was performed for suspicion of bile duct tumor. In exploration of the common bile duct by a choledochoscope, a large pedunculated lump protruding into the common bile duct from the left hepatic duct was discovered. This lump was completely removed from the root of the lump by endoscopic needle-knife electrotomy without active hemorrhage (Fig. ). The morphology of the lump was similar to the gallbladder with a capsule wall and capsular space (Fig. ). The choledochoscope was inserted into the hepatic duct from the stump of the lump and there was no viscous liquid secreted by cystadenoma or cystadenocarcinoma. Postoperative pathology of the lump suggested a hepatic cyst wall without heterocysts or tumor cells (Fig. ). A T tube was retained in the common bile duct in order to prevent bile leakage and observe the recovery of the root incision of the lump and postoperative bleeding.\nTwo months later, all of the liver function indices recovered to the normal range (Table ). Epigastric US and CT showed a normal hepatic duct and the hepatic cyst was the same size compared with preoperatively. A choledochoscopic examination was performed through a T-tube fistula. There was no evidence of bile duct tumor. The choledochoscope could enter into the cyst from the defect of the bile duct wall, and the mucosa around the incision recovered well, with no signs of stricture.\nThree years after the procedure, the patient was in a good general condition, without signs of cholestasis or bile duct stones. The cyst had barely changed (Fig. ).","A 41-year-old woman was admitted to the hospital after experiencing persistent yellowing of her skin and eyes, along with itching, for three months. Tests revealed a large cyst in her liver was blocking her bile ducts, causing jaundice and liver damage. Doctors successfully drained the bile from her system using endoscopic procedures and removed the cyst. Following the procedure, her liver function improved, and she was able to return to a good general condition."
557,"Prior to this admission, a 27-year-old woman sought medical attention at a local hospital because of facial redness and edema caused by eating a mango. She was treated with dexamethasone (5 mg intravenously daily) for five days. The patient gradually developed a round face, acne, hirsutism, hypokalemia, and 5 kg of weight loss over the course of one month since receiving dexamethasone. Upon hospitalization, the patient presented with a one-month history of facial edema, weight loss, and acne. She had no family history of Cushing’s syndrome, pheochromocytoma, or multiple endocrine neoplasia type 2. A 5.5-cm mass located in the right posterior lobe of the liver was detected by ultrasound in a routine physical examination 16 months ago at a local hospital; however, the patient had no symptom and did not seek further medical attention at that time.\nThe patient’s blood pressure was 120/75 mmHg in both arms in the supine position, with a regular pulse of 76 bpm. Her height was 164 cm and her weight was 48 kg (body mass index:17.8 kg/m2). The patient had “moon face” and severe facial edema, beard, central deposition of fat with slim extremities and atrophic muscles, and no pretibial edema. The skin was diffused with acne and both armpits had hyperpigmentaion.\nLaboratory tests revealed marked hypokalemia (2.1 mmol/L; normal range, 3.5–5.5 mmol/L), which could not be normalized with oral and intravenous potassium supplementation until spironolactone was added. A 75-g oral glucose tolerance test confirmed diabetes mellitus with a fasting blood glucose level of 9.19 mmol/l and a 2-h glucose level of 21.66 mmol/L, with a HbA1c level of 6.3%. She was started on insulin aspart30 (48 U daily).\nEndocrinological investigation identified severe hypercortisolism with loss of circadian rhythm. Plasma ACTH level was elevated to 1157 pg/mL, confirming ACTH-dependent Cushing’s syndrome. Except for testosterone, the catecholamine metabolites, growth hormone, calcitonin, and prolactin levels were all within the normal range (Table ).\nMRI and contrasted MRI revealed no pituitary adenoma and EAS was considered. Abdominal contrast-enhanced CT scanning revealed that the mass lesion was located in the right adrenal gland and not in the right hepatic posterior lobe. The mass was a heterogeneous solid tumor which was mildly enhanced with some patchy nonenhancing areas (Fig. ). PET/CT showed moderate fluorodeoxyglucose (FDG) uptake in the mass (Fig. ). Both contrasted CT and PET-CT revealed bilateral adrenal hyperplasia, but the neck, thorax, and pelvis were normal.\nWe prepared to do inferior petrosal sinus sampling (IPSS) for ACTH assays, adrenal vein sampling for ACTH assays to make out the origin of ACTH. But the patient’s general condition rapidly deteriorated after admission, Adrenal tumorectomy was performed. During surgery, when the right adrenal mass was mobilized, no hypertensive crisis occurred. A 6.5-cm black mass was found arising from the medial branch of the right adrenal gland (Fig. ). Hematoxylin-eosin staining of the tumor revealed that most of the cells were chromaffin-like cells. In addition, there were multifocal oval eosinophilic cells under the tumor capsule (Fig. ). Immunohistochemical staining showed positive staining for chromogranin A (CgA) (Fig. ) and CD56 (Fig. ), with a Ki67 labeling index of approximately 16% (Fig. ) for chromaffin-like cells. No positive ACTH immunostaining was noticed (Fig. ). Positive immunostaining for CRH and Melan-A (Fig. and ) and negative immunostaining for CgA and CD56 were found in the eosinophilic cells, indicating that CRH production is indeed derived from the tumor’s peripheral cells.\nOne day after surgery, ACTH levels had decreased from 715 to 14.3 pg/ml and serum cortisol level had decreased from more than 50 to 10.4 μg/dl. One week postoperatively, blood potassium and glucose levels normalized without the need for medication. The signs and symptoms of Cushing’s syndrome gradually disappeared within two months, and the hydrocortisone supplementation (initial dosage was 60 mg daily and tapered gradually) was discontinued seven weeks postoperatively. CT revealed that the left adrenal gland was almost reduced to the normal size three months later. The patient is currently under regular follow-up and remains well nine months after surgery.","This 27-year-old woman was admitted to the hospital after experiencing facial swelling and redness following eating a mango. She was treated with a medication called dexamethasone, but unfortunately, it caused her to gain weight, develop acne, and experience other symptoms of Cushing’s syndrome, a condition where the body produces too much cortisol. Further tests revealed a tumor in her right adrenal gland, which was the source of the excess cortisol. Surgery to remove the tumor was performed, and it was found to be a rare type of adrenal cancer. After the surgery, her symptoms improved significantly, and her hormone levels returned to normal. She is now being closely monitored to ensure the tumor doesn't return."
558,"A four-year-old girl with a history of asphyxiating thoracic dysplasia (Jeune syndrome) and end-stage renal disease, with a post-renal transplant, was on immunosuppressive therapy for chronic rejection. She was admitted to our tertiary care pediatric intensive care unit (PICU) with cardiorespiratory failure. The child was intubated and started on mechanical ventilation. The child was on daily peritoneal dialysis for renal failure and on dapsone for Pneumocystis prophylaxis for the last six months. During the hospital stay, she developed multiple complications, including sepsis, electrolyte imbalance, and thrombocytopenia. She was on various medications, including fentanyl, midazolam, mycophenolate, prednisolone, pantoprazole, cefepime, and dapsone. During the third week of hospital stay, the child had diarrhea and then developed metabolic acidosis. She was also noted to have cyanosis, low oxygen saturation (82% to 87%) by pulse oximetry (peripheral capillary oxygen saturation (SPO2)), despite the escalation of inspired oxygen to 100%. A blood gas analysis consistent with metabolic acidosis (ph 7.23, oxygen partial pressure (PaO2) 125 mmHg, partial pressure of carbon dioxide in arterial blood (PaCo2) 42 mmHg, HCo3 14.6, bases excess (BE) -9). With cyanosis, low oxygen saturation by pulse oximetry (SPO2) in the setting of normal oxygen partial pressure (PaO2) methemoglobinemia was suspected. Co-oximetry was ordered, which revealed a methemoglobin level of 21.5% (normal range 0 to 2%). Laboratory workup ruled out hemolysis. Since the child had end-stage renal disease, methylene blue was not considered as a treatment option, and she was started on daily ascorbic acid 500 mg via a gastrostomy tube. Dapsone was discontinued and pentamidine nebulization monthly started for Pneumocystis prophylaxis. Serial methemoglobin levels on co-oximetry showed an improving trend. Within 48 hours, the methemoglobinemia resolved and the level came down to 0.4 mg/dl. Ascorbic acid was continued for four more days and discontinued. Off ascorbic acid, the methemoglobin level remained less than 0.3 mg/dl. After a meticulous review of the medications and workup, we concluded that dapsone was the probable cause of methemoglobinemia in this child.","A four-year-old girl with a complex medical history, including a kidney transplant and a condition affecting her lungs, was admitted to the hospital with breathing problems and low oxygen levels. She was treated with medications to help her breathe and manage her kidney function, but she also developed a serious condition called methemoglobinemia, which made it difficult for her blood to carry oxygen. After careful investigation, doctors discovered that a medication she was taking, dapsone, was the cause of the problem. Thankfully, the methemoglobinemia resolved with treatment, and she was able to go home after a few days."
559,"A 15-year-old girl presented to the emergency department with a history of progressive lower limbs weakness for 10 days, inability to pass urine and intermittent grade 6/10 lower abdominal pain for two days. She had no associated symptoms like fever, rash, headache, backache or blurring of vision. No antecedent respiratory tract infection or diarrheal illness. Past medical history was insignificant. About 24 days prior to these symptoms, she received neural tissue sheep brain anti-rabies vaccine following a dog bite. On physical examination, the patient was not in acute distress. She was afebrile and her vitals were: a) Blood pressure: 130/80 mm Hg; b) Respiratory rate: 18 breaths/minute; c) Heart rate: 102 bpm.\nNeurological examination revealed no facial asymmetry and intact cranial nerves. Motor system examination of the lower limbs revealed following: a) Power of the knee: grade ⅕ below the knees; b) Power of the hips: grade ⅕ of the hip flexors and extensors bilaterally; c) Tone and reflexes of the legs: hypotonia of legs bilaterally with loss of knee and ankle reflexes bilaterally; d) Joint position and vibration sense: reduced joint position and vibration was noticed, and there was hyperesthesia of the soles bilaterally.\nUpper limbs were normal on physical examination. The cardiorespiratory system was unremarkable on examination. On abdominal examination, urinary bladder was palpable above the symphysis pubis and was tender on palpation.\nLaboratory investigations revealed hemoglobin of 12.5 g/dl with the hematocrit of 44%; TLC was 6500/cmm and ESR at the first hour using Westergren method was 23 mm. Cerebrospinal fluid examination (CSF) revealed clear fluid, normal opening pressure, glucose 75 mg/dl (normal range: 45–100 mg/dl), proteins 2.5 g/l (normal range: 0.18–0.45 g/dl) and WBCs 2/cmm (albuminocytologic dissociation).\nBased on physical examination and CSF findings a provisional diagnosis of Guillain–Barre syndrome was made. Electrophysiological study (confirmatory test) revealed demyelinating polyneuropathy consistent with Guillain–Barre syndrome. Plasmapheresis and physical therapy sessions resulted in a nearly complete recovery in six to eight months.","A 15-year-old girl went to the hospital because she had been feeling weak in her legs for ten days and experiencing pain in her lower abdomen. She had recently received a rabies vaccine after being bitten by a dog, which may be related to her symptoms. Tests showed she had a nerve problem called Guillain-Barre syndrome, and her spinal fluid confirmed this diagnosis. With treatment, including plasmapheresis and physical therapy, she made a nearly full recovery over several months."
560,"A 68-year old gentleman presented with an isolated right axillary mass in October 2014. An axillary biopsy revealed poorly-differentiated (Grade 3, Ki67 85%) neuroendocrine carcinoma of unknown origin (positive for CD56, chromagranin and synaptophysin; negative for TTF1, CDX2 and Merkel Cell Polyomavirus). Positron emission technology/computed tomography demonstrated a right axillary mass and no other identifiable sites of disease. The patient received six cycles of carboplatin and etoposide chemotherapy between Oct 2014 and April 2015 with RECIST partial response after three cycles but with evidence of tumor growth after cycle 6. He was referred to the Experimental Cancer Medicine Team and consented to TARGET in June 2015. In parallel he was referred for surgical resection of the isolated axillary mass which achieved a complete resection and permitted access to fresh tissue for PDX and translational research in July 2015.\nFollowing a brief disease-free period, the patient relapsed with metastatic nodules within the retroperitoneum in December 2015. The patient was treated with capecitabine and temozolamide between March and October 2016 with best response of progressive disease. Treatment was switched to interferon and sandostatin and his disease has remained stable by RECIST 1.1 criteria on this combination at the time of writing.","A 68-year-old man was diagnosed with a concerning, aggressive type of cancer in his arm in After initial chemotherapy, the cancer spread, and he underwent surgery to remove the original mass. Despite this, the cancer returned, and he has been receiving a combination of medications to keep it under control. Currently, the cancer is stable, and he is being closely monitored."
561,"A 40-year-old Caucasian male, with a prior diagnosis of PAN, presented in January 2016 with large volume hematemesis from esophageal varices related to portal hypertension. Throughout his childhood he had chronic arthralgia and swelling of his ankles diagnosed as juvenile idiopathic arthritis. By age 4, he manifested developmental delays, including difficulty with ambulation. At the age of 20, he developed recurrent ischemic strokes causing persistent dysarthria, ataxia, and weakness. During that time, he developed recurrent violaceous subcutaneous lesions that would ulcerate, but were responsive to glucocorticoids. He was diagnosed with PAN based on skin biopsies. He required a long-term maintenance dose of prednisone 7.5 mg daily, as reduction would led to recurrent skin lesions. In childhood, he developed poorly explained visual loss without evidence of inflammatory eye disease on physical exam. His visual acuity had been stable for the last couple years prior to presentation. His history was also remarkable for recurrent mouth and genital ulcers, which were also responsive to glucocorticoids. He had lymphopenia (absolute lymphocytes count 400/μl [1000-4800], CD3 422/μl [600-2990], CD4 370/μl [440-2160], CD8 49/μl [120-1320], CD19 11/μl [100-700]), yet normal lymphocyte proliferation studies to mitogens. Additionally, he had hypogammaglobulinemia (IgG 383 mg/dl [762-1488], IgA 46 [70-390], IgM <20 [38-328]), but had protective antibody levels to both tetanus and pneumococcus. There was no history of recurrent infections.","This 40-year-old man has a complex medical history, including a previous problem with bleeding from his esophagus due to high blood pressure and several strokes. He also has a history of joint pain, developmental delays, and skin problems, including ulcers and unusual skin lesions that respond to steroid medication. He was diagnosed with a rare condition called PAN, and he takes daily medication to manage it. Despite having a weakened immune system, he has not experienced frequent infections."
562,"A 43-year-old Caucasian male, brother to Patient 1, had a similar presentation. At 5 years of age, he was diagnosed with juvenile idiopathic arthritis after presenting with polyarthritis. At the age of 8, he was diagnosed with stage IV Hodgkin’s lymphoma (records were not available). He was initially treated with radiation therapy alone, but later transitioned to chemotherapy [initially with MOPP (mechlorethamine, vincristine, procarbazine, and prednisone) and later converted to ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine)] with remission achieved 1 year later. At age 24, he developed sudden onset left-sided persistent numbness followed by bilateral sensorineural hearing loss with near complete left-sided deafness. At age 26, he presented with bright red blood in his stool, which required resection of 6 inches of small intestines for reasons that were unclear. By age 29, he developed heart failure and pulmonary hypertension presumed secondary to ABVD treatment. His history was also significant for superior vena cava occlusion, presumed secondary to a port catheter, and esophageal varices. Immunologic evaluation revealed normal immunoglobulins, lymphocyte proliferation to mitogens, and lymphocyte subsets except for a slightly low CD8 count (83/μl). He had protective antibody levels to tetanus.\nBased on these presentations, there was a high suspicion for DADA2. Written informed consent was obtained for each patient, as well as their parents for genetic testing, and for the publication of this case report. ADA2 exon sequencing was performed on both patients and their unaffected parents as previously described (). Adenosine deaminase 2 levels were tested only in Patient 2 with comparisons made to DADA2 patients, adult carriers for ADA2 mutations and adult healthy controls.","This patient’s brother had a similar set of health problems, including arthritis, lymphoma, and hearing loss. He underwent chemotherapy for his lymphoma and later developed heart failure and other complications. He also had issues with bleeding in his intestines and problems with his blood vessels. Because of these shared issues, doctors suspect that both patients may have a genetic condition called DADA2, and they are planning genetic testing to confirm the diagnosis."
563,"A 35-year-old female was admitted to our hospital complaining of an intermittent headache for 5 years, menopause for more than 2 months, and blurred vision and lactation for more than 1 month. Five years prior, the patient began suffering from intermittent and gradually deteriorating occipital and frontal headaches. One month prior to admission, she had impaired vision and an absent right temporal view and simultaneous bilateral galactorrhea, without purulent or bloody secretions. Since the onset of symptoms, the patient experienced no nausea or vomiting, sleepiness, chills, hair loss, or fatigue. Computed tomography (CT) showed an irregular cystic solid mass in the suprasellar area. The size of the mass was 1.4*1 cm and could be markedly enhanced. The CT value of the solid part of the lesion on plain CT was 40 Hounsfield units (HU); in the enhancement, the value was 74 HU, and the cystic part showed no enhancement. The boundary of the lesion was less clear, the density was not uniform, and no obvious calcification was observed. The cavernous sinus was involved. There were no abnormalities in the ventricular system, sulcus, brain split and pool. Moreover, there was no shift in the midline structure, and no abnormalities were found in the skull structure. The sinuses exhibited no obvious abnormalities. Magnetic resonance imaging (MRI) of the brain and pituitary showed an irregular cystic solid mass in the suprasellar area, involving in the pituitary stalk, and the boundary between the lesion and pituitary was unclear. In addition, the mass pressed on the optic chiasm, and the solid part of the lesion was significantly enhanced, while the cystic part showed no enhancement. There was no abnormal signal in the brain or paranasal sinuses nor signs of calcification (Figure ). Auxiliary examinations revealed that the patient's urine volume and specific gravity were within the normal range (urine volume: 1,800 mL/day; urine specific gravity: 1.015). A pituitary function evaluation showed hyperprolactinemia (45.47 μg/L, normal range for female: 2.8–29.2 U/L) and intact growth hormone (GH) and gonadotropin function. The insulin-like growth factor 1 (IGF-1) level was 120 ng/ml (109–284 ng/ml). The pituitary-thyroid and pituitary-adrenal axis were intact (Tables –). The blood sedimentation and CRP level were normal, and indicators related to tuberculosis and other infections were negative. Because of the thickening of the pituitary stalk, CSF analysis was performed to identify the nature of the mass lesion before any invasive procedures were performed. The results of CSF analysis were normal except for a significantly increased human HCG (18.54 U/L, normal range: 0–5 U/L) level. However, the serum HCG and α-fetoprotein (AFP) levels were normal. A biopsy was recommended to clarify the diagnosis, but the patient declined. Considering the possibility of a germinoma, radiotherapy was recommended. No regression of the intracranial mass and no resolution of the headache were observed after 10 radiotherapy treatments (total 10 Gy). Therefore, the patient was admitted a second time. Apart from a deficiency in GH and hypogonadotropic hormones, the other axes were intact (Tables –). The IGF-1 level was 89 ng/ml (109–284 ng/ml). A repeated CSF analysis indicated there was no obvious decrease in the HCG level in the CSF (17.41 U/L, normal range: 0–5 U/L). An MRI showed no signs of shrinkage of the mass (Figure ). Due to the enlarged intracranial mass, the patient was transferred to the neurosurgical department for total resection of the mass. The pathological analysis was indicative of a craniopharyngioma (Figure ).\nUnder a microscope, the lateral fissure was separated at the saddle area, and CSF was released. The brain tissue collapsed, and a portion of the temporal lobe was removed. The tumor was located in the suprasellar area. The tumor was removed from the second gap along the longitudinal axis of the pituitary stalk and was frozen for analysis. The tumor and hypothalamus were closely related. Hemostasis was achieved, and the gauze inventory was correct. The surgery was complete following strict closure of the dura mater, bone flap reduction and fixation, suturing of the temporalis muscle and scalp, and placement of an external dura mater drain. The tumor specimens were sent for pathological analysis.\nAfter surgery, the patient did well and had no complaints of headache, vomiting, polyuria, polydipsia, sleepiness, or anorexia. She did not receive any hormone replacement. One year later, she was admitted to our endocrinology department for re-evaluation. Her urine volume and specific gravity were within the normal range (urine volume: 1,500 mL/day; urine specific gravity: 1.017). The urine osmotic pressure was 752 mOsm/L (0–1,000 mOsm/L), the blood osmolality was 292 mOsm/L, and a pituitary stimulation test revealed normal frontal lobe function except for an impaired GH level (Tables , ). The IGF-1 level was 92 ng/ml. HCG levels in the serum and CSF were within normal limits (serum HCG: 0.1 U/L; CSF HCG: 0.31 U/L). The slightly increased prolactin detected preoperatively had decreased to within the normal range. Cranial and pituitary MRI showed no expansion of the sellar region, no signs of a local tumor, no abnormal enhancement, a clear boundary of the optic chiasm, and no signs of tumor recurrence (Figure ).","This 35-year-old woman has been dealing with persistent headaches for five years, along with changes in her vision and unusual milk production. Recent tests revealed a small, growing mass in the area of her brain that’s affecting her pituitary gland, leading to elevated levels of prolactin and a decrease in growth hormone. Further investigation, including a brain biopsy, confirmed that the mass is a craniopharyngioma, a type of tumor. After surgery to remove the tumor, she is now recovering well and her headaches and other symptoms have improved."
564,"A 54-year-old male businessman presented to our clinic with progressive shortness of breath for the last three months. He also complained of intermittent cough with yellowish sputum and dyspnoea upon exertion for half a year. He had a history of well-controlled hypertension and smoking one pack of cigarettes per day. Family history was remarkable for adenocarcinoma of the lung. Travel history was remarkable for travel to Yunnan, China, in the past year.\nHe first visited a pulmonologist in a local clinic a month prior to admission, where chest X-ray demonstrated increased infiltration over bilateral lung fields. Oral azithromycin was administered, but his dyspnoea progressed. He then visited our clinic for a second opinion. Upon examination, he denied fever, joint pain, dry eyes or mouth, muscle weakness, pitting oedema over lower legs, and skin rash. Physical examination was unremarkable, except chest auscultation demonstrating fine crackles over bilateral lung fields. Chest X-ray showed diffuse interstitial infiltration bilaterally (Fig. ). He was admitted for further workup and monitoring of his respiratory performance.\nDuring hospitalization, the patient had no fever, and respiratory pattern was smooth. Peripheral capillary oxygen saturation (SpO2) was 95% under ambient air. Serum white blood cell count showed no leucocytosis or left shift. Autoimmune markers, including rheumatoid factor, antinuclear antibody, anti-Smith antibody, anti-ribonucleoprotein antibody, anti-SSA, anti-SSB, and anti-Scl-70, were all negative. Polymerase chain reaction of Pneumocystis jiroveci from induced sputum was negative. One of three sets of sputum culture demonstrated non-tuberculous mycobacteria, which was later attributed to environmental contamination given his symptoms and image findings. Chest CT disclosed extensive patchy ground-glass opacities superimposed with thickened interlobular septa and intralobular lines over both lung fields, demonstrating a “crazy paving” pattern (Fig.). Pulmonary function test showed normal spirometry and moderately reduced diffusing capacity of the lungs for carbon monoxide (DLCO) on single breath diffusing capacity test (5.42 mmol/min/kPa, correlated with 57.7% of predicted value).\nBronchoscopy indicated no endobronchial lesions. A radial probe endobronchial ultrasound (EBUS) showed a “blizzard sign”, suggesting ground-glass opacity over the superior segment of the right lower lobe (Fig.) . Brush cytology, bronchoalveolar lavage (BAL), and biopsy were performed. Brush cytology was negative for malignant cells.\nMacroscopically, the BAL specimen was clear in appearance. Microbiological workup of BAL was negative for presence of bacteria, tubercolosis (TB), non-tuberculous mycobacteria (NTM), fungus, and Pneumocystis jiroveci. We performed both a forceps biopsy and a cryobiopsy on the larger-sized lesions.\nPathological report of the four 0.2 × 0.2 × 0.1 cm tissue fragments obtained by forceps biopsy indicated only chronic inflammation.\nOn the other hand, the four 0.5 × 0.5 × 0.2 cm tissue fragments acquired by cryobiopsy revealed periodic acid-Schiff (PAS)-positive granular proteinaceous exudate with mild interstitial lymphocytic infiltration filling the alveolar spaces consistent with PAP (Fig.).\nWe discussed the treatment options with the patient, and whole lung lavage was advised. The patient declined our suggested treatment and opted for close surveillance. He was discharged with outpatient follow up. Later, his shortness of breath spontaneously resolved, and serial chest X-rays showed resolution of the interstitial infiltration of the lungs.","This 54-year-old man has been struggling with shortness of breath and a cough for several months, and his symptoms have been getting worse. He has a history of high blood pressure and smoking, and his family has a history of lung cancer. Tests revealed unusual changes in his lungs, including patchy inflammation and reduced lung function. After further investigation, including biopsies, the doctors found evidence of protein accumulation in the lungs, which is a condition called PAP. Fortunately, his symptoms have improved on their own, and he will continue to be monitored."
565,"A 5-year-old male initially came to the attention of the immunology service at 2 years of age with a history of recurrent sinopulmonary infections and a family history of XLP1 (nonsense mutation c. 191G>A in the SH2D1A gene). Of note, his uncle with XLP1 had a history of EBV-related central nervous system (CNS) lymphoma. SAP expression was found to be absent in NK and CD8 cells, confirming the diagnosis of XLP1. NKT cells were undetectable. Profound hypogammaglobulinemia was also noted. He was monitored and maintained on monthly intravenous immunoglobulin (IVIG) infusions with only minor breakthrough sinopulmonary infections. Adherence to IVIG was suboptimal due to social reasons. The family deferred stem cell transplantation. At the age of 5 years, he presented with acute behavioral changes, manifesting as uncontrolled aggression, requiring inpatient psychiatric treatment. Basic labs including complete blood count and comprehensive metabolic panel were normal. Specifically, no cytopenias, no transaminitis, and normal inflammatory markers including C-reactive protein (CRP) and platelet count were noted. MRI of the brain showed multiple small nonenhancing foci on T2 FLAIR sequence, mostly in the frontal lobes and scattered throughout the subcortical white matter (). The lesions remained unchanged on repeat MRI at 2 weeks and again at 4 months following presentation, suggesting that these lesions may be consistent with glial scars without acute inflammation of the brain. PCR revealed presence of EBV in the blood (2300 copies/ml). Cerebrospinal fluid (CSF) also demonstrated EBV (73 copies/ml). CSF was otherwise normal apart from only slightly elevated CSF WBC (8 cells/mm3), of which 79% were lymphocytes and 21% monocytes. Intravenous gamma globulin 1 g/kg and intravenous ganciclovir 10 mg/kg/day were started immediately. However, despite 14 days of ganciclovir treatment, EBV counts remained significantly elevated. Rituximab was administered on days 16 and 23 of admission. Prednisolone 1 mg/kg/day was given daily on days 16 through 23, and IVIG 1 g/kg was repeated on day 23. Valganciclovir 45 mg/kg/day was continued for 4 months after ganciclovir treatment. Prior to rituximab infusion, EBV counts had reached 2300 copies of EBV genome/mL of blood. Five days after the second rituximab infusion CD19+ and CD20+ lymphocytes and EBV PCR were found to be absent in the blood and remained absent for 5 months, at which time hematopoietic stem cell transplant (HSCT) was performed ( and ). IgM to EBV was undetectable at the time of presentation, and the patient never seroconverted.","This 5-year-old boy has a rare genetic condition called XLP1 that makes him more likely to get infections. He’s been receiving regular IVIG treatments to help protect him from illness. Recently, he started showing concerning behavioral changes and developed an EBV infection in his brain, which was confirmed by tests. Doctors started him on medications and a stem cell transplant to fight the infection and address the changes in his behavior, and thankfully, the EBV infection was successfully treated."
566,"A 12-year-old girl (height, 144 cm; weight, 40 kg) was presented to the hospital with an episode of seizure with impaired consciousness. Her BG at that time was 60 mg/dL (normal value of casual BG: 70–200 mg/dL). Glucose was administered and she recovered consciousness. She had no significant comorbidities prior to hospital admission. From her family history and further investigation, she was diagnosed as having insulinoma and hyperparathyroidism in MEN-1. Arterial phase images of computed tomography showed a vascularity-rich tumor of 20 mm in diameter located in the head of the pancreas. The feeding artery of the tumor was not clearly demonstrated by angiography and selective arterial calcium injection. We considered that extended surgical procedures such as pancreatoduodenectomy might be required depending on intraoperative findings; therefore, open abdominal surgery rather than laparoscopic surgery was scheduled. Her intact parathyroid hormone level was 66.0 pg/ml (normal value: 10–65 pg/mL) and her adjusted serum calcium level was 10.3 mg/dL (normal value: 8.7–9.9 mg/dL), although parathyroid ultrasound examination revealed no parathyroid tumor. Other tumors complicated with MEN-1 were not detected. The results of other preoperative examinations were unremarkable. On the day before surgery, an Enlite™ sensor of MiniMed™ 620G (Medtronic Diabetes, Northridge, CA, USA) SCGM system was inserted into her upper arm. Although MiniMed 620G was combined with an insulin pump, we did not use the pump. The SCGM system was calibrated as recommended by the manufacturer with capillary BG measured by OneTouch® UltraVue™ Blood Glucose Meter (Johnson & Johnson, New Brunswick, NJ, USA).\nNo premedication was given and she walked into the operating room. Capillary BG was 80 mg/dL and the SCGM system was calibrated. Continuous glucose infusion was started at 4.6 g/hr, and general anesthesia was induced with 80 mg of propofol, 50 mcg of fentanyl, and 0.02 mcg/kg/min of remifentanil. Muscle relaxation was obtained by administration of 30 mg of rocuronium, and the trachea was intubated. After induction of general anesthesia, an epidural catheter was inserted at the eighth and ninth thoracic interspace. Anesthesia was maintained with 1.7% sevoflurane, 0.01–0.02 mcg/kg/min of remifentanil, and intermittent thoracic epidural administration of 0.25% levobupivacaine (3 ml). An arterial catheter was inserted, and arterial BG measured by an ABL800 Flex Blood Gas Analyzer (Radiometer, Brea, CA, USA) was 71 mg/dL, which was compatible with IG of 73 mg/dL. An additional 2 g of glucose was given intravenously before the start of surgery, and IG increased from 77 mg/dL to 101 mg/dL at 20 minutes (). After that, IG mildly decreased and returned to almost the same level after 1 hour. The surgeon informed us that the tumor would be removed by enucleation shortly. Just before tumor resection, arterial BG was 76 mg/dL and IG was 80 mg/dL. At 20 minutes after tumor resection, IG showed a rapid increase. We therefore decreased the continuous glucose infusion rate to the usual dose of 1 g/hr, and IG gradually decreased and was stabilized at about 140 mg/dL. During surgery, blood pressure was between 90/50 and 120/70 mmHg, and heart rate was between 60 and 90 bpm. She was extubated in the operating room and transferred to the general surgery ward. The operation time was 2 h and 24 min, and the anesthesia time was 3 h and 53 min. Postoperative pain was controlled well by continuous epidural analgesia (12 mcg of fentanyl and 0.2% levobupivacaine at 4 ml/hr) and administration of 600 mg of acetaminophen (every 6 hr). Her postoperative course was uneventful and she was discharged on POD 10.","This 12-year-old girl was admitted to the hospital after experiencing a seizure and low blood sugar. She was diagnosed with insulinoma and hyperparathyroidism, both related to a genetic condition called MEN-Doctors found a small tumor in her pancreas and planned to remove it surgically. During the surgery, they carefully monitored her blood sugar levels and provided support to ensure a smooth and successful procedure, and she was discharged home a week later."
567,"An 83-year-old woman was referred to our department for further examination of a left renal tumor 20 mm in diameter in her horseshoe kidney. She had no remarkable medical history except for uterine cancer at 49 years of age. The laboratory data showed slight anemia and a low liver function (Hb 12.3 g/dL, AST 52 IU/L, ALT44IU/L, and LDH 201 IU/L).\nContrast-enhanced computed tomography (CT) showed a renal tumor covered with a capsule and buried by nearly 30%. The tumor was supplied by four arteries toward the left kidney (). We set the laparoscopic ports as shown in . We first approached intraperitoneally and then cut the peritoneal and approached to the renal helium. We encountered a thin artery and cut it after confirming the supplied area by clamping. We then clamped the main artery and cut the tumor with a 1 mm surgical margin. No urinary tract leakage was observed. After coagulation using bipolar forceps, we sutured using 1-0 Vicryl (Ethicon, Cincinnati, OH, USA) then sprayed with Arista AH (BARD; Warwick, RI, USA). We then fixed the thread as shown in . After confirming the lack of active bleeding, we placed a drainage tube and closed the incision. The total operation time was 2 h 39 min, with 11 min of clamping.\nA histopathological examination revealed clear cell carcinoma (grade 2, INFα, v[−], pT1a). The patient was discharged 11 days after surgery and has been free from recurrence for 43 months.","An 83-year-old woman was seen for a small tumor found in her kidney. She has a history of uterine cancer but otherwise is generally healthy. Tests showed slightly low blood counts and some liver function changes. Doctors removed the tumor laparoscopically, carefully cutting off the blood supply and ensuring no leaks. The tumor was confirmed to be a type of cancer, but the patient is doing well and has been cancer-free for over four years."
568,"A 50-year-old man underwent live laparoscopic donor nephrectomy (LLDN), through transperitoneal approach, on his left kidney as a donor for his sister. This procedure went uneventful and the patient was discharged 3 days after surgery. However, within one week after the procedure the patient experienced pain at the left side of the scrotum and left upper leg. During physical examination, a hypersensitive scrotum (allodynia) was noted whereas no other abnormalities were seen. The urologist was consulted but no urologic complications were found. The neuropathic pain area befitted the genitofemoral nerve (GFN). Conservative treatment was initiated with neuroleptic drugs and blocks of the L1/L2 dorsal roots with local anaesthetics and pulsed radiofrequency lesioning. These treatments did not result in significant pain relief and he could not work. Three years after the start of the neuropathic pain, operative treatment aiming at pain relief was initiated. In a multidisciplinary meeting it was concluded that a nerve entrapment of the GFN was suspected to be the most likely cause of the patient's neuropathic pain. Eventually, an exploratory transperitoneal laparoscopy was performed. After extensive adhesiolysis, a hemoclip used for closure of the ureter at the time of nephrectomy was identified in close relation to the GFN and removed. Due to the local scarring it could not be surgically assessed to which extent the GFN was actually damaged or a neuroma was formed. It seemed, however, highly likely that the pain originated from the GFN in this area. We therefore cut the GFN proximally in a normal looking segment (). Pathological analysis of the tissue confirmed this to contain nerve tissue. Within 2 weeks following surgery, the patient was completely pain-free and could return to his normal activities.","This 50-year-old man donated one of his kidneys to his sister. After the surgery, he developed persistent pain in his scrotum and upper leg. Doctors discovered that the pain was likely caused by a nerve being trapped near the kidney area. By carefully removing the nerve and scar tissue, the patient was able to completely eliminate his pain and return to his normal life."
569,"A 32-year-old Caucasian male with a known history of depression presented to the emergency department accompanied by police after a violent outburst following prolonged DFE abuse during a suicide attempt. On admission, he was emotionally labile and had contusions on his left shoulder and upper extremities due to a physical altercation with police. He was initially confused and verbally abusive, but within six hours he was oriented, cooperative, and able to provide a reliable history.\nThe patient stated that he had huffed keyboard cleaner several days per week for the past year; however, the frequency had increased over the last month to several times daily. Other than depression, the patient had no other known medical problems. He denied any personal or family history of kidney disease. The patient denied drinking alcohol, and he reported smoking one-half pack of cigarettes per day for the previous two years. He denied any other substance abuse except for DFE. He reported no use of nonsteroidal anti-inflammatory (NSAID) medications. During this episode of difluoroethane abuse, he had no loss of consciousness, but experienced frightening visual and auditory hallucinations as well as anxiety that persisted even after returning to his self-reported baseline mental status.\nInitial labs revealed a WBC count of 21,000 with polymorphonuclear predominance of 81%, an elevated creatinine of 1.5mg/dL with no known baseline, BUN of 10mg/dL, GFR of 54 mL/min/1.73 m2, lactic acidosis, creatine kinase of 350 U/L, and a carboxyhemoglobin level of 3.1%. Urinalysis was significant for 3+ proteinuria, moderate blood, 14 RBCs, 3 WBCs, and 3 hyaline casts. He had a negative urine drug screen.\nThe patient received single renally adjusted doses of Vancomycin and Piperacillin-Tazobactam, intravenous 0.9% saline, and oxygen by nasal cannula while in the emergency department prior to admission. Over the next 24 hours, he received intravenous fluids and oxygen with subsequent normalization of lactic acid, creatine kinase, and leukocyte count. His creatinine down trended to 1.3mg/dL. However, 36 hours into admission, his serum creatinine inexplicably rose to 2.3 with a rise in carboxyhemoglobin to 3.6%, while his urine output remained stable (). Repeat urinalysis showed a small amount of blood with RBC of 2, negative proteinuria, and no evidence of infection.\nHis normal saline infusion was transitioned to bicarbonate with saline, but was discontinued after the patient developed pruritus. Ultimately, he was transitioned to normal saline at 75 ml/hour. A renal ultrasound showed increased echogenicity of both kidneys consistent with medical renal disease, without change in size, atrophy, or cystic lesions (). Thus, it is likely that the patient had some degree of chronic kidney disease (CKD) prior to admission. For the next three days, he continued to receive intravenous normal saline with subsequent improvement of creatinine and normalization of his urinalysis and was discharged with a creatinine of 1.6.","This 32-year-old man was brought to the hospital after a serious incident involving a suicide attempt and a violent outburst. He had been abusing keyboard cleaner, which caused dangerous symptoms like hallucinations and lactic acidosis. Initial tests showed he had some kidney problems and a high level of toxins in his blood, but with treatment, his condition improved. He was eventually discharged with stable kidney function and a plan for continued monitoring."
570,"A 44-year-old female patient reported to the Department of Oral and Maxillofacial Surgery, and Periodontology at the Dental School of Ribeirao Preto, University of São Paulo for a placement of implants. During the initial examination, it was observed that the right upper central incisor had an unsatisfactory temporary Richmond type crown that was changed by a metal core, post, and new temporary crown. The new temporary crown was fitted to the removable partial denture that was used by the patient (). Surgical guides were made and the patient was referred for implant placement, in the region of teeth 12, 35, 36, 44, and 46.\nAfter approximately 8 months, it was verified that the implant located in the anterior region was positioned further apical and buccal () making it impossible to fabricate a successful implant crown from an esthetic point of view.\nThe implant was gradually removed using the implant removal instrument (Implant Retrieval Tool; Nobel Biocare), with antirotational movement (). Following this surgical procedure, a new implant was installed (cone morse 3.5 × 11 mm, Conexão, Brazil), with a torque of 30 N, and positioned in the mesiodistal direction. Specifically, the implant was placed closer to tooth 14 in the buccal-palatine direction on the bone ridge (located around 1 mm for the palatal from an imaginary curvature passing through the buccal surfaces of the present teeth and 1 mm infraosseo in the coronoapical direction) ().\nAfter the surgery, the removable partial denture was removed. This was followed by the fabrication of a temporary fixed partial prosthesis with retention used during the osseointegration phase on teeth 11 and 14 (): this was only for esthetic purpose. This was in addition to the installation of prostheses on the implants in the regions of teeth 35, 36, 44, and 46.\nImmediately after the second stage surgery, the temporary fixed partial denture was performed using the healing cap as an abutment and a lateral incisor as a mesial cantilever. Following the rehabilitation procedures, this temporary fixed partial denture was changed to a provisional fixed prosthesis with the mesial cantilever screwed into the implant. During this phase, periodic acrylic resin implements were added to the temporary prosthesis to promote gingival conditioning (Figures and ) and black triangle closure (Figures and ) and also to improve gingival esthetics.\nAt the end of two months, the implant prosthesis with a mesial cantilever was installed (). This case report was approved by the institution's Ethics Committee on Human Research and followed the ethical principles of the Declaration of Helsinki, in addition to complying with specific legislation.","The patient came to the dentist to have implants placed in her mouth. During the initial examination, a temporary crown was replaced, and new guides were made for the implant placement. Unfortunately, the first implant was positioned incorrectly, so it was carefully removed and a new one was placed in a better location. The dentist then created temporary and permanent dentures to ensure a good fit and beautiful smile, and the patient’s mouth is now fully restored."
571,"A 26-year-old Yemeni male patient from Taiz Province was referred to the restorative dental clinics at King Saud University, Saudi Arabia. His chief complaint was an unpleasant smile caused by generalized tooth discoloration. His medical history was irrelevant. The fluoride level in the water around Taiz Province is >3.6 mg/l [].\nClinical examination revealed generalized fluorosis with loss of the outermost enamel in irregular areas involving less than half of the entire surface, as well as changes in the morphology caused by merging pits and marked attrition (). In this case, based on the Thylstrup and Fejerskov index (TFI) for dental fluorosis classification, the dental fluorosis was classified as TFI = 7 [].\nAfter the clinical examination, radiographs, preoperative photographs, and upper and lower alginate impressions for diagnostic models were taken. The patient was presented with treatment options, which included ceramic or composite veneers, along with the advantages and disadvantages of each option. The patient agreed to smile enhancement using ceramic veneers for his upper teeth given that he desired an optimum aesthetic and a long-term result. The veneers would be placed on the patient's upper teeth, from his upper right 2nd premolar to upper left 2nd premolar. The patient decided to postpone veneering his lower teeth, given his limited financial capacity. Diagnostic models were analyzed to evaluate the occlusion, and a diagnostic wax-up was made of white-colored wax. The use of the wax-up allows the patient to preview the desired appearance of his teeth, and this wax-up is also essential for the fabrication of a clear matrix for temporary restorations.\nThe desired shade was selected using the VITAPAN classical shade guide (VITA Zahnfabrik, Germany). The enamel of the eight maxillary teeth was prepared using a flat-end tapered diamond bur to a depth of 0.5–0.75 mm facial reduction with 1.5 mm incisal reduction (). A chamfer finish line was maintained at the level of the gingival margin. The proximal margin was extended into the facial and gingival embrasures.\nFollowing tooth preparation, gingival retraction was achieved using retraction cords (Ultrapak Cord #00, Ultradent Products Inc., South Jordan, UT, USA) soaked in a hemostatic agent. Impressions were taken with a polyvinylsiloxane material (Virtual, Ivoclar Vivadent, Amherst, NY). The impression material was manipulated according to the manufacturer's instructions. Temporization was performed by spot etching on the facial surface of each prepared tooth with 37% phosphoric acid (Total Etch, Ivoclar Vivadent, Schaan, Liechtenstein). Bonding agent (OptiBond Solo Plus, Kerr, Orange, CA, USA) was applied on the enamel-etched spots and light cured for 20 seconds using a high-intensity light-emitting diode (LED) curing light (Elipar S10, 3M ESPE, MN, USA). The clear matrix that was previously fabricated was loaded with a temporization material (Protemp Plus, 3M ESPE, MN, USA) and placed over the prepared teeth. Light curing was done for 10 seconds per tooth. Then, the matrix was gently teased away from the prepared teeth. A number 12 scalpel blade was used to remove the partially cured temporization material. Facial and lingual embrasures were refined with a thin diamond disk, the occlusion was adjusted, and the temporary restorations were polished using polishing discs and points ().\nCeramic veneers were fabricated with a lithium disilicate-reinforced glass ceramic material (IPS e.max Press, Ivoclar Vivadent, Schaan, Liechtenstein). Temporary veneers were removed, and the teeth were cleaned using pumice. Ceramic veneers were tried-in using a transparent shade try-in paste (Variolink Veneer try-in paste, Ivoclar Vivadent, Schaan, Liechtenstein) to assess marginal adaptation and shade.\nAfterwards, veneers were prepared for bonding. Fitting surfaces of the veneers were etched with hydrofluoric acid (Porcelain Etchant 9.5%, Bisco Inc., Schaumburg, IL, USA) for 60 seconds, washed under running water for another 60 seconds, and dried with an air syringe. A layer of silane coupling agent (Monobond Plus, Ivoclar Vivadent, Schaan, Liechtenstein) was applied on the veneers' fitting surfaces and gently air-dried after one minute. Then, the prepared teeth were etched using 37% phosphoric acid for 30 seconds, rinsed, and dried. A clear mylar strip was placed interproximally to prevent inadvertent bonding to the adjacent tooth and to facilitate the subsequent removal of excess resin cement in the embrasures. A layer of bonding agent (Adhese Universal, Ivoclar Vivadent, Schaan, Liechtenstein) was applied on the prepared tooth surfaces and air-thinned. Then, Heliobond (Ivoclar Vivadent, Schaan, Liechtenstein) was placed on the prepared tooth surfaces. The inner surface of the veneers was covered with light-cured resin cement (Variolink Veneer, transparent shade, Ivoclar Vivadent, Schaan, Liechtenstein). Veneers were positioned appropriately on the teeth by applying gentle pressure, following which excess resin cement was carefully removed with an explorer before light curing. Light curing was first performed for 2 seconds, and the excess resin cement was removed with a microbrush. After that, each veneer was light-cured from the facial aspect for 40 seconds and from the lingual aspect for 40 seconds. The two veneers of the central incisors were first simultaneously cemented. This was followed by cementation of the veneers of the two lateral incisors. Then, the veneers of the two canines were cemented. Finally, veneers for the first and second premolars were cemented simultaneously on each side.\nMinimal gingival flash of the resin luting cement was removed with a number 12 scalpel blade. A flame-shaped fine diamond bur was used to finish the ceramic margins and to contour the embrasure surfaces. Occlusion was assessed and adjusted. Flossing was performed to ensure interproximal contact patency. Ceramic polishing was performed using a series of polishing cups and points (OptraFine polishing system, Ivoclar Vivadent, Schaan, Liechtenstein). Interproximal contacts were finished with finishing and polishing strips. Final surface lustre was achieved by using a diamond polishing paste with a rubber prophylaxis cup. The postoperative clinical photographs are shown in . The patient was satisfied with the final result ().","This 26-year-old man from Yemen came to the dentist because he wasn't happy with the color of his teeth. After examining his teeth, the dentist found that he had fluorosis, a condition caused by too much fluoride in the water, which affected the enamel. To improve the appearance of his smile, the dentist planned to place ceramic veneers on his upper teeth, which are custom-made to match his natural teeth. The veneers were carefully bonded to his teeth using a special adhesive, and the dentist made sure the fit and color were perfect before finishing the procedure."
572,"A 75-year-old male visited a urological practitioner because of nocturia. An elevated serum prostate specific antigen (PSA) level of 76.2 ng/mL was observed, and digital rectal examination showed diffuse induration of the prostate. Pelvic MRI demonstrated extensive high signal of the prostate in diffusion-weighted imaging (DWI) (). Fluorodeoxyglucose-positron emission tomography/CT revealed multiple spine and pelvic bone and para-aortic and pelvic lymph node metastases (Figures and ). Based on these findings, the patient was diagnosed with metastatic prostate cancer (cT3aN1M1b) and treated without prostate needle biopsy, with primary androgen deprivation therapy (ADT), including a GnRH antagonist (degarelix) and anti-androgen agent (bicalutamide). The PSA level immediately declined and reached nadir (0.23 ng/mL) after 8 months. However, 15 months after the start of ADT (PSA level, 2.33 ng/mL), the patient was diagnosed with CRPC and referred to our hospital.\nTo evaluate the patient's current disease status, we performed CT, bone scan, and WB-MRI. We observed discrepancies between the WB-MRI, bone scan, and CT. CT showed multiple osteoblastic lesions in the spine and pelvic bone (Figures and ) and shrunken para-aortic lymph nodes. Bone scan similarly showed multiple accumulations at the same bone sites as the CT (). However, these osteoblastic lesions showed almost no high signal in DWI of WB-MRI, suggesting that the lesions did not have viable tumor cells (Figures –). In contrast, the primary lesion had a diffuse high signal remaining in DWI of WB-MRI ().\nWe then performed histopathological examinations of both the prostate and the vertebra. The prostate needle biopsy demonstrated that 10 of 12 cores had viable prostate cancer cells (). Meanwhile, we performed the CT-guided needle biopsy from the osteoblastic lesion of the second lumbar vertebra, which was diagnosed with bone metastasis by CT and bone scintigraphy, to exclude vertebral bone metastasis of prostate cancer by an orthopedic unit of another hospital, which the patient visited owing to lumbago and bilateral lower limb paralysis. Histopathological examination of the osteoblastic lesion demonstrated no malignant cells (). Considering the results of the bone needle biopsy, we comprehensively reviewed the patient's image findings. A CT scan in the second lumbar vertebra revealed an osteoblastic change (885.4 Hounsfield unit), T1- and T2-weighted imaging of MRI revealed low signal intensity, and the ADC value was low (0.498 × 10−3 mm2/s); however, b = 0 and b = 1200 of DWI exhibited no high signal intensity, suggesting a benign osteoblastic change induced by the treatment effect (Figures –). With orthopedic conservative therapy in accordance with the diagnosis of lumbar hernia, the lumbago and bilateral lower limb paralysis completely resolved.\nBased on the findings of our WB-MRI and histopathological examinations, we reasoned that viable tumor cells inducing disease progression may primarily exist in the primary lesions and not in the metastatic lesions. Then, to control the overall disease, we changed the patient's medication from bicalutamide to enzalutamide for potentially existing micrometastases and added prostate RT (74 Gy). After this, his elevated PSA immediately declined and was controlled at a level of <0.2 ng/mL.","This 75-year-old man came to the urologist because he was experiencing frequent nighttime urination. Tests revealed a high level of PSA, indicating prostate cancer that had spread to his bones and lymph nodes. He was initially treated with medication to block testosterone, which lowered his PSA. However, further tests showed that the cancer was still progressing, and he was diagnosed with castration-resistant prostate cancer. To control the cancer, his treatment was changed to include a new medication and radiation therapy, which has now successfully lowered his PSA to a very low level."
573,"A 64-year-old-man with an eighty-pack-year smoking history presented with insidious onset of worsening shortness of breath over a period of 6 months. He had previously undergone ablation for atrial fibrillation, as well as surgery and radiation for prostate cancer. Six months before presenting to our institution, he had fallen and fractured 2 right-sided ribs and developed an upper respiratory tract infection one month later. Over the next 3 months, the patient continued to experience intermittent fevers, chills, cough, wheezing, and exertional dyspnea.\nThe patient was initially treated with oral antibiotics for presumed community-acquired pneumonia; however, his symptoms persisted despite multiple courses of antibiotics and inhaled bronchodilators, and he was ultimately hospitalized. Serum serology for coccidioidomycosis was negative. Sputum cultures grew normal upper respiratory flora. His chest radiograph showed a left lower lobe (LLL) infiltrate. A computed tomogram (CT) of the chest showed a LLL consolidation, with evidence of narrowing of the LLL bronchus (). There was an abrupt cutoff in the LLL bronchus, but no endobronchial lesion was detected (). A bronchoscopy performed at the hospital showed a “rounded, nonulcerating, pink mass-like growth” obstructing the LLL bronchus. Brushings from the growth were negative for malignancy, but no biopsies were obtained. He was referred to our center for evaluation of this endobronchial lesion.\nUpon further examination, the patient recalled a significant decline in his exercise tolerance about 6 months prior to the current presentation. His pulmonary function tests showed severe airflow obstruction, with FEV1 of 1.53 L (42% predicted), which was a decline from his previous FEV1 of 2.2 L (63% predicted) 6 months earlier. The patient's physical examination revealed decreased breath sounds at the lower left lung base, with wheezing limited to the LLL. Although he had continued to smoke for the past 6 months, he had quit smoking 3 weeks prior to the current presentation. Based on his symptom complex, worsening airflow obstruction, and the mass seen on prior bronchoscopy, the likely differential diagnoses included primary lung cancer, lung carcinoid tumor, and foreign body aspiration. Although foreign body aspiration was unlikely, the development and progression of his symptoms over just 6 months would also be unusual for primary lung cancer or for lung carcinoid tumor.\nThe patient underwent another bronchoscopy, which revealed a narrow LLL bronchus, with a fleshy endobronchial mass lesion (). Multiple fine needle aspirates were obtained using a Wang needle (CONMED, Utica, NY), but every pass was positive for dense acute inflammation on rapid onsite evaluation. Multiple endobronchial biopsies were then obtained, and 2 pearly white structures with a brown detached cover were revealed (). The object was extracted in its entirety using a Zero Tip Airway Retrieval Basket (Boston Scientific, Marlborough, MA) and biopsy forceps; the object was identified as an aspirated almond. An ultrathin bronchoscope was then used to examine the airways distal to the aspirated almond, and there was no evidence of any additional foreign material. A bronchoalveolar lavage was performed at the end of the case, and specimens were sent for culture. Pathology of the extracted foreign body was consistent with vegetable matter (), and bronchoalveolar lavage cultures were negative for bacterial or fungal organisms.\nA 10-day course of amoxicillin-clavulanate was prescribed due to the imaging evidence of aspiration pneumonia secondary to almond aspiration. On follow-up examination one month later, he reported drastically increased exercise tolerance and his cough and wheezing had disappeared; meanwhile, pulmonary function tests showed an increase in his FEV1 to 2.12 L (60% predicted), and a repeat flexible bronchoscopy revealed no bronchial stricture. The patient shared that while he was recovering from his rib fractures, his favorite snack (enjoyed in the supine position) was almonds. While foreign body aspiration for 6 months could lead to irreversible parenchymal changes, a follow-up CT scan at 3 months revealed complete resolution of parenchymal infiltrates in this case (). At the time of his 6-month follow-up, his exercise tolerance had returned to baseline and he had successfully quit smoking.","This 64-year-old man had been struggling with shortness of breath for months, and doctors discovered a small mass in his lung. He has a history of smoking, atrial fibrillation, and prostate cancer, and had previously been treated for pneumonia. After several tests, they found that he had accidentally inhaled an almond, which caused the mass and his breathing problems. The almond was removed, and he started to feel much better, with his breathing returning to normal and his exercise tolerance improving."
574,"A healthy 11-year-old girl was presented to the National University of Malaysia (UKM) Paediatric Dental Clinic with a referral for further management of pulp necrosis of an immature lower right second premolar (tooth 45), secondary to the fractured tubercle of dens evaginatus. Two weeks earlier, she had treatment at a general dental clinic for pain related to tooth 45. Tooth 45 had spontaneous and lingering pain following cold and thermal stimuli. The tooth was diagnosed to have symptomatic irreversible pulpitis, and root canal therapy was initiated. The canal was accessed, and pulp extirpation performed before the placement of intracanal nonsetting calcium hydroxide by the general dental practitioner (GDP).\nAt the time of current assessment, her tooth-related symptoms had completely resolved. General oral examination showed the presence of generalised mild gingivitis with a basic periodontal examination (BPE) score of 1 in all sextants. The patient's oral hygiene was fair with a plaque score of 30%. The patient is still in her mixed dentition with the presence of the primary maxillary canines. Her upper dental arch was well aligned, and mild crowding of anterior teeth was noted in the lower arch. Tooth 45 has an occlusal glass ionomer dressing of the access cavity made for the pulp extirpation earlier by the GDP (). Cold and electric pulp sensibility testings showed positive responses to all fully erupted premolars indicative of tooth vitality expect for tooth 45. Tooth 45 also has slight tenderness to percussion. Periapical radiograph of tooth 45 showed an immature root with convergent open apex and small periapical radiolucency. The pulp space of tooth 45 is of an even width from the coronal to the apical portion (). Based on the assessments, tooth 45 was diagnosed with pulp necrosis secondary to fractured dens evaginatus and symptomatic apical periodontitis.\nOn the day of initial assessment, tooth 45 was isolated with rubber dam after infiltration of local anaesthetic solution (2% lidocaine with 1 : 80000 adrenaline). Pulp chamber was reentered through the previously prepared access cavity. The root canal was exposed and irrigated with saline. After that, the canal was dried with paper points and the tooth working length was estimated with a K-file No. 60. A working length, 2 mm short of the apical opening, was determined (17 mm). The canal was gently prepared with the K-file No. 60 and then irrigated with a copious volume of 1.5% sodium hypochlorite (NaOCI). After drying the wet canal with paper points, nonsetting calcium hydroxide was placed into the canal and the access cavity was double sealed with Cavit™ 3M, USA, and glass ionomer cement (GIC) (Riva Self Cure™ SDI, Australia).\nTwo weeks later, the tooth was reassessed for any signs and symptoms of infection. The tooth was no longer tender to percussion, and there was no indication of infection-related signs and symptoms. After isolation with a rubber dam, the root canal of tooth 45 was reaccessed and irrigated with a copious volume 1.5% NaOCl to remove the nonsetting calcium hydroxide. Then, the canal was irrigated with sterile water and dried with paper points. Subsequently, the canal was irrigated with 17% EDTA (Pulpdent™, Watertown, Massachusetts) for a minute and dried with paper points. Finally, under the guidance of a dental operating microscope (Carl Zeiss Surgical GmbH, S100), the apical region was filled using the EndoSequence (BC RRM-Fast Set Putty, Brasseler, USA) material up to 4 mm thickness to create an apical seal (). The orifice of the root canal was double sealed with a cotton pellet, temporary filling material (Cavit 3M, USA), and GIC (Riva Self Cure SDI, Australia).\nOnce again, the root canal was reaccessed two weeks later, irrigated with 1.5% NaOCl, and dried with paper points. Next, the dried canal was obturated with thermoplasticised gutta-perca using the Obtura III Max System (Obtura Spartan® Endodontics) (). After that, the access cavity was double sealed with GIC (Riva Self Cure SDI, Australia) and nanohybrid composite (AURA™ SDI, Australia), respectively.\nFollowing the obturation, tooth 45 was reviewed at three-month and six-month intervals. During both reviews, tooth 45 was asymptomatic. However, at the six-month review, a periapical radiograph of tooth 45 showed an unexpected finding. The apical root of tooth 45 continued to grow beyond the apexification level with a normal periodontal ligament space and lamina dura. No evidence of periapical radiolucency was noted (). However, regular annual monitoring of tooth 45 is essential to ensure that the coronal seal is intact and no apical complication further arises.","This 11-year-old girl came to the dentist because she had pain in a lower tooth that had been previously treated. The tooth had a damaged part, which led to an infection in the inside of the tooth. The dentist cleaned out the infected area and filled the tooth with special materials to prevent further infection. After a few weeks, the pain was gone, and the tooth was healthy again. The dentist will continue to monitor the tooth to make sure it stays healthy for the long term."
575,"The patient is a 50-year-old African American female with a history of bilateral breast reduction twelve years ago, iron deficiency anemia, and obesity, who presented to the surgeon's office complaining of tenderness of her right breast. The patient reported that recently she had been developing keloids along the scar of the right breast with some areas having a blue hue; her left breast was unremarkable. She noticed that after wearing a sports bra there was increased pressure and abrasions to the keloid, leading to cellulitis and edema. She was previously treated with two courses of antibiotics for what was presumed to be an infected keloidal scar of her right breast but with minimal improvement. On exam, she had a large 10 cm diameter keloidal region on the inferior and lateral aspect of the right breast with edema and cellulitis. The keloidal area had no palpable fluctuance; she exhibited no nipple discharge or palpable adenopathy of the right axilla ().\nThe patient had a benign-appearing mammogram 8 months prior, and all of her screening mammograms since her breast reduction have been without signs of malignancy. Another mammogram was ordered but was not performed due to patient discomfort. An ultrasound of the breast was preformed and suggested marked edema and skin thickening suggestive of infection but no definitive fluid collection or underlying suspicious mass was observed.\nThe patient underwent a right breast partial mastectomy for cosmesis and resection of the infected keloidal area. Intraoperatively, the mass was highly vascular, firm, but not fixed to the chest wall. Postoperatively, the pathology revealed a high-grade primary angiosarcoma of the breast with negative margins.\nPatient underwent a computed tomography of the chest, abdomen, and pelvis, which did not show any evidence of gross metastatic disease. The patient then underwent completion mastectomy and scheduled for adjuvant chemotherapy with combination gemcitabine and Taxotere, followed by radiation.","This 50-year-old woman came to the doctor because she had a painful, keloid-covered area on her right breast that had become infected. She had previously tried antibiotics for this area, but it didn't improve. Examination revealed a large, inflamed keloid with signs of infection, and imaging showed significant swelling. After further investigation, a rare and aggressive type of cancer, called angiosarcoma, was found in the breast, requiring a partial mastectomy and now plans for chemotherapy and radiation treatment."
576,"The 41-year-old male patient F.C.F. showed no comorbidities and denied alcoholism, smoking, and taking regular medication. He started having fever, myalgia, anorexia, vomiting, jaundice, and colure on July 14, 2016. He took antiviral drugs with monohydrate dipyrone (750 mg/day) associated to chlorpheniramine maleate (6 mg/day) and paracetamol (>4 g/day) at his peril. He denied taking any other drugs on that occasion. Because his clinical condition had not improved, he went to the hospital, where he underwent medical tests such as complete blood count (CBC): hemoglobin 13.2 g/dL, hematocrit 39.6%, platelets 343,000/mm3, white blood cells (leucocytes 5200/mm3), liver function Aspartate transaminase (AST) 39.8 U/L, Alanine transaminase (ALT) 40.1 U/L, gamma-glutamyl transferase (GGT) 346 U/L, and alkaline phosphatase 98.5 U/L. In addition, the NS1 test for dengue was negative and the abdominal ultrasound showed the liver with diffuse increase in parenchymal echogenicity, indicating mild/moderate steatosis. The patient was diagnosed with hepatitis due to the clinical signs assessed, results of laboratory tests, and excessive intake of paracetamol. The patient was hospitalized, and after 4 days of intravenous hydration and clinical assessment, he was discharged from the hospital.\nFifteen days after his discharge, the patient related that he had daily high fever, appetite loss, sweating, weight loss (8 kg), and progressive worsening. On August 6, 2016, the infectious disease specialist ordered new medical tests when examining the patient. The results revealed that blood count was hemoglobin 10.2 g/dL; hematocrit 30.7%; red blood cells 4.16 million/mm3; and platelets 200,000/mm3. It also revealed anisocytosis, microcytosis, and hypochromia; white blood cells were leucocytes 9700/mm3, banded neutrophils 6%, segmented neutrophils 76%, and lymphocytes 10%. Liver function tests indicated aspartate transaminase (AST) 64 U/L, alanine transaminase (ALT) 85 U/L, gamma-glutamyl transpeptidase (GGT) 560 U/L, and lactic dehydrogenase 248 U/L. C-reactive protein was 134.29 mg/L. Serologies were ordered for leptospirosis, rickettsialpox, hepatitis, syphilis, Zika virus, Chikungunya, Epstein-Barr virus, cytomegalovirus, toxoplasmosis, and human immunodeficiency virus (HIV). All of them had negative results.\nFurthermore, blood cultures were conventionally performed using the Bact/Alert 3D 120 automated microbial detection system. The initial incubation was performed with chocolate agar medium, in 5–10% CO2 at 35°C–37°C for 24 hours. Three blood samples were collected in the first 24 hours, at a minimum 15-minute interval between them, using venous puncture in different places. The colonies isolated on chocolate agar plates were identified by colonial morphology, gram stain, and oxidase reaction. Colonies appeared smooth, round, uniform, grey/brown, and 1 mm in diameter. The oxidase test and tributyrin agar test were positive. This result was positive for the presence of the microorganism M. osloensis in the three samples.\nTransesophageal echocardiography (TEE) was ordered, and it showed prolapsed mitral valve involving the posterior leaflet (P2 segment) with an echogenic structure adhered to the atrial face, with approximate dimensions of 0.47 × 0.85 cm, compatible with endocardial vegetation, determining failure of coaptation and a moderate degree of valvular insufficiency and eccentric jet. Intracavitary thrombi were absent, and systolic and diastolic functions of the left ventricle were normal. According to the criteria of Habib et al. [], the diagnosis of IE was confirmed, and the patient was hospitalized for intravenous antibiotic therapy.\nAntibiotic therapy was prescribed from August 8 to September 28 with the administration of vancomycin for four days, gentamicin for 10 days, and ampicillin for 6 days, and after the result of blood culture, ceftriaxone was introduced for 36 days. Remission of signs and symptoms was observed as of August 12. Laboratory tests results were better, and blood cultures were sterile. Theses exams were repeated on August 21 and on September 15. Their results showed no alteration.\nAfter completing the therapeutic treatment with specific antibiotics, the patient underwent serial laboratory tests and was hospitalized. Another TEE was carried (09/21), and it revealed chordal rupture and moderate mitral regurgitation. Surgical correction was performed.\nOn September 26, the patient underwent implantation of a mitral valve prosthesis. Surgery was performed without any complications. However, neither the culture nor the pathological anatomy of the surgically removed valve was performed. On October 06, the patient was discharged from the hospital with a prescription for oral anticoagulant warfarin.\nEighteen months after the endocarditis episode, the patient was asymptomatic, in good physical condition. He attends follow-up visits with a cardiologist at the same hospital.","Mr. F.C.F. initially experienced symptoms like fever, stomach problems, and jaundice, leading to a diagnosis of hepatitis. He was treated with medication and fluids, but his condition worsened, and further tests revealed a serious infection in his heart. Blood tests and an echocardiogram showed a damaged mitral valve and vegetation, requiring surgery to replace it. After the surgery, he was fitted with a blood thinner and is now doing well, attending regular check-ups with his cardiologist."
577,"A 50-year-old Asian male with a past medical history of supraventricular tachycardia and obstructive sleep apnea on CPAP at night presented with one month of intermittent flu-like symptoms, orthopnea, and dyspnea on exertion. At the onset of these symptoms, he presented to a walk-in clinic and was diagnosed with influenza. He was treated symptomatically and noted improvement, but one week later he had a recurrence of symptoms while playing volleyball. From that time on, he noticed dyspnea on exertion, continued malaise, fevers, and diffuse joint pains so he presented multiple times to outpatient providers. He received doxycycline without improvement, and follow-up testing showed a mild leukocytosis, negative EBV, and an unremarkable chest X-ray. He was diagnosed with lingering postviral symptoms from influenza. He ultimately presented as a walk-in patient to the cardiology clinic when he started having chest tightness, palpitations, and his dyspnea progressed to occurring at rest, relieved only with a tripod position.\nEKG on presentation () showed right axis deviation and abnormal ST-T wave segments in V1 through V3 which was new compared with a prior EKG. Due to the concern for pulmonary embolism, a CT angiogram of the chest was obtained which displayed moderate bilateral pleural effusions, a mass in the right ventricle, and a mass in the left atrium extending through the mitral valve invading into the left ventricle (). Echocardiogram exhibited normal LVEF but some mitral valve occlusion due to the mass. Cardiac MRI was obtained () and confirmed the masses. The patient required debulking of the left atrial tumor, and pathology revealed an undifferentiated, high-grade pleomorphic sarcoma. Due to tumor infiltration into the left pulmonary veins, as well as focal areas of uptake in the small bowel at a site of intussusception, he was started on pembrolizumab chemotherapy with concurrent radiation therapy to the heart and small bowel.","This 50-year-old man initially thought he had the flu, but his symptoms kept returning and worsened, leading him to seek multiple doctor visits. Tests revealed he had lingering symptoms from a previous flu infection, along with some inflammation in his blood. Further investigations showed a concerning mass in his heart and lungs, which was later determined to be a rare type of cancer. He is now receiving chemotherapy and radiation to treat the cancer and is closely monitored."
578,"A 64-year-old white male with no prior medical history presented to his primary care physician for routine follow-up. There was no history of hypertension. During work-up for elevated liver transaminases, he was found to have hepatitis C. Before initiation of Harvoni, he underwent CT imaging of the abdomen with contrast which found a 5 × 6.7 × 7 cm right adrenal mass (). On physical examination, he was afebrile with a pulse of 47 and normotensive at 118/68. His abdominal exam was nontender, nondistended, without masses, or hernias. Review of systems was negative for abdominal pain, hypertension, weakness, palpitations, headache, diaphoresis, or weight gain. He was a current smoker with a 33 pack-year history. He had no history of endocrine disease. His family history was significant only for a father with pancreatic cancer. His remaining laboratory values were within normal values including a normal potassium value. The patient was seen by the endocrine service for evaluation, and biochemical work-up revealed that the ACTH level was 9.1 pg/ml (nl 7.2–63.3); AM cortisol was normal at 10.01 mcg/dl, and 24-hour urine metanephrines was less than 50 mcg (nl).\nOn CT imaging, the right adrenal mass contained scattered calcifications with small regions of fat. It enhanced in a peripheral globular fashion with central progression. The absolute contrast washout of 22.9% was indeterminate for adrenal adenoma (). The mass was noted to abut but did not appear to invade the adjacent liver, right kidney, and inferior vena cava. There was no adenopathy or free fluid. There was no evidence of metastatic disease.\nDue to the size and atypical features of the mass, right adrenalectomy was performed. An open thoracoabdominal approach was chosen due to the patient's low lying costal margin, the size of the mass, and retrocaval extension of the mass medially towards the vertebral body. The patient recovered well postoperatively and was discharged four days after surgery. The resected specimen weighed 126 grams and measured 7.5 × 6.5 × 4.7 cm on gross pathology (). Within the specimen was a 6.4 × 5.5 × 4.7 cm intraparenchymal nodule, which on histologic examination proved to be a cavernous hemagioma (). The patient has had no evidence of recurrence for nearly 18 months.","This 64-year-old man came in for a routine check-up and was found to have hepatitis C. During testing, a growth was discovered on his right adrenal gland. After surgery to remove the growth, it was determined to be a benign cavernous hemagioma. He is doing well and has been closely monitored since the surgery."
579,"A 34-year-old Gravida 11 Para 3073 at 16 weeks and 1 day gestation presented to the emergency room of an outside hospital with a 2-day history of progressively worsening nausea, vomiting, and diarrhea, exacerbated by eating. The pregnancy had been unremarkable. Her past medical history included endometriosis and infertility. Her past surgical history was significant for two cesarean sections and left salpingo-oophorectomy secondary to an ectopic pregnancy. Physical exam elicited severe, diffuse abdominal tenderness. Fetal heart tones were taken to be in the 140s and positive fetal movement was reported. Laboratory investigations, including complete blood count, comprehensive metabolic panel, amylase, and lipase, were within normal limits. The ER physician's leading differential diagnosis was of gastrointestinal etiology. An MRI and MRCP were performed to rule out appendicitis and gallbladder disease. The MRI was notable for a large amount of intraperitoneal fluid of unknown etiology; an intrauterine fetus was visualized.\nThe patient continued to experience intractable pain, worse with movement and breathing, despite IV pain medication. At that point she has been at the outside facility for approximately 12 hours. The patient was transferred to our facility under the joint care of the Obstetrics/Gynecology and General Surgery teams. Upon arrival, the patient's hemodynamic status had deteriorated. She presented with tachycardia, dyspnea, chest pain, and worsening abdominal pain. Her hemoglobin had fallen from 11.7 g/dL to 7.9 g/dL. Transabdominal ultrasound imaging revealed a single intrauterine pregnancy that was positioned low in the uterus, with marked thinning of the anterior myometrium at the site of the pregnancy, and significant hemoperitoneum. Fetal heart tones were steady in the 140s. The MRI images were reevaluated prior to surgery (see ).\nAt this point, the patient was taken for emergency laparotomy and the staff Gynecologic Oncologist was consulted. The patient underwent a modified radical hysterectomy with right ureteral lysis and cystotomy with bladder repair. The intraoperative findings were consistent for a placenta percreta and uterine rupture with a 2 x 1 cm defect in the right lower uterine segment. There were significant intra-abdominal blood and evidence of invasion of the placenta into the posterior aspect of the bladder. Total estimated blood loss for the surgery was 3,150 mL. The patient received 900 mL of cell saver and 1 unit packed red blood cells (PRBC) intraoperatively.\nThe patient was admitted to the ICU following surgery. She was transferred out of the unit on postoperative day 1. Two more units of PRBC were transfused over the course of the postoperative period. She was discharged on postoperative day 4 after having met her postoperative milestones. Due to the cystotomy, she was discharged with Foley urinary catheter in place for a minimum of 7 days with cystogram scheduled prior to removal. Patient was referred for grief counseling.\nPathologic examination of the uterus included placenta percreta with uterine rupture (see for gross specimen). There was absence of decidua identified in the lower uterine segment in the area of the uterine rupture.","This patient is 16 weeks pregnant and experienced a sudden and severe worsening of nausea, vomiting, and diarrhea. During a visit to an outside hospital, she was found to have a large amount of fluid around her abdomen and a concerning pregnancy position. After her condition worsened, she was transferred to our hospital for emergency surgery, where doctors discovered a placenta percreta – a rare complication where the placenta grows too deeply into the uterine wall. The patient underwent a hysterectomy to remove the placenta and stop the bleeding, and she is now recovering at home with a catheter and support."
580,"The first case concerns a 26-year-old prelingually Deaf male, with a prior history of Tourette's syndrome, bipolar disorder, and HIV, who was placed under a Baker Act at a local hospital for “acting erratic and psychotic.” A Baker Act is a 72-hour involuntary psychiatric hold within the state of Florida that can be initiated by healthcare professionals and police officers in the event of a patient being a danger to self or others. The preliminary diagnosis on the involuntary form, as per the emergency room physician, was “psychosis.” The patient was subsequently given an emergency treatment order of intramuscular lorazepam and was transferred to a psychiatric hospital where he was observed by nursing as “calm and nonthreatening.”\nPrior to initial psychiatric interview, an ASL-interpreter was called to assist. The patient asked where he was at and became angry after discovering the truth of his hospitalization. He reported he initially came to the hospital as he had been having anxiety and physical pain attributed to his Tourette's Disorder. He reported his neurologist had him on carisoprodol and diazepam to help relieve these symptoms, but that they were stopped one month prior. The family was called and stated there was questionable abuse of medications but they were adamant that he was safe for himself and others.\nWhen the patient was seen by the ED physician initially there was no interpreter present. The patient reported becoming frustrated and was trying to sign aggressively which he believes was misinterpreted. He also expressed in spoken word to the staff there that he had been “hearing voices” secondary to his pain level. He purportedly was never told what was occurring prior to seeing the interpreter at the transfer facility nearly 12 hours later. The patient adamantly denied SI, HI, AVH, or mania and maintained a linear and coherent thought process. He expressed a history of bipolar disorder which had been diagnosed after a similar incident in the past. He had been on several antipsychotics previously but had not taken any for several years without incident. He had only been taking anxiolytics and pain meds for multiple years which he felt stable on, as well as antiretrovirals for his HIV diagnosis.\nThe patient later admitted that he had been buying oxycodone off the street since his neurologist had stopped prescribing medications due to questionable abuse. A clinical opiate withdrawal scale was performed and was only positive for minor anxiety elevation. A full medical workup was performed and excluded any medical causes to his admission. Through further interview, OCD was excluded as a diagnosis but substance use disorder remained high on the differential for his current and past behavior. The patient was kept overnight for observation and discharged the next morning following positive report from staff. He was given extensive education on substance use as well as coping strategies to prevent readmissions. Upon discharge “unspecified psychosis” was given as his diagnosis.","A 26-year-old man who is Deaf was brought to the hospital after staff believed he was acting erratically and potentially a danger to himself. He was initially diagnosed with psychosis and treated with medication, but he was confused about why he was there and became angry when he learned he was being hospitalized. He had a history of Tourette's, bipolar disorder, and HIV, and had recently stopped taking his prescribed medications, leading to a possible opioid withdrawal. After a thorough evaluation, he was discharged with education about substance use and coping strategies to prevent future hospitalizations."
581,"The second case involves a 30-year-old Deaf, Hispanic male who presented to the Emergency Department after his mother reported that the he was behaving oddly and not taking his risperidone. Per reports, the patient was talking to his mother about going places in a UFO and exhibiting disorganized and illogical behaviors. He was subsequently placed under a Baker Act by the emergency room physician who documented that the patient was exhibiting auditory hallucinations. Initially an interpreter was brought to the hospital prior to his admission. Per the ASL-interpreter, the patient stated that he felt “fine and not crazy” and that all of these events are happening because his mother does not “understanding Deaf culture.” He also conveyed that he did not like to take his meds because they interfered with him being able to drink alcohol and caused drowsiness.\nUpon initial psychiatric interview an interpreter was not present as the hospital only agreed to set periods of time for the interpreter. As an effort to communicate, questions were prepared for the patient to answer via written responses. highlights a portion of the questions and answers that were constructed. From the responses he maintained bizarre delusions but denied current SI, HI, or AVH. When the ASL-interpreter arrived, the patient appeared jovial and yearned to express himself. The interpreter stated she had difficulties reading his rapid signing at first and had to have him slow down several times. However she did note that this was a common occurrence when addressing Deaf individuals.\nWith the interpreter's assistance, the patient was answering questions logically with a linear thought process. He reported that he had been diagnosed with schizophrenia as a teenager after having several interpersonal issues with his mother. She is Spanish speaking only and he stated that she has never fully understood how to communicate effectively with him. He had been taking risperidone for several years but was tired of continuing with the medication due to the side effects of drowsiness and weight gain, which he was never able to fully discuss with his psychiatrist. Patient reported he was in an ASL school and learning a career in massage therapy. After meeting a girlfriend there he began to develop a sense of independence that he reported his mother disapproved of. This caused an altercation that he reports his mother misinterpreted which precipitated his admission.\nThe patient continued to express that he was abducted by aliens as a child and could understand their language, but besides this he expressed no other psychotic processes. He was observed for two days without medications and remained calm/cooperative but was unable to participate in most activities due to limitations of the interpreter availability. After a family session was completed the patient was discharged home with plans to follow up with his community psychiatrist. The patients' diagnosis was changed to delusional disorder upon his discharge.","A 30-year-old man who is Deaf and Hispanic was brought to the hospital because his mother was worried about his behavior, which included talking about UFOs and seeming confused. He was experiencing auditory hallucinations and was placed under a Baker Act for safety. While initially resistant to medication, he eventually communicated that he felt fine and that his mother’s lack of understanding of Deaf culture was the reason for his experiences. After working with an interpreter, he shared a history of mental health challenges, including a previous schizophrenia diagnosis, and expressed frustration with his mother’s disapproval of his newfound independence. He was discharged with a plan to continue seeing his psychiatrist and was diagnosed with delusional disorder."
582,"A 36-year-old male, a seasoned cyclist with no past medical history, presents to the emergency department with complaints of lightheadedness and diaphoresis after a bicycle fall. Patient was participating in a bicycle race when another rider ahead of him fell causing the patient to swerve to avoid him. Patient states that he fell on his left side and hit a tree with his right leg. Patient was wearing a helmet and did not suffer any chest or head trauma. After the fall, he felt lightheaded and diaphoretic and complained of mid back pain. Patient denied any chest pains or shortness of breath. Patient was subsequently brought to the hospital directly following the accident by ambulance.\nIn the emergency department, patient was noted to be in no acute distress; initial blood pressure was 128/69 mmHg with pulse of 65 beats per minute. He was afebrile, not tachypneic, and well appearing with marked right thigh swelling and tenderness to his medial thigh. Given the dizziness and diaphoresis initially, patient had an ECG performed which showed lateral ST segment elevation () and had a subsequent troponin I that was positive, 0.49ng/mL, with a Creatine Phosphokinase (CPK) of 617 U/L.\nThere was initial concern for a possible cardiac contusion, although the patient had no chest wall trauma and thus was admitted for further evaluation. As an inpatient, an echocardiogram was performed demonstrating normal right and left ventricular function and trace pericardial effusion while the patients troponin continued to trend upwards towards a maximum of 21ng/mL. He was loaded with Aspirin and Clopidogrel as well as initiation of a heparin infusion, Lisinopril, and a Beta Blocker. Coronary angiography was subsequently performed demonstrating a spontaneous coronary artery dissection of left anterior descending coronary artery. No further diagnostic study was performed at that time. Further history revealed that he took multiple caffeine Jello shots and drank a large cup of coffee prior to participation in the race. He denied cocaine, amphetamine, or other performance enhancing drug use ().\nThe patient's CPK and troponin trended downwards on conservative medical management and his back pain resolved; therefore a stent was not placed. The patient was visiting from outside the area; discharge planning included repeat coronary angiography in 6 weeks and instructions that he will not be able to perform competitive cycling again. Should his dissection extend at that period of time or patient become symptomatic, stent placement would be considered. Patient was to continue the Aspirin and Clopidogrel until the repeat angiography was performed. Patient was discharged with plans to follow up with a cardiologist in his home state. Multiple follow-up phone calls made us unable to reach the patient and he was subsequently lost to follow-up.","A 36-year-old cyclist was brought to the hospital after falling off his bike during a race. He experienced dizziness and sweating, and later developed pain in his back and swelling in his right thigh. Tests revealed a tear in a blood vessel in his heart, which caused elevated levels of enzymes indicating heart damage. The patient was treated with medications and will need to undergo further testing in six weeks to monitor his heart."
583,"A 30-year-old male with history of active smoking (1 pack per day for 10 years) and external hemorrhoids came to the preop anesthesia clinic for anesthesia evaluation fitness and was found to have high blood pressure (BP) (234/144). He was referred immediately to the emergency room (ER) for BP control. In the ER, BP was 221/125 and heart rate (HR) was 50 beats/minute. Routine electrocardiogram (EKG) showed 3rd-degree heart block (TDHB) and left ventricular hypertrophy (LVH) with strain pattern (). He denied chest pain, palpitation, dyspnea, dizziness, or syncope. The patient was started on antihypertensive medication for BP control and was admitted to the cardiology ward for evaluation and management of complete heart block. Further physical exam revealed absent arterial pulses except the left radial pulse which was weak. BP was significantly different between both upper limbs and between upper and lower limbs (right upper limb 126/86 and lower limb 85/54, left upper limb 145/85 and lower limb 75/50). His initial blood work showed mild renal impairment.\nComputerized tomography (CT) thoracic aortogram was done to rule out coarctation of the aorta, which was normal; CT coronary angiogram showed no evidence of coronary artery disease (CAD). Magnetic resonance imaging (MRI) of the heart was normal as well. Transthoracic echocardiogram (TTE) showed moderate hypokinesia of the left ventricle (LV), ejection fraction (EF) 35–40%, grade 2/4 diastolic dysfunction, and moderate concentric LVH. Holter monitoring did not reveal any pauses. Ultrasound/Doppler of the kidneys showed increased parenchymal echogenicity with poorly defined corticomedullary junction impressive of renal parenchymal disease. CT abdominal aortogram showed large thrombus seen in the abdominal aorta starting at the level of renal arteries completely occluding the aorta and common iliac arteries with no blood flow seen beyond the renal artery level up to the aortic bifurcation; moderate to severe stenosis is noted at the origin of both renal arteries because of thrombus (Figures and ). Multiple abdominal collaterals are seen with multiple collaterals in the anterior and lateral abdominal wall and paraspinal collaterals (). Extensive blood work-up including thyroid function test and autoimmune and thrombophilia work-up was all unremarkable. No cause of aortic thrombosis and TDHB was identified.\nInitial recommendation of the vascular surgeon was to follow up in the clinic with no intervention as it is a chronic process, and the patient was asymptomatic. Since the patient had uncontrolled hypertension despite being on maximum doses of four antihypertensive medications, eventually he underwent percutaneous stenting of bilateral renal arteries which was followed by an improvement in the BP and renal function and reduction in doses of antihypertensive medications. The patient also underwent permanent pacemaker insertion for TDHB. The patient was also placed on warfarin and was advised to see the vascular surgeon after 3 months. Unfortunately, the patient did not follow up.","This 30-year-old man with a history of smoking and hemorrhoids was rushed to the hospital because his blood pressure was dangerously high. Doctors discovered he had a serious heart problem, including a complete heart block and weakened heart muscle, which caused his heart rate to slow down significantly. After tests, they found a large blood clot in his aorta, which was blocking blood flow to his kidneys. He underwent a procedure to open up the blocked arteries, and was then fitted with a pacemaker to help regulate his heart rhythm."
584,"Case 1 concerned a 71-year-old male presenting with gait difficulties and vertigo. Cranial MRI revealed a low and focal contrast-enhancing nodular tumor in the left cerebellar hemisphere and upper vermis (). The lesion appeared hypointense relative to gray matter on T1 weighted (T1w) images and moderately hyperintense on T2w images. Tumor margins were best displayed on diffusion weighted images (DWI). The suspected clinical diagnosis was metastasis from an unknown primary tumor. Microsurgical resection of the tumor was performed. Histopathological work-up revealed a highly cellular tumor consisting of small cells with scant cytoplasm and round-oval or pleomorphic, hyperchromatic cells in the cerebellum. A part of the tumor showed a nodular architecture and a desmoplastic component (Gomori staining). Some tumor cells expressed the neuronal differentiation marker synaptophysin. The diagnosis was paucinodular desmoplastic MB (WHO grade IV). Tumor cells showed nuclear YAP1 and cytoplasmic GAB1 staining, while nuclear staining for ß-catenin and staining for p53 was negative. There was no MYC- or MYCN-amplification detectable (). The patient's condition was stable in the continuing course; however a week later he was affected by a severe pneumonia and died due to respiratory insufficiency.","This 71-year-old man came to the hospital because he was having trouble walking and felt dizzy. A scan of his brain showed a small tumor in his cerebellum, which doctors suspected might be from another cancer. The tumor was surgically removed, and tests revealed it was a type of aggressive cancer called MB. Sadly, the patient later developed a severe pneumonia and passed away due to breathing problems."
585,"Case 2 was a 72-year-old male who was referred to the hospital because of change of personality and loss of weight. Cranial MRI showed a large low contrast-enhancing mass in the right cerebellar hemisphere consisting of a lateral solid component and a small medial cystic. The tumor caused occlusive hydrocephalus but no surrounding edema (). MR revealed diffusion restriction of the solid tumor part and peripheral susceptibility effects, e.g., hemosiderin deposits. Once again, the first suspected diagnosis was metastasis without presence of any neoplasm in the patient history; the second radiological diagnosis was MB. The possibility of a high-grade glioma was discussed but neglected due to its rare occurrence in the cerebellum in this age group. Prior to surgery an external ventricular drainage was inserted. Complete tumor resection was performed. Histopathological examination showed a highly cellular cerebellar tumor consisting of sheets of uniform cells with a high nuclear/cytoplasmic ratio and round to oval hyperchromatic nuclei. Many tumor cells reacted for synaptophysin. There was no evidence of a nodular or desmoplastic component in the Gomori staining. The diagnosis was that of a classical MB (WHO grade IV) (). The tumor cells did not show staining for YAP1, GAB1, and p53 or nuclear staining for ß-catenin. Evidence of MYC- or MYCN-amplification was not found. The postoperative course was uneventful and the ventricular drainage was removed without evidence of an enlarged ventricular system. However, the patient was found dead seven days later in his room. The cause of unexpected death could not be clarified, since an autopsy was not allowed.","This 72-year-old man was admitted to the hospital because he was acting differently and had lost weight. A scan of his brain showed a large tumor in the back of his brain that was causing fluid buildup. Surgery to remove the tumor was successful, and tests confirmed it was a type of aggressive brain cancer called medulloblastoma. Despite the surgery, the patient unfortunately passed away just a week later, and the cause of his death remains unclear."
586,"An 86-year-old female with a history of metastatic ovarian cancer presented to the ED with painful bilateral lower extremity edema and a left lateral leg ulceration. Her metastatic ovarian cancer had been diagnosed by malignant pleural effusion five months earlier, and she had completed neoadjuvant chemotherapy with carboplatin and Taxol approximately one week prior to this presentation. She was admitted to the hospital and started on cefazolin for left lower extremity cellulitis on hospital day one.\nOn admission, plain films and ultrasound did not reveal any evidence of osteomyelitis, fracture, DVT, or abscess to the left lower extremity. On exam, she had 3+ pitting edema below the knee bilaterally as well as chronic venous stasis changes. The patient also had a venous ulcer (approximately 2 cm in diameter) on the anterolateral aspect of the distal third of her left lower leg. At the time of admission, this venous ulcer had some serous weeping but no purulent drainage or fluctuance on examination. Her initial Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score was 4, suggesting a low risk for necrotizing fasciitis; however, on hospital day 3, her CRP began to uptrend and she became febrile. At this point, her antibiotics were switched from cefazolin to vancomycin to cover MRSA.\nOn hospital day five, the patient was noted to have a new erythematous area over the anterior left knee, inferior to the patella (). Ultrasound revealed a small fluid collection superficial to the patellar tendon in the infrapatellar region measuring 3.3 × 2.5 × 0.4 cm (). The infrapatellar bursa was aspirated and sent for culture. The patient was started on piperacillin-tazobactam, given the patient's immunocompromised status and subsequent risk for atypical and gram-negative organisms.\nAn MRI was performed on hospital day seven (this was delayed due to the patient's pacemaker) but did not reveal any evidence of osteomyelitis. The patient was clinically improved after starting piperacillin-tazobactam, and vancomycin was discontinued on hospital day seven. On hospital day eight, aspirate cultures returned with Pseudomonas aeruginosa; she was stable for discharge at that time and was sent out with a ten-day course of levofloxacin (culture was pan-sensitive) and close follow-up with infectious disease.","An 86-year-old woman with a history of cancer was admitted to the hospital because of painful swelling and an open sore on her leg. She had recently finished chemotherapy for her cancer and was experiencing complications from the swelling and the sore, which was later found to be infected with a bacteria called Pseudomonas. Doctors initially treated her with antibiotics, but switched to a stronger medication when the infection worsened. After treatment, she began to improve and was discharged with a prescription for an antibiotic to finish the infection. The doctors ruled out other possible causes for the leg problems, such as a bone infection or blood clot."
587,"A 48-year-old female with known breast carcinoma was screened for possible dissemination with whole-body computed tomography (CT) and a bone scintigraphy scan. The bone scan revealed a tumor in the entire right tibia. The patient reported no symptoms from the tibia tumor. A plain X-ray and magnetic resonance image (MRI) confirmed an intraosseal tumor that extended from 4 cm below the knee joint proximally to about 4 cm from the ankle joint distally (). An open biopsy confirmed an adamantinoma histology. Different treatment options were thoroughly discussed with the patient, including a lower leg amputation with disarticulation of the knee, a total tibia resection and reconstruction with a tibia allograft, or a custom-made tibia EPR, which was eventually selected.\nThe tumor was resected with an extensive anteromedial approach, and the defect was reconstructed with a custom-made, silver-coated, modular endoprosthesis of the Modular Universal Tumor and Revision System (Implantcast®, Buxtehüde, Germany) (). The knee joint was reconstructed with a metal-on-poly articulation with a (unique) metal-on-metal hinge mechanism (). The ankle joint was reconstructed with a metal-on-poly hinge joint with a talar replacement, stabilized with a trans-talar and trans-calcanear hydroxyapatite-coated stem. A supplementary screw was used to add stability in the subtalar joint. The endoprosthesis was enveloped in a Trevira (Implantcast®) tube to facilitate the attachment of soft tissues and the patella tendon (). A microvascular latissimus dorsi musculocutaneous flap was anastomosed to the tibia artery (end-to-side) and concomitant vein and wrapped around the prosthesis to avoid dead space and allow tension-free closure. In addition, a medial gastrocnemius muscle flap was transposed to cover the patellar tendon region; this was covered with a meshed split-thickness skin graft.\nA histological analysis of the resected specimen showed an adamantinoma that had spread throughout the entire tibia and the margins were wide: an unaffected periosteum as an anatomic barrier and a minimum of clear soft tissue margin of 3 mm. Due to intracortical location of the tumor, no massive muscle excision was needed.\nThe knee joint was immobilized in extension for 6 weeks to facilitate patella ligament attachment to the tube. Then, the joint was gradually mobilized. After 6 months, the patient's gait was almost normal, with mild limping. Local MRI and chest radiographs performed during the 8 years of follow-up showed no signs of local recurrence or distant metastasis. No loosening of the stem or other mechanical problem was reported. In a routine follow-up, in addition to radiographs, we used a local MRI with a metal artifact reduction sequence (MARS) technique [], which enables to observe local recurrences of the tumor around the massive titanium endoprosthesis.\nEight years postoperatively, the patient had most of the time no pain and could mobilize freely. The patient resumed working full-time as a kindergarten teacher, and she has maintained her previous active lifestyle (except downhill skiing). On her latest follow-up visit (at 8 years), the knee range of motion was 0–105 degrees, ankle dorsiflexion was 5 degrees, and ankle flexion was 35 degrees. In the latest follow-up visit, we used 4 patient-related outcome (PRO) measures. The Musculoskeletal Society Tumor Score (MSTS) was 77%; the Oxford Knee score (OKS) was 35/48; the Toronto Extremity Salvage Score (TESS) was 80/100; and the 15D was 0.87/1.\nThe metal-on-metal prosthesis caused an increase in metal ion concentrations: cobalt was 6 ppb and chromium was 8 ppb. The silver coating created a mild, local skin argyria pigmentation, with cosmetic discomfort [] ().","This 48-year-old woman with a history of breast cancer had a tumor found in her leg bone. After a biopsy, doctors determined it was a rare type of tumor called an adamantinoma and decided to remove it completely with a custom-made implant to rebuild her leg. The surgery involved extensive reconstruction of the knee and ankle joints, and a flap procedure to cover the area. Eight years later, she is walking well and returning to her normal activities, though the metal implant has caused some metal ion levels to increase and a slight skin discoloration."
588,"A 26-year-old male healthy Asian student presented to an emergency department in Australia with sudden onset generalised weakness affecting predominantly his lower limbs after eating dinner. The patient reported difficulty standing and difficulty lifting his arms without any muscle pain or paraesthesia, headache, or back pain. Although he had experienced multiple similar episodes over the past month, these had been less severe and always self-resolved. It was unclear to the patient if these episodes of weakness were associated with food intake or exercise.\nOn further questioning, the patient reported 15-kilogram weight loss over the past three months and a four-day history of nonbloody diarrhoea, which resolved one week prior to presentation. He had otherwise been well and was playing soccer regularly. He had no relevant family history, was on no regular medications, and denied using illicit drugs.\nOn examination, the patient appeared mildly diaphoretic but was afebrile. Heart rate was irregular, at 92 beats per minute and blood pressure was 118/60 mmHg. He had a normal respiratory rate at 18 breaths per minute with oxygen saturations of 98% on room air. Neurological examination revealed symmetrical proximal weakness of upper and lower limbs with normal tone, reflexes, and sensation. The decrease in power was more noticeable in the lower limbs compared to the upper limbs. In addition, there was a mildly enlarged painless thyroid gland, with a slight hand tremor, but no signs of thyroid acropachy or thyroid eye disease. Heart sounds were normal with no murmurs, gallops, or rubs, and lung fields were clear to auscultation and percussion. There was no abdominal tenderness to palpation.\nBedside electrocardiogram revealed atrial flutter with a variable ventricular rate. Initial biochemistry showed hypokalaemia with potassium of 2.3 mmol/L (reference range: 3.5–5.2 mmol/L) and hypomagnesaemia with magnesium of 0.59 mmol/L (reference range: 0.70–1.10 mmol/L). Plasma glucose level was 7.1 mmol/L. Complete blood count, urea and creatinine, liver function, phosphate, and creatinine kinase were unremarkable.\nHypokalaemic periodic paralysis was suspected and thyroid function testing was performed. This revealed hyperthyroidism with a TSH of <0.01 mU/L (reference range: 0.40–4.00 mU/L), T4 of 44 pmol/L (reference range: 9–19 pmol/L), and T3 of 25 pmol/L (reference range: 3.0–5.5 pmol/L), suggesting a diagnosis of TPP. TSH receptor antibodies were elevated at >40 U/L (reference range: <1.8 U/L), confirming a diagnosis of Graves' disease. This was further supported by a radionuclide thyroid scan showing diffuse uptake in the thyroid gland. Antithyroperoxidase antibodies, antithyroglobulin antibodies, and urinary electrolytes were not measured in this case.\nInitial treatment in the emergency department included 40 mmol of intravenous KCl and 10 mmol of intravenous MgSO4. The patient's weakness resolved during inpatient admission. Potassium was 4.7 mmol/L and magnesium was 0.88 mmol/L on repeat testing 6 hours after the initial biochemical tests. Thereafter, potassium remained normal throughout the admission, without rebound hyperkalaemia or the need for further replacement. The patient was discharged home the next day on Carbimazole 20 mg twice a day and Propranolol 20 mg three times a day. No potassium supplementation was prescribed on discharge and he was not started on anticoagulation for the atrial flutter due to his low risk of thromboembolism.\nOne month after discharge, the patient reported no further episodes of weakness. His T4 and T3 had normalised, although TSH remained at <0.01 mU/L. His potassium was normal at 4.1 mmol/L. Ongoing monitoring of thyroid function was recommended, with a plan for radioiodine ablation or thyroid surgery if antithyroid medical therapy was unsuccessful. However, further follow-up information was not attainable as the patient returned to his home overseas.","This 26-year-old man experienced sudden weakness in his legs after eating, which had been happening occasionally over the past month. He also reported weight loss and diarrhea, and a physical exam revealed an enlarged thyroid gland and an irregular heartbeat. Blood tests showed low potassium and magnesium levels, and confirmed he had an overactive thyroid, leading to a diagnosis of Graves’ disease. He was treated with medication to control his thyroid and his weakness resolved, and his thyroid levels have normalized."
589,"Thirty-three-year-old male with diabetes and seizure disorder presented to the emergency department (ED) with worsening dyspnea and hemoptysis. Two weeks prior to his ED presentation, he was treated with antibiotics for community acquired pneumonia with minimal improvement. Upon further inquiry, patient admitted to vaping for the past 2 months with overtly increased exposure time and has experimented on new flavors. He denied previous or current recreational drug use. CT scan of the chest showed diffuse ground glass opacities and bilateral patchy consolidation (). He had worsening hypoxia that required noninvasive ventilation. His echocardiogram was otherwise normal. Bronchoscopic examination failed to demonstrate airway lesions. Bronchoalveolar lavage (BAL) revealed increasing blood in four sequential aliquots confirming diagnosis of DAH (). BAL cell count showed greater than 30,000 RBCs and 800 WBCs, 42% neutrophils, 36% lymphocytes, 1% eosinophils, and 21% macrophages. All inflammatory serologies were unremarkable: erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), rheumatoid factor (RF), antinuclear antibody (ANA), and anti-antineutrophil cytoplasmic antibodies (ANCA). In addition, serum eosinophil count, anti-glomerular basement membrane (GBM) antibodies, and anti-phospholipid antibodies were all normal. Urine toxicology screen which includes amphetamines, cannabinoids, and cocaine was negative. There was no microbiologic growth on all BAL specimens. Patient was treated with pulse dose steroids after DAH was confirmed with BAL aliquots (). He underwent right wedge resection lung biopsy which revealed evidence of bland pulmonary hemorrhage () with no evidence of capillaritis or diffuse alveolar damage (DAD). Prussian blue iron staining was also noted which reflects old hemorrhage (). His symptoms improved with complete resolution of alveolar hemorrhage on chest CT scan after 2 weeks (). His steroids were tapered quickly and he has not used a personal vaporizer since then.","This 33-year-old man with diabetes and a history of seizures came to the hospital because he was having trouble breathing and coughing up blood. He recently started vaping, and tests showed he had a serious lung condition called diffuse alveolar hemorrhage (DAH). The tests revealed blood in his lungs and showed evidence of old bleeding. After receiving treatment with steroids, his symptoms improved, and the bleeding stopped, and he has since stopped vaping."
590,"A 41-year-old man without any underlying diseases such as cardiovascular disease was hospitalized for spontaneous gum bleeding, epistaxis, and lower limb ecchymosis. Laboratory data on the admission date showed leukocytosis (WBC, 15,820/mm3; promyelocyte, 66%, with Auer rod), anemia (Hb, 9.5 g/dL), thrombocytopenia (PLT, 22,000/mm3), and abnormal coagulation profile (fibrinogen, 29 mg/dL; fibrin degradation product (FDP), 68.5 mcg/mL; d-dimer, 19.81 mcg/mL; prothrombin time (PT), 20.5 sec; international normalized ratio (INR), 1.92; partial thromboplastin time (PTT), 29 sec). Other laboratory data were as follows: C-reactive protein (CRP), 8.97 mg/dL; total bilirubin (T-bil), 0.73 mg/dL; aspartate aminotransferase (AST), 60 U/L; alanine transferase (ALT), 100 U/L; and serum creatinine (Scr), 0.9 mg/dL. His baseline electrocardiogram (ECG) was normal (). Bone marrow aspiration and biopsy disclosed APL with PML-RARα. ATRA at a dose of 45 mg/m2/day (40 mg twice daily) was administered. On the third day of therapy, oxygen saturation abruptly dropped to 90% without oxygen supplementation. Chest X-ray (CXR), ECG, and echocardiography did not show any abnormalities. In order to prevent DS, intravenous methylprednisolone was administered at a daily dose of 80 mg–120 mg according to the clinical signs and symptoms. Idarubicin (12 mg/m2/dose) was administered starting on the fourth day for four doses. WBC progressively elevated to 46,830/mm3; therefore, 1000 mg hydroxyurea twice daily was also added starting on the seventh day.\nOn the 11th day of therapy, WBC and promyelocyte decreased to 6,310/mm3 and 14%, respectively. ATO infusion at a dose of 0.15 mg/kg was initiated; however, dizziness and chest pain occurred during infusion. ECG showed complete AV block with a heart rate (HR) of 40–50 beats/min (bpm) (). The electrolytes and liver function tests were all within normal limits (Na, 138 mmol/L; K, 4.6 mmol/L; Ca, 2.29 mmol/L; Mg, 0.95 mmol/L; T-bil, 0.77 mg/dL; AST, 15 U/L; ALT, 14 U/L). Thus, ATO and ATRA were immediately discontinued, and aminophylline was administered.\nAfter interruption of ATRA for three doses, complete AV block persisted, but his HR partially recovered to 60 bpm. ATRA was rechallenged with 40 mg once daily on the 13th day of therapy under relatively stable vital signs. On the following morning, his HR decreased to 30 bpm and he required a nonrebreathing mask to maintain oxygen saturation. CXR showed bilateral pulmonary edema, and echocardiogram revealed a large amount of pericardial effusion with signs of cardiac tamponade. In addition, ECG showed complete AV block with QRS widening, and acute renal failure also occurred (Scr, 1.4 mg/dL; blood urea nitrogen (BUN), 45.2 mg/dL; Na, 133 mmol/L; K, 5.2 mmol/L; Ca, 2.22 mmol/L; Mg, 0.93 mmol/L; P, 4.9 mg/dL; T-bil, 1.22 mg/dL; ALT, 17 U/L). Therefore, ARTA was immediately postponed, and a temporary pacemaker was inserted. Steroid treatment was switched to dexamethasone (8 mg twice daily) because of suspected DS. Pericardiocentesis and thoracentesis were performed. Two days after stopping ATRA, ECG showed sinus bradycardia with a first-degree AV block and diffuse ST elevation. The Naranjo adverse drug reaction probability scale [] indicated a score of 7 for ATRA, suggesting it was the probable cause of arrhythmia in this episode.\nThe PML-RARα mutant was still detected using polymerase chain reaction (PCR) of blood sampled on the 19th day of therapy. Consequently, ATRA was resumed the following day with 10 mg twice daily. His HR decreased from 70–90 bpm to 60–70 bpm with stable vital signs. ECG demonstrated sinus rhythm with first-degree AV block. The dose of ATRA was gradually titrated up to 30 mg twice daily on the 24th day. Sinus tachycardia with occasional premature ventricular complexes was noted. The pacemaker was removed on the 27th day. During follow-up, ECG showed normal sinus rhythm (), and echocardiogram revealed minimal pericardial effusion.\nComplete molecular remission was documented by bone marrow aspiration done on the 38th day of therapy using flow cytometry and PCR for PML-RARα. Thereafter, the patient received three cycles of consolidation with ATRA-based chemotherapy. No further bradycardia episode was reported. The patient has been free of leukemia for 1.5 years after the diagnosis.","This man was hospitalized after experiencing gum bleeding, nosebleeds, and bruising. Tests showed he had a high number of white blood cells, low red blood cells and platelets, and some liver issues. He was diagnosed with Acute Promyelocytic Leukemia (APL) with a complication called PML, and started on a specific chemotherapy drug called ATRA. During treatment, he experienced a dangerous heart rhythm problem and fluid buildup in his lungs, requiring a temporary pacemaker and other supportive care. The doctors carefully adjusted his medication and monitored his condition closely. After several weeks of treatment and supportive care, his leukemia was in remission, and his heart rhythm returned to normal. He was eventually able to go home and continue with follow-up appointments."
591,"A 20-year-old fit and healthy man presented with sudden collapse after running for 3 hours under the hot sun (ambient temperature 39°C) during a marathon competition. After finishing his run for 35 km, he felt unwell and collapsed. He was brought to the hospital immediately. No measures were taken to lower his temperature during the 30-minute transferral to the hospital. Upon arrival to the hospital, he was confused (Glasgow coma scale: 13/15, E4M5V4). His blood pressure was 100/60 mmHg, heart rate 120/min, rectal temperature 42.2°C (axillary temperature 41.5°C). He was given 2 L of intravenous normal saline and rapidly cooled with ice pillow and ice water-soaked towel. He was then transferred to ICU for close monitoring. His rectal temperature was brought down to 39°C (axillary temperature 38.5°C) in an hour time. He was then transferred to ICU.\nHis cerebral CT scan, electrocardiogram, chest X-ray, and bedside echocardiogram were unremarkable. Laboratory results was shown in .\nHe remained oliguric (urine output < 10 ml/hour) despite initial fluid resuscitation. Hemodialysis was commenced on Day 1 of admission. On Day 3 of admission, his arterial blood pH, renal panel, and electrolytes were all normalized and he had regained urine output (urine output 10–20 ml/hour). He was fully conscious and alert (GCS 15/15).\nUnfortunately, on Day 4 of admission, he started to complain of severe headache followed by 6 episodes of generalized tonic-clonic seizures, each episode lasting for 1-2 minutes, in a one-hour time. There was no regaining of consciousness in between seizures. The episodes were associated with drooling of saliva, tongue biting, and postictal drowsiness. He was intubated and started on intravenous midazolam infusion, intravenous phenytoin, and intravenous levetiracetam.\nElectroencephalogram done on the same day showed generalized continuous slowing. However, no epileptic activity was observed during the bedside EEG monitoring. MRI brain done on the same day () showed bilateral, symmetrical vasogenic oedema involving both frontal and posterior parieto-occipital, cerebellar hemispheres, and predominantly the cortex and subcortical white matter regions. DWI and ADC sequence showed no area of restricted diffusion. The finding was consistent with posterior reversible encephalopathy syndrome (PRES).\nHe had another 2 episodes of generalized tonic-clonic seizures on Day 5 of admission that lasted less than 1 minute. Loading dose of intravenous sodium valproate 1 g was added. His repeat blood tests, including renal panel, complete blood count, pH, and electrolytes, were all normal.\nOn Day 6 of admission, he was able to produce 3 L of urine per day and hemodialysis was stopped. His midazolam infusion was weaning off slowly. He was fit-free since then. On Day 7 of admission, he was extubated and transferred to general ward. His antiepileptics agents were changed to oral form. His seizure was well controlled with oral phenytoin, valproate, and levetiracetam.\nA repeat MRI brain done on Day 14 of admission () showed near complete resolution of the previous vasogenic oedema. He was discharged well with oral valproic acid and levetiracetam.","This 20-year-old man collapsed during a marathon due to overheating and dehydration. He was brought to the hospital and was initially confused and very hot, requiring rapid cooling and intravenous fluids. After a period of instability, he developed seizures and brain swelling, which was identified as posterior reversible encephalopathy syndrome (PRES). With treatment, his condition stabilized, and he was eventually discharged home on medication to prevent future seizures."
592,"A 63-year-old woman was transferred to our department from the internal medicine clinic of the hospital with a diagnosis of acute abdomen due to possible rupture of the bladder.\nThe patient was admitted in the internal medicine clinic three days earlier due to acute abdominal pain. She had a known medical history of uncontrolled type 2 diabetes and cirrhosis of the liver with extensive ascites. The bladder had been drained and urinary retention was observed (over 2 liters of urine). The white blood cell count was 21300, C-reactive protein (CRP) was 14,83 mg/dl, and procalcitonin was 1,1 ng/ml. Intravenous empiric antibiotic treatment with ciprofloxacin (800 mg/day) and amikacin (1000 mg/day) was immediately initiated, with good recovery until the third day.\nOn day 3, the patient presented rebound tenderness, involuntary guarding, and a completely rigid “washboard” abdomen with percussion tenderness. Bowel sounds were absent. She was haemodynamically unstable. Blood pressure was 85/42 mmHg and heart rate was 114 beats per minute. Urine analysis was normal and urine culture was negative. The blood findings were as follows: WBC was 11,900 and CRP was 8,35 mg/dl. Although there was amelioration in the blood tests (as compared with baseline values), the clinical symptoms and the condition of the patient deteriorated. The computed tomography (CT) scan of the abdomen indicated presence of gas within the anterior bladder wall. The latter was not enhanced with contrast material, indicating necrosis (Figures and ). Instillation of contrast solution in the bladder through the indwelling catheter during CT revealed extravasation in the peritoneal cavity (). Based on these results, emergency surgery was decided.\nDuring laparotomy, we initially encountered extensive necrosis of the perivesical fat with presence of pus in the retropubic space. After the incision of the bladder, full thickness necrosis of the wall was revealed, with the exception of the anatomical area of the trigone. A partial cystectomy with debridement of the necrotic tissue and preservation of both ureters was decided (Figures –). The abdominal cavity was further explored and peritoneal lavage was performed due to the presence of diffuse peritonitis. A suprapubic catheter and two surgical drains (one in the hepatorenal fossa and the other in the rectouterine pouch) were used.\nThe patient was transferred to the intensive care unit in a critical condition and succumbed 12 hours later due to multiple organ dysfunction and septic shock. Histology revealed extensive necrosis of the entire bladder wall.","63-year-old woman was admitted to the hospital with severe abdominal pain and was found to have a ruptured bladder. She had a history of diabetes and liver problems, which made her more vulnerable. After initial treatment, her condition worsened, and scans showed significant damage to her bladder. Unfortunately, despite surgery, she developed a severe infection and died due to complications."
593,"Our case is of a 9-year-old Caucasian male who lives with his mother, stepfather, older brother (15 years), and sister (12 years). He has preexisting diagnoses of Autistic Spectrum Disorder (ASD) and Attention Deficit Disorder (ADD), inattentive subtype, but was not taking any regular medication at the time of presentation. He presented to our inpatient service as an emergency from a local acute hospital due to concerns regarding minimal dietary intake. He was detained under Section 2 of the Mental Health Act for assessment and treatment, due to his resistance of treatment in the community and lacking Gillick competence. He was admitted to a specialist psychiatric ward for children under 12 years. At the time of presentation his most prominent and concerning symptoms were refusing food and fluids, mutism, school refusal, and self-neglect, including refusal to engage in his personal care regime. It was the severity of these symptoms that was particularly concerning to his family and to professionals. His restriction of dietary intake resulted in severe weight loss and admission to hospital for nasogastric (NG) tube feeding, his body mass index (BMI) being less than 12kg/m2 at the time of admission (less than 0.5th percentile). His sole method of communication was typing on an iPad to his mother and his personal care was restricted to wearing his pyjamas and a coat for several days without washing or changing. His mother reports that prior to admission there were also incidents of urinary and faecal incontinence. He would not sleep in his bed but was instead sleeping on the floor outside his parents' bedroom in the “foetal position”. When family members attempted to touch him, he became physically aggressive and hit out at them. He was diagnosed with Pervasive Refusal Syndrome at this time.\nIn May 2015, his stepfather gave him a haircut, which he particularly disliked. This appears to have been a trigger for the subsequent observed behaviours. Following this, he became more controlling of his dietary intake and became selectively mute. In particular, he stopped talking to his stepfather and eating any food that he had prepared. This progressed in severity to the extent that, in January 2016, he completely stopped eating and drinking and became completely mute. He was admitted to the local acute hospital for NG tube insertion and intravenous (IV) fluids at this point. Blood biochemistry indicated acute reduced renal function which resolved with administration of IV fluids. Subsequently, he began to eat and drink small amounts and was discharged after a few days. However, a pattern of refusal to eat and drink developed, resulting in further six admissions to hospital with similar symptoms of dehydration and acute kidney injury over the following 6 months. During this time, his presentation deteriorated to the severity of behaviours described above. He also communicated via letters to his mother and typing on his iPad that he would like to die and return as a Labrador dog or as a superhero. On two occasions, during his admissions to the acute hospital, attempts were made to administer medication to him. However, on each occasion he refused and only one dose of fluoxetine was administered via his NG tube. He also refused administration of aripiprazole and no doses were given. Intramuscular administration of psychotropic medication did not seem proportional given the lack of evidence for medication being successful in PRS cases []. Given that he began to accept oral intake and made attempts to remove his NG tube, it was also not appropriate or practical to continue with administration of psychotropic medication via this route.\nOur patient was born at full term by Caesarean section with no complications. The pregnancy was described as uneventful and there was no exposure to alcohol, tobacco, or illicit substances. He achieved his developmental milestones within a normal timeframe. No concerns were raised prior to starting nursery school (age 4 years). He was noted to be “different” to other children in his class and was diagnosed with ASD at the age of 6 years using Autism Diagnostic Observation Schedule (ADOS) and extensive interviews with his mother. A cognitive assessment, the Wechsler Intelligence Scale for Children (WISC-IV), was completed at age 6, which showed an IQ of 78, indicating a low normal intelligence (and therefore no intellectual disability).\nIn this case it is pertinent to consider the nature and extent of his preexisting ASD symptoms prior to his symptoms at presentation in 2016. In his records, he is described as struggling with understanding abstract questions, complex reasoning, and problem solving skills. He displayed formal speech at times and had difficulties with reciprocal conversation. He spoke quietly with deficits in prosody and struggled to contribute in class at school, but there was no history of muteness at the time of ASD diagnosis. He could misinterpret instructions and had a literal understanding of language. He showed avoidance of eye contact and limited facial expressions. He preferred to be alone, struggled with imaginative play, and did not initiate interaction with other children. He had specific interests in superheroes and dressing in these costumes. In younger childhood, he showed specific interest in vacuum cleaners and washing machines. He also was observed to make repetitive movements in the form of a “jerking” of his neck.\nSince admission to the ward in July 2016, he made comparatively quick progress and was discharged into the community in November 2016. Initially he began to eat only a small amount of prepackaged food. He was reviewed by the dietician, who advised prescribing supplement drinks, which he initially accepted two of. The rationale of the admission to hospital was clearly explained to him and that, as long as he was not eating or drinking, he would need to be in hospital. We believe that this triggered his decision to begin to accept diet and fluids. He accepted a change in clothes and in his personal hygiene routine with the assistance of nursing staff. He made it clear to his mother (via written letters) that he wanted to be at home and that he believed that there was no need for him to be in hospital as he was accepting diet and fluids and had changed his clothes. He also attended his tribunal himself to appeal his detention under Section 2 of the Mental Health Act but did not communicate to the tribunal panel, verbally or otherwise.\nHe made steady progress with regard to dietary intake and gradually accepted full meals served on the ward. His engagement with nursing and allied therapy staff began to improve; he began to communicate with hand gestures (“thumbs up or down”) and use written notes. We therefore agreed to allow him to leave the ward to his family home at weekends. He also started to very quietly verbally communicate with his mother. He began to smile and laugh appropriately with staff and peers. However, he would hide his mouth with his fingers to avoid others seeing this. When asked if he would like to participate in activities, he declined, but when asked if he would, he engaged in a full timetable and appeared to enjoy some of the activities. We believe this is an indication of his preference to avoid activities and a symptom of his ASD. After a period of assessment, speech and language therapy (SALT) advised staff on the phrasing of questions to avoid confusion. Occupational therapy (OT) guided nursing staff, with whom he had established a better therapeutic relationship, with regard to reestablishing his daily skills including dressing and self-care.\nHe progressed to having day leave from the ward and started to attend a specialist school with support in the classroom to assist his communication and learning needs. Although he is not verbally communicating other than with his mother, we believe that his prognosis is good based on his current progress. We expect him to make a full recovery to his premorbid functioning with support in the community. It is difficult to comment upon what his ultimate level of communication will be like as his ASD is likely to have an impact upon this. However, given his young age, with specialist support for his ASD he has the potential to develop coping strategies. At discharge from hospital, he indicated no signs of distress with his level of communication and interaction (i.e., verbally with his mother). However, he will require further input from the community team to accurately assess his long term goals regarding verbal communication for the future.","This 9-year-old boy has a history of Autism Spectrum Disorder and Attention Deficit Disorder, and he’s been struggling with refusing to eat and drink for a long time. Recently, he became completely mute and started refusing to take care of himself, leading to several hospital stays. During his most recent stay, he expressed a desire to die and was experiencing severe anxiety and self-neglect. Doctors believe this was triggered by a haircut his stepfather gave him. Now, he’s making good progress, eating regularly, and starting to communicate with his family and therapists, and he’s being supported to return to school and a normal life."
594,"A 46-year-old Japanese woman with known SLE was admitted to our hospital due to sudden weight loss and respiratory distress. Her vitals on arrival were a temperature of 37.1°C, heart rate of 96 bpm, blood pressure of 148/82 mmHg, respiratory rate of 18 per minute, and oxygen saturation of 95% in room air. The patient was given 50 mg of oral prednisolone immediately after admission.\nApproximately 3 weeks after the start of treatment, the dosage of prednisolone was reduced to 45 mg/day. However, the patient suddenly developed hemoptysis and respiratory distress. A chest X-ray () revealed infiltrative shadows in both lung fields, with greater density in the right lung. A computed tomography (CT) scan showed pulmonary infiltrates along the peripheral bronchovascular bundles and ground-glass opacities (). We diagnosed the patient with DAH-induced hypoxemia accompanying SLE and initiated high-dose corticosteroid therapy (methylprednisolone 500 mg/day). However, respiratory distress worsened, and we began immunosuppressive therapy (cyclophosphamide 750 mg/day) 3 days after the start of high-dose corticosteroid therapy. The patient was also transferred to the intensive care unit (ICU) and placed on mechanical ventilation. Although the patient produced copious quantities of bloody secretions after intubation, the secretions were reduced when APRV was set to the highest airway pressure (35 cmH2O). We administered oxygen through an oxygen mask (flow rate: 6 L/min) while in the ICU. Under these conditions, the arterial oxygen partial pressure (PaO2) was 55.1 mmHg, and the ratio of arterial oxygen partial pressure to fractional inspired oxygen (P/F ratio) was 125.2. APRV resulted in substantial improvements to respiratory function, with a fraction of inspired oxygen (FiO2) of 0.60 and a PaO2 of 117.6 mmHg.\nHigh-dose corticosteroid therapy was administered using 500 mg/day of methylprednisolone for 3 days, 250 mg/day of methylprednisolone for 3 days, and 125 mg/day of methylprednisolone for 3 days, after which the patient was given 50 mg/day of intravenous prednisolone every day while in the ICU. During approximately 3 consecutive weeks of APRV and steroid therapy, the patient demonstrated gradual improvements in respiratory function, and a CT scan after extubation showed that the pulmonary infiltrates and ground-glass opacities had disappeared (). Mechanical ventilation was discontinued 25 days after admission to the ICU. The patient was moved to the general ward and continued on oral steroid therapy (methylprednisolone 16 mg/day) until discharge. The patient's respiratory and hemodynamic parameters from admission to ICU discharge are summarized in . The patient was successfully discharged from hospital 102 days after admission.","This 46-year-old woman with a history of lupus (SLE) was admitted to the hospital because she was experiencing sudden weight loss and difficulty breathing. After initial treatment with steroids, her breathing worsened, and doctors found signs of infection in her lungs on X-rays and CT scans. To help her breathe, she was put on a breathing machine and received high doses of medication. After several weeks of treatment, her breathing improved, and she was able to be weaned off the machine and eventually discharged home on a lower dose of medication."
595,"A 38-year-old North African man, with no past medical history, consulted our out-patient clinic for a painless left scrotal mass. There was no history of previous orchitis or scrotal contusion. He noted the mass a month ago. A physical examination found a 2 cm palpable mass in the upper pole of his left testis. There were no signs of scrotal inflammation. The mass had a firm consistency and regular margins. Palpation of his right testis and the lower pole of his left testis were normal. Routine blood tests were normal. As a testicular tumor was strongly suspected, a bioassay of testicular tumor markers was ordered. Alpha-fetoprotein, human chorionic gonadotropin (hCG), and lactate dehydrogenase (LDH) were in the normal ranges. There was no bacterial growth in urine analysis, including Mycobacterium tuberculosis screening. A scrotal ultrasound showed a homogeneous testicular parenchyma, with a conserved vascularization on Doppler. An extratesticular mass was observed, attached to the upper pole of his testis. The mass was isoechoic to the testis parenchyma, and poorly vascularized Doppler (Fig. ).\nHe underwent a radical inguinal orchiectomy. We first performed a high ligation of the spermatic cord. The operative specimen included the testis and the tunica vaginalis in one piece (Fig. ). The macroscopic aspect of the supratesticular mass looks similar to splenic tissue (Fig. ). There were no macroscopic lesions of the testis and the spermatic cord. His postoperative course was uneventful. He was discharged on the second postoperative day.\nHistological examination of the operative specimen confirmed the presence of regular splenic tissue in the suspect mass, without any signs of malignancy. The splenic proliferation had its proper and regular capsule, demarcating it from the testis. Testicular pulp, the albuginea and the tunica vaginalis had a preserved microscopic architecture (Fig. ). He was examined 3 weeks after orchiectomy and he was examined again 2 months after the orchiectomy in our out-patient unit. An abdominal ultrasound showed an habitual location with standard measurements and a regular aspect of his spleen.","A 38-year-old man came to the clinic because he found a painless lump in his left testicle. After a physical exam and some blood tests, doctors suspected a testicular tumor and ordered an ultrasound. The ultrasound showed a mass attached to the testicle, and he underwent surgery to remove it. The surgery revealed that the mass was actually normal splenic tissue, and he is now recovering well."
596,"The present case involves a 62-year-old woman admitted to surgical oncology unit for a planned transanal excision of a large polyp of the mid rectum. Following a positive faecal occult blood test, colonoscopy detected the presence of a large flat neoplastic lesion, 50 mm in maximum diameter, tending to grow laterally and involving one-third of the rectal lumen (Fig. ). The lesion was located in the mid rectum, 8 cm from the anal verge and, based on its detailed endoscopic appearance during chromoendoscopy, was labelled as a lateral spreading tumour granular type (LTS-G). The endoscopic biopsy demonstrated a tubular adenoma with high-grade dysplasia. In view of the size of the lesion, endoscopic mucosal resection was considered unfeasible and it was decided to proceed with surgical excision transanally by TAMIS. The day before surgery, patient had standard mechanical bowel preparation and at the time of anaesthetic induction received preoperative antibiotics (Cefazolin 2 g and Metronidazole 500 mg). The procedure was performed under general anaesthesia and the single incision laparoscopic surgery port (SILS™ Port, Covidien) was adopted and traditional laparoscopic instruments were used. The surgery lasted 2 h with no intraoperative complications. The rectal wall defect was washed with a povidone-iodine solution (Fig. ) and then closed by a running suture performed with a barbed suture (Covidien V-Loc™).\nPatient had unremarkable past medical history and on admission routine laboratory profile was in normal range: WBC, 6.34 × 103/μL (reference value, 4–10 × 103/μL); platelets, 231 × 103/μL (reference value, 150–400 × 103/μL); prothrombin time (PT), 11.4 s (reference value, 10.0–13.4 s); activated partial thromboplastin time (APTT), 34 s (reference value, 22.0–43.0 s); fibrinogen, 301 mg/dL (reference value, 160–450 mg/dL). Following surgery, the patient was allowed to be mobilised and to have a regular diet with no restriction, and standard prophylaxis for venous thrombosis was started with low molecular weight heparin (LMWH).\nOn day 3, the patient developed a spike in temperature without any suspicious clinical evidences. She was passing flatus associated with the mucous discharge, the abdomen was soft and not tender, and digital rectal examination did not show any lump or collection. Laboratory data disclosed both a deranged coagulation profile with marked APTT prolongation (126 s), PT 12.5 s, fibrinogen 897 mg/dL, raised white blood cells count (WBC 21.00 × 103/μL) and procalcitonin 0.52 ng/mL (reference value, < 0.5 ng/mL). Cross-mixing studies of patient plasma and normal pooled plasma (25, 50, and 75%) insufficiently corrected the APTT (99, 71, and 56 s, respectively) after 2 h of incubation at 37 °C. Lupus anticoagulant assay was negative by dilute Russel viper venom test (DRVVT). FVIII, FXI, and FIX were in normal range whereas coagulant activity of FXII was < 1%, tested using one-stage APTT-based clotting assay.\nConsidering the spike in temperature and laboratory data, a computed tomography (CT) of abdomen and pelvis was performed to rule out any collection and source of infection. The CT scan did not show any pelvic abscess, but there was evidence of perirectal fat suffusion related to the recent procedure. A rigid proctoscopy was performed showing the evidence of the partial dehiscence of rectal wall defect suture; no other abnormalities were noticed. Antibiotic therapy was started with intravenous ciprofloxacin and metronidazole (500 mg) three times a day. From a therapeutic point of view, there is a general consensus that patients with an FXII inhibitor do not need any correction of the APTT and so our patient did not receive any therapy in addition to antibiotics. LMWH standard prophylaxis for venous thrombosis, initially suspended, was resumed. In the next 7 days, the patient had no more fever and laboratory data improved while APTT was still prolonged (70 s). She was discharged home with no further intervention. The histopathological report demonstrated a tubular adenoma with low- and high-grade dysplasia with free excision margins. 45 days later endoscopy showed a complete mucosal healing, APTT and FXII activity were back to the normal value (38 s and 50%, respectively). Time course of APTT and FXII activity during follow-up after surgery is pictured in Fig. .\nBlood and tissue samples used were prepared and stored by CRO-Biobank (CRO National Cancer Institute, Aviano, Italy).","This 62-year-old woman was admitted for a polyp in her rectum that was found during a routine test. The polyp was removed surgically through a small incision, and the area was carefully closed. After the surgery, she developed a fever and some changes in her blood clotting tests, which led to further tests and antibiotics. Thankfully, the cause of the fever was identified, and she recovered well, with the polyp completely healed after 45 days."
597,"A 20-year-old male patient was referred to our clinic in December 2017 for acute liver failure (ALF) of unknown origin. History revealed no pre-existing medical conditions, anamnesis was empty for exposition with predisposing agents such as previous drug-use, promiscuity, pork consumption or autoimmune disorders. The initial presentation at the peripheral hospital occurred due to indolent jaundice and fatigue-syndrome. Physical examination of the patient showed distinct jaundice and hepatic encephalopathy grade I. Laboratory studies revealed massively elevated transaminases with an alanine aminotransferase (ALT) level of 4645 U/l and an aspartate aminotransferase (AST) level of 4956 U/l (normal < 50 U/l) while cholestatic liver enzymes were merely elevated (alkaline phosphatase (AP) 216 (normal 25–124) U/l and gamma-glutamyl-transferase (γ-GT) 91 (normal < 55 U/l)). Furthermore, liver synthesis parameters were significantly impaired with a total bilirubin of 14.8 (normal 0.3–1.2) mg/dl, an international normalized ratio (INR) of 2.39, and a factor V activity of < 35 (normal 70–120) % with a consecutive MELD score of 28 points. Additionally, there was no serological evidence for autoimmune hepatitis, viral hepatitis (A-E), Wilson’s disease or hemochromatosis. Laboratory parameters on admission are presented in Table . According to the above-mentioned parameters and circumstances, the patient was diagnosed with a cryptgenic ALF and treated supportively by substitution of vitamin K, ursodeoxycholic acid, and lactulose.\nAccordingly, liver maximum capacity (LiMAx) test on admission revealed significant impairment of enzymatic liver function of 147 (normal > 315) μg/h/kg. Two days later, laboratory parameters further deteriorated: the patient now fulfilled the KCC for non-acetaminophen-induced ALF ((1) unknown etiology; (2) INR > 3.5; (3) bilirubin > 17.4 mg/dl), and he was considered for urgent liver transplantation at Eurotransplant. Three days later, the patient became anuric and hemodialysis was necessary with a MELD score of 40 points. However, a suitable donor organ was not available. Two days later, the patient developed hepatic encephalopathy grade III-IV accompanied by increasing inflammatory parameters in blood (increase of leukocytes, C-reactive protein, and procalcitonin) and respiratory insufficiency, which made mechanical ventilation necessary. Computed tomography scan revealed bilateral pneumonia and antibiotic (imipenem) and antifungal (caspofungin) therapy was initiated (Fig. ). Bloodstream infection with evidence of Staphylococcus aureus was ascertained concurrently. Due to pulmonary Staphylococcus aureus sepsis the patient was no longer suitable for urgent LT at that critical juncture.\nInterestingly, LiMAx test, which was performed under mechanical ventilation, showed an improvement of enzymatic liver function to 169 μg/h/kg indicating liver regeneration, though laboratory tests (deterioration of conventional liver synthesis parameters, rapid increase of inflammatory parameters in blood) and clinical course (multi-organ system failure: liver and renal insufficiency, encephalopathy, sepsis due to Staphylococcus aureus pneumonia) indicated lethal outcome. However, from then on, clinical condition of the patient improved, inflammatory parameters were declining, urine excretion resumed, and extubation was possible after 6 days of mechanical ventilation. Accordingly, CT scan showed regressive pulmonary infiltrations (Fig. ). Slowly, with significant time delay of 11 days, liver synthesis parameters recovered, and the patient could be moved out from the intensive care unit after 21 days of treatment. At that time point, the LiMAx test demonstrated distinct improvement to 299 μg/h/kg. The following mini-laparoscopy revealed cholestatic changes of liver parenchyma with regenerative nodules and capsular fibrosis (Fig. ). Histopathology of the obtained liver biopsy illustrated advanced connective tissue of the parenchyma in sense of septal fibrosis (F3) accompanied by severe cholestatic liver damage without evidence for precise etiological correlation (Fig. ).\nFinally, the patient could be dismissed from our clinic in markedly ameliorated condition after 38 days. Outpatient three-month follow-up examination showed complete clinical recovery while biochemical parameters were entirely within normal ranges (AST: 21 U/l, ALT: 34 U/l, total bilirubin 0.4 mg/dl, INR: 1.07, MELD score: 7). Accordingly, LiMAx illustrated complete restitution of enzymatic liver function with a value of 614 μg/h/kg (normal > 315 μg/h/kg). Figure shows the timespan between events and summarizes the diagnostic/ therapeutic measures that were taken.","This 20-year-old man was admitted to the hospital after developing a sudden and severe liver failure with no clear cause. Initially, he had symptoms like jaundice and fatigue, and his liver function tests were extremely high. Doctors determined he needed a liver transplant, but a suitable donor wasn't found in time. Despite treatment and dialysis, he developed a serious infection and needed to be put on a ventilator. Fortunately, after several weeks, his condition improved, and his liver began to recover, eventually allowing him to be discharged home with completely normal liver function tests."
598,"The second patient is a 5-year old boy, born as a first child to healthy non-consanguineous parents. The mother reported two previous early spontaneous abortions. Otherwise, the family history is unremarkable. He was born after an uneventful pregnancy in the 37th week of gestation after a spontaneous start of the delivery. The boy’s birth weight was 2430 g (10-25P), birth length 46 cm (10-25P), and head circumference 34.5 cm (75-90P). He had gastroesophageal reflux in the first few months, the abdominal ultrasound was normal. Due to apnoic attacks the boy was administered to hospital at the age of 5 months. The pH-metry confirmed gastroesophageal reflux, ECG and CMCRF were normal. The neurologist described a mild hypertonus and related mild motor delay. He sat independently at 9 months of age and he started walking at 20 months of age. The parents noted shortness of breath and tiredness after simple physical tasks, therefore, he was evaluated by a paediatric cardiologist. Two haemodynamically significant ASDs were noted and a slightly dilated right ventricle; corrective surgery is planned. The tests of acylcarnitine profiles and aminoacids in blood and organic acid in urine were normal. At the age of four his height and weight were in the normal range (height 99.2 cm (17P), weight 16.1 kg (46P)), however, the head circumference showed macrocephaly - 53.8 cm (>97P).\nMicroarray analysis (180 K CGH array, Agilent Technologies- Fig. ) revealed a de novo microduplication of 2.06 Mb in chromosome 2p16.1p15 region (arr[GRCh37] 2p16.1p15(60308869_62368583)× 3 dn). No other pathogenic genomic imbalance was detected in the proband’s sample.","This 5-year-old boy was born a little early and has a family history of early miscarriages. He initially had some problems with breathing and reflux, and later developed some difficulties with movement and coordination. A cardiologist found two heart defects, and he’s scheduled for surgery to correct them. Recent tests revealed a small genetic change that may be responsible for some of his developmental challenges, and doctors are continuing to monitor his growth and development."
599,"The patient is a 43-year-old woman who was admitted for the first time for a progressive non-painful, mobile mass of the right inguinal fold evolving for 7 months. The medical history of the patient included childhood asthma, chronic tonsillitis, seven pregnancies and four children, caesarean section and abortions. Pelvic ultrasound showed a heterogeneous suspicious non-circumscribed mass measuring 5 cm in its longer axis. It was localised in the right inguinal region and showed cutaneous adhesions. CT scan confirmed the presence of this inguinal mass, measuring 5.8 × 4.9 × 3.2 cm and extending within the right femoral triangle in contact with the long adductor muscle, without enhanced contrast, and without locoregional lymph node (Fig. ).\nThe patient underwent a chirurgical biopsy. The pathological analysis diagnosed a granular cell tumor (Abrikossoff’s tumor) without any malignant signs (absence of mitosis, necrosis and cytonuclear atypias). Tumorectomy of this inguinal mass were performed three weeks later. At the gross pathology examination, the tumoral tissue was homogeneous with a greyish stain. Its margin was not well defined and the hypodermic, dermic were involved. One lymph node was discovered and was invaded. The epidermis was not ulcerated (Fig. ). Histologically, collagen bundles were infiltrated by cords of large, polygonal cells with inconspicuous cell membrane and homogenous finely granular cytoplasm. Nuclei were round or oval and presented large nucleoli, vesicular of dark chromatin and sometime an intranuclear vacuole. Mitosis were rare and the mitotic index was low (1 mitosis/ 10 High Power Field). There was a slight increase of the nucleo-cytoplasmic ratio. We observed no necrosis (Fig. ). Fanburg-Smith score of malignancy was of 3: nuclear pleomorphism, tumor cell spindling, vesicular nuclei with large nucleoli. Immunohistological finding showed a cell expression of S-100 protein, vimentin, calretinin (slight), α-Inhibin, CD56, CD57, CD68 and neuron specific enolase (NSE). (Fig. ) Cytokeratin AE1-AE3, EMA, calponin, caldesmon, desmin, smooth muscle actine, myosin, myogenin, chromogranin, synaptphysin, Neurofilament proteins, Glial fibrillary acidic protein, CD1a, renal cell carcinoma antibodies were all negative. Therapeutic marker, estrogen receptors, progesterone receptors, androgen receptor, HER2, CD117 ALK, C-MET, ROS1 and PDL-1 were negative too.\nDue to microscopic resection and the nodal status, a large surgical revision with an inguinal curage was then decided at the oncology committee. Pathological evaluation did not reveal tumor tissue in the tumorectomy region but showed metastatic invasion by granular cells in four of twelve lymph nodes, without capsular rupture. Adjuvant chemotherapy was excluded because of very low mitotic activity. To reduce the risk of local recurrence, adjuvant radiotherapy on the tumor bed and right inguinal area of ​​50 Gy in conventional fractionation was delivered. After 10 months after the end of radiotherapy, the patient is in complete remission. Due to the unpredictable tumor, the follow up strategy is a physical examination with a CT scan every 4 months for the first 2 post-operative years, then every 6 months for up 5 years and yearly thereafter, as a sarcoma.","A 43-year-old woman was admitted to the hospital after noticing a growing, painless lump in her groin that had been developing for several months. Imaging tests, including ultrasound and CT scans, revealed a suspicious mass, and a biopsy confirmed it was a type of tumor called a granular cell tumor, which is not cancerous. The tumor was surgically removed, and a nearby lymph node was also removed and found to contain cancer cells. Following the surgery, the patient received radiation therapy to the area to help prevent the cancer from returning, and she is currently in remission and will continue to be monitored with regular scans."
600,"Patient: a 9-year, 7-month-old Japanese girl (height 127 cm, body weight 33 kg, body mass index 20.5 kg/m2).\nPrimary complaint: severe deformity of the femur.\nPast medical history: no notable history.\nThe patient was referred to our facility with complaints of progressive deformity of her right femur associated with an SBC and pathological fractures. The girl experienced a pathological fracture of her right femur due to bone tumor when she was 4-years, 6-months old (Fig. ), which her previous physician treated with lesion curettage and fixation (Fig. ). Pathological findings confirmed the presence of an SBC. Bone healing was confirmed 6 months later, at the age of 5 years. The fixator was removed and steroids were injected simultaneously with an artificial bone graft into the lesion (Fig. ). She wore a functional brace after the surgery. However, 1 week after removing the fixator, a new fracture developed in the same location of the bone, following a minor external injury (Fig. ). After a 5-week trial of conservative treatment using steel wire skeletal traction, she underwent fixation with application of a hip spica cast. Five months after the second fracture, at the age of 5 years and 5 months, weight-bearing on the affected limb was progressively initiated, and she was discharged with full weight-bearing status at the age of 5 years and 7 months. She was monitored as an out-patient (Fig. ).\nSubsequently, she developed a fracture again at the age of 6 years and 4 months, following a fall (Fig. ). She was once again treated with a steel wire skeletal traction. Once bone healing was achieved, the inside of the cyst was curetted and a cannulated screw was inserted to reduce localized pressure (Fig. ). Approximately 6 weeks after surgery, a hip spica cast was applied, which was followed by the use of a functional brace. During the follow-up period, severe bowing of her right femur developed, which was progressive (Fig. ). At the age of 9 years and 6 months, the femur fractured, with the fracture originating at the cannulated screw. The screw was subsequently removed (Fig. ) and the patient was referred to our facility at the age of 9 years and 7 months.\nOn first examination at our facility, the femur was deformed, with a bowing of approximately 90° in the central area of the femur and an internal rotation of 60°, with incomplete fractures observable in the same area (Fig. ). We proceeded with resection of a 7-cm portion of the bone, which included the SBC, corrected the alignment and applied an Ilizarov fixator to gradually lengthen the thigh. We also cut a portion of healthy bone from the proximal femur (approximately 10 cm from resected lesion), for use as bone extension at the site of resection (Fig. ). One week after surgery, extension of the bone was initiated at a speed of 1 mm/day, completing the process in 4 months. Fixation was then maintained until the callus matured (Fig. ). Once callus maturation was achieved, the external fixator was gradually removed to prevent re-deformation.\nOn histopathologic examination of the surgically resected bone, fibrillation of the medullary cavity was increased, and a small formation, identified as inorganic-like material, was scattered throughout; these findings were consistent with a fracture due to a bone cyst (Fig. ).\nAt present, 3 years after surgery, correction of the deformity has been maintained, and our patient does not experience any limitations in daily activities or regular exercise (Fig. ).","This 9-year-old girl has had several fractures and deformities in her right femur due to a bone tumor that she had as a young child. After multiple surgeries and treatments, including a hip spica cast, she has now undergone a more complex procedure to correct the bone deformity. A portion of her femur was removed and replaced with bone from her own leg, and a fixator was used to gradually lengthen the bone. Currently, her bone is properly aligned, and she is able to live a normal life."
601,"We describe the case of a 27-year-old white woman who had experienced an emergency caesarean delivery at 39 weeks for fetal distress with no postpartum complications. As part of our ongoing study “Vaginal delivery after caesarean section”, she underwent saline contrast sonohysterography 6 months after the caesarean section. The caesarean scar had a small indentation and the remaining myometrium over the defect was 7.5 mm (Fig. ).\nIn the current pregnancy, she had a dating scan at around 11 weeks with no remarks. She came for a transvaginal ultrasound examination at around 13 weeks as part of our study. This scan revealed a duplex pregnancy with one viable intrauterine fetus with normal anatomy and placenta located high on the anterior wall and a small gestational sac (8 mm) with a yolk sac without embryo was located in the caesarean scar (Fig. ). There was no extensive vascularity surrounding the sac. One corpus luteum was found in each of the two ovaries. She was asymptomatic.\nShe was informed that not enough evidence existed to advise a specific management of this condition. After discussion with her and her husband, expectant management was chosen with a new ultrasound examination after 5 weeks.\nShe came to our ultrasound department at 18 weeks, 22 weeks, and 30 weeks of gestation. She remained asymptomatic. The ectopic gestational sac was not visualized with transvaginal or transabdominal scans at the 18 weeks examination (Fig. ). The niche in the scar and the thickness of the thinnest part of the remaining myometrium appeared unchanged at all visits. The intrauterine pregnancy developed normally with no signs of abnormal placentation. At 30 weeks of gestation the ultrasound appearance of the scar area did not indicate any contraindications for vaginal delivery. The thickness of the lower uterine segment (LUS) was 4.9 mm (Fig. ). In agreement with our patient, vaginal delivery was planned. The staff of the labor ward was fully informed.\nShe was admitted to the labor ward with irregular contractions in week 37 + 0. Her cervix dilated to 3 cm with no further progress. Due to that oxytocin augmentation was administered for 3 hours. The duration of active labor was 6.5 hours. A healthy male neonate weighing 2985 g was delivered, with Apgar scores 9–10 at 1 and 5 minutes and umbilical cord pH 7.27. The placenta delivered spontaneously and total blood loss was 250 ml. The postpartum period was without any complications, and she was discharged home the next day.\nAt a follow-up visit 6 months postpartum, saline contrast sonohysterography showed no signs of the previous CSP, and the remaining myometrium over the hysterotomy scar defect was 5.7 mm (Fig. ).\nEthical approval for the ongoing study was obtained by the Ethics Committee of the Medical Faculty of Lund University, Sweden, reference number 2013/176. Our patient has given permission for publication of this case report in a scientific journal.","This woman had a planned C-section and has since been following up with ultrasounds as part of a study. During one ultrasound, they found a small pocket of fluid in the area where her C-section was performed, which contained a tiny sac but no embryo. The fluid pocket didn't grow or cause any problems during her subsequent pregnancies, and she was able to deliver a healthy baby vaginally at 37 weeks. At a follow-up six months after the birth, the ultrasound showed the area where the C-section was performed had returned to normal, and she was doing well."
602,"A 60-year-old female patient met with a traffic accident while walking. The patient had a coma and was taken to a nearby hospital for emergence treatment, then shifted to our hospital for further treatment after 2 weeks. On presentation, the patient was conscious, oriented and hemodynamically stable. The left lower limb was tracting, there was contusion over the hip. The right elbow was casting. The radiographs revealed a fracture of the right Ulna olecranon, a combination of ipsilateral left femoral neck and sub-trochanteric fracture. His neurovascular examination is normal. Evaluations are femoral neck fracture Garden II (nondisplaced) and sub-trochanteric fracture Seinsheimer IIIB (AO 32A1.1), AIS 14, ISS 27 (). Eventually the fracture was treated with a long Gamma3 nail () with ORIF to the sub-trochanteric fracture, and the femoral neck fracture was treated with closed reduction. During fluoroscopy, care was taken to ensure that all the screw threads crossed the fracture lines and compression was obtained at the femoral neck region. After 12-month follow-up, the fracture was union and has no evidence of avascular necrosis (). But at 4-year follow-up, the patient began to complain of the pain. The X-ray showed an evidence of the necrosis of the femoral head (). One year later, she had a total hip arthroplasty for the severe pain.","This 60-year-old woman was brought to the hospital after being involved in a car accident and spending two weeks in another hospital. She had a broken arm and leg, and a fracture in her hip and thigh bones. After initial treatment, her leg and arm healed with surgery, and the fractures were stabilized. However, several years later, she began experiencing pain and the hip bone started to break down, eventually requiring a total hip replacement."
603,"The patient was a healthy 25-year-old man with a one-year history of right ankle pain following trauma.\nHe had met a car accident while walking. One year earlier, he had undergone open reduction and internal fixation on his right ankle for fracture at another hospital. A tibia diaphysis spiral fracture was fixed by the anterograde intramedullary nail with infra-patellar approach. An ankle malleolar fracture was fixed by the locking plate and cannulated cancellous screws with direct lateral and medial approach. He had finally consulted us because of worsening ankle pain while walking. On physical examination, there was tenderness in the anteromedial joint space of the right ankle. Slight ankle swelling was noted. Dorsiflexion of the right ankle was 10°, similar to that of the left ankle, but plantarflexion was restricted to 38°, compared with 60° on the left, but the ankle instability test was negative.\nThe first three months, even though we performed intra-articular injections, arthroscopic synovectomy for osteoarthritis, and the fixation implant removal in order to release implant irritation, his ankle pain persisted.\nThe AOFAS ankle score at that point in time was 50 . Radiographs showed moderate narrowing of the ankle joint and forward displacement of the talus (-A, B). Computed tomography of the right ankle showed an osteochondral defect on the anterolateral surface of the distal tibial plafond (). This was diagnosed as progressive osteoarthritis caused by an osteochondral defect on the anterolateral surface of the distal tibial plafond, and surgical repair of the osteochondral defect was recommended. Three months later, the osteochondral graft was performed on the patient's right ankle. The patient was placed in the supine position under general anesthesia. The lower extremity was prepared and draped in the standard sterile fashion. We inserted the 2.0 mm K-wire in his right calcaneus, then skeletal traction was done in order to open his right ankle joint space if necessary. Next, 10 mL of fluid was injected intra-articularly to distend the ankle. The anterolateral ankle arthroscopy portal was established in the routine fashion, and global arthroscopy was performed. The osteochondral defect was identified about the anterolateral plafond which site was enthesis of the anterior tibiofibular ligament (), and osteochondral lesions of the tibia and talus were identified. An anteromedial portal was established. The osteochondral lesions were probed, and the osteochondral lesions were diagnosed as grade 2 according to the classification of the International Cartilage Repair Society . We performed multiple OAT in the anterolateral plafond at the anterolateral wall with extension of the anterolateral portal. The anterolateral osteochondral defect was measured 9 mm × 20 mm (). The plug donor site was the lateral corner of the patellar groove in the right knee joint. Under direct vision, we harvested 2 bone plugs from the normal bone deeper than the osteochondral defect for the preparation of the recipient site in order to accept the osteochondral plugs from the donor site in maximum plantarflexion. Then, the dilator was interpositioned into the recipient site, and a radiograph was taken to calculate the inclination angle between the dilator and tibial plafond. Therefore, we thought that the smooth surface was obtained by the inclination of the grafted plugs. According to the angle required, two 8-mm osteochondral autograft plugs ramped 35° and 45° were harvested from the donor site (), and transferred into the anterolateral plafond at an angle to re-contour the articular surface and make it smooth. Both harvest and transplantation of osteochondral autograft plugs were performed by open approaches for transplantation in the exact slope. After this procedure, the surface of the anterolateral wall of plafond was almost completely smooth (). The depth of these plugs was 20 mm, deeper than the osteochondral defect. It was confirmed that the deepest area of the plugs consisted of a macroscopically normal bone. We used press-fit technique in order to stabilize the osteochondral plugs. Stabilization of the osteochondral fragments relied on tight interference with the host bone. In case of difficulty with press-fit technique, a screw made of polylactic acid and hydroxyapatite was prepared, but it was never used.\nPostoperatively, the foot was placed in a short leg cast, and the patient was advised to ambulate non-weightbearing for a 4-week period. The patient had an uneventful postoperative course, and a range of motion exercises were initiated immediately after cast removal at 4 weeks, with partial progressive weightbearing allowed during the next 4 weeks. The patient was able to return to standing work 10 months after surgery. The most recent magnetic resonance imaging scans at 2 years after surgery showed that the cartilage in the anterolateral joint surface was almost intact, and there was no osteonecrosis (). At 3 years the post-operative X-rays of the ankle joint depicted a larger osteophyte, compared with the pre-operative situation, in the anterolateral corner of the right tibial plafond. No forward displacement of the talus was evident on the lateral view of the right ankle joint, while the respective joint space was preserved (). On the latest physical examination, the patient complained of mild pain in his right ankle while walking, but could walk for 30 min. Dorsiflexion of the right ankle was 0°, compared with 10° on the left, and plantarflexion was 30°, compared with 60° on the left. The postoperative AOFAS ankle score was 80. The patient remained asymptomatic with respect to the donor sites of the right knee. He was able to return to carpenter's work on a five-day week, but remained with minor ankle pain on standing up and walking for more than half an hour over the 3-year observation period.","The patient had ankle pain for a year after a car accident, during which time he had several procedures to try to fix the problem. After these procedures, an MRI revealed a damaged area of cartilage in his ankle. To address this, he underwent surgery to replace the damaged cartilage with healthy cartilage from his own knee. Following the surgery, he had a period of rest and rehabilitation, and is now able to return to work and walk comfortably, although he still experiences some mild ankle pain."
604,"A 35 year-old male was referred to our unit with a right distal tibial non-union, which had progressively deviated into varus deformity. He had initially sustained a high-energy, multifragmentary compound (Gustilo–Anderson Type 3A) fracture in a road traffic accident 4 months earlier. After successful treatment of the soft tissue injuries he had been managed in a below-knee, weight-bearing Sarmiento cast. His non-union (, ) was stabilised with a retrograde 10 mm × 340 mm expandable nail. Regular clinical and radiological follow-up revealed that the non-union had solidly united within 6 months of the operation (). The patient was able to walk comfortably and return to work as a carpet fitter.\nNine months after insertion of the nail there were radiological appearances suggestive of calcaneal resorption around the neck of the nail, therefore we recommended removal of the nail. This was attempted under general anaesthesia using the kit and technique described by the manufacturer. After deflation of the nail, attempts were made to “back-slap” the nail out of the tibia. This failed as the nail fractured at the junction between the valve and the metal fins (). Further attempts to remove the nail using grabbers and a mole wrench were unsuccessful and the procedure was abandoned.\nThe patient returned to theatre for a second attempt. Initial attempts at removal through the entry point using grabbers and large diameter crown drills were unsuccessful as the broken end of the nail had split, with the fins separating, like an inverted cone. A further attempt at pushing the nail down using an antegrade nail was also unsuccessful. Eventually, the nail was removed piecemeal through a 6 cm × 2 cm antero-medial tibial window using a Midas Rex® burr (Medtronic Ltd., Watford) (). The tibia was protected in a below-knee walking cast for 6 weeks and the patient made an uneventful recovery with no further complications.","This man had a serious broken leg four months ago that didn't heal properly, leading to a significant bend in his ankle. He had a surgery to insert a nail to help the bone heal, and it eventually did, allowing him to walk again. However, nine months later, the nail started to cause problems with bone changes, and attempts to remove it were unsuccessful, requiring a second surgery. Finally, the nail was removed piece by piece, and he made a full recovery with no further issues."
605,"A 67 year-old female was referred with a right distal tibial non-union with progressive valgus deformity following a fragility fracture five months previously. It was initially treated with locked antegrade intra-medullary nailing.\nThe initial nail was removed and an expandable retrograde nail was inserted. Two months later an Ilizarov frame was applied over the nail to provide further compression. The non-union united and the frame were removed three months later. The nail was left for six more months in order to allow further consolidation of the non-union.\nThe first attempt to remove the expandable nail was made ten months after insertion. The standard technique to remove the nail was attempted, but again the procedure failed because the nail fractured at the junction between the valve and the fins. Further attempts to remove the nail with grabbers at that time were unsuccessful and the procedure was abandoned. Two months later, a second attempt was undertaken as the patient was complaining of ankle pain. The track from her primary nail was re-opened from the proximal end and the expandable nail was successfully pushed out using an antegrade nail. This procedure passed without incident and she made a good post-operative recovery.","A 67-year-old woman had a broken bone in her right ankle that didn't heal properly. Initially, doctors tried to fix it with a metal rod, but it didn't work. They then used a special frame to apply pressure and help the bone heal, which eventually succeeded. After removing the frame, they tried to take out the metal rod, but it broke, so they had to try again, and they were successful in removing it without any problems."
606,"A 73 year-old male presented to our unit with a multifragmentary Pilon fracture and was treated with primary retrograde expandable Fixion® nail. Regular follow-up revealed good union at 22 weeks and the patient was able to walk without significant pain. Although we recommended the removal of the nail, the patient didn't consent on it.\nTwo and a half years later the patient requested removal of the nail because of chronic heel pain presumably due to slight prominence at the bottom end of the nail. Using the standard extraction technique an attempt to remove the nail was performed but resulted in fracture of the nail at the junction of the valve and fins (). The end cup and valve were removed but attempts to remove the remaining part of the nail with grabbers failed and the procedure was abandoned.\nThe patient was advised to keep the nail remnant unless it becomes symptomatic. He is still under follow-up and currently asymptomatic.","A 73-year-old man broke his ankle and had a special nail inserted to help it heal. After two and a half years, he started experiencing pain in his heel, which the doctors believe is related to the nail. He asked to have the nail removed, but the procedure was unsuccessful and the nail broke. The doctors now recommend leaving the remaining nail in place unless it causes problems, and he will continue to be monitored."
607,"A 79-year-old male with a past medical history significant for pancreatic pseudocyst secondary to idiopathic acute pancreatitis diagnosed two years ago presents with severe acute gastrointestinal bleed with a hemoglobin of 5.8 g/dL. Throughout the week prior to admission, the patient had been experiencing melena. He was admitted to the intensive care unit for supportive care including pantoprazole infusion, blood transfusion and close monitoring. CT scan of the abdomen revealed a complex pancreatic mass representing a bleeding pancreatic pseudocyst with an interval increase in size when compared with the previous CT scans (Figure ).\nThe patient underwent esophagogastroduodenoscopy (EGD)/endoscopic ultrasound (EUS) which showed submucosal bulging likely from extrinsic compression on the proximal gastric body and fundus with small clean-based ulcer on the top of this bulge (Figure ). No fresh or old blood was noted in the stomach, and the esophagus and duodenum both appeared normal. EUS confirmed the presence of anechoic lesion with hyperechoic shadowing suggestive of cyst/pseudocyst with bleeding located in the tail of the pancreas. The lesion measured approximately 50 x 50 mm in the maximal cross-sectional diameter.\nThe patient continued to have melena and a decrease in hemoglobin to 6.3 g/dL for which an additional two units of packed red blood cells (PRBCs) were provided. Interventional radiology was asked to perform mesenteric angiogram with hope that may help identify and control the source of bleeding, however, angiogram did not demonstrate any active bleeding vessel (Figure ). Ultimately, the bleeding stopped spontaneously. The patient had another EGD/EUS which again noted two openings over the greater curvature of the stomach suggestive of fistulous communication of the lesion with the stomach lumen. A bullet-tipped catheter was inserted into the lumen and aspiration showed blood consistent with hemorrhagic pancreatic pseudocyst fistulizing into the stomach and causing severe upper gastrointestinal bleed. Follow-up CT abdomen one year later revealed calcification in the head and uncinate process of pancreas along with scarring in the uncinate process, consistent with chronic pancreatitis (Figure ). Additionally, the pseudocyst that was abutting the stomach wall had resolved.","This 79-year-old man was admitted to the hospital after experiencing significant bleeding in his stomach and a drop in his blood count. Tests revealed a large, bleeding cyst in his pancreas that was pressing on his stomach and causing a connection between the two organs. Doctors performed several procedures to try to stop the bleeding, but it eventually stopped on its own. Finally, they found that the cyst was leaking into the stomach, which was the source of the bleeding, and the cyst has resolved."
608,"A 73-year-old white man presented to our emergency department with a 3-day history of left lower extremity swelling and acute-onset shortness of breath. On evaluation, he was tachycardic with a pulse of 113, hypertensive with a systolic blood pressure of 130-170 mmHg, and demonstrated poor oxygen saturation of 81% on room air. He was supported with continuous positive airway pressure (CPAP) and supplemental oxygen while a computed tomography angiogram (CTA) was obtained, which revealed a saddle PE (Fig. ).\nTissue plasminogen activator (tPA) was administered and he was started on a heparin infusion and admitted to our intensive care unit (ICU) for management. He remained on the heparin infusion for 3 days, during which he continuously improved and was eventually weaned to 3 L oxygen via nasal cannula. On hospital day 2, he was transferred to intermediate level of care. Per hematology recommendations, he would have to be on indefinite anticoagulation due to the massive PE he had sustained, the source of which was a left lower extremity popliteal deep vein thrombosis (DVT). The plan was to transition him from the heparin infusion to enoxaparin twice per day with hematology follow-up in 1 month.\nOn the day of discharge, however, he had sudden onset of right leg numbness and weakness below the level of his hip. He had previously been working with physical therapy and had been able to walk 200 feet with the assistance of a walker during each session. A physical examination revealed decreased sensation to light touch, 2/5 strength in right hip flexion and right knee extension and flexion, and loss of right patellar reflex. Left leg physical examination was normal at that time.\nAn emergent head computed tomography (CT) scan was ordered due to concern for a possible stroke, and neurology was consulted. The head CT was negative for infarction or hemorrhage. Neurology was concerned for spinal cord infarction versus hematoma and recommended emergent magnetic resonance imaging (MRI) of his thoracic and lumbar spine. The MRI revealed a left psoas hematoma (Fig. ). A CT of his pelvis performed the same day also showed a right psoas and iliacus hematoma. Due to these findings, hematology recommended discontinuing enoxaparin and reverting to a low intensity heparin infusion, as well as placement of an inferior vena cava (IVC) filter. The following day his left leg began exhibiting the same symptoms as his right leg. There was concern regarding the risk of progressive and irreversible nerve damage due to compression if the hematomas were not promptly drained. IR was consulted and advised that if the drainage were to occur while our patient was on anticoagulation, the risk of rebleeding into the retroperitoneum would be high and potentially nullify any benefit from drainage. Our patient would also be at risk of hemodynamic instability if the current hematomas were acting as tamponades against further bleeding. An additional complicating factor was the risk of further thrombosis due to the presenting saddle PE. Hematology was consulted for recommendations on pausing anticoagulation, but they were hesitant to offer a timeframe as there was no established safe period to enable this type of procedure to take place. Eventually, a window period of pausing the heparin infusion for 3 hours pre-procedure and up to 6 hours post-procedure was decided upon in the event that our patient agreed to have the drainage performed.\nThroughout this sequence of events, our patient and his wife were aware of the plans and considerations on how to proceed. They were informed of the recommendations and concerns made by neurology, hematology, and IR, as well as the risks and benefits of intervention versus non-intervention.\nAfter speaking with his wife, our patient decided to undergo the procedure. The low intensity heparin infusion was stopped 3 hours beforehand and the IR team then performed drainage of the right retroperitoneal hematoma, placing two pigtail catheters in our patient’s right flank (Fig. ). In total, the hematoma was drained of 215 milliliters of blood, 10 milliliters of which were drained during the procedure itself. The left psoas hematoma was not found to be amenable to drainage.\nOur patient tolerated the procedure well, and the heparin infusion was restarted 6 hours after it was completed. A repeat neurological examination demonstrated improved lower extremity strength bilaterally as well as the return of sensation to light touch. Hip flexion improved to 3/5 bilaterally, and knee flexion and extension improved to 4/5 bilaterally. Deep tendon reflexes remained absent. Four days later, the pigtail catheters were removed. His recovery was complicated by anemia requiring blood transfusions totaling 4 units of packed red blood cells (PRBC). Other sources of potential bleeding were evaluated and not found. A repeat CT on hospital day 10 (Fig. ) showed a stable right-sided hematoma, and our patient did not experience any further neurologic deficits. He was transitioned again from the heparin infusion to enoxaparin after 3 more days. His hemoglobin and hematocrit remained stable. During this time, he worked with physical therapy, who recommended discharge to a skilled nursing facility where his strength began to improve somewhat. Follow-up was scheduled with neurology and hematology. On hospital day 18, he was safely discharged.","This 73-year-old man was admitted to the hospital after experiencing sudden shortness of breath and swelling in his leg. Tests revealed a large blood clot in his leg, which caused a serious complication called a psoas hematoma. To treat this, doctors performed a procedure to drain the blood from the hematoma, which helped improve his breathing and leg strength. After the procedure, he received additional treatment and support, and he was eventually discharged to a skilled nursing facility to continue his recovery."
609,"A nine-year-old entire male greyhound presented with head trauma resulting from a collision with a park bench. The dog had no previous significant clinical history. On examination, cardiovascular parameters were stable overt distress in the animal not apparent. The dog was ambulatory with normal gait and posture devoid of proprioceptive deficits.\nThorough head inspection revealed subcutaneous emphysema between the eyes and a superficial cut to the right dorso-orbital region. Mild right unilateral epistaxis was noted. The dog resented palpation of the right frontal bone and a communication with the sinonasal cavity was inferred by the presence of a flail segment movement of the bone synchronous with respiration. Cranial nerve examination demonstrated bilateral delayed pupillary light reflex (PLR) and normal pupil size, the remainder of the neurological examination was within normal limits.\nA right-sided frontal bone depression fracture was suspected founded on clinical findings. Radiographs and Computer Tomography (CT) imaging with a three-dimensional reconstruction of the skull were performed under general anaesthesia (see Fig. -). Radiographs of the cervical spine were unremarkable.\nCT imaging revealed a comminuted, depressed fracture of the frontal bone that extended from the level of the maxillary recesses up to the caudal aspect of the frontal sinuses at the level of the dorsal aspect of the right maxillary, nasal and frontal bones. Surgical repair of the defect was warranted to reestablish sinus architecture and mechanical stability []. Further, fracture comminution is associated with soft tissue contracture leading to cavitation with connective tissue scarring and sequestrum formation [], fracture repair addresses soft tissue injury and may minimize long-term risks of complication []. Surgery was carried out three days after admission.\nA standard dorsal approach to the frontal bone was taken (Fig. -). A malleable highly porous Ti mesh (0.2 mm thickness with 1.4 mm by 0.6 mm elongated pores) was contoured to the patient’s skull (Fig. -).\nThe Ti mesh was firmly seated against the skull and a 1.6 mm drill bit, with a 6 mm stop, was used to drill pilot holes through the mesh into the skull. A 2.1 mm diameter resorbable poly-L, D-lactic acid (PLDLA) thermoplastic pin was placed into the pilot hole and the ultrasonic trode applied to the proximal end of the pin (Fig. -). The ultrasonic device was activated melting the outer surface of the pin and allowing it to be advanced into the pilot hole. Further pilot holes were drilled around the periphery of the Ti mesh at 20-25 mm intervals and thermoplastic pins inserted until the Ti mesh was adequately secured.\nThe surgical site was flushed with copious volumes of sterile saline and a sheet of gentamycin-impregnated collagen (Collatamp®, EusaPharma) was overlaid on the implant to prevent infection and assist in achieving a pneumatic seal. Prophylactic intravenous antibiotic cover was provided by clavulanated amoxicillin (20 mg/kg; Augmentin, GSK) given 30 min pre-operatively and every 90 min thereafter for surgery duration. Recovery from anaesthesia was uneventful. Post-operative analgesia was provided with intravenous methadone (0.2 mg/kg q4hours; Comfortan, Dechra). Postoperative radiographs and CT images showed satisfactory positioning of the Ti mesh and adequate coverage of the calvarial defect (Fig. -).\nThe dog was discharged two days after surgery with a 10 day course of oral carprofen (2 mg/kg q24hours; Rimadyl, Zoetis) and oral clavulanic acid-amoxicillin (20 mg/kg q12h; Synulox, Zoetis).\nThe dog re-presented two weeks after discharge, with hyperthermia and swelling around the surgical site. Subcutaneous oedema was present in the absence of emphysema. Sampling for culture and sensitivity was prevented by the absence of nasal and surgical site discharge, exposure of the wound to obtain a swab sample was deemed inappropriate. Antibiotic therapy effective against common nasal pathogens [] was introduced with oral cefalexin (20 mg/kg q 12 hours; Therios, Ceva) for ten days. Clinical signs had resolved at 1 week post treatment.\nAt the six weeks recheck, the physical examination was within normal limits. No pain was elicited upon palpation of the surgical site and no indications of infection recurrence was found. Radiographs and CT of the skull revealed a slight uplift of the mesh at its most rostral aspect from the frontal bone. Mesh uplift was not a clinical concern at this stage. Radiographs and CT scan at 6 months revealed soft tissue swelling between the rostral mesh portion and the skull (see Fig. -). Decision was made to trim and re-contour the uplifted mesh. The procedure and recovery were uneventful and no further complications were experienced.\nAt 2 years assessment, radiographs and CT scan confirmed adequate contouring and positioning of the Ti mesh. Radiographs revealed a bone-dense opacity between the rostral part of the mesh and the skull potentially associated with mesh micromotion stimulating periosteal reaction (Fig. -). The degree of new bone formation around the mesh periphery was difficult to ascertain radiographically, however, clinical examination was unremarkable and cosmetic result was satisfactory. The owners did not raise any further concerns regarding the patients ability to participate in normal lifestyle and activity.","The greyhound was brought to the hospital after a serious injury from hitting a park bench. X-rays and a CT scan showed a broken bone in his forehead, and surgery was needed to repair the damage and prevent complications. During the surgery, a special mesh was used to hold the bone fragments together and restore the shape of his sinuses. The dog is now recovering well and is expected to make a full recovery, although there is some slight bone growth around the mesh."
610,"A ten-year-old neutered female Cavalier King Charles Spaniel presented for a gradually enlarging mass on the right frontal bone with no associated clinical signs. The mass was bone-like and non-painful upon palpation. The remainder of clinical examination was within normal limits. Fine needle aspirates of the mass revealed evidence of bone remodeling compatible with a neoplastic process yet were not diagnostic, further investigation was declined and mass excision by surgery was planned. Radiography and CT imaging of the skull were performed under general anaesthesia to advise surgical planning and custom 3D Ti mesh design for use in reconstruction following tumour resection. Thoracic and abdominal CT scan were also taken for staging and were negative for metastatic disease. CT imaging of the skull revealed a 25 mm (h) × 20 mm (diam.) ovoid mass arising from the right frontal bone above the right orbital globe (see Fig. -). Surgery was implemented a week later.\nThe same surgical approach was performed (Fig. -). A craniotomy with resection margins guided by the use of customised template was performed (Fig. -). The resection guide was computer modelled from CT imaging and printed using a sterolithographic system. A pre-contoured Ti mesh aided by computer modeling and computed manufacture was firmly seated to the patient’s skull covering the defect (see Fig. -). The protocol for thermoplastic pin placement and application of gentamycin-impregnated collagen sheet was as described for patient 1. Non-resorbable methacrylate-butadiene-styrol (MBS) pins were used in case 2 for fixation in contrast to resorbable PLDLA described in case 1.\nProphylactic intravenous antibiotic cover was provided by cefuroxime (20 mg/kg; Zinacef, GSK) given 30 min pre-operatively and every 90 min thereafter for surgery duration. Recovery from anaesthesia was uneventful. Post-operative analgesia was provided with intravenous methadone (0.2 mg/kg q 4 h) and in travenous paracetamol (10 mg/kg q12hours; Perfalgan, BristolMyers squibb). Postoperative radiographs and CT images showed satisfactory positioning of the Ti mesh and adequate coverage of the calvarial defect (Fig. -). The dog was discharged six days after surgery after uneventful hospitalisation.\nThe dog re-presented two weeks after discharge for suture removal. Surgical wound has healed and physical examination was within normal limits. At the ten weeks recheck, physical examination was within normal limits and the owner reporting no restrictions on the patient’s activity. There was no pain elicited upon surgical site palpation. Radiographs and CT of the skull revealed satisfactory positioning of the mesh. A histological diagnosis of multilobular osteochondrosarcoma was made with potential of local recurrence advised.\nAt 7 months assessment, owners reported a small bone-like mass near the resection site. This clinical finding was consistent with either local recurrence or reactive osseus proliferation. CT scan identified the region of proliferation as dorsal and to the right side of the Ti mesh. The mesh remained well localised and confluent with the skull. The remaining clinical examination was unremarkable and cosmetic result was satisfactory. Owners did not raise concerns regarding overall quality of life and further investigation of the mass was declined. A request to examine the patient at 1 year follow up was again declined by the owner due to long travel distance.","A ten-year-old dog presented with a growing lump on her forehead that was not causing any pain. After taking samples of the lump, doctors determined it was likely a type of bone cancer and planned to remove it surgically. During surgery, a custom-made metal mesh was used to rebuild the skull after the tumor was removed. The dog recovered well and was discharged after six days, but follow-up scans revealed a small area of regrowth, which the owners chose not to investigate further."
611,"A 44-year old male was referred following investigation for chest pain and dyspnoea. He had no pre-existing co-morbidities. Physical examination revealed feeble femoral pulses and he was found to be hypertensive with marked differences between the upper and lower limbs (systolic blood pressure upper limb 190mmmHg, lower limb 75 mmHg, with an ankle brachial index (ABI) of 0.39). Electrocardiogram revealed evidence of severe left ventricular hypertrophy. This was confirmed with echocardiography which also demonstrated a tricuspid aortic valve with significant aortic regurgitation in the presence of an aortic root aneurysm of approximately 9 cm. Left ventricular function was preserved. Computerised tomography angiography (CTA) was performed to evaluate the aortic pathology in further detail (Fig. ). The scan noted an aortic root aneurysm (8.8 cm), in addition to the presence of severe aortic coarctation, with subtotal occlusion and a lumen less than 6 mm in size. The coarctation was just distal to the left subclavian artery, at the aortic isthmus. There was clear evidence of collateral circulation to the descending thoracic aorta via the subclavian and intercostal arteries. Coronary angiography confirmed a right dominant coronary system with no significant coronary disease.\nA multidisciplinary team meeting took place and a consensus was agreed to proceed with a two staged hybrid approach, with the first phase involving an endovascular approach to stent the coarctation, followed by a second stage to perform the surgical repair of the aortic root aneurysm. The first stage to stent the coarctation was unsuccessful via the femoral approach, as the guidewire could not cross the coarctation. Assessment through angiography via the left brachial artery showed complete obstruction at the aortic isthmus. The decision was then made to proceed to a single stage surgical approach to treat both lesions.\nAfter induction of anaesthesia, arterial lines were placed in the left radial and left femoral artery. A right infraclavicular incision and a right groin incision was made this was to establish peripheral arterial cannulation access to the right axillary and right femoral artery. An 8 mm dacron graft was anastomosed to each vessel for indirect cannulation. Median sternotomy was performed to access the mediastinum and expose the heart and aorta. Following heparinisation cardiopulmonary bypass (CPB) was established with venous return from bi-caval cannulation. The body temperature was cooled to 25 degrees Celsius. The right superior pulmonary vein was used for venting. Once the cross clamp was applied, complete cardiac arrest was achieved using Custodiol 25 ml/kg crystalloid cardioplegia via a retrograde cannula through the coronary sinus. A further top up of cardioplegia was given once the aorta was opened through direct cannulation of the coronary ostia.\nThe aortic root, valve and ascending aorta were excised. The coronary ostia were fashioned as buttons from the native aortic root. The coronary ostia were noted to be significantly displaced, with distorted anatomy due to the patient’s disease process. Therefore, 8 mm dacron grafts were attached end-to-end to each ostia, with view to performing the modified Cabrol technique later following replacement of the root. The heart was then retracted in a cephalad position to access the posterior pericardium. A vertical incision was made to expose the descending thoracic aorta (DTA). An end to side anastomosis was formed with a 20 mm dacron graft to the DTA (Fig. ). This graft was then routed posterior to the inferior vena cava (IVC) and anterior to the right inferior pulmonary vein (RIPV), adjacent to the right atrium (RA). Root replacement was then performed with a 25 mm biological valved-conduit, as this was favoured by the patient over a mechanical prosthesis, despite the risk of a difficult redo procedure in the future. The 8 mm dacron grafts attached to the coronary ostia were anastmosed to the root conduit as neo coronary ostia. The distal part of the valved-conduit was anastomosed to the proximal arch under selective antegrade cerebral perfusion (SACP). Finally, an end to side anastomosis was fashioned between the 20 mm extra-cardiac graft (attached to the descending thoracic aorta) and the ascending portion of the valved-conduit. Valve-sparing root replacement was not considered in this patient due to the grossly abnormal aortic anatomy.\nFollowing rewarming and deairing the patient was successfully weaned off CPB. The bypass time was 160 min, the cross-clamp time was 120 min, and the SACP time was 40 min. Haemostasis was achieved and thereafter a routine closure of all incision sites. The patient remained in ICU for less than 48 h, and made excellent progress on the ward. Minimal anti-hypertensives were required and the patient was discharged on 8th day post operatively neurologically intact and independent. At 3 months follow up the patient underwent a repeat CTA scan which showed complete patency in the extra-anatomical graft and resolution of the collateral arterial network (Fig. ).","The patient was admitted to the hospital after experiencing chest pain and shortness of breath. Tests revealed a serious problem with his aorta, including a narrowing and an aneurysm, and significant heart enlargement. To fix these issues, doctors decided on a complex surgical approach involving both endovascular and open surgery to repair the aorta and improve blood flow. The surgery was successful, and the patient is recovering well at home."
612,"A 79-year-old Japanese woman with a weight of 72 kg who has been maintained on anti-hypertensive drugs, including hydralazine, for more than 10 years, was advised on acute onset of proteinuria and microscopic hematuria by her family doctor. At this time, her serum creatinine (Cr) level was within normal range (0.8 mg/dl). One month later, however, the Cr level was elevated to 1.6 mg/dl. Therefore, she was referred to our hospital for admission.\nOn admission, vital signs revealed body temperature of 36.9 °C, blood pressure of 150/70 mmHg, and pulse rate of 80 per minute. The white blood cell count was 8700/μl with 2.0% eosinophils, red blood cell count was 307 × 104/μl, and platelet count was 26.6 × 104/μl. The following values indicated renal dysfunction; blood urea nitrogen: 25.0 mg/dl, Cr: 1.9 mg/dl, urinary protein: 2.5 g/day, and the presence of microscopic hematuria. Dysmorphic red blood cells were noted in the urine sample microscopically. In the serum, MPO-ANCA was 107 IU/ml (normal limit, 3.5 IU/ml), whereas C-reactive protein (CRP) was 0.2 mg/dl. PR3-ANCA and other ANCAs, including anti-elastase and anti-lactoferrin antibodies, were negative. The titer of anti-nuclear antibody (ANA) was less than 1:40. Anti-DNA antibody was negative. Complement values were as follows: C3 162.2 mg/dl (normal range, 71–135 mg/dl) and C4 37.7 mg/dl (normal range, 11–34 mg/dl). Renal biopsy revealed pauci-immune necrotizing crescentic glomerulonephritis (Fig. ).\nHydralazine-induced MPO-AAV was considered regardless of absence of skin involvement, elastase- and lactoferrin-ANCAs, anti-nuclear and anti-DNA antibodies, and hypocomplementemia, which are usually observed in the disease []. By discontinuation of the causative drug, the serum Cr level decreased gradually. At 10 months later, the serum Cr and MPO-ANCA levels recovered to 1.1 mg/dl and 13 IU/ml, respectively. Proteinuria and microscopic hematuria also disappeared. Since the clinical course was consistent with hydralazine-induced MPO-AAV and the serum CRP level was not high throughout the clinical course, no additional medication was administered.\nAfter another 6 months of observation, the serum Cr and MPO-ANCA levels were re-elevated (Cr, 2.0 mg/dl; MPO-ANCA, 195 IU/ml) and proteinuria and microscopic hematuria were re-appeared. The titer of ANA was 1:40. Other ANCAs, including PR3-ANCA, and anti-DNA antibody were negative even at this time. Complement values were as follows: C3 149.4 mg/dl and C4 38.5 mg/dl. Renal biopsy was performed again and revealed cellular crescents in some glomeruli (Fig. ). These findings suggested the relapse of MPO-AAV. Since she had fever (38.3 °C), and the serum CRP level was elevated to 10.0 mg/dl at this time, administration of 30 mg prednisone (0.5 mg/kg body weight) was initiated. The illness improved rapidly, and remission was achieved 5 months after the beginning of treatment. The patient has remained in remission thereafter (Table ).\nThis study was approved by the Ethical Committee of Osaka General Medical Center (Permission No. 29-C0313) and the Ethical Committee of Faculty of Health Sciences, Hokkaido University (Permission No. 15–90). After acquisition of written informed consent from the patient, serum samples were obtained at the disease onset (Serum A; MPO-ANCA, 107 IU/ml), at relapse (Serum B; MPO-ANCA, 195 IU/ml), at 3 months after treatment (Serum C; MPO-ANCA, 4.5 IU/ml), and at remission (Serum D; MPO-ANCA, 2.4 IU/ml).\nTo assess the involvement of NETs in the pathophysiology of this patient, we determined the NET degradation activity in the serum samples at first. In brief, peripheral blood neutrophils from a healthy volunteer were seeded in slide chambers (1 × 106/ml), incubated for 15 min at 37 °C, and then made to react with 100 nM phorbol myristate acetate (PMA; Sigma-Aldrich, St. Louis, MO) for 3 h at 37 °C. We have confirmed that this stimulation induces NETs conspicuously []. After washing with PBS, the cells were incubated in 10% Serum A, B, C, or D for 6 h at 37 °C. For positive control, 10% serum of a healthy volunteer (49 years old, male) was employed. This sample exhibited the average value for NET degradation in our previous study []. To stop the serum nuclease activity, 2 mM EDTA was added, and then the remaining cells on the slides were fixed with 4% paraformaldehyde (PFA) followed by mounting with the solution containing DAPI. Photomicrographs (magnification, × 200) were taken randomly under a fluorescent microscope (6 fields/well of chamber slides), and then the residual NET area was determined using Image J software. NET degradation rate (%) was calculated as follows; {(residual NET area, incubated with PBS) – (residual NET area, incubated with serum) / (residual NET area, incubated with PBS)} × 100. As a result, the NET degradation activity was entirely low in Sera A, B, C, and D compared with the healthy control (Fig. ). Correspondingly, the DNase I activity as determined using ELISA kit (Orgentec GmbH, Mainz, Germany) was low in Sera A (21.7%), B (28.3%), C (22.8%), and D (33.5%) compared with the healthy controls {mean ± standard deviation (SD), 52.6 ± 12.1%}.\nNext, we determined the NET induction activity of IgG, which was isolated from the serum samples, using immunoadsorbent columns (Protein G HP SpinTrap, GE Healthcare, Tokyo, Japan). Contamination of endotoxin in the IgG samples was ruled out using the Limulus test kit (Wako Pure Chemical, Osaka, Japan). Peripheral blood neutrophils from a healthy volunteer were seeded in slide chambers (1 × 106/ml), pre-treated with 5 ng/ml TNF-α for 15 min at 37 °C to express MPOs on the cell surface, and then made to react with 250 μg/ml of the IgG samples. Serum IgG samples from a 65-year-old woman patient with MPO-AAV (MPO-ANCA, 93.2 IU/ml) and the healthy volunteer were employed as positive and negative controls, respectively. These samples exhibited the average values for NET induction in our previous study []. After incubation for 3 h at 37 °C, the supernatants were removed and the remaining cells on the slides were fixed with 4% PFA. Finally, the remaining cells were mounted with the DAPI-containing solution. Photomicrographs (magnification, × 200) were taken randomly under a fluorescent microscope (6 fields/well of chamber slides), and then the rates of NET-forming neutrophils were determined using ImageJ software. As a result, the NET induction activity was high in Sera A, B, and C, whereas that in Serum D was equivalent to the healthy control (Fig. ).\nLastly, we conducted immunofluorescent (IF) tests to determine the presence of ANETA in the serum samples. Briefly, peripheral blood neutrophils from a healthy volunteer were seeded in slide chambers (1 × 106/ml), incubated for 15 min at 37 °C, and then made to react with 20 nM PMA for 2 h at 37 °C. After washing with PBS, the cells were fixed with 4% PFA, and then made to react with 250 μg/ml of the IgG samples for 1 h at 37 °C. After washing with PBS, the cells were next allowed to react with 1:5000 dilution of FITC-conjugated anti-human IgG antibodies for 1 h at 37 °C followed by mounting with the solution containing DAPI. As shown in Fig. , ANCA was detected in Sera A and B but not in Sera C or D; thus, these findings were consistent with the ELISA titers of MPO-ANCA. On the other hand, ANETA was detected in Sera B and C but not in Serum A or D.","This patient experienced a challenging autoimmune condition that came and went several times. Initially, she developed kidney problems and a severe infection linked to a medication she’d been taking for high blood pressure. Tests revealed a rare autoimmune disease called MPO-AAV, which caused inflammation in her kidneys and led to a buildup of antibodies. After treatment, her condition improved, but it later flared up again, requiring further medication. Finally, after a period of treatment, she achieved a sustained remission and is now doing well."
613,"A 54 year-old Caucasian male without significant comorbidities was diagnosed with IgG kappa multiple myeloma in 2005. Initial treatment consisted of doxorubicin, vincristine, and dexamethasone followed by an autologous stem cell transplant (SCT) with melphalan 200 mg/m2 conditioning. He remained in remission for 2.5 years, at which time he relapsed and was treated with a series of doublet regimens followed by a second autologous SCT in 2011, with melphalan 200 mg/m2 conditioning. He relapsed 4 months after the second transplant and was treated with carfilzomib but quickly progressed. The patient eventually achieved a very good partial response with bendamustine and dexamethasone and underwent reduced intensity conditioning with fludarabine 30 mg/m2 on days − 6 to − 2 and melphalan 50 mg/m2 on days − 3 to − 2, followed by a 9/10 matched unrelated allogeneic SCT in November 2012. GVHD prophylaxis consisted of sirolimus and tacrolimus starting day − 3 as well as methotrexate on days + 1, 3, 6, and 11. On day + 27 post-transplant the patient developed acute kidney injury (creatinine of 2.6 mg/dL from a baseline of 0.7) that was attributed to calcineurin inhibitor toxicity. The patient was switched to mycophenolate mofetil and corticosteroids for GVHD prophylaxis, with normalization of kidney function. By day + 130 the patient was felt to be in at least very good partial remission based on negative serum protein immunofixation and 99.8% peripheral blood donor chimerism.\nOn day + 132, the patient returned to the hospital with diarrhea with scant blood. He underwent colonoscopy with biopsy. Histologic analysis demonstrated findings consistent with CMV colitis and GVHD: crypt apoptotic bodies, ulcerations, and CMV inclusions were noted. He was started on ganciclovir, and prednisone was increased from 60 mg daily to 60 mg twice daily. He was discharged 2 weeks later, at which time the platelet count had decreased from 93,000/μL on admission (normal 150,000–450,000/μL) to 29,000/μL. The thrombocytopenia was attributed to a combination of antiviral medication and CMV infection.\nHe returned to the hospital 1 week later (day + 146) with recurrence of profuse diarrhea with small amounts of blood and associated abdominal cramping. Diarrhea was attributed to worsening GVHD. He was restarted on tacrolimus but continued to have maroon-colored stool output. A colonoscopy was repeated and was notable grossly for pancolitis with scattered ulcerations, ileocecal valve ulceration, mild ileitis, anorectal junction ulcers, and internal hemorrhoids that were not bleeding. The pathology report again suggested CMV infection and GVHD (increased crypt apoptotic bodies, focal erosions, and a positive CMV immunostain). During that admission, a diagnosis of TMA was considered due to a persistently low hemoglobin of approximately 9 g/dL (normal 13–17 g/dL) and thrombocytopenia that persisted in 30,000–50,000/μL range. A peripheral smear at that time showed 2–4 schistocytes/HPF in the setting of a haptoglobin of 22 mg/dL (normal 36–195 mg/dL) and a lactate dehydrogenase (LDH) of 731 U/L (normal < 260 U/L). Overall, the patient met 6 out of 7 of the TA-TMA criteria proposed by Cho et al. His tacrolimus was again discontinued, and he was maintained on a combination of sirolimus (goal level 5–7), mycophenolate mofetil, and steroids for GVHD treatment. At the time of discharge, his hemoglobin was 8.7 g/dL and platelets were 20,000/μL.\nOn day + 156 the patient returned to the hospital with multiple episodes of bright red blood per rectum. He had diffuse patchy ecchymoses on physical exam. The platelet count was 17,000/μL. A colonoscopy was repeated, and per visual inspection the GVHD was felt to be improved. Hemorrhoidal bleeding was suspected as the cause of the bleeding, and colorectal surgery was consulted. He underwent sclerotherapy for hemorrhoids prior to discharge.\nOn day + 188 TMA was again considered in the setting of an LDH of 1147 U/L. Sirolimus was discontinued. A peripheral blood smear, however, showed no schistocytes, and haptoglobin returned within low-normal range at 43 mg/dL. TMA was therefore felt to be unlikely. A CT scan of the abdomen and pelvis, performed for another indication, revealed an unexpected finding of pneumatosis intestinalis involving the ascending and transverse colon. This finding was attributed to GVHD and CMV and, as the patient was felt to be relatively asymptomatic, no specific intervention was performed. Sirolimus was restarted given the concerning imaging findings and lack of strong evidence for TMA.\nThe patient was re-admitted on day + 211, this time with melenic stool. A tagged red blood cell scan identified the ascending colon as a source of bleeding. An angiogram with attempted embolization was unsuccessful due to inability to identify a bleeding vessel. Hemolysis labs were repeated given persistent cytopenias, including a hemoglobin of 7.1 g/dL and a platelet count of 37,000/μL. Haptoglobin was undetectable, and LDH was elevated at 1254 U/L. A repeat peripheral smear demonstrated a normocytic, normochromic anemia without increase in schistocytes. Sirolimus was again discontinued. The patient remained anemic and intermittently refractory to red blood cell transfusions, prompting two additional attempts at angiogram/embolization, neither of which successfully identified a bleeding vessel. A bone marrow biopsy was repeated and revealed a hypocellular marrow without evidence of myeloma relapse.\nOn day + 217 the patient was transferred to the medical ICU for high volume bloody stool output, a hemoglobin of 6.3, and lightheadedness. The INR was 1.0 with a PTT of 22. A wireless capsule endoscopy and a repeat tagged red blood cell scan were both unsuccessful at identifying a source of bleed. A colonoscopy with biopsy was repeated; 2 visible vessels were identified and clipped. Histologic analysis did not show evidence of persistent GVHD or CMV colitis. An EGD with biopsy revealed no abnormalities on histologic analysis. He was managed supportively with transfusions as needed but then began to develop confusion, agitation, and increasing anger. Psychiatry was consulted for delirium. LDH was 767 U/L, and the haptoglobin was low at < 35 mg/dL. Platelets were 47,000/μL, and the INR was 1.1. A peripheral smear was repeated and did not show schistocytes. An ADAMTS13 level was ordered given recurrent suspicion for TMA, and therapeutic plasma exchange (TPE) was initiated empirically pending that result. The patient’s confusion was noted to improve following TPE, and he completed a total of 5 exchanges. LDH improved to 372 U/L. ADAMTS13 level ultimately returned at 60% (normal > 66%). Shortly thereafter, the patient developed copious hematochezia associated with a drop in hemoglobin from 8.9 to 6.9 g/dL. He was taken urgently to the operating room for visceral angiogram, which did not identify a bleeding vessel. An ileocolectomy was performed, but the patient suffered a cardiac arrest intra-operatively and expired shortly thereafter.\nA post-mortem analysis of the resection specimen revealed TMA involving numerous arteries and arterioles in the ileal and colonic submucosa as well as few thrombosed arterioles in the muscularis propria and deep lamina propria of the mucosa (Figs. and ). Rare thrombosed arterioles were identified in the appendix. The TMA in many of the vessels was active, characterized by endothelial cell swelling, endothelial cell detachment with intimal expansion by pale plasma protein material (“mucoid intimal edema”), and accumulation of fibrin and red cell fragments within expanded intimal zones and vascular lumina. A smaller number of arteries and arterioles showed features of persistent or chronic microangiopathic changes, such as concentric layers of new basement membrane material alternating with intimal edema well as focal presence of foam cells within artery intimal zones. All venous structures were patent without thrombosis. In areas of more severe TMA, there were foci of perivascular hemorrhage and deep ulceration extending into the superficial muscularis propria. Non-ulcerated mucosa showed architectural distortion and crypt regenerative features, consistent with previous injury. Additionally, there were areas of crypt dropout with replacement of mucosa by healing granulation tissue and an overlying layer of regenerative epithelium. Scattered CMV inclusions were visible within endothelial cells of the granulation tissue. However, there was no evidence of CMV infection by light microscopy or immunohistochemistry within endothelium of vessels affected by TMA. There was no significant increase in crypt apoptosis, arguing against GVHD.\nA retrospective review of the patient’s previous colonic biopsies was performed. In addition to the initially reported findings, biopsies from days 146 and 217 showed subtle features of TMA (Fig. ); these included rare thrombosed arterioles in the deep lamina propria and superficial submucosa surrounded by perivascular hemorrhage as well as few arterioles with subendothelial expansion by pale material accompanied by underlying layers of new basement membrane material.","This patient had a long and complex battle with multiple myeloma and then relapsed several times despite multiple treatments. He eventually achieved a partial recovery, but continued to experience problems with bleeding in his colon, leading to several hospitalizations and ultimately, a cardiac arrest. Investigations revealed a rare condition called TMA, which caused blood clots to form in his small blood vessels, leading to significant bleeding. Despite multiple treatments and interventions, the patient sadly passed away."
614,"A 25-year-old woman was hospitalized due to frequent premature ventricular beats of high grade (17,000 per day) and repeated episodes of bidirectional non-sustained ventricular tachycardia without syncope. Echocardiography revealed enlarged left ventricular dimension and local ventricular wall thinning. Upon routine clinical examination bilateral symmetrical hand abnormality was noted, namely the fifth finger camptodactyly (Figure ). Additionally, hypoplasia of the breast with inverted nipples was observed (Figure ). Facial features included wide-set eyes, a broad nasal tip and thin upper lip vermilion and strabismus (Figure ). Dental abnormalities were represented by tooth malalignment and hypoplasia involving canines and back teeth (Figures ). No defects were documented in her lower limbs. Apart from physical defects, intellectual deficit was noted and included mild mental retardation and learning disabilities. Family history reported that proband’s mother died due to congenital heart defect and congestive heart failure at the age of 30. Grandmother from mother side was not affected. No other relatives were available for examination. Due to the lack of family data, it is hard to conclude the mode of inheritance unambiguously. However, keeping in mind the mother’s phenotype, the dominant inheritance could be suggested (Supplementary Figure ).","This 25-year-old woman was admitted to the hospital because of a rapid heart rate with occasional dangerous heart rhythm problems. Tests showed her heart was enlarged and she had some physical differences, including a curved fifth finger, underdeveloped breasts, and specific facial features. She also has some learning challenges and dental issues. Her mother had a similar heart condition that led to her early death, suggesting a possible inherited condition."
615,"The patient, a 52-year-old female, was admitted to the department of gastrointestinal surgery of Peking University Cancer Hospital & Institute in September, 2016, due to space-occupying lesions in the colon found by colonoscopy during medical examination 2 weeks before. Histopathological examination of endoscopic biopsy specimens indicated moderately differentiated colonic adenocarcinoma. Contrast-enhanced computer tomography (CT) of her abdomen demonstrated that the intestinal wall was thickened about 14 mm in the transverse colon, and several small lymph nodes (7 mm) scattered around the intestine were detected (Fig. and ). Laboratory examination revealed that the levels of CEA and CA72.4 increased to 15.17 ng/ml and 20.88 U/ml respectively. Laparoscopic examination confirmed the tumor (6 cm × 5 cm) was located in the hepatic flexure of the colon (Fig. and ).\nThe patient (III7) had no other major medical history, except a family history of colon cancer in 3 out of 5 first-degree relatives (mother II2; sister, III5; brother, III6). In particular, the patient’s mother (II2) was diagnosed two separate primary colon cancer at the age 54 and 61 at different sites. Her sister (III5) was diagnosed with endometrial cancer and colon cancer at the age of 54 and 61 respectively (Fig. ). The patient was referred to our cancer genetic counseling clinic for LS genetic testing. Based on Amsterdam II criteria, the proband was diagnosed with LS.\nTo confirm the diagnosis, all affected individuals (III5, 6 and 7) underwent genetic testing of a 101-gene panel by next generation sequencing. Peripheral blood was collected to extract genomic DNA (gDNA). The gDNA was then used to generate libraries according to the protocols suggested by Illumina. A custom targeted capture kit, covering all exons of the 101 genes, was designed (Agilent Technologies, Additional file : Table S1) []. Qualified libraries were subsequently sequenced on the Illumina HiSeq 2500 platform with 2 × 150 bp configuration. Reads were aligned to the reference human genome GRCh37 with BWA and PCR duplications were marked using Picard tools (version 1.57). To further increase the specificity for mutation calling, realignment and base recalibration were conducted using Genome Analysis tool kit (GATK). All samples were tested at least in an average depth of 200-fold coverage. Bases with a minimum of 30-fold coverage was required at every targeted position (Additional file : Table S2). The missense, nonsense, indel and splice site mutations that located at the upstream or downstream 1-2 bp of exon, whose frequency are below 5% in at least one pubic population database were retained (Additional file : Table S3). According to the American College of Medical Genetics (ACMG) standards and guidelines for the interpretation of sequence variants, all the gene variants were classified into 5 grades. Therefore, 14 mutations at least carried by two first-degree relatives were listed. A pathogenic variant (class 5) in MLH1 (c.1852_1854delAAG, p.K618del) was identified in all patient’s blood samples (Table ).\nLaparoscopy-assisted colectomy was performed on the proband to resect right colonic mass and its surrounding tissue followed by ileocolonic anastomosis. In addition, the clinicopathologic stage was pT3N0M0 and no complications occurred in the perioperative period. Conventional hematoxylin and eosin staining and immunohistochemistry were performed on resected specimens to confirm the malignancy (Fig. and ). Additionally, abdominal CT of III5 showed a obstructing mass in the same location of colon as the proband (III7) (Fig. and ). Moreover, according to the medical record for the proband’s brother (III6) in another hospital, a tumor was found in his hepatic flexure of colon. Immunohistochemistry results showed MMR deficiency in all tumor tissues of the 3 cancer patients (Table ). Subsequently, MSI testing was performed using MSI Analysis System Version 1.2 (Promega). Tumor DNA was extract from formalin fixed paraffin embedded sections. Genomic DNA extracted from white blood cell was used as normal control. Seven markers were amplified using fluorescent PCR. The PCR products were separated by capillary electrophoresis using Applied Biosystem 3130 Genetic Analyzer. GeneMapper Analysis Software was used to analyze the output data. The MSI results indicated that the 3 siblings with cancer (III5, 6 and 7) were all microsatellite instability-high (MSI-H) (Table ). Based on the results of MSI and the proband’s clinical stage, no adjuvant chemotherapy was given after surgery.\nTo screen and evaluate the cancer developing risk in the offspring, children of affected individuals were also enrolled for genetic testing. And 1 (IV8) out of 3 carries the same MLH1 mutation to the proband. However, this carrier has no symptoms or confirmed diagnosis of cancer. Endoscopy was performed on IV8 for further examination and the ileocecal mucosa showed signs of dysplasia, including chronic inflammation with erosion, lymphoid hyperplasia and mild atypical hyperplasia of glandular epithelium (Fig. ). The asymptomatic individual was given oral administration of aspirin as a preventative treatment, and 6-months follow-up showed improved appearance with colonoscopy examination (Fig. ). A healthcare plan was proposed to this offspring including colonoscopy and urine test once a year, and gastroscopy every 3~ 5 years after 35 years old. All individuals carrying the MLH1 mutation in this family will be monitored on a long term basis.\nThe CARE guidelines were followed in reporting this case.","This woman was diagnosed with colon cancer after a routine screening colonoscopy. The cancer had spread to nearby lymph nodes, and she has a family history of colon cancer, with several relatives also diagnosed with the disease. Genetic testing revealed that she carries a specific gene mutation that increases her risk of cancer, and her siblings also carry the same mutation. After surgery to remove the tumor, she is being monitored closely for any signs of recurrence."
616,"Patient 2, a 1-year-old boy, presented with microcephaly (OFC 43.5 cm; −2.8 SD) and attacks of abnormal arm extension. His motor and speech development were delayed. He showed truncal hypotonia on physical examination. His length was 84 cm, he weighed 10 kg. EEG results were normal. MRI showed a short and hypoplastic corpus callosum of which the splenium was affected more than the rostrum (Figure ). He had an upward slant, a small and somewhat sloping forehead, depressed nasal bridge, small and upturned nose tip and nostrils, elongated philtrum and a thin upper lip. A de novo nonsense variant was found: Chr1(GRCh37):g.244217655G>A, NM_205768.2(ZBTB18): c.579G>A (p.(Trp193*)) that leads to a premature stop codon.","This 1-year-old boy came to the doctor because his head is smaller than expected and he’s having trouble moving his arms properly. He’s also behind in learning to talk and has weak muscles in his trunk. An MRI scan showed a small connection between his brain’s two halves, and a genetic change was identified that may be contributing to these issues. The doctors will continue to monitor his development and provide support."
617,"Patient 3, a 13-year-old boy, presented with mild to moderate speech and developmental delay and attention deficit disorder (ADD). He did not have hypotonia. His OFC was 52.5 cm (−1.25 SD). He was 156 cm tall (−0.75 SD). No structural brain anomalies were seen on MRI. He had retrognathia, mild hypertelorism, and a slightly elongated philtrum and thin upper lip. His hands were broad and short. Mild syndactyly of the second and third toe with a sandal gap were seen in both feet. WES analyses showed a de novo frameshift variant Chr1(GRCh37):g.244217335del, NM_205768.2(ZBTB18):c.259del(p.(Leu87Cysfs*21)), that leads to a premature termination codon located more than 400 codons upstream of the canonical termination codon.","This 13-year-old boy came to the clinic because he has some challenges with speaking and learning, and he has attention difficulties. His physical exam showed some features like a small jaw, wide hands, and slight differences in his feet. Brain scans showed no problems with his brain structure. Genetic testing revealed a new change in a gene that may be contributing to his developmental differences."
618,"Patient 4, a 4-year-old boy, presented with severe speech delay, motor delay, and hypotonia. MRI showed agenesis of the splenium of the corpus callosum. At 3 years of age, an OFC of 49 cm was measured (−1 SD). His height was 98 cm (0 SD). He had hypertelorism, a prominent nasal tip, and a bulbous nose, a small mouth and retro- and micrognathia. His fingers showed broad tips. He carried a missense variant in ZBTB18 (Chr1(GRCh37):g.244218467G>A, NM_205768.2(ZBTB18):c.1391G>A(p.Arg464His)). This heterozygous de novo missense variant is predicted to be deleterious (SIFT score 0; Polyphen score 0.991) and affects a highly conserved amino acid residue located in the ZNF domain of the ZBTB18 protein (conserved up to Tetraodon). This variant has not been found in individuals from the ExAC database.\nWe reviewed four patient cohorts containing one or more patients with pathogenic variants in ZBTB18 (Cohen et al., ; Depienne et al., ; Lopes et al., ; Rauch et al., ) and included one case report (de Munnik et al., ). So far, a total of 25 patients with a pathogenic ZBTB18 variant have been reported in literature and in this study. All patients presented with developmental delay in varying degrees with prominent speech delay. Fifteen patients underwent an MRI scan. Nine of them showed corpus callosum abnormalities. Results of clinical evaluation of congenital anomalies in 13 patients were present: dysmorphic facial features were seen in 10 patients, epilepsy was described in five patients, hypotonia in seven, and dystonia in two. Data about growth, development, neurological, or congenital anomalies was incomplete in 13 cases. Clinical data of cases included in this study and patients from literature are presented in Table . Variants in the ZBTB18 gene are schematically depicted in Figure .","This 4-year-old boy has significant delays in speech and motor skills, along with very low muscle tone. An MRI scan revealed a missing part of his brain, and genetic testing identified a new variation in a gene called ZBTB18 that may be responsible for these issues. Other children with the same genetic variation have also experienced developmental delays, including speech problems and brain abnormalities. Doctors are continuing to monitor his development and provide support to help him reach his full potential."
619,"A 60-year-old man was transferred to our department with complaints of chest distress and wheezing for 3 d. The patient reported no significant comorbidities apart from a 3-year history of hypertension. An examination revealed the following: temperature, 36.8 °C; blood pressure, 162/98 mm Hg; pulse rate, 78 beats/min; and respiratory rate, >20 breaths/min. He was thin and had no eyelid edema. The breath sounds over both lungs were rough, and a few moist rales were heard. The abdomen was soft, without tenderness or rebound pain.\nThe laboratory findings were as follows: serum creatinine, 1283 μmol/L (25–123 μmol/L); blood urea, 40 mmol/L (2.29–7.2 mmol/L); white blood cells, 12.6 × 109/L (3.5–9.5 × 109/L); red blood cells, 4.12 × 1012/L (3.8–5.1 × 1012/L); hemoglobin, 107 g/L (115–150 g/L); platelets, 269 × 109/L (125–350 × 109/L); B-type natriuretic peptide, 146 pg/mL (0–100 pg/mL); prothrombin time, 12.00 s (10–14 s); activated partial thromboplastin time, 35.50 s (23–35 s); fibrinogen, 4.01 g/L (2–4 g/L); K+, 6.2 mmol/L (3.5–5.3 mmol/L); Na+, 133 mmol/L (135–145 mmol/L); calcium, 2.05 mmol/L (2.2–2.7 mmol/L); phosphorus, 2.13 mmol/L (0.5–1.5 mmol/l); and parathyroid hormone, 472 pg/mL (16–65 pg/mL). Tumor marker, thyroid-function, chest X-ray, and electrocardiographic assessments yielded normal results. Chest computed tomography (CT) revealed inflammatory areas in both lungs and bilateral pleural effusion. Urinary tract color ultrasonography showed that the left kidney measured 9.0 cm × 5.1 cm and had 1.2-cm-thick parenchyma; the right kidney measured 8.8 cm × 5.0 cm, and had 1.3-cm-thick parenchyma. No abnormalities were observed in the ureters or bladder. Owing to the risk of cardiac arrest because of hyperkalemia, hemodialysis was performed. The right femoral vein was catheterized under local anesthesia, and 5000 IU low-molecular weight heparin was administered for anticoagulation.\nFour hours after LMWH was administered, the patient felt increasing pain in the lower abdominal region after straining at stool and an urgent desire to micturate. A physical examination revealed a tender mass in the lower abdomen. Urgent bedside urinary tract ultrasonography showed large amount of fluid in the bladder. A 20-Fr in-dwelling catheter was inserted, but there was no obvious outflow of urine through the catheter, and the abdominal pain was not improved. Therefore, abdominal and pelvic CT was performed, which revealed marked hematoma that extended to the prevesical space and bilateral rectus sheath hematomas (RSHs; type III). The following emergency measures were undertaken immediately: bed rest, ice bag application, and compression bandaging. In addition, a blood coagulation profile was obtained, which showed the following: prothrombin time, 12.80 s (10–14 s); activated partial thromboplastin time, 47.40 s (23–35 s); and fibrinogen, 3.04 g/L (2–4 g/L). Given the abnormal coagulation function and risk of continuing hemorrhage, we transfused the patient with 180 mL fresh frozen plasma, supplemented with the injection of 2 U hemocoagulase agkistrodon for hemostasis and 2 U red blood cell suspension.\nWe then performed heparin-free continuous renal replacement therapy (CRRT) 3 times per week, and closely monitored the patient. After 1 week, a repeat CT showed that the hematoma had not expanded. After six sessions of heparin-free CRRT, the hematoma was slightly reduced, and did not recur when 2500 IU LMWH was administered for hemodialysis. We then increased the heparin dose to 5000 IU without further complications. A follow-up clinical examination 6 months later showed that the abdominal mass had completely resolved.","This 60-year-old man came to the hospital with chest pain and difficulty breathing. Tests showed inflammation in his lungs and fluid around them, along with high levels of potassium in his blood, which led to a need for immediate dialysis. A CT scan revealed a large hematoma (blood collection) in his abdomen, which was causing pain and preventing urine flow. After receiving blood transfusions and continuous dialysis, the hematoma began to shrink, and he was closely monitored for several months until the problem completely resolved."
620,"Patient 1 is a 74-year-old male who underwent HoLEP for refractory bladder outlet obstruction and bladder stones. His medical history included BPH complicated by recurrent urinary tract infections and bladder stones, elevated prostate specific antigen (biopsy negative), hyperlipidemia, and hypertension. Preoperative transrectal ultrasound (TRUS) estimated prostate volume to be 150 cc. HoLEP was performed utilizing a two-incision technique. Owing to the large amount of adenoma, extended time was spent during morcellation (120 minutes) because of poor observation secondary to bladder neck bleeding. There was noted to be an area of capsular perforation at the 5 o'clock position in the mid gland. When the operative drapes were removed, significant abdominal distention was noted.\nIn discussion with anesthetist, the patient's airway pressures upon induction ranged from 10 to 20, however, at this point in the procedure, the airway pressures had increased >30. The patient was also experiencing systolic pressures ranging from 80 to 90, whereas preoperatively he was >110 systolic. The drapes were removed at this time and the abdomen appeared distended and was firm on examination. Given the significant abdominal distention and concern for a bladder injury secondary to poor observation during morcellation, general surgery was consulted intraoperatively. Per the recommendation of general surgery, they elected to proceed with a subumbilical minilaparotomy, after initial laparoscopy was unsuccessful because of increased opening pressures with the Veress needle. Less than 400 cc of bloody-colored fluid was suctioned out of the abdomen, and an intraoperative cystogram was performed that revealed retroperitoneal extravasation without intraperitoneal bladder injury or perforation. As there was not a significant amount of intraperitoneal fluid, it was theorized that the capsular perforation resulted in extraperitonealization of intraoperative saline. Thus, a 10F Jackson Pratt drain was placed in the pelvis, and the fascia and skin were closed. He was given 20 mg of IV Lasix intraoperatively.\nThe patient was extubated effectively and transferred postoperatively to the ICU for hemodynamic monitoring and observation. In the ICU, cardiology was consulted because of a prolonged PR interval and bradycardia that ultimately warranted no further work-up. On postoperative day (POD) 1, the patient was progressing well, and he was transferred to the floor in stable condition. The Jackson-Pratt drain output was 710 cc on POD 0, 81 cc on POD 1, and then removed on POD 2 after draining 20 cc. The patient was discharged on POD 3. His catheter was removed on POD 10. Pathologic analysis of the specimen revealed no evidence of malignancy and 167 g specimen.","This 74-year-old man had a blocked bladder due to prostate enlargement and stones, and underwent a surgery called HoLEP to clear the blockage. During the surgery, the doctor noticed some bleeding and had to carefully cut and remove the prostate tissue, which took longer than expected. Because of the bleeding and a buildup of fluid in his abdomen, the surgical team had to perform additional surgery to drain the fluid and ensure there was no damage to the bladder. The patient recovered well and was discharged home on day 3 after his catheter and drain were removed, and the pathology report showed no cancer."
621,"Patient 2 is a 78-year-old male who was experiencing persistent lower urinary tract symptoms despite combined medical therapy with alpha blockade and 5-alpha reductase inhibitors. His medical history included coronary artery disease status post-coronary artery bypass grafting and percutaneous coronary intervention, and a history of pneumonia. Preoperative cystoscopy revealed enlarged median and lateral lobes, as well as severe trabeculations of the bladder with a TRUS measuring a 41 cc prostate. HoLEP was carried out utilizing a two-incision technique. Upon completion of morcellation, it was noted that the patient's abdomen was distended, but his peak airway pressures were normal, the abdomen was soft, and the catheter drainage was noted to be clear. In addition, there was no suspicion for a significant mismatch between irrigation used and fluid output collected in the drainage system.\nGiven the previous similar presentation in Case 1 with no suspicion of bladder injury, we suspected that the patient had extraperitoneal extravasation of the saline irrigation through a capsular perforation as occurred in Case 1. The decision was made for the patient to be awakened, extubated, and transferred to the recovery room where he was further monitored. A stat noncontrast abdominal CT scan was performed that revealed a moderate amount of free fluid in the pelvis and upper abdomen; the fluid in the pelvis and lower abdomen was distributed in the extraperitoneal region with no evidence of hematoma (). The patient remained hemodynamically stable and was transferred to the floor with continuous bladder irrigation. The patient was given a 40 mg dose of Lasix ∼8 hours after the operation was completed. Overnight, there were no acute events. On POD 1, the patient's abdomen was soft and significantly less distended. The Foley catheter drained 3950 cc of urine overnight without evidence of hematuria. The patient was discharged with a catheter on POD 1. The patient had his catheter removed on POD 9. A postoperative CT cystogram revealed no evidence of leak with resolution of the pelvic and perivesical fluid (). Thirty grams of benign prostate tissue was removed on final pathology analysis. The patient was noted to have a bladder neck contracture seen on cystoscopy 4 months after his procedure for which he underwent cystourethroscopy and laser incision of bladder neck contracture.","This 78-year-old man was having trouble with his bladder and was treated with surgery to remove excess prostate tissue. During the surgery, there was a small leak of fluid outside the bladder, which was discovered and monitored closely. After the surgery, he was closely watched and treated for fluid buildup around his bladder. He recovered well and was eventually discharged home with a catheter, which was removed after a few weeks. Finally, a follow-up scan showed that the leak had stopped, and he underwent a procedure to address a bladder neck issue."
622,"A 19-day-old male infant was urethral catheterized with a 6F infant feeding tube followed by a VCUG for evaluation of vesicoureteral reflux. After the procedure, the catheter could not be removed. Therefore, the pediatric unit consulted us regarding the problem. Under fluoroscopic guidance, a guidewire was inserted through the feeding tube to uncoil it, but this failed (). Therefore, we planned to cut the knot endoscopically under general anesthesia. The patient was taken to the operating room and put under general anesthesia. Considering the relaxation effect of the anesthesia before cystoscopy and the danger of urethral trauma, it was then planned to remove the catheter by gently pulling it out. Since there was no resistance encountered and no serious tension on the catheter was observed, the operation proceeded as planned. The catheter seemed to be removed with ease. There are cases in the literature that report of removing knotted catheters using the said method. After the catheter was removed fully with the knot at the tip (), cystoscopy was performed to check for any potential urethral injury. Mucosal integrity of the urethra was observed to be intact and the patient was discharged after a short follow-up and observing spontaneous micturition.","A 19-day-old baby was seen because he had a blocked catheter in his bladder. Doctors used X-rays and a special procedure to try to loosen the knot in the catheter. The catheter was successfully removed under anesthesia, and the baby’s bladder was examined to make sure it was healthy. The baby was discharged home after a short check-up and is now urinating normally."
623,"The patient was a 65-year-old woman who presented with a 1-month history of bloody stool. A digital colonoscopy with biopsies revealed adenocarcinoma in the sigmoid colon. The patient elected to undergo primary laparoscopic colon resection, and the procedure was reported to be uneventful. However, on the 6th postoperative day, the patient noticed a large amount of yellow fluid coming out of a left side abdominal drain. The fluid appeared to be urine. An abdominal ultrasonography showed a collection of fluid in the patient's pelvis. A contrasted computed tomography (CT) scan showed contrast extravasation in the pelvis and around the descending colon (). The patient had decreased serum protein and albumin; however, complete blood count, creatinine, liver functions, and urine analysis were normal. On the 8th postoperative day, the patient was taken to the operating room for a ureteroscopy. The ureteroscopy revealed that the left ureter was completely severed about 4 to 5 cm from the ureteral orifice (, transected distal end of the ureter). The bowels could be seen through the ureteroscope (, intraabdominal cavity with bowels seen through the distal end of the transected ureter). No other obvious injury was identified. With patience, persistence, and some difficulty, the severed upper end of the ureter was identified and entered (, the proximal end of the transected ureter). We estimated that there was a 3- to 4-cm gap between the two ends of the ureter. Two 0.035″ guidewires were first passed, followed by the placement of two 4.5F Double-J ureteral stents. After placing the Double-J stents, the abdominal drainage quickly subsided. An abdominal ultrasonography 6 days after tube placement showed complete resolution of the abdominal fluid collection. The abdominal drain was removed and the patient was discharged. At the 3-month follow-up, a repeat CT scan showed no hydronephrosis, no abdominal fluid collection, and no contrast extravasation. The patient, however, had an asymptomatic urinary tract infection from Klebsiella pneumoniae, which was treated and resolved. A follow-up ureteroscopy over a guidewire showed excellent healing and realignment of the disrupted ureter. The only obvious sign of the transacted ureter was that the mucosa was paler than normal. Because of this unconventional treatment, we decided to continue stenting the ureter with two fresh 4.5F Double-J stents for an additional 4 weeks. Stents were removed 4 months after the injury. Follow-up contrast-enhanced CT scans taken 8 and 14 months after the initial endoscopic treatment showed mild but unchanged residual dilation of the renal pelvis and ureter with good drainage. There were no other abnormalities.","A 65-year-old woman was found to have cancer in her colon and underwent surgery to remove the affected part. After the surgery, she experienced a leak of urine into her abdomen, which was caused by damage to her ureter. Doctors performed a procedure to repair the damaged ureter and placed stents to help drain the urine. The leak stopped, and the patient’s condition improved, but she developed a urinary tract infection which was treated. After several months of follow-up, the ureter healed, and the stents were removed, with no further problems."
624,"A 12-year-old Cameroonian girl from the ""Baka"" ethnic group and residing in a remote area of the East region of Cameroon presented with a progressively extensive, pruritic, and painless pigmented skin lesion on her back, persistent since she was 2-days old. She was born through normal vaginal delivery at term from an uneventful pregnancy. Her past medical and family histories were unremarkable. On our initial physical examination, she had normal anthropometric characteristics for age, as well as normal vital parameters. Examination of her skin revealed a large, irregular, well-demarcated and unequally pigmented (bluish-brown to black) multinodular hypertrophic nevus occupying almost all her back (Fig. ). The largest diameter of this lesion was 45 cm. Its surface was rough and had several excoriation marks. No other malformation was apparent. An examination of her lungs, heart, abdomen, and extremities was otherwise normal. Despite the unavailability of histopathology in our setting, the aforementioned clinical findings were highly suggestive of a GCMN. She was scheduled for a free of charge surgical campaign due within the same year in her community. This surgical excision would provide several benefits namely the reduction of the risk of melanoma, improvement in aesthetics, and obtaining histopathology samples. Taking into consideration the psychosocial aspect of this pathology, our patient and her parents were also oriented to the consult of a psychologist. Meanwhile, her parents were counseled on signs of complications which should warrant urgent admission. At 3-month follow-up, she was still pending surgical intervention. Currently, she is being followed-up clinically and psychologically on a weekly basis while waiting for surgery.","A 12-year-old girl from Cameroon has a large, unusual, and dark-colored skin growth on her back that has been present since she was a baby. The growth is itchy and doesn’t hurt, but it’s quite large and has some rough spots. Doctors believe it’s likely a genetic cutaneous mosaic nevus, a rare type of birthmark. She is scheduled to have the growth surgically removed, and she is also receiving support from a psychologist to help her cope with the situation while she waits for the surgery."
625,"A 52-year-old menopausal woman complained of intermittent vaginal spotting for 1 month. She denied any systemic disease, dysmenorrhea, menorrhagia, body weight loss, abdominal pain, or abdominal fullness. Gynecologic history was gravida 2 and para 2. Transvaginal ultrasound revealed a 10-cm multi-lobular cystic pelvic mass containing the mixed heterogeneous solid component, fluid and papillary growth in the inner surface of cystic wall Significant venous flow was detected in the solid part and papillary growth lesion. Serum AFP (< 20 ng/ml), cancer antigen (CA)-125 (< 35 U/ml), and carcinoembryonic antigen (< 5 ng/ml), and CA19–9 (< 37 U/ml) were 60,721, 38.1, 84, and 97 ng/ml, respectively. All of these tumor markers from serum have their own specific cut off values and sensitivities, and they come from the same assay methods and from the same laboratory. All are elevated. Computed tomography (CT) showed a 9-cm heterogenous mass probably developed from the left adnexa (Fig. ) and a 4-cm well-defined mass located at the right subphrenic region (Fig. ), suggesting the diagnosis of left ovarian carcinoma with peritoneal seeding.\nThe patient underwent optimal debulking surgery, including total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, pelvic lymphadenectomy, and para-aortic lymphadenectomy (Fig. ). All gross tumors were almost completely resected. Histologically, sections of the left ovarian tumor showed a clear cell carcinoma (Fig. ). The tumor is composed of polygonal, cuboidal to columnar cells with clear cytoplasm arranged in solid nests and tubule-cystic growth patterns. Numerous hyaline globules are present. Some tumor cells also showed high-grade anaplastic nuclear features. Right ovarian tumor showed metastatic clear cell carcinoma (Fig. ). Typical yolk sac tumor differentiation and Shiller-Duval body were absent. Sal-like protein 4 (SALL4) was strongly positive (Fig. ). Immunohistochemically, the tumor cells were positive for glypican-3 (GPC3) and AFP and negative for CD30. Meanwhile, the tumor cells showed weak positive staining for hepatocyte nuclear factor1-beta (HNF-1beta) (Fig. ). The tumor cells were focally positive for cytokeratin (CK) 20 and Cdx2 (caudal type homeobox 2) and negative for CK7 (Fig. ). To rule out potential artifacts due to antibody issues, we titrated the antibody used for AFP staining (Fig. ). For every immunohistochemical stain, positive controls were included. As a negative control, we also stained the classical clear cell carcinoma (Fig. ). Combination of all made a diagnosis of AFP-producing clear cell type EOC with fetal gut differentiation, FIGO (International Federation of Gynecology and Obstetrics) IIIc.\nAfter surgery, the patient received adjuvant multi-agent chemotherapy (carboplatin and paclitaxel). After treatment, CT showed a 2-cm hypodense nodule at the right subphrenic region and wedge-shaped hypoperfusion over segment 7 of the liver, suspicious tumor seeding, and liver metastasis (Fig. ) and serum level of AFP was 1831 ng/ml. Tumor excision for liver metastasis was done. Dose-intensity chemotherapy with weekly paclitaxel was given. CT scan, 1 year later, showed a lobulated mixed solid and cystic lesion at the right paracolic gutter measuring 5 cm in size in favor of peritoneal seeding (Fig. ). Exploratory laparotomy was done, including the use of Cavitron Ultrasonic Surgical Aspirator. After operation, chemotherapy was given through intraperitoneal route. After additional 18 months, tumor recurrence was found, including persistent liver metastases (Fig. ), with increasing serum level of AFP of 330,014 ng/ml. The patient finally died of disease.\nBased on our search of PubMed (from January 1960 to July 2018; search terms: “Alpha-fetoprotein”, “clear cell”, “ovarian cancer”; ), there are a few cases of AFP-producing clear cell type EOC available in the literature [–].\nA total of three articles are included and the detailed information, including microscopic finding and immunohistochemical staining results are summarized in Table .\nAll patients were middle aged (range, 54–63 years). Tumor size ranged from 14 cm to 22 cm, but all were advanced FIGO stage (IIb–IIIc). Among these [–], including the current case, half of patients died of disease.","This woman was experiencing irregular bleeding and was found to have a large, complex growth in her abdomen. Tests revealed high levels of certain substances, indicating a type of ovarian cancer. Surgery to remove the growth and surrounding tissues was performed, but unfortunately, the cancer had spread to other parts of her body. Despite chemotherapy, the cancer eventually returned and caused her death."
626,"In December 2006, a 31-year-old woman was referred to a neurologist because of consciousness disorder and fainting. Her main problems were obesity, snoring and waking up with a feeling of suffocation in the middle of sleep. The intraoral examination showed a large soft palate (). The soft palate was scored as class III according to the Mallampati classification (visualization of the soft palate and the base of the uvula) []. The electroencephalogram (EEG) showed focal dysrhythmia during hyperventilation with scattered sharp waves (). The patient was depressed and had sleep disorders such as sleep apnea and myoclonus, especially at the onset of sleep. She had experienced several occurrences of complete loss of consciousness during swimming and at work. The patient was on anticonvulsants and antidepressants (at first, she had been prescribed with Lamotrigine for 5 months, but later she was given 500mg Sodium valproate per day).\nOne of the best treatments for snoring during sleep is UUUP. The success rate of this type of surgery is reported to be between 16% and 83% [].\nWe chose a minimally invasive surgical procedure for the present case since the patient had a proper facial profile and a large soft palate (class III according to the Mallampati classification) [].\nIn May 2007, after analyzing the lateral cephalogram, we evaluated the craniofacial and pharyngeal airway morphology before the surgery. Under general anesthesia, 1cm of the soft palatal mucosa, from the right tonsil to the left tonsil, was removed. The patient’s tonsils were also removed during the surgery, and the anterior and posterior tonsillar pillars were sutured together ().\nThe symptoms were significantly decreased after the recovery. The patient no longer had sleep apnea, and antidepressants and antiepileptic drugs were discontinued. After the surgery, sharp waves were detected on the EEG at the level of the trachea (), but the patient was clinically asymptomatic. The 10-year follow-up showed no symptoms of sleep apnea or seizure. The patient did not lose any weight during the follow-up period.","This 31-year-old woman was referred to a neurologist because she was experiencing episodes of fainting and waking up feeling like she couldn't breathe. Doctors discovered that her large soft palate and snoring were contributing to her problems, along with sleep apnea and other issues. To help with her breathing, she underwent a minimally invasive surgery to remove part of her soft palate and tonsils. After the surgery, her breathing problems improved significantly, and she no longer needed medications for sleep or seizures."
627,"A fit and active 38-year-old female presented to the Accident and Emergency Department with a four-day history of worsening right shoulder pain radiating down the right arm, with swelling around the shoulder. This was accompanied by intermittent fevers for the preceding two days. The patient graded the pain to be 8/10 on a visual analogue scale for pain. The patient reported an episode of right shoulder pain three weeks prior to current presentation which developed while she was boxing with a punch bag and resolved spontaneously in 2-3 days without seeking any medical advice.\nThe patient denied any history of infections in the previous 6 weeks. She had a significant past medical history of cellulitis around the leg 6 months prior and a Bartholin cyst that was treated conservatively 8 months before this presentation. She was not on any routine medications and did not have any predisposing medical conditions such as immunosuppression or diabetes.\nAt presentation, all her observations were essentially unremarkable except temperature which was recorded to be 38.6°C. On examination, the right shoulder was tender and swollen with severely restricted active and passive range of movements. No cellulitis, erythema, or differential warmth was noted.\nHaematological investigations showed mild leukocytosis with a white cell count of 11.1 × 109/L with predominant neutrophilia and a C-reactive protein (CRP) level of 233 mg/L. Liver functions tests, urea and electrolytes, bone profile, and coagulation studies were all within normal limits. Plain radiographs of the chest and shoulder were essentially unremarkable. Shoulder aspirate analysis was negative for any organisms, however showed some scanty pus cells. The patient was started on IV flucloxacillin 1 g intravenous four times a day as she was continuing to have temperature spikes, although shoulder aspirate cultures and blood cultures were negative.\nDue to the patient's severe symptoms and markedly elevated CRP level, urgent magnetic resonance imaging (MRI) of the right shoulder was performed. This revealed marked oedema throughout the subscapularis muscle with a relatively well-defined ovoid area of hyperintensity on short-tau-inversion-recovery (STIR) () and isointensity to muscle on T1 (). The area measured 9 cm on the oblique axial diameter, almost 3 cm in depth, and over 3.5 cm craniocaudally, with fluid extending inferiorly from the subscapular region overlying the chest wall axially measuring over 5 cm transversely and 1.5 cm in depth on T2-weighted images (). This MRI confirmed abscess formation within the subscapularis muscle as the cause of the presentation.\nThe patient underwent surgical open drainage of the right subscapularis abscess under general anaesthesia via a standard deltopectoral approach. During mobilisation of the conjoined tendon, approximately 150 mL of blood-stained pus exuded from the subscapularis muscle. The subscapularis muscle was left with a defect but subscapularis tendon integrity was maintained. Following irrigation, the wound was closed. Cultures of the evacuated pus grew PVL-positive S. aureus, sensitive to flucloxacillin. No per operative signs of intraarticular infection were found, and an on table aspirate yielded no organisms on gram stain and cultures. The case was discussed with musculoskeletal microbiologist, and the patient was given a further two-week course of flucloxacillin.\nAt the 6-week follow-up to assess improvement, the patient's wound had healed well and shoulder pain had resolved with no signs of recurrence of the infection. She still had some restriction in the movement of her shoulder for which she was referred to physiotherapy.","A 38-year-old woman came to the hospital with a painful and swollen right shoulder that was radiating down her arm, along with a fever. She had a similar shoulder problem a few weeks earlier that resolved on its own. Tests showed she had an infection in the muscle of her shoulder, confirmed by an MRI, and the pus was found to be caused by a type of bacteria. She received antibiotics and had the infected area drained surgically. After treatment, her pain and swelling went away, but she still has some limited movement in her shoulder. She is now being referred to physical therapy to help her regain full range of motion."
628,"A 50-year-old female (BMI = 35) presented to our clinic in Salt Lake City, Utah, USA, with a chief complaint of right shoulder pain. She has a history of non-insulin-dependent diabetes, hypertension, anxiety, depression, and fatigue. Her medications included hydroxyzine for anxiety, ibuprofen for joint pains, lisinopril for hypertension, and pioglitazone tablets and liraglutide (Victoza®; Novo Nordisk A/S, Bagsvaerd, Denmark) subcutaneous injections for diabetes. She had a several-year history of intermittent low-grade right shoulder pain that was attributed to subacromial bursitis. This had been treated with subacromial corticosteroid injections and physical therapy, which only gave moderate pain relief.\nHer right shoulder pain worsened acutely in April 2016 after her dog jerked on the leash, almost causing her to fall. Radiographs obtained one month later demonstrated a hooked acromion and subtle decrease in trabecular bone density adjacent to the greater tuberosity, but no distinct bone lesions were noted (). The subtle decrease in trabecular bone was considered to possibly reflect disuse osteopenia associated with a long-standing rotator cuff tear [–]. Subsequent MRI with intra-articular contrast was obtained which demonstrated what was interpreted as a small full-thickness tear of the supraspinatus tendon. The MR images also revealed multiple quasi-circular lesions within the proximal humerus that were suggestive of metastases or multiple myeloma (). Bone lesions were also in close proximity to the insertion of the supraspinatus tendon.\nTen days prior to the radiographs, she had an unrelated skin biopsy of a facial lesion that was diagnosed as sarcoidosis. A biopsy of the humeral lesions seen on MRI revealed noncaseating granulomatous inflammation, confirming osseous sarcoid of the humerus (). Additionally, mediastinal and hilar adenopathy seen on a subsequent chest computed tomography (CT) were consistent with the diagnosis of sarcoidosis.\nWith osseous sarcoid lesions, there is concern that pathologic disturbances in bone architecture could make surgical repair involving cortical bone difficult []. For example, Ungprasert et al. [] reported an increased incidence of fragility fracture in the proximal humerus in patients with sarcoidosis of the humerus. However, given that our patient's humeral lesions involved cancellous bone, while cortical bone was spared, we concluded that the lesions were not a contraindication to a standard surgical repair of the rotator cuff tear. This is because only a small amount of cortical bone needs to be removed during the repair of a small rotator cuff tear [, ]. However, larger tears and/or cases with more invasive sarcoid lesions might require advanced repair techniques [, ]. This was not a concern in our patient's case because during surgery only a low-grade partial tear was found. Surgical treatment was done by JGS and included arthroscopic debridement, bursectomy, and acromioplasty [, ]. The shavings from the surgical procedure were not obtained for histological analysis. However, the tissues did not appear to be grossly abnormal.\nDespite being enrolled in a physical therapy program at two weeks after surgery, she had an unusually prolonged recovery because of flares of pain in addition to shoulder stiffness. Treatment for this included a one-time seven-day course of an oral corticosteroid on a tapered dose schedule (methylprednisolone tablets; Medrol® dose pack; Pfizer Inc., New York, USA). Because the improvement with this was less than satisfactory, two sets of subacromial and glenohumeral corticosteroid injections (Depo-Medrol injectable suspension; Pfizer Inc., New York, USA) were administered two months apart []. Supervised physical therapy was also continued.\nBecause of continuing shoulder and generalized pain flares coupled with a great sense of fatigue, the patient consulted with a rheumatologist who prescribed adalimumab (Humira®, AbbVie Inc., North Chicago, Illinois, USA) subcutaneous injections once every other week. Adalimumab is a tumor necrosis factor-alpha (TNF-α) inhibitor that was selected over other first-line therapies for sarcoidosis because of its greater effect on sarcoidosis-associated fatigue []. Our patient reported that the adalimumab injections greatly improved her fatigue and thereby enhanced her ability to participate in physical therapy. At final postoperative follow-up at 48 months after her shoulder surgery the patient reported that she had an excellent result that included complete pain relief and restoration of full range of motion and strength.","This 50-year-old woman has a history of several health conditions, including diabetes, high blood pressure, and anxiety, and she was experiencing persistent right shoulder pain. After a recent injury, imaging revealed a small tear in her rotator cuff and signs of sarcoidosis, a condition causing inflammation in the bones. She underwent surgery to repair the tear and improve her shoulder function, and has been taking medications to manage her sarcoidosis and fatigue. At her 48-month follow-up, she reported complete pain relief and full shoulder movement."
629,"We present a case of 40-year-old building and construction male worker who slipped and fell from a height of three (3) meters and sustained a deep penetrating wound on the right side of the anterior neck a week prior to presenting at our facility. He was apparently working from the above height when he slipped and fell on a sharp piece of iron rod which penetrated deep into the right anterior neck. He quickly pulled the sharp iron rod out when he got up from the floor. According to him, the bleeding was not profuse and stopped when he arrived at the local hospital to search for remedy (). He did not have hemiplegia, paraplegia, or quadriplegia when we saw him. He is not known to be hypertensive. He did not take alcohol prior to the fall although he takes alcohol occasionally. He had a left femoral fracture at the age of 24 and a right femoral fracture at the age of 32; both incidences were operated on successfully. On examination at our facility we saw a middle aged man who was conscious and alert but however acutely ill with his neck fixed in cervical collar. General as well as systemic examination did not yield much. All the systems where grossly normal. Neurological examination revealed normal pupils which reacted normally to light. Cranial nerves examination was unremarkable. Power on four limbs as well as reflexes was normal. Digital rectal examination revealed a normal spinster tone. Routine laboratory as well as other ancillary (ECG, CXR, etc.) investigations were normal.\nNeck CT-scan done at the local hospital revealed C2-C4 transverse process fractures on the right side, fracture at the right lamina of C3, and right common carotid artery dissection. CT-scan of the head showed no abnormalities (Figures and ). Explorative three-dimensional reconstruction plain and enhanced scan imaging of the cervical spine, chest, and abdomen done at our facility revealed two segmental stenoses of the right common carotid artery with very pale V1 and V3 segment of the right vertebral artery as well as blockage at V2 segment (Figures –) as well as fracture at the right lamina of C3 and C2-C4 transverse processes with free bone fragments and peripheral soft tissue swelling (Figures –). The skin at the right anterior cervical region is discontinuous, with adjacent soft tissue swellings and gas accumulation. The bilateral carotid artery sheath lymph nodes slightly enlarged. At the upper lobe of the right lung there were multiple calcifications, some of which were adjacent to the pleura. There was also slight thickening of the left pleura. The heart was not enlarged but we observed slight accumulation of gas in the anterior mediastinum. Multiple low-density lesions were seen in the liver which we think are constant cysts. A working diagnosis of right common carotid artery dissection with C1-C4 fractures was made.\nAfter preoperative education and counselling of the patient as well as the relatives, surgery was scheduled the next day. Intraoperative cerebral angiography showed right carotid artery dissection and right vertebral artery occlusion. There was some reparation at the distal end of the right vertebral artery. The left vertebral artery was however normal. We introduced the guiding catheter guide wire to the proximal end of the right common carotid artery with continued infusion of heparinized saline, after which we introduced a guide wire with a Cordis stent (10 ∗ 60mm) to completely cover the right common carotid artery dissection site with stenosis and released the stent gradually until it completely filled the stenosis area (Figures –)). We delivered contrast agent into right common carotid artery to make sure it was patent before removing the guiding catheter followed by withdrawal of the femoral arterial sheath. Control contrasted angiograph done revealed stenting was successful (Figures and ). The patient recovered markedly and was discharged home a week after. Scheduled outpatient visit every 6 months for 2 years revealed no neurological complications.","This 40-year-old man was seriously injured when he fell three meters and a sharp piece of metal pierced his neck. He quickly removed the metal, but the injury caused significant damage, including fractures in his neck bones and a tear in his carotid artery. CT scans revealed more problems, including blockages in his arteries and some lung issues. After surgery to repair the artery with a stent, he made a good recovery and was discharged home after a week."
630,"A 45-year-old male from the Netherlands presented with a painless right parotid swelling that was progressively increasing in size for the past 8 months. Though occasionally he suffered from jaw lock, other symptoms associated with neurologic deficit such as drooling, facial weakness, paresthesia, or auditory defects were absent. Apart from being a social alcohol consumer, there was no history of smoking, prior radiation, or significant family medical history, especially in regard to his present illness.\nPhysical examination revealed a tender right parotid swelling below the ear lobule, which extended inferiorly to the angle of the mandible (). The skin overlying the swelling was slightly erythematous, thickened, and nodular. The swelling was firm, diffused, and fixed to the underlying muscles, and there was no associated lymphadenopathy. Otoscopic examination of both ears was within normal limits.\nThe patient initially had a neck ultrasound and then a magnetic resonance imaging (MRI) to characterize the nature of the lesion. The neck CT scan revealed a well-defined altered signal enhancing mass measuring 3.5 × 2.2 × 2.0 cm at the posterior aspect of the superficial part of the right parotid gland (). The radiologist's impression was an altered signal enhancing mass lesion, likely to be a benign pleomorphic adenoma. The patient then underwent MRI of the parotid glands, which showed a well-defined focal lesion of altered signal intensity at the posterior aspect of the superficial part of the right parotid gland, measuring 3.5 × 2.2 × 2.0 cm along its maximum transverse, craniocaudal, and anteroposterior diameters, respectively. The impression was again benign pleomorphic adenoma of the right parotid gland. However, the radiologist could not exclude other diagnostic possibilities and recommend fine-needle aspiration cytology.\nFine-needle aspiration cytology of the mass was nonconclusive as the smears only showed polymorphous population of lymphoid cells in keeping with intraparotid lymph node. In view of these clinical findings, a superficial parotidectomy with facial nerve monitoring and preservation was planned.\nA modified Blair incision was done, with elevation of a skin flap and control of hemostasis during the surgery. The dissection was rather difficult! The superficial parotidectomy was done in piecemeal as the mass was unexpectedly adherent to the skin and underlying fascia (). The facial nerve was intact and was examined branch by branch. No frozen section was done during the operation.\nHistopathologic examination showed a widely infiltrative tumor involving the parotid parenchyma and extending into the surrounding adipose tissue and skeletal muscles (). The tumor was predominantly composed of bland spindle cells with wavy nuclear contours, embedded within a fibrillary, pale pink matrix, which resembled a neurofibroma (). Of concern, there were evidently distinct scattered more cellular foci of epithelioid tumor cells with nuclear atypia and increased mitotic activity (). The tumor was diffusely and intensely positive for the S100 protein and neuron-specific enolase (NSE) immunohistochemical stains, with a low Ki-67 proliferation index. In addition, the CD34 immunohistochemical stain was focally positive in tumor cells. On the other hand, the tumor lacked immunoreactivity for AE1/AE3, p63, EMA, Melan-A, and HMB-45 immunohistochemical stains, thus excluding carcinomas, clear-cell sarcoma, melanoma, and other salivary gland tumors. Based on morphologic and immunophenotypic features of the tumor, the diagnosis rendered was low-grade malignant peripheral nerve sheath tumor (MPNST) arising in a diffuse neurofibroma.\nGiven the rare presentation and the association of such tumors with NF1, the patient was evaluated clinically for features of the syndrome. Multiple café au lait macules were subsequently discovered on his trunk (). The patient was referred to a cancer center for further management and follow-up.\nFurther clinical examination of the patient and MRI revealed no distant metastasis. The patient underwent several courses of radiotherapy and is currently eighteen months' disease free.","A 45-year-old man from the Netherlands noticed a growing lump in his cheek that wasn't painful. After tests like ultrasounds and MRIs, doctors determined it was likely a benign tumor, but they couldn't be completely sure and recommended a surgical removal to be sure. During the surgery, the tumor was found to be more complex than expected and was diagnosed as a low-grade malignant peripheral nerve sheath tumor, which is a rare type of cancer. Fortunately, the patient is currently doing well and is disease-free after treatment."
631,"This 34-year-old male, who was medically free, was presented to the emergency department by the Red Crescent after an assault injury. He was conscious, alert, oriented, and complaining of right shoulder pain and bleeding due to assault by a cleaver. On examination, there was a wound around 20 cm on the posterior aspect of the right shoulder extending to the glenohumeral joint, acromion was exposed, and no active bleeding was present. There was no vascular or neurological injury, and passive motion and active motion of the shoulder were painful and limited. Computed tomography (CT) scan with 3D reconstruction was done prior to surgery, which confirmed a minimally displaced coronal-oblique fracture at the base of the acromion (Figures and ). Informed consent was taken from the patient to publish this case report.","This man was brought to the hospital after being assaulted with a cleaver, resulting in a significant wound on his right shoulder. The wound was deep and exposed the bone, and he was experiencing pain and limited movement in his shoulder. A CT scan confirmed a fracture of the bone at the top of his shoulder. He underwent surgery to repair the fracture, and the doctors plan to publish a report about his case."
632,"A 53-year-old male presented with hoarseness of 12-year duration. He gave no history of breathing or swallowing difficulty. On enquiring further, he had complaints related to gastric acid reflux. He was a smoker but had quit smoking 6 months back. He is a politician with a history of voice abuse. On flexible fibreoptic evaluation, there was a 0.5 cm polypoidal, cystic mass pedicled on the medial free edge of the middle 1/3 of the right true vocal fold. There was no abnormality of vocal fold mobility. Rest of the ENT examination was normal.\nBased on a history of long-standing hoarseness, voice abuse, and presence of a solitary polypoidal lesion over the true vocal fold, a preoperative diagnosis of a laryngeal polyp was made. No preoperative radiology was taken due to the unambiguous nature of the clinical findings. The patient was taken up for microlaryngeal surgery (MLS), and the lesion was excised with cold instruments. Postoperative period was uneventful with patient reporting near-normal voice during first follow-up after one week. Surprisingly, the postoperative histology showed features consistent with laryngeal myxoma.\nOn histological examination, our case showed a polypoidal tumour lined by hyperplastic stratified squamous epithelium (). A subepithelial unencapsulated lesion was noted. The latter was paucicellular formed by small, bland, spindle to stellate cells having indistinct cytoplasmic margins and hyperchromatic nuclei (). No significant atypia or mitotic activity or any necrosis was noted (). These cells were embedded within an abundant myxoid matrix. Immunohistochemically (IHC), the cells were negative for CD34, smooth muscle actin (SMA), and S100 (Figures –). Thus, a final diagnosis of laryngeal myxoma was rendered. The absence of stromal vasculature, hemorrhage, hemosiderin-laden macrophages, and hyalinization of basement membrane helped to differentiate it from a vocal fold polyp [].","A 53-year-old man came to the doctor because he’d had a persistent hoarse voice for over 12 years. The doctor found a small growth on his voice box during an examination, which was removed surgically. Surprisingly, the growth turned out to be a rare type of tumor called a laryngeal myxoma, not a simple polyp. The surgery was successful, and the patient’s voice has improved significantly after a week of follow-up."
633,"A 68-year-old man who was diagnosed with myasthenia gravis three months prior to admission presented with acute nonpruritic painless 1 cm erythematous papules over the upper torso, accompanied with subjective fevers, chills, nausea, vomiting, and frontal headache for 2 days. His past medical history was significant for heart failure with preserved ejection fraction of 65% and mechanical mitral valve replacement for which he was on warfarin. He was started on prednisone 40 mg daily and pyridostigmine 120 mg four times daily, two and a half months prior to admission, and azathioprine 150 mg daily, 10 days prior to admission. Upon presentation, he was found to have a temperature of 102.7 degrees Fahrenheit, with a heart rate of 107 beats per minute, blood pressure of 159/87 mmHg, and oxygen saturation of 95% on room air.\nA complete blood count with differential was remarkable for a white blood cell count of 15,000 cells/mm3, with 89% neutrophils and venous lactate of 2.6 mmol/L. All other laboratory parameters including electrolytes, blood urea nitrogen, creatinine, blood glucose, and liver function tests were within normal limits. Given the fever, leukocytosis, and elevated lactate, the initial concern was for sepsis. Infectious workup included blood cultures, chest X-ray, urinalysis with urine culture, respiratory viral panel, Lyme titers, and procalcitonin. The chest X-ray showed a possible new left lower lobe basilar opacity, procalcitonin was 0.59 ng/mL, and the patient was started on antibiotics with ceftriaxone and azithromycin for suspected lower respiratory tract infection. Of note, his azathioprine was discontinued on presentation, due to concern for continued immunosuppression and possible infection. Two days after presentation, given the improvement in clinical symptoms the azathioprine 150 mg was reinitiated. Within a few hours, he became acutely ill, febrile to 103.7 degrees Fahrenheit and tachycardic to 115 beats per minute, with return of the initial presenting symptoms and new onset photophobia. Initially, there was concern for worsening sepsis; repeat procalcitonin was ordered along with C-reactive protein and erythrocyte sedimentation rate (ESR), with antimicrobial therapy broadened to vancomycin, piperacillin/tazobactam, and intravenous acyclovir. Notably, a diffuse 1 cm papulopustular rash erupted over the scalp, head, neck, thorax, abdomen, and upper and lower extremities including the palmar and dorsal aspects of the hand (). As the cutaneous findings were nonspecific, the differential remained broad and infectious workup included bacterial, fungal, viral, or drug hypersensitivity. Drug hypersensitivity was suspected given the return of symptoms along with rash after rechallenge of azathioprine and the temporal response to the symptoms. The repeat procalcitonin was now elevated further to 5.36 ng/mL along with an elevated C-reactive protein of >270 mg/L and an ESR of 44 mm/hr.\nThe azathioprine was discontinued and the symptoms subsided with the pustules reduced in size and number. Biopsy of the pustule showed suppurative folliculitis, which is expected from a neutrophil driven process, consistent with azathioprine hypersensitivity (). All pustule stains, bacterial, viral, including herpes zoster and varicella zoster, and periodic acid-Schiff-diastase (PAS-D) stains, were negative. Repeat liver function tests including AST/ALT remained within normal limits, and a complete blood count revealed a white blood cell count of 9,300 cells/mm3 with 0% eosinophils. Antimicrobial therapy was deescalated. Over the next few days, the rash and symptoms resolved and the CRP decreased to 108 g/L. We utilized the Naranjo algorithm to estimate the probability of azathioprine causing hypersensitivity and found that our patient had a probable hypersensitivity reaction to azathioprine [].","This 68-year-old man was admitted to the hospital with a fever, chills, and a rash that appeared after he started a new medication for his myasthenia gravis. Initial tests suggested a possible infection, but the symptoms worsened after restarting the medication. Further investigation revealed that he was having a reaction to the azathioprine, causing the rash and other symptoms to return. The medication was stopped, and his symptoms improved, and the cause was confirmed by a skin biopsy. He is now recovering at home with continued monitoring."
634,"An 80-year-old female presented to clinic with a mass over the superior aspect of the right scapula. The mass was achy but did not interfere with performing activities of daily living. However, it was bothersome for the patient and she stated that it had been enlarging over the previous few months. She denies any local injuries or recent surgeries on the affected side. She denied having weakness in the left arm when compared to the contralateral side. She denied having trouble with overhead activities. She had not noticed any constitutional symptoms or nighttime pain. She did give a history of having a similar mass on the contralateral side, which was excised 10 years previously with a favorable result. She was very interested in having the new mass excised as well.\nInspection of the area is unremarkable, but palpation of the area demonstrates a firm, nonmobile, and nonpulsatile mass in the area of the upper trapezius overlying the scapula. The mass is longer in its medial to lateral dimension than craniocaudad. With deeper palpation, slight tenderness can be elicited. Examination of the shoulder does not yield signs of rotator cuff weakness or shoulder pain with provocative maneuvers.\nPlain X-ray demonstrates narrowing of the posterior glenohumeral joint space with sclerosis secondary to osteoarthritic changes. The acromiohumeral interval is measured to be 8 mm without signs of superior migration of the humeral head.\nMagnetic resonance imaging (MRI) shows an elongated lesion arising from the AC joint and tracking medially to superficial and within the trapezius muscle (Figures and ). It measures 2 cm (AP) × 13 cm (transverse) × 1.8 cm (craniocaudad). The lesion appears cystic with peripheral enhancement. There is also suspected full-thickness tear of the anterior fibers of the supraspinatus.\nAlthough the mass did not prevent the patient from performing her activities of daily living, it was bothersome enough for her that she wanted it removed. Under a general anesthetic, an incision was made directly over the palpable mass. With careful dissection, a stalk emanating from the AC joint was identified. The mass extended laterally from the AC joint within the trapezius muscle for approximately 13 cm (). Once it was dissected free, the mass was removed en bloc. A distal clavicle excision was then performed using an oscillating saw. After the cyst was excised, it was incised revealing thick mucoid content (Figures and ).\nThe patient was followed up at three weeks and three months postoperatively. She said she felt pain relief immediately after the surgery and she continued to have full, pain-free range of motion with no signs of recurrence.","An 80-year-old woman came to the clinic because she noticed a growing, painful lump on her shoulder blade. The lump didn't interfere with her daily activities, but she wanted it removed because it was bothersome. Tests, including an X-ray and MRI, showed the lump was a cyst-like growth connected to the AC joint and a tear in the supraspinatus muscle. The doctor removed the mass and a portion of the clavicle, and the patient reported immediate pain relief and full range of motion after the surgery."
635,"A 74-year-old male patient was admitted to our hospital in March 2017 to undergo liver resection to treat a malignant hepatic lesion diagnosed with CT and PET and a fine-needle biopsy positive for squamous carcinoma. The hepatic tumour discovered during follow-up for a previous bladder cancer submitted to endoscopic surgery three years before measured 22 mm in diameter and was located in the VIII Couinaud's segment [] of the liver in association with three smaller hypodense liver lesions with a focal dilatation of peripheral biliary tree ().\nThe case is discussed with radiologists, oncologists, and pathologists of our hospital. Even if the lesion had been the single site of disease; due to the proximity/doubtful infiltration of the lesion to the biliary tree, we decided to submit the patient to an explorative staging laparotomy and possible palliative surgery.\nOur internal protocol states that during the preadmission every patient who is a candidate for a liver resection is subjected to a routine liver function test with ICG to determinate the most appropriate surgical procedures []: 0,5 mg/Kg ICG are routinely injected intravenously up to seven days before surgery to evaluate the ICG retention rate at 15 min (R15). In our case 45 mg of ICG was intravenously administrated to test hepatic function, ten days before the surgery (patient R15 = 8.9).\nThanks to the ICG property of being fluorescent with the light emitted from the photodynamic eye of the laparoscopic system in our possession, it is possible to visualize the lesion during the surgical procedure. To this target, timing of administration and dose of ICG are key points.\nSeveral studies have demonstrated that the effective dose of ICG depends on the timing of injection; in particular, if the function liver test had been performed more than 7 days before surgery it would have been necessary to administer an adjunctive dose (0,1 mg/Kg) the day before []. In this case, it was necessary to administrate an adjunctive dose of ICG the day before the surgery (9 mg of ICG injected intravenously). After laparotomy, exploration of the abdominal cavity, and exposure of the liver, we easily confirmed the superficial lesion in the VIII Couinaud's segment. The liver surface has been analysed with the fluorescent imaging system. The fluorescing tumour has been clearly identified and defined on the liver surface, as shown in . We have also identified that a large area of fluorescent parenchyma that gets from the peripheral of the lesion up to the portal pedicle such as the neoplasia would interest the right biliary tree in the form of neoplastic lymphangitis (). This datum was not preoperatively known.\nA right hepatectomy would have been the oncologically correct surgical procedure due to the infiltration of right biliary duct. Considering the probable metastatic nature of the lesion, the absence of a clearly primary lesion, the age, the comorbidities, and the small size of residual liver, we have decided to perform an atypical segmental resection of S8 associated with cholecystectomy and lymphadenectomy of the hepatic pedicle nodes, including the area of impaired biliary excretion.\nAt the histological examination, the lesion, the lymph nodes of the hepatic pedicle region, and the right biliary branch, respectively, resulted in hepatic metastases from squamous cell carcinoma and sites of metastatic location. As expected the resection margin was interested by neoplasia.\nIn particular, the histological examination showed the following:Macroscopical exam: the neoplasia, in a site, appears to be in contact with the resection margin Microscopical exam: parenchymal hepatic section that showed metastatic localization of squamous carcinoma moderately differentiated. The neoplasia interest the surgery resection margin.\nIn this case, fluorescent imaging has revealed a fluorescing ring around the hepatic metastasis (). The fluorescence of the cholestatic area was shown on the cut surface ().","A 74-year-old man was admitted to the hospital in March 2017 to have a part of his liver removed to treat a cancerous growth. Doctors used special imaging techniques, including fluorescent lights, to precisely locate the tumor and determine if it was spreading to nearby blood vessels. Because of this, they decided to perform a more extensive surgery to remove the tumor and a portion of the gallbladder and nearby lymph nodes. The final examination confirmed that the cancer had spread to the bile ducts and surrounding tissues, and the surgical team was able to remove the affected area."
636,"A 45-year-old woman was admitted to an emergency department with dyspnea and swelling on her hands and face for at least three days. She was nonsmoker and did not have any chronic disease. Her dyspnea and hypoxemia were getting worse and she was accepted to ICU. Noninvasive mechanical ventilation (MV) was used for initial treatment but hypoxemia was worsened; hence, she was intubated and invasive MV was used. On her physical examination, we auscultated mild crackles bilateral on lower lung zones. Her chest X-ray showed bilateral nonhomogenous infiltration at middle and lower zones (). While initial fraction of inhaled oxygenation (FiO2) was 80% on MV, her PaO2 was 65 mmHg and lung protective MV strategies were applied. An appropriate fluid replacement, antibiotics, and other medical treatments were applied. Undergoing MV, FiO2 level was decreased gradually and she was weaned from MV on her fifth day of ICU stay and MV. After weaning, we observed that her oral secretions increased and her left nasolabial sulcus wiped out. On her neurological examination, abnormal findings were not found except left facial paralysis. We did not study out any pathological imagination neither on her cranial computed tomography (CT) nor on cranial magnetic resonance imaging (MRI). Peripheral facial paralysis (PFP) was diagnosed and intravenous steroid treatment 1 milligram per kilogram (methylprednisolone) was added to her therapy by neurologist. At the same time, dermatological lesion occurred and, on her dermatologic examination, oedema on her face, pustular lesions on her skin, and fissure on her tongue were detected; therefore labium mucosal biopsy was taken and mucositis was reported (). When we talked to the patient about her symptoms, she informed us that she had recurrent and spontaneous facial paralysis in previous years. According to her medical history, signs of orofacial oedema, fissure on the tongue, and PFP, MRS was diagnosed. She was transferred from ICU to department of neurology and then she was discharged from the hospital.","A 45-year-old woman was admitted to the hospital because she was having trouble breathing and swelling in her hands and face. She needed help breathing and was initially given oxygen and a machine to assist her breathing, but her breathing continued to worsen. Doctors found fluid in her lungs and diagnosed her with a respiratory infection and facial paralysis. After treatment with medication and supportive care, she was able to breathe on her own and was discharged home with a neurologist to monitor her condition."
637,"A healthy 7-year-old girl of Indian descent presented with one-year duration of hypochromic linear bands in two regions. The lesions were present on the right forearm and left leg and buttocks. Neither the patient nor her parents were able to recall any inciting illness, allergy, or environmental or social exposure that may have preceded the onset, which was gradual. There was no associated pruritus, pain, hair loss, or nail involvement. No recent growth had been noted. The patient had not received any previous topical or systemic treatment for the lesions. The patient's past medical history was negative for atopy and otherwise unremarkable, as was her family history.\nOn examination 2 mm hypopigmented lichenoid macules were noted coalescing into a linear patch on the dorsal aspect of the patient's right forearm (Figures and ). The eruption ended at the distal forearm, sparing the right hand, fingers, and nails. The distribution was consistent with BL. Similar lesions were also noted on the left buttock, though somewhat more diffuse, but also progressing distally along a BL to the left posterior thigh (Figures and ). The lesions in both locations were nonscaling, nonpainful, nonpruritic, and stable in appearance according to the patient's parents.\nNo biopsies were taken at the request of the patient's parents. A diagnosis of LS was made clinically, and observation was recommended with explanation of the disease course. A follow-up visit was scheduled but the patient did not return to the clinic.","This 7-year-old girl has unusual, light-colored streaks on her arms and legs that have been developing over the past year. Her parents don't know what caused them, and there are no other symptoms like itching or pain. Doctors believe she has a condition called Linear Sclerosing Band (LS), which is a harmless skin change. They recommended watching the streaks to see if they change, and a follow-up appointment was scheduled, but the patient did not return."
638,"A 58-year-old man underwent pancreatoduodenectomy and right hepatic lobectomy with choledochojejunostomy for a duodenal gastrointestinal stromal tumor with multiple liver metastases. Ten years after the operation, he developed recurrent fever and upper abdominal pain with hepatobiliary enzyme elevation. He underwent double-balloon endoscopy (DBE) and anastomotic stenosis was revealed. There was no evidence of malignancy, and we diagnosed cholangitis due to benign anastomotic stenosis. Balloon dilation for stenosis and biliary stenting with a plastic stent (PS) was performed. As relapsing cholangitis occurred 6 times a year, he underwent EUS-HGS with MS. We used a GF Type UCT 260 (Olympus Medical Systems, Tokyo, Japan) endoscope. The B3 duct was visualized from the stomach. After the absence of blood vessels crossing the puncture route was confirmed, the bile duct was punctured with a 19-G needle (EZ shot 3; Olympus) (). Then, a 0.025-inch guidewire (VisiGlide 2; Olympus) was introduced into the jejunum in an antegrade manner. Subsequently, the puncture site was dilated with a 3.6-Fr double-lumen catheter (Uneven Double Lumen Catheter; PIOLAX, Tokyo, Japan), and another 0.035-inch wire (Revowave; PIOLAX, Tokyo, Japan) was introduced into the jejunum (). An 8 mm covered MS (Niti-S; TaeWoong Medical Inc., Seoul, Korea) was placed (). No adverse events occurred. Before EUS-HGS, fever and hepatobiliary enzyme elevation frequently recurred. After EUS-HGS, the enzymes normalized, and cholangitis has not recurred in 5 months.","This 58-year-old man had surgery ten years ago to remove a tumor in his pancreas and liver. Recently, he experienced recurring fever and abdominal pain, which led doctors to discover a narrow spot in the connection between his bile duct and the intestine. To fix this, he underwent a procedure called EUS-HGS, where a small opening was made in his intestine to widen the blocked area. After this procedure, his symptoms improved, and his liver enzymes returned to normal, with no further problems in the last five months."
639,"A 68-year-old man underwent extended right hepatectomy and bile duct resection with choledochojejunostomy for hilar cholangiocarcinoma. Relapsing cholangitis occurred because of anastomotic benign stenosis after the operation. Biliary stenting with PS had repeatedly been performed, but the stenosis did not improve. Thus, he underwent EUS-HGS with MS. The B3 duct was punctured with a 19-G needle (Expect; Boston Scientific, Natick, MA, USA). Then, a 0.025-inch guidewire (Radifocus; Terumo, Tokyo, Japan) was introduced into the jejunum. Subsequently, the puncture site was dilated with a 6-Fr diathermic dilation catheter (Cysto-Gastro-Set; ENDO-FLEX, Voerde, Germany). The wire was changed to another 0.035-inch wire (THSF; Cook Medical, Winston-Salem, NC, USA), and an 8 mm covered MS (Niti-S) was placed. No adverse events occurred. Nine months after EUS-HGS, cholangitis occurred only once due to debris and granulation. We performed balloon sweeping for debris and placed a PS into the MS. Twelve months after EUS-HGS, we replaced the PS with an MS. As in case 1, hepatobiliary enzymes normalized, and cholangitis has not recurred in 11 months.","This 68-year-old man had surgery to remove a cancer in his liver and reconnect his bile duct. After the surgery, he developed a blockage in the bile duct, leading to recurring infections. To address this, he underwent a minimally invasive procedure called EUS-HGS to clear the blockage. The procedure successfully improved his bile duct and prevented further infections, and his liver function has returned to normal."
640,"A 33-year-old man was involved in an automobile accident and was brought to our hospital by ambulance. He had been in the front passenger seat and had been wearing a three-point seatbelt. He reported severe back pain, but showed no neurological deficit.\nAnteroposterior and lateral radiographs of the spine showed an increased gap between the 1st and 2nd lumbar spinous processes and 2nd lumbar vertebral fracture (figures not shown). Magnetic resonance imaging (MRI) of the spine also demonstrated an L2 vertebral fracture and disruptions of the posterior ligamentous complex between L1 and L2, in combination with extensive subcutaneous hematoma ().\nComputed tomography (CT) of the spine in the sagittal orientation and 3-dimensional (3D) CT further revealed the involvement of the superior end plate of the L2 vertebra, comprising horizontal splitting from the left pedicle, through the left transverse process, and reaching the center of the neural arch (). The right-sided L2 pedicle was intact.\nAfter checking the general condition of the patient and excluding intra-abdominal injury by enhanced CT and ultrasonography, the patient underwent L1-L2 single-level instrumented fusion using a posterior approach. Initially, monoaxial pedicle screws with conventional trajectory were placed at L1 and L2 pedicles on the right side (intact pedicle side). A rod slightly bent in lordosis was then introduced and connected with these pedicle screws with a compression force applied between screws. This procedure achieved reduction and the fracture gap at the left L2 pedicle and lamina was completely closed. Polyaxial pedicle screws were used on the left side. A pedicle screw with a conventional trajectory was placed at the left L1 pedicle. A CBT pedicle screw was then inserted through the fractured L2 pedicle under fluoroscopy. This CBT screw was used as an alternative to an osteosynthesis screw. A rod was introduced on the left side, bilateral facet fusion with local bones obtained from the lower one-third of the L1 spinous process was performed, and the wound was closed. Although the merits of cross connectors for CBT screws remain unclear, we applied the connector in this case because connecting bilateral pedicle screws along conventional trajectories has been reported to increase the pullout strength of these screws []. Postoperative X-rays showed good reduction by this single-level fixation ().\nThe postoperative period was uneventful. Although rigid fixation was obtained with this procedure, a thoracolumbosacral orthosis (TLSO) was applied for 6 weeks, since this case was our first experience. Physical activities were not restricted with the TLSO. Sagittal CT and 3D-CT obtained at 6 months and 1 year postoperatively showed proper trajectory of the CBT pedicle screw and complete bone union ().","A 33-year-old man was brought to the hospital after a car accident, experiencing severe back pain. X-rays and an MRI revealed a fracture in his lower spine, along with some bruising. He underwent surgery to stabilize the fracture with a fusion, using screws and plates to hold the bones together. The surgery was successful, and he is recovering well with a brace for six weeks, and follow-up scans will be done to ensure proper healing."
641,"A 20-year-old male presented with worsening bloody diarrhea of 4 months' duration associated with cramping abdominal pain and weight loss of 4 Kg. On admission, he was hemodynamically stable. Physical examination showed mild tenderness to deep palpation in the left lower quadrant. Laboratory tests were consistent with anemia (hemoglobin = 10.5 mg/dl, hematocrit = 33.5%), thrombocytosis (platelets = 568000/mm3), low iron level (iron = 25mg/dl), and normal C-reactive protein (CRP). Stool analysis, ova and parasite test, Clostridium difficile toxin assay, and stool culture were negative. Colonoscopy revealed left-sided colitis with marked erythema, absent vascular pattern, and friability erosions (Mayo score 2). Biopsies showed chronic active colitis consistent with UC. Based on the clinical presentation and laboratory, endoscopic, and pathologic findings, the patient was diagnosed with moderate left-sided UC and was started on oral and topical 5-aminosalicylic acid (5-ASA) without any response to treatment: bloody diarrhea (more than 5 bowel movement per day), severe abdominal pain, low grade fever, and additional weight loss in addition to severe anemia (hemoglobin = 7.3g/dl) and high CRP with negative stool tests. High dose steroids therapy was started with marked improvement. Steroid tapering caused recurrence of symptoms and anemia at 20mg prednisone per day. Relying on the findings above, the patient had left-sided UC and is steroid-dependent, so Infliximab 5mg/kg was initiated at 0, 2, and 6 weeks, then every 8 weeks without any improvement after 4 months of treatment with persistent bloody diarrhea and severe iron deficiency anemia. Repeated colonoscopy showed severe inflammatory mucosa with deep ulcerations and pseudopolyps formation at the splenic flexure and the distal part of the left colon, separated by healed mucosa. Biopsies from the pathologic area revealed severe chronic active colitis consistent with inflammatory bowel disease/UC without any evidence of cytomegalovirus inclusions. Given the lack of response to Infliximab and the worsening endoscopic findings, resistance to therapy was suspected. Additional laboratory tests showed low Infliximab through level with high antibodies level to Infliximab. The patient was switched to combination therapy with Adalimumab 160mg at week 0, 80mg at week 2, and 40mg every other week plus Azathioprine 2.5mg/Kg with only partial response: improvement of diarrhea but persistence of severe nocturnal colicky abdominal pain. Adalimumab trough level was very low with the absence of anti-drug antibodies. So, Adalimumab was increased to 40mg per week. Two months later, he continued to have severe colicky abdominal pain and distension with weight loss. New colonoscopy revealed complete mucosal healing and a giant pseudopolyp in the left colon and another obstructive one at the level of the splenic flexure (Figures and ). Biopsies were consistent with chronic inflammation with architectural distortion and cryptic abscess. Abdominal CT scan confirmed the presence of two giant pseudopolyps with evidence of obstruction at the splenic flexure (Figures and ). The patient had total colectomy () with ileoanal anastomosis and J pouch.","This 20-year-old man has been struggling with persistent, bloody diarrhea and abdominal pain for four months, leading to weight loss and anemia. Tests revealed inflammation in his colon, and a colonoscopy confirmed he has ulcerative colitis (UC). Despite trying several medications, including Infliximab and Adalimumab, his symptoms continued to worsen. Ultimately, he underwent a total colectomy with ileoanal anastomosis and J pouch to remove the diseased colon and restore normal bowel function."
642,"A 71-year-old male, previously healthy, was seen for the first time in May 2011 for diarrhea and rectal bleed. His physical examination was unremarkable. Laboratory tests were within normal range. Ileocolonoscopy showed mucosal inflammation and ulcerations over a segment of 7cm at the level of transverse colon. Biopsies were in favor of chronic active colitis. The patient was treated as colonic IBD and was started on Mesalamine 4g per day but he was lost to follow-up. Four years later, he was seen again in January 2015 for the same previously described symptoms. He stated that he took Mesalamine for 6 months and stopped by his own after marked improvement and he was asymptomatic since then until the reappearance of symptoms associated with abdominal pain few days prior to the presentation. Physical examination and lab tests were normal. Colonoscopy revealed an obstructive giant pseudopolyp () at the level of the transverse colon; biopsies showed chronic inflammation with architectural distortion and granulation tissue formation. Abdominal CT scan confirmed the presence of giant pseudopolyp (). The patient was treated with segmental colonic resection and the surgical pathologic report was CD. The final diagnosis was colonic CD complicated by an obstructive giant pseudopolyp.","6 This 71-year-old man had a long-standing problem with diarrhea and bleeding from his rectum, which he first noticed in After an examination and colonoscopy, doctors diagnosed him with chronic inflammation in his colon. He tried medication, but stopped taking it when he felt better. However, his symptoms returned, and further tests revealed a large, abnormal growth (pseudopolyp) blocking his colon, requiring surgery to remove it. The final diagnosis was inflammatory bowel disease with a complication of a large growth in his colon."
643,"A 32-year-old male presented to the emergency department with 20 mins of cramping retrosternal chest pain radiating to his left shoulder accompanied by sweating and shortness of breath. He did not have a history of any cardiovascular risk factors, such as a history of smoking, diabetes, or hypertension. He did not have any family history of cardiac events in family members at an early age. He had a self-reported diagnosis of hypothyroidism for which he was self-administering 120 mg of Armour Thyroid daily.\nAt the time of presentation, his blood pressure was 171/106 mm of Hg, heart rate was 88 beats per minute, and respiratory rate was 16 breaths per minute. Physical exam was notable for well-developed musculature and cystic acne. Other physical examination findings were unremarkable. A 12-lead electrocardiogram (ECG) (Figure ) demonstrated ST-segment elevations in leads aVL, I, and v1-v6, as well as ST segment depressions in leads II, III, and aVF, suggestive of an acute ST elevation myocardial infarction (STEMI).\nInitial lab work reported markedly increased levels of cardiac troponin. Urine drug screen was negative, eliminating cocaine as a potential etiology.\nTransthoracic echocardiography (TTE) displayed a moderate increase in left ventricular (LV) wall thickness, reduced ejection fraction (EF) of 40%, grade 1 diastolic dysfunction, and hypokinetic anterior and anteroseptal walls in the distribution of the left anterior descending (LAD) coronary artery. Emergent left heart catheterization was performed via the right radial artery using the Seldinger technique. An LV pressure of 117/5 mm of Hg with an LV end-diastolic pressure of 14 mm of Hg was noted. A coronary angiogram revealed a complete occlusion of the LAD at the ostium (Figure ).\nThe remainder of the coronary arteries were patent without evidence of atherosclerotic changes. Manual thrombectomy of the LAD was performed, and a XIENCE Alpine 3.25 mm x 15 mm drug-eluting stent (Abbott Laboratories; Abbott Park, IL, USA) was positioned leading to return of TIMI-III flow (Figure ).\nThe patient was started on dual antiplatelet therapy with aspirin and clopidogrel, in addition to heparin and eptifibatide infusions. His subsequent fasting lipid profile was normal with low-density lipoprotein of 127 mg/dL, a high-density lipoprotein of 31 mg/dL, and triglycerides of 44 mg/dL. Focused questioning to elicit the potential cause of the myocardial infarction led to the revelation that the patient participated in recreational bodybuilding for which he self-administered exogenous testosterone therapy and was using Armour Thyroid as a weight loss supplement.\nHis testosterone levels were elevated at 1,311 ng/dL with luteinizing hormone (LH) and follicle stimulating hormone (FSH) levels below trace levels of 0.20 mIU/mL, confirming exogenous testosterone supplementation. Free triiodothyronine (T3) was high at 4.08 pg/mL with a suppressed total thyroxine (T4) at 1.2 mcg/dL and a thyroid stimulating hormone (TSH) at 0.20 mIU/mL, confirming Armour Thyroid administration.","A 32-year-old man went to the hospital because of sudden, severe chest pain, shortness of breath, and sweating. Tests revealed a blocked artery in his heart, causing a heart attack, and showed signs of heart muscle thickening and reduced function. The blockage was cleared with a stent, and he was given medications to prevent further clots. Doctors discovered he was taking too much testosterone and Armour Thyroid, which may have contributed to the heart attack."
644,"A 64-year-old African-American male presented with an elevated PSA of 9.3 ng/mL and no previous history of prostate biopsy. He had a systematic TRUS-guided extended sextant biopsy with two of 12 cores demonstrating prostate cancer, one with GS 4+3 and a second with GS 3+4, both in the left apical region. He had no baseline urinary or bowel problems, but did have erectile dysfunction adequately managed with sildenafil taken as needed for sexual performance. His AUA urinary symptom score was 3 and SHIM score was 14 without use of PDE5 inhibitors. Using the web-based Memorial Sloan Kettering Cancer Center nomogram, his risk of nodal involvement was estimated to be 7% []. He was in good overall health and his age-adjusted life expectancy was estimated to be 19.4 additional years using the Social Security Administration life tables. After discussion of all treatment options with the multidisciplinary team, he elected to pursue definitive treatment with prostate SBRT.\nDiagnostic multi-parametric prostate MRI and review by the multidisciplinary prostate imaging conference previously demonstrated a 1.7 cm T2-weighted hypointense lesion with corresponding restricted diffusion in the anterior apical transition zone left of midline that was considered high suspicion based on imaging parameters but associated well to the systematic biopsy cores positive for prostate cancer (Figure ). The whole prostate volume and the area of MRI cancer suspicion were segmented using post image processing software. Following the MRI, three gold fiducial markers were placed in the urology office via a TRUS-guided approach. The radiation therapy planning CT scan was scheduled two weeks after fiducial placement. No urinary catheter, rectal balloon, or rectal spacer was used.\nAfter the simulation CT scan was completed, axial T2-weighted and post contrast T1-weighted MRI were fused with the CT images using radiation therapy planning software. Target volumes of the prostate and the high-risk lesion were then generated on the CT based on MRI segmented regions of suspicion. The radiation oncologist and urologist met to review the image fusion and target volumes prior to proceeding with final treatment planning. In this case, the highest suspicion lesion was located in the left anterior apical prostate as visible on T2-weighted MRI. Hence the prostate surrounding the high-risk lesion was included in the high-risk target volume for planned SIB (Figure ). For transition zone and central gland lesions, limiting dose to the urethra presents the greatest challenge to radiation therapy planning. However, most prostate SBRT protocols recommend achieving normal tissue dose limits over complete coverage of the high-risk target volume. In this case, adequate coverage was possible while limiting urethral dose to an acceptable range.\nThe high-risk volume received 40 Gy SIB in five fractions while the entire prostate received 36.25 Gy. He was placed on tamsulosin during radiation therapy after developing dysuria, diminished urinary stream, and frequency corresponding to an increased AUA symptom score of 14. These irritative urinary symptoms were his only radiation-associated side effects. He did not develop any significant lower gastrointestinal symptoms. Six months after his treatment, he continued to have irritative urinary symptoms and remained on tamsulosin.","A 64-year-old man had a high PSA level, leading to a prostate biopsy that revealed cancer in two areas of his prostate. The cancer was located in the left side of his prostate and was confirmed by MRI scans. After discussing treatment options, he decided to undergo SBRT radiation therapy to target the cancerous areas, with careful planning to minimize damage to surrounding tissues. He is currently receiving radiation and experiencing some urinary irritation, which is being managed with medication."
645,"A 65-year-old Caucasian male presented with an elevated PSA of 8.98 ng/mL and a history of TRUS-guided extended sextant biopsy negative for prostate cancer three years prior to presentation. At the time of his prior prostate biopsy, his PSA was 7.25 ng/mL. He had mild baseline lower urinary tract symptoms with an associated AUA urinary symptom score of 14. He denied any erectile dysfunction and had a SHIM score of 25. MP-MRI was performed, and review by the multidisciplinary prostate imaging conference revealed patchy diffuse abnormal signal that is often seen in patients with prior biopsy history and/or inflammation. Despite this diffuse irregularity in signal in the right posterolateral peripheral zone, there was a focal area of well-defined hypointensity with corresponding diffusion restriction suspicious for harboring prostate cancer. There was notable central gland hyperplastic nodules with regional areas of low T2 signal intensity in the left anterior transition zone that was low suspicion for representing malignancy more likely representing benign prostatic hyperplasia nodularity. With these findings, he underwent MRI/TRUS fusion-targeted biopsy that demonstrated GS 4+3 adenocarcinoma in 80% of the specimen cores sampled from the MRI-targeted lesion in the posterior peripheral zone. After discussion of all treatment options with the multidisciplinary team, he elected to proceed with prostate SBRT.\nThe patient then had TRUS-guided fiducial markers placed followed by the radiation therapy planning CT scan two weeks after fiducial marker insertion. CT and MRI fusion was performed to allow for CT generation of the target volumes of the prostate gland and the high-risk intraprostatic lesion which was used for MRI-targeted biopsy proven to represent the intermediate-risk prostate cancer. The radiation oncologist and urologist met to review this image fusion between treatment simulation CT and MP-MRI as well as target volumes for SBRT and SIB. In this case, the highest suspicion lesion was located in the posterior peripheral zone, well visualized on T2-weighted MRI around which the SIB region was drawn. Dose limitations in the region of the rectum were considered posteriorly, and the ipsilateral neurovascular bundle exposures were considered but not considered dose limiting (Figure ).\nThe SIB volume in the posterior peripheral zone received 40 Gy in five fractions while the entire prostate gland was administered 36.25 Gy over the same time course. The patient was being managed with tamsulosin prior to starting radiation therapy due to his history of lower urinary tract symptoms. He developed moderate dysuria during the final two fractions of radiation therapy that was managed by increasing his tamsulosin to twice daily dosing. One week after radiation was completed, he developed a self-limited diarrhea lasting three days. By his one-month follow-up visit, his urinary and gastrointestinal symptoms had returned to baseline.","A 65-year-old man had a slightly elevated PSA level, and a previous prostate biopsy didn't show cancer. Further tests, including an MRI scan, revealed a suspicious area in his prostate that was confirmed by a targeted biopsy to be prostate cancer. He decided to undergo SBRT radiation therapy to treat the cancer, and the radiation team carefully planned the treatment to minimize damage to surrounding organs. After completing radiation, he experienced some temporary urinary and digestive issues, which resolved on their own."
646,"A 58-year-old Caucasian woman, with past medical history significant for a 2.4 cm GIST diagnosed with esophagogastroduodenoscopy (EGD) (Figures , ) (performed for persistent epigastric pain despite therapy) six months earlier and s/p laparoscopic partial gastrectomy, presented to the emergency department (ED) with new-onset jaundice initially observed by her son four days prior to arrival. Also, she reported generalized weakness, fatigue, and itching for the past several days. The patient reported no previous history of alcohol consumption, intravenous drug use, acquiring tattoos or non-sterile piercings, receiving transfusions of blood or blood products, sexual promiscuity, residence in a developing country, occupational exposure to toxins (she worked as a school teacher), or prior liver diseases. She reported no family history of liver diseases. The earlier biopsy had shown GIST, histologic grade G2 (high grade; mitotic rate > 5/50 per high-power field (HPF)), with spindle cells and no necrosis (Figures , ). The margins were negative. The tumor cells were positive / immunoreactive for CD34, CD117, and DOG-1 (Figures , ). A recent follow-up computed tomography (CT) scan showed no recurrence.\nAt presentation, the patient appeared icteric with yellowish discoloration of the skin and sclera. She was afebrile (temperature 97.4) and hemodynamically stable (heart rate 72 beats per minute (BPM), blood pressure 110/85 mm Hg). On physical exam, the abdomen was soft and not distended, with no tenderness and normoactive bowel sounds. Murphy’s sign was negative and there was no guarding nor rigidity.\nInitial laboratory testing showed significantly elevated transaminases with aspartate aminotransferase (AST) of 1450 U/L (10-35 U/L) and alanine aminotransferase (ALT) of 1632 U/L (10-55 U/L). Also, there were increased total bilirubin of 4.9 mg/dL (<1.2 mg/dL) and increased direct bilirubin of 2.7 mg/dL (<0.6 mg/dL). The alkaline phosphatase (ALP) was 162 U/L (40-150 U/L), the gamma-glutamyltransferase (GGT) was 95 U/L (5-50 U/L), and the international normalized ratio (INR) was elevated at 1.9 (0.8-1.2). The serum total protein, serum albumin, serum electrolytes, and complete blood count (CBC) were within normal limits. The patient’s Model for End-Stage Liver Disease (MELD) score was calculated to be 28 upon arrival.\nFurther laboratory testing showed that the serum iron level, total iron-binding capacity (TIBC), serum ferritin, ceruloplasmin level, and thyroid function tests (TFTs) were within normal limits. An acetaminophen level and an autoimmune workup, including antinuclear antibody (ANA), anti-mitochondrial antibody (AMA), and anti-smooth muscle antibody (ASMA), were negative. And, viral serologies for hepatitis B virus (HBV), hepatitis C virus (HCV), cytomegalovirus (CMV), and Epstein-Barr virus (EBV) were negative. An abdominal ultrasound revealed mild hepatomegaly.\nUpon medication reconciliation, it was discovered that the patient was taking imatinib, prescribed by her hematologist-oncologist, for the past four months. The medication was discontinued on hospital admission due to the concern for hepatotoxicity. No other potentially hepatotoxic medications were noted. Prior liver function tests (LFTs), before initiation of imatinib, were all within normal limits. As the LFTs were not improving with conservative management, an interventional radiology (IR)-guided liver biopsy was performed for the purpose of diagnosis as well as to assess the extent of liver injury (Figures -).\nAfter seven days, the patient’s transaminases began to decrease, ultimately falling to AST of 483 U/L and ALT of 544 U/L during the hospital admission. The transaminases, as well as the rest of the LFTs, continued to trend down and were within normal range two months later.","This 58-year-old woman came to the hospital because she was suddenly feeling very weak and yellow, and her skin was also itchy. She had a previous surgery to remove part of her stomach due to a type of cancer, and she was taking medication for that. Blood tests showed her liver was not working properly, and an ultrasound revealed some enlargement. After stopping the medication that was causing the problem, her liver function improved, and her symptoms gradually went away."
647,"A six-year-old-African American female presented to our care in April 2010 for the evaluation of sleep attacks and apnea during sleep. According to her mother, the patient experienced cataplexy episodes with laughter. At that time, the patient underwent a nocturnal polysomnogram (NPSG) for further investigation. According to the results of the sleep study, patient slept 474.00 minutes out of 539.3 minutes in bed for a sleep efficiency of 87.9% (n=89%). Her sleep latency was decreased at 7.3 minutes and 68.1% of the total sleep time was spent in the supine position. In addition, rapid eye movement (REM) sleep and latency were logged at 17% and 68.5 minutes (n=136-156), respectively. Furthermore, the NPSG illustrated that the patient experienced five central apneas, one mixed apnea, five hypopneas, and an apnea-hypopnea index of 1.4 events/hour consistent with mild obstructive sleep apnea (OSA) by pediatric criteria [].\nThe patient underwent another NPSG with a multiple sleep latency test (MSLT) in November 2010 for persistent symptoms. Results indicated that the patient slept for 395.50 minutes out of the 411.8 minutes in bed, which translated to a sleep efficiency of 96.0%. Similar to the previous study, the patient experienced decreased sleep latency at 3.3 minutes. In addition, REM sleep and latency were logged at 20.4% and 106.5 minutes, respectively. In contrast to the earlier NPSG study, this evaluation illustrated three central apneas, six mixed apneas, nine obstructive hypopneas, and an apnea-hypopnea index of 2.9 events/hour with <1 being normal.\nAn MSLT is the primary diagnostic tool for narcolepsy and is typically performed following an NPSG to measure sleep latency, which is the time it takes an individual to go from wakefulness to sleep. Additionally, it measures how quickly the patient enters REM sleep, known as sleep onset REM periods (SOREMPs). This test includes four or five nap periods of 20 minutes each []. The patient is instructed to lie in the darkroom and attempt to fall asleep. Sensors transmit data such as the amount of time needed to fall asleep and enter REM sleep. Once asleep, the patient will be woken up fifteen minutes later; if 20 minutes pass and the patient has not fallen asleep, the nap trial is terminated. Next, the patient is given a two-hour break before the second nap trial can begin. During this break, the patient must stay awake and this process is repeated four more times []. The results of the study include the mean sleep latency (MSL) and the number of SOREMPs recorded. The diagnostic criterion requires an MSL of ≤8 minutes and ≥2 SOREMPs on an MSLT []. Our patient underwent a series of five nap trials with a sleep latency of 3, 2, 3, 6, and 2 minutes, respectively, and a mean sleep latency of 3.2 minutes. However, the patient did not enter REM sleep during any nap trials, thus having 0 SOREMPS. Additionally, laboratory workup for HLA-DR15 and DQ0602 was positive, and the patient had documented cataplexy.","This six-year-old girl has been having trouble sleeping and experiencing unusual events like laughing during sleep. Tests showed she was sleeping too much and entering sleep too quickly, along with some breathing problems during sleep. Further tests, including a sleep study, confirmed these findings and showed she has a high likelihood of narcolepsy. Because of these findings, the doctor is continuing to investigate and monitor her condition."
648,"We present the case of a white, Hispanic, 58-year-old, non-smoker female. She has a past medical history of obstructive sleep apnea and chronic obstructive pulmonary disease (chronic bronchitis). Her travel history includes the Dominican Republic and Caribbean Islands. She worked for several years in an automobile repair shop and was exposed to lead and paint. Her current occupation is as a telephone operator in a call center, which requires her to speak continually.\nFifteen years ago, she developed a progressive cough. During the following four years, she was evaluated by more than eight pulmonologists who were unable to make a diagnosis. She developed a productive cough with white sputum and blood titer. Her alpha-1-antitrypsin serum, perinuclear anti-neutrophil cytoplasmic antibody (P-ANCA), cytoplasmic antineutrophil cytoplasmic antibody (C-ANCA), and rheumatoid factor were all within normal limits. Her purified protein derivative (PPD) and fungal infection tests were negative. Lung function tests revealed an obstructive pattern. Her forced expiratory volume in one second (FEV1)/forced vital capacity (FVC) was 70% and total lung capacity (TLC) was 72%. A computed tomography (CT) scan showed mild ground glass infiltrates in the lung bases (Figure ).\nFinally, in December 2011, a lung biopsy via assisted thoracoscopic surgery (VATS) was performed, and she was diagnosed with constrictive bronchiolitis and diffuse idiopathic neuroendocrine cell hyperplasia with carcinoid tumorlets. She was treated with octreotide; however, the treatment was interrupted several times due to issues with insurance and difficulties visiting the medical center. Her treatment was restarted in our hospital after three years in May 2014. Since then, CT scans taken every six months show a stable disease. She currently has a stable radiographic disease with no new complaints during the over two years of follow-up.","This 58-year-old woman has a history of sleep apnea and COPD, and she was diagnosed with constrictive bronchiolitis and a type of lung cancer after years of being misdiagnosed. She worked in a shop where she was exposed to lead and paint, which may have contributed to her condition. Tests showed a blockage in her small airways and some scarring in her lungs. Despite treatment with medication, her condition has remained stable over the past two years, and she is currently being monitored regularly."
649,"A nine-year-old girl presented to the emergency department with abdominal pain and distention for the past one week, with sudden increase in intensity of pain for the last four hours. The patient had not yet reached the age of menarche. There was no associated nausea or vomiting and her bowel habits were not affected. Past medical, surgical, and family history was also insignificant. An abdominal examination revealed tenderness in the lower abdomen with a firm palpable mass occupying the right side of the abdomen. Her blood counts showed an elevated total leukocyte count of 13,000 cells/dL with neutrophilic predominance. Initial clinical assessment raised the possibility of an appendicular mass.\nThe patient therefore immediately underwent a contrast-enhanced computed tomography (CT) scan of the abdomen and pelvis, which revealed a large soft tissue mass measuring approximately 80 x 150 x 170 mm in anteroposterior, transverse, and craniocaudal dimensions, respectively, and was predominantly occupying the right mid and lower quadrant. The mass showed some areas of low attenuation, suggestive of necrosis/intratumoral edema (Figure ). There was free fluid noted adjacent to the lesion and in the pelvis (Figure ). The right ovary was separately identified and appeared normal (Figure ).\nAnteromedially, the mass had a tortuous, twisted vascular pedicle that was likely originating from the left adnexa (Figure ).\nAdditionally, few speckled calcifications were noted in the mass (Figure ). No enhancing fibrovascular septa were noted in the lesion.\nNo evidence of regional lymphadenopathy or distant metastases was found on the CT examination. On the basis of the radiological picture, an impression of left ovarian tumor with torsion was suggested.\nThe patient then underwent an exploratory laparotomy and left salpingo-oophorectomy along with partial omentectomy. Intraoperative findings included a large bilobed edematous mass weighing approximately 1.5 kg with a twisted, thickened vascular pedicle and varicosed vessels. The left fallopian tube was slightly thickened as well. No lymphadenopathy or invasion into the surrounding structures was seen.\nThe surgically resected specimen was then sent for histopathological analysis, which revealed a neoplastic lesion in the left ovary arranged in nests and trabeculae separated by fibrous septa. The cells were polygonal with moderate amount of clear to eosinophilic cytoplasm. The nuclei were round to oval, showed moderate plemorphism with prominent eosinophilic nucleoli and frequently visible mitotic activity. Scattered plasma cells and lymphocytes were present within the fibrous septa. In areas, tumor cells were seen scattered against edematous stroma. Thin-walled dilated and congested blood vessels were seen, suggestive of vascular compromise secondary to torsion. The neoplastic cells showed diffuse nuclear positive expression for octamer-binding transcription (OCT) 3/4 immunohistochemical stain. Intracytoplasmic glycogen was highlighted on Periodic acid-Schiff special stain (Figure ).\nThe overall findings confirmed mature ovarian dysgerminoma that was limited to the left ovary without capsular invasion (TNM stage: T1A, N0, M0 according to FIGO staging). The excised left fallopian tube, omentum, and peritoneal washout were all negative for malignancy.\nPostoperatively, the patient showed satisfactory progress and was therefore discharged in a stable condition. She is planned for on oncology follow-up visit as an outpatient.","A nine-year-old girl came to the hospital because of severe abdominal pain and a large, firm mass in her lower right abdomen. Doctors used a CT scan to find that the mass was a twisted ovarian tumor, and it was causing problems with blood flow. She underwent surgery to remove the tumor and her left fallopian tube, and the pathology report confirmed it was a type of ovarian cancer. The surgery was successful, and she is now recovering at home and will be seen by an oncologist for follow-up care."
650,"Informed consent was obtained from the patient prior to the submission of this paper.\nA 74-year-old Caucasian woman presented with fever, fatigue, and painful erythematous nodules. Her oncologic history was significant for MDS (refractory cytopenia with multilineage dysplasia subtype) diagnosed three years previously. She received 23 cycles of azacitidine (AZA). On initial presentation, her temperature was 101.4° F with tachycardia. Physical examination was significant for conjunctival pallor, tender erythematous vesicles on her right temple and bilateral ear lobes extending to the right periocular area, and tender erythematous nodules on her buttocks. A complete blood count showed pancytopenia (white blood cell count of 2.0 x 10­9/L with an absolute neutrophil count of 1,500/mm3, hemoglobin of 8.1 g/dL, and platelet count of 16 x 109/L). Given the concern for sepsis, she was started on antibiotics (1 gm of vancomycin and aztreonam every 12 hours) and antiviral medications (650 mg of acyclovir every eight hours). Despite that, she was persistently febrile with worsening of her condition and development of new erythematous plaques and nodules over her shoulders, forearms, and lower extremities (Figure 1).\nA 5-mm x 5-mm x 6-mm punch biopsy of an erythematous nodule over her right shoulder showed subcutaneous lobular and septal infiltrates of neutrophils and scattered histiocytes with sparing of the dermis, consistent with NP (Figure 2).\nSpecial stainings (Gram's method, Ziehl-Neelsen, and Periodic acid–Schiff stains) and tissue cultures for bacteria, mycobacteria, and fungal infections were negative. Blood cultures were negative for infection. Bone marrow biopsy did not show evidence of transformation of her MDS to acute myeloid leukemia (AML). A diagnosis of MDS-related SSS was made. She was started on oral prednisone, 60 mg/day, and had marked clinical improvement with resolution of her fever within 24 hours. Although she had relapsed painful erythematous nodules and plaques with a rapid steroid taper, she reported resolution of her cutaneous lesions weeks later at follow-up with a slow steroid taper. In the meantime, AZA was withheld.","This 74-year-old woman came to the hospital with a fever, fatigue, and painful bumps on her skin. She has a history of a blood disorder called MDS and had been receiving treatment for it. After tests, doctors found she had a skin infection called SSS, likely related to her MDS, and started her on antibiotics and antiviral medications. Fortunately, she responded well to treatment, and the skin lesions cleared up after a few weeks."
651,"A 68-year-old Caucasian man presented with generalized weakness, dizziness without syncope, polyuria, and dyspnea on exertion. He had a past medical history of hypertension, hyperlipidemia, and coronary artery disease. Physical examination was as follows: temperature 99.3°F, pulse 84 per minute, blood pressure 168/80 mmHg, respiratory rate 18 per minute. A grade IV/VI systolic murmur was heard over the apex radiating to left axilla and back, and a grade III/VI systolic murmur was best heard at the aortic area, bibasilar crackles, hepatomegaly and pitting edema of the bilateral lower extremities were noted. Laboratory data included hemoglobin of 6.5 g/dL and blood urea nitrogen (BUN)/creatinine 71 md/dL/6.3 mg/dL, white blood cell, platelet count and lactate dehydrogenase (LDH) levels were normal. Two months previously, hemoglobin and renal function studies were normal. Urinary protein excretion was increased, but not in the nephrotic range (Microalbumin/Cr ratio = 2.00). Hepatitis B and C serology, antineutrophil cytoplasmic antibodies (ANCA), antinuclear antibody (ANA), SSA, SSB, antistreptolysin O, and anti-glomerular basement membrane (GBM) antibodies were negative and C4 complement level was normal, rheumatoid factor (RF) was 2048 IU/M and serum C3 level was 65 mg/dL (ref 80-180 mg/dL). Renal ultrasound was normal. Complete evaluation for multiple myeloma was negative.\nTransthoracic echocardiogram demonstrated severe mitral regurgitation and multiple hyperechoic masses on the tips of both mitral leaflets with a small mobile mass on the posterior mitral leaflet (Figure ).\nIt also demonstrated aortic regurgitation and a mobile echogenic structure (4 mm x 4 mm), attached to ventricular side of aortic valve (Figure ).\nSubsequently, Streptococcus parasanguinis was isolated from blood cultures. The organism was sensitive to penicillin and ceftriaxone. Antibiotics treatment for endocarditis was initiated. Hemodialysis was required due to worsening kidney function BUN/Cr 114 mg/dL/10.3 mg/dL. Electron microscopy of renal biopsy showed crescent formation (Figure ).\nImmunofluorescence showed immune complex deposition (Figure ).\nDespite antimicrobial therapy, there was no improvement in kidney function, and the patient remained dialysis dependent. Corticosteroids and cyclophosphamide were administered. Double valve replacement surgery was performed because of severe aortic and mitral valve regurgitation. Echocardiogram after surgery did not demonstrate mitral or aortic valve regurgitation, and the bioprosthetic mitral and aortic valves were normally functioning. The patient remained on dialysis despite antibiotics, steroids and cyclophosphamide. An arteriovenous (AV) fistula was placed in the left arm and maintenance hemodialysis was recommended three times per week.\nOn follow-up at one year, the patient remained on hemodialysis in otherwise stable condition.","68-year-old man came to the hospital with feeling weak, dizzy, and having to urinate more often. Tests revealed he had low red blood cells and kidney problems, along with an infection in his heart. An echocardiogram showed significant problems with his heart valves, and bacteria were found in his blood. After treatment with antibiotics and surgery, he remained on dialysis, but is otherwise stable."
652,"A 53-year-old white man presented to our Department with a 2-month history of a painful and moderately swollen left wrist. His past medical history was unremarkable. Standard anteroposterior and lateral X-rays of his left wrist revealed two osteolytic lesions involving the distal ulna and the lunate fossa of the distal radius without any joint involvement (Fig. , ). Subsequent biopsy of his left ulna under regional anesthesia produced brown spongy material, histologically characterized by the presence of large numbers of multinucleated giant cells and spindle cells in a dense collagenous background. These findings were histologically consistent with a diagnosis of GCT and correlation with the clinical and radiological findings was recommended by the pathologist. As he had no other skeletal manifestations, a complete resection of the distal ulna (9.5 cm length) followed, along with curettage and cementoplasty of the distal radial metaphysis, to support the articular surface (Fig. , ). The resected distal ulna specimen and the curettings from the distal radius were submitted for histopathological evaluation; our patient was discharged 2 days later, with a forearm cast and instructions to attend the clinic in 2 weeks’ time for re-evaluation and removal of sutures.\nTwo weeks postoperatively, he was re-admitted to our orthopedic department with diffuse musculoskeletal soreness, anorexia, constipation, nausea, and localized abdominal pain. He also reported weight loss of approximately 5 kg. On palpation he had tenderness in the thoracic wall, the second and fifth metacarpals of his right hand, the left tibia, the pelvic ring, and the left shoulder girdle and humerus. Plain radiographs revealed multiple osteolytic lesions in his ribs, right hand, left tibia, and scapula (Fig. –).\nA histopathological examination of both the resected ulna (Fig. –) and the curettings of the radius (Fig. ) revealed similar findings: numerous, multinucleated, osteoclast-type giant cells were noted amid a mononuclear, spindle cell, histiocytoid component (Fig. ). Many of the giant cells were clustered in large nodular aggregates separated by fibrous septa containing fibroblast-like spindle cells. The spindle cell component showed no evidence of atypia or sarcomatoid features (Fig. ). There were prominent foci of hemorrhage with relatively restricted hemosiderin deposition (Fig. ). Mitoses were observed (up to five mitotic figures/ten high power fields) but no atypical mitoses or necrosis were seen. On the resection specimen of the ulna, the lesion focally disrupted the cortex producing periosteal reaction with woven bone trabeculae, extending in the surrounding adipose tissue and skeletal muscle (Fig. ). Based on the similar findings of both lesions and the rarity of multifocal GCT of bone the histopathology report included in the differential diagnosis a BT of hyperparathyroidism, either primary or in the setting of a paraneoplastic PTH-like protein production and suggested further patient evaluation.\nOur patient’s laboratory examination showed high total serum calcium (14.5 mg/dl, normal range 8.8–10.4), low serum phosphorus (2.3 mg/dl, normal range 2.5–4.5), and low 25-hydroxyvitamin D (9.74 ng/ml, normal range > 30). PHPT was suspected and confirmed by the elevated PTH levels of 1453 pg/mL (normal range 15–65). Serum potassium and sodium concentrations and thyroid hormone levels were in reference range as well as the main cancer indicators: cancer antigen (CA) 15-3, carcinoembryonic antigen (CEA), CA 125, and prostate-specific antigen (PSA). Serum protein electrophoresis was also normal. His human chorionic gonadotropin (hCG) was elevated (25.3 mUl/ml, reference level < 5). An isotope bone scan showed multiple sites of uptake over his ribs bilaterally, the lower pole of both scapulae, multiple foci in his pelvis, the metacarpal bones of his right hand, and his right tibia. At subsequent radiological work-up, both computed tomography (CT) and ultrasonography of his neck revealed a 4.5 × 2.5 × 3.2 cm mass emanating from the right lobe of his thyroid gland. Parathyroid subtraction technetium-99m (99mTc) sestamibi (MIBI) scintigraphy showed extensive uptake in his right lower parathyroid gland (Fig. ). Multiple endocrine neoplasia was excluded because of the normal MRI imaging of his pituitary gland.\nAppropriate medical care was given to our patient including hyperhydration and high doses of diuretics and diphosphonates. After his health status improved and his serum calcium nearly normalized, a specialist surgeon was consulted for further surgical treatment. Surgery consisted of extensive resection: total thyroidectomy with removal of the parathyroid glands. A mass measuring 4.8 cm in greatest diameter, abutting the thyroid gland was documented at surgery. The mass was surrounded by a thick capsule, had a tan-brown, solid, and microcystic cut surface, and rubbery consistency. On histologic examination, the tumor comprised small cells with minimal to scanty cytoplasm and round nuclei, arranged in an organoid pattern, with frequent perivascular pseudorosettes. Thick fibrous septa emanating from the capsule were noted within the tumor. There was capsular invasion, with extension of neoplastic groups in the surrounding loose connective tissue adjacent to striated muscle, and foci of vascular invasion in the tumor capsule. The histologic findings were consistent with a parathyroid carcinoma. The neoplasm did not appear to invade the adjacent thyroid lobe and did not involve the margins of resection.\nOur patient experienced postoperatively persistent hypocalcemia requiring calcium and vitamin D replacement. His condition was characterized as “hungry bone syndrome.” He is now recovering 12 months after surgery, with a serum PTH level of 7.1 pg/mL and serum calcium level of 10.7 mg/dl and he is under calcium and vitamin D replacement therapy. The lytic bone lesions have almost disappeared (Fig. –) and no other additional orthopedic intervention is necessary. He is closely followed by general surgeons, oncologists, and endocrinologists.","This patient initially presented with pain and swelling in his wrist, leading to a surgery to remove a cancerous growth. However, after the surgery, he developed new, similar bone lesions in other parts of his body, including his ribs, hands, and legs. Further investigation revealed that he had a rare type of cancer called parathyroid carcinoma, which was causing his high calcium levels. After a total thyroidectomy and removal of the cancerous parathyroid gland, his condition has improved, and he is now receiving ongoing monitoring and treatment to manage his calcium levels."
653,"On March 2017, a male, caucasian, 56-year-old, non-smoker patient came to our observation. He was employed as a truck driver. Remote clinical history included hepatitis, nasal polyposis treated with functional endoscopic sinus surgery (FESS), chronic sinusitis, gastroesophageal reflux disease (GERD) undergone laparoscopic GERD surgery (fundoplication). In 2016, a diagnosis of non-allergic asthma was made. Skin prick tests were negative. Respiratory function tests showed a moderate obstruction (FEV1 2.20 L, 68% of predicted; FEV1/FVC 67%) without bronchial reversibility after 400 μg of inhaled salbutamol. A few months later, after normalization of lung function parameters following maximal therapy, a positive bronchial provocation test with methacholine showed a degree of bronchial hyperresponsiveness congruent with the diagnosis of bronchial asthma (PDV20 FEV1 136 mcg). Antineutrophil cytoplasmic antibodies (ANCA) were negative. The therapy included formoterol/fluticasone metered-dose inhaler 250/10 μg, two inhalations twice daily and as needed (twice a day on an average), tiotropium bromide 2.5 μg soft mist inhaler, montelukast 10 mg/day. Due to frequent exacerbations and poor control of asthma, systemic corticosteroids (either oral or parenteral) had to be prescribed for over 6 months and 7 unscheduled visits were required in the previous year.\nOn February 2017 a chest high-resolution computed tomography (CT) scan showed only mild fibrotic scarring in the anterior basal segment of the lower right lung lobe. On September 2017 the patient received a first session of BT, in the lower right lobe, without any tolerability problem. Three days before the procedure systemic oral corticosteroids (prednisone 50 mg/day) were administered according to usual protocol, to control potential exacerbation of airway inflammation. Immediately before bronchoscopy, inhaled bronchodilators (nebulized salbutamol and ipratropium bromide) were given, along with nebulized lidocaine to provide topical anesthesia. Atropine 0.4–0.6 mg was administered intravenously 15–30 min before the procedure to minimize secretions. According to our procedure, the patient is placed under moderate sedation, a peripheral intravenous (IV) line is taken and supplemental oxygen is administered. The bronchoscope is positioned at the first treatment site, usually the most distal airway in the targeted lobe and the Alair Catheter is introduced through the working channel. The electrode array at the tip of the Catheter is expanded to contact the airway wall. The bronchoscopist activates the RF Controller to deliver low-power, temperature-controlled RF energy to the airway; the energy transfer ends automatically upon completion of the cycle (about 10 s). A single activation of the Catheter delivers RF energy over a distance of approximately 5 mm. After each procedure there is a 1–2 days hospitalization with monitoring of vital parameters and control of chest radiography. On October 5, 2017 a second BT session was performed in the left lower lobe, according to the same procedure. On October 23, persistent haemoptysis appeared requiring patient admission to our ward. On physical examination there was a reduction of breath sounds at the left pulmonary base. The patient underwent a new chest CT scan: around the basal anterior segmental branch line of the left lower lobe, mild varicoid bronchiectasis and distal parenchymal infiltrate were present, in addition to mild signs of airway inflammation in the right lower lobe (Fig. a). A fibreoptic bronchoscopy showed two small nodular neoformations in the sub-segmental branches of the same lobe (Fig. b). Endobronchial ultrasound (EBUS) with radial probe exploration of the left lower lobar bronchus was also performed with evidence of peripheral infiltrate, sampled with four trans-bronchial biopsies. These changes were not present on endoscopy at the time of the BT procedure, and were detected only after the appearance of haemoptysis.\nOn histological examination bronchial mucosa showed mild non-specific inflammation. Some large fragments of peribronchial pulmonary parenchyma (Fig. d) presented an area of haemorrhagic necrosis, with blood and fibrin, hemosiderin deposits and organizing pneumonia (Fig. d, e). No neoplastic cells were observed. Infectious agents were excluded after histochemical staining with Grocott and Ziehl–Nielsen methods. Microbiological cultures for aerobe and anaerobe bacteria were negative as well as cultures for mycobacteria. The patient was treated empirically with co-amoxiclav 1 g three times and azithromycin 500 mg daily for 6 days and prednisone 25 mg per day for 10 days. On December 18, a new chest CT showed a complete resolution of the parenchymal opacities (Fig. f). On January 9, 2018, the patient underwent the third session of BT, without recurrence of haemoptysis or subsequent radiological changes. Before performing the last BT session a fibreoptic bronchoscopy showed a complete resolution of the previously reported endoscopic findings.","This 56-year-old truck driver has a history of asthma, GERD, and nasal problems, and has struggled to control his asthma symptoms over the past few years, requiring frequent hospital visits and steroid medications. After trying various medications, doctors decided to use a procedure called BT to treat a localized area of inflammation in his lungs. During the procedure, a flexible tube was inserted into his airways to deliver targeted energy, and he was given medication to help him relax. Unfortunately, after the third BT session, he started coughing up blood, and further tests revealed inflammation and some abnormal tissue changes in his lungs. After treatment with antibiotics and steroids, the inflammation resolved, and he was able to complete the BT procedure."
654,"A 54-year-old Caucasian male presented with an enlarging right neck mass in November, 2015. Fine-needle aspiration (FNA) was performed on the mass at that time which showed malignant cells consistent with squamous cell carcinoma. The patient did not have follow-up or further treatment at that time due to socioeconomic issues. His past medical history is significant for alcoholism, tobacco abuse, noninsulin-dependent type 2 diabetes mellitus, and osteoarthritis. For the next sixteen months, he reported three flares of painful neck adenopathy. He sought treatment, and short courses of antibiotics and steroids were administered each time.\nIn March of 2017, his latest flare of right-sided neck adenopathy did not respond to antibiotics and steroid treatment course. He presented to the Emergency Department and found to have a grossly palpable mass in the right neck. He reported no symptoms of fevers, chills, night sweats, fatigue, or weight loss. Computed tomography (CT) revealed multiple low-density cystic structures in the right neck consistent with necrotic lymph nodes. The lymph nodes ranged in size from 1.4 cm to 2.9 cm in greatest dimension. No additional masses were detected in nasopharynx, oropharynx, or larynx. At this point, the patient was admitted for further workup and management. PET-CT showed right neck hypermetabolic uptake ranging from SUV of 4.3 to 4.5, and a CT of the chest showed no obvious disease and no evidence of lymphadenopathy. Following an FNA suggestive of either an anaplastic carcinoma or a hematolymphoid neoplasm, an excisional biopsy of the neck mass was performed.\nHematoxylin and eosin- (H&E-) stained right neck mass excisional biopsy material demonstrated lymph node and soft tissue with sinusoidal infiltration of large atypical monomorphic cells with round nuclei, occasional prominent central nucleoli, and abundant amphophilic cytoplasm. The lymph node was mostly effaced by tumor cells, but the uninvolved areas appeared unremarkable and showed residual small mature lymphocytes ().\nAn extensive immunohistochemical panel was performed to aid the diagnosis with appropriate reactive controls (Figures and ). The tumor cells expressed CD45, weak CD38, CD138, and EMA. ALK-1 showed a restricted granular cytoplasmic staining pattern highly suggestive of CLTC-ALK fusion protein expression. MIB-1/Ki-67 was approximately 80%. Interestingly, the tumor cells also showed strong and diffuse CD33 expression. The tumor cells were negative for PAX-5, myeloperoxidase (MPO), TIA-1, EBV, HHV8, CD2, CD4, CD5, CD7, CD8, CD20, CD30, CD34, CD79a, and kappa/lambda light chains. Other nonhematopoietic immunostains including CK OSCAR, CK7, melan-A, CK5/6, p40, and p63 were negative. Flow cytometry, cytogenetics, and FISH studies were not performed on the sample. Based on the morphology and immunohistochemistry, a diagnosis of ALK+ LBCL was rendered. The case was also reviewed at NIH, and the diagnosis was confirmed. Due to the aberrant CD33 expression, additional molecular studies were performed in an attempt to identify any myeloid malignancy-associated mutations. A myeloid malignancies mutation panel by next generation sequencing (NGS) was performed and did not reveal any significant mutations. Currently, the patient is under treatment protocol, which includes three cycles of CHOP chemotherapy to be followed by radiation therapy.","A 54-year-old man was initially diagnosed with squamous cell carcinoma in his neck in 2015, but didn't receive further treatment. Over the next few years, he experienced recurring painful swelling in his neck, which was treated with antibiotics and steroids, but didn't fully resolve. Recent tests revealed a more aggressive type of lymphoma (ALK+ LBCL) in the neck, confirmed by a biopsy and further testing. He is now undergoing chemotherapy and radiation therapy to treat the lymphoma, and doctors are continuing to monitor his progress."
655,"A 41-year-old man, a native from Rio de Janeiro and an HIV and HCV carrier, without criteria for the treatment for HCV (detectable viral load, without cirrhosis and with normal transaminase levels), who had abandoned ART, had attended the Gaffreé and Guinle University Hospital's immunology clinic complaining about continuous epigastric burning pain without irradiation and with diffuse abdominal pain that was mild and continuous, which had started approximately two months prior to admission. He also complained about intense hematochezia that had started three weeks before, with intense flow and with “pure blood” appearance without clots. He presented with daily hyperthermia since the abdominal symptoms had started with intermittent high fever and an over 10% body weight loss in the same period. The physical examination revealed oral candidiasis, bleached mucous membranes, and cachexia. At the admission time, the HIV viral load was recorded at 905,569 copies per ml, and the TCD4 lymphocyte count was 144 cells/dL. Prophylactic sulfamethoxazole-trimethoprim 400/80 mg 2 IV ampoules once daily and fluconazole 200 mg IV once daily for treatment of the oral candidiasis were prescribed. The patient's condition evolved without major occurrences or complaints, presented with high fever, above 38°C almost every day. Blood counts revealed thrombocytopenia, neutrophilia, lymphopenia, anemia, microcytosis, and anisocytosis (). The medical team requested upper digestive endoscopy () and colonoscopy (), which verified the presence of ulcer with irregular and raised edges, fibrinonecrotic base, measuring approximately 3 cm in the middle third of the esophagus and 30 cm from the incisors and the mild antrum gastritis, and swollen, irregular, and fibrinous ulcers in the ileocecal valve, descending colon, and all other segments. The lesions were similar to those found in the esophagus, which could suggest the same etiology. It was suggested by the internal medicine team that the diagnosis could be a coinfection (tuberculosis, cytomegalovirus, and herpes simplex virus disease). The diagnosis of tuberculosis and cytomegalovirus coinfection of the gastrointestinal tract was confirmed by the histopathological report (Ziehl–Neelsen staining of acid-fast bacilli, CMV intracytoplasmic inclusions in Giemsa staining, and immunohistochemical study with positive labeling for CMV in cells with clear halos), and some time later, culture with the growth of M. tuberculosis (). Treatment was started with an RIPE scheme (rifampicin + isoniazid + pyrazinamide + ethambutol) 4 tablets daily and ganciclovir 350 mg IV for 21 days with a weight gain of 4 kg and clinical and laboratory improvement. He was discharged from the hospital with ART lamivudine, tenofovir, and efavirenz (TDF + 3TC + EFV) one tablet per day and was referred to a clinical follow-up for tuberculosis and HIV/HCV coinfection monitoring. At the end of the treatment for tuberculosis and 6 months after ART was restarted, the patient's viral load was <40 copies/dL and the CD4+ T-cell count was 356 cells/dL, asymptomatic.","A 41-year-old man with a history of HIV and Hepatitis C, who had stopped taking his HIV medication, came to the hospital with stomach pain, bleeding, and a fever. Tests revealed ulcers in his esophagus and colon, likely caused by a combination of tuberculosis and a virus. After receiving treatment for both infections, his condition improved, and his HIV medication was restarted, leading to a significant drop in his viral load and an increase in his immune cell count. He was discharged home with ongoing monitoring and support."
656,"A 45-year-old male patient gazed at the sun several times during a baseball game that took place on a sunny day at 7 weeks prior to his first visit to our clinic. Immediately after gazing at the sun, the subject reported having bilateral central scotoma and decreased vision. At the time of the incident, the patient was taking etizolam for a psychiatric condition (panic disorder). At the first visit, his decimal best corrected visual acuity was 0.8 (logMAR conversion: 0.10) (with -3.00 diopters, cylinder -1.00 diopters axis 5°) in the right eye and 0.7 (logMAR conversion: 0.15) (with -3.00 diopters, cylinder -1.00 diopters axis 180°) in the left eye. Slit lamp examinations showed no abnormalities in the anterior segments and media of both eyes. Fundus examinations showed a tiny, yellowish spot in the fovea bilaterally (). FAF (Spectralis HRA; Heidelberg Engineering, Heidelberg, Germany) (), fluorescein angiography, and indocyanine green angiography all indicated that there were no remarkable abnormalities in either of the eyes. OCT (Cirrus HD-OCT; Carl Zeiss Meditec AG, Dublin, CA, USA) images showed an elevated and blurred ellipsoid zone along with loss of the interdigitation zone at the foveal area bilaterally (). There was also no vitreomacular adhesion or traction seen in either of the eyes (). When the findings were taken together, the patient was diagnosed with solar retinopathy due to the characteristic symptoms and bilateral findings present after an episode of sun gazing. Treatment was started at the first visit, with the patient given a posterior sub-Tenon triamcinolone injection in his right eye followed by being placed on oral prednisolone therapy (30 mg per day) on the same day. The prednisolone therapy was decreased over a 12-week tapering period. There were no changes noted in the decimal best corrected visual acuity at 2, 4, and 6 weeks after starting the medication. However, at 9 weeks, there was improvement to 1.2 in the right eye and 1.0 in the left eye, with this good visual acuity sustained and observed at the examinations at 12 and 21 weeks. Fundus examinations performed at 12 weeks after the initial treatment showed the tiny, yellowish spots were diminished in both eyes. Sequential OCT images obtained during the follow-up examinations showed that the blurred ellipsoid zone that was visible in both eyes at 2 weeks after initiation of the therapy along with the elevated ellipsoid zone both improved to nearly normal at 4 weeks in the right eye and at 21 weeks in the left eye. However, loss of the interdigitation zone was observed after 12 weeks in the right eye and after 21 weeks in the left eye ().","This patient experienced vision problems after looking directly at the sun, which caused temporary blind spots and blurry vision. Tests revealed a small spot in his eyes, indicating solar retinopathy. He started taking medication to reduce inflammation and promote healing, and his vision gradually improved over several months. Follow-up exams showed the spots faded, and his vision returned to near normal."
657,"A 55-year-old woman with a history of type 2 diabetes mellitus, hyperlipidemia, obesity, and depression was referred to an endocrinologist with complaints of weight loss, palpitations, and diarrhea. The patient also had hypertension and was taking α-adrenergic receptor antagonists and a calcium channel blocker. She was found to have a thyroid-stimulating hormone (TSH) level of <0.10 (normal: 0.34 to 4.82) µlU/ml and a free T4 concentration of 4.28 (normal: 0.6 to 1.6) ng/dL. I123 thyroid scan revealed elevated, diffuse uptake bilaterally, without nodules, consistent with the diagnosis of Graves' disease. The patient was treated with 11.9 mCi of radioactive iodine. Ten days after the ablation treatment, the patient presented to a local hospital by ambulance after experiencing lightheadedness, diffuse abdominal pain, and one episode of bilious emesis.\nUpon arrival, she was hypotensive (77/44 mm Hg), pale, bradycardic, and febrile (39.4°C). An electrocardiogram (ECG) revealed accelerated junctional rhythm at a rate of 53 beats/min. The patient was given atropine 0.5 mg intravenously without effect, followed by initiation of external cardiac pacing. Continuous intravenous infusions of dopamine and norepinephrine were started along with fluid resuscitation of 4 L of normal saline over a 2-hour period. She received one ampule of calcium gluconate with no change in her blood pressure, heart rate, or rhythm. Computed tomography of the abdomen was unrevealing. The patient was endotracheally intubated and transferred via helicopter to our facility.\nUpon arrival to our facility, she was receiving intravenous infusions of dopamine at 20 µg/kg/min and norepinephrine at 10 µg/kg/min and remained hypotensive (92/55 mm Hg) and bradycardic (59 beats/min). ECG showed an accelerated junctional rhythm. Laboratory findings included serum sodium of 139 mEq/L, potassium of 5.3 mEq/L, chloride of 108 mEq/L, and total CO2 content of 15 mmol/L, serum glucose of 208 mg/dL, urea nitrogen of 38 mg/dL, creatinine of 1.5 mg/dL, ionized calcium of 1.15 mmol/L, total bilirubin of 0.4 mg/dL, serum alkaline phosphatase of 141 U/L, aspartate aminotransferase of 2196 U/L, and alanine aminotransferase of 2010 U/L. Plasma troponin I was repeatedly undetectable. The peripheral leukocyte count was 15.9 x 109 cells/L with no immature forms. There was evidence of an anion gap metabolic acidosis with a serum lactate concentration of 6.2 mmol/L. Blood cultures revealed no microbial growth. Thyroid function testing showed an undetectable TSH (<0.10 µlU/ml), a free T4 of 12.8 (normal: 0.6 to 1.6) ng/dL, total T4 of 21.9 (normal: 5.6 to 13.7) µg/dL, and a total T3 of 0.94 (normal: 0.8 to 1.8) ng/mL. The patient was treated for thyroid storm with 1000 mg of propylthiouracil by orogastric tube as a loading dose followed by 300 mg every 6 hours, 5 drops saturated solution of potassium iodide (SSKI) every 8 hours by orogastric tube, and 100 mg intravenously of hydrocortisone every 8 hours. The hypotension resolved, vasopressors were stopped, and the patient was extubated 25 hours after her initial presentation. During her stay in the intensive care unit, the patient exhibited fever (39.6°C maximum), tachycardia, and tremulousness. These manifestations resolved over a period of 22 hours. The patient was discharged in satisfactory condition on the fourth day of hospitalization on 100 mg of propylthiouracil orally every 8 hours. Outpatient testing days later demonstrated normalization of her thyroid function tests.","This 55-year-old woman was referred to an endocrinologist because she was losing weight, experiencing heart palpitations, and having diarrhea. Tests revealed she had Graves’ disease, an overactive thyroid, which was causing her symptoms. After a radioactive iodine treatment, she developed a serious reaction with low blood pressure and a rapid heart rate, requiring intensive care and support with medications and a pacemaker. Fortunately, with treatment for her thyroid storm, her condition improved, and she was able to return home on medication to manage her thyroid function."
658,"A 69-year-old Caucasian man with schizophrenia represented to our emergency department (ED) from a psychiatric hospital with catatonia, notable for agitation and altered mental status requiring physical restraints. Limited physical exam was revealing for increased tone and rigidity in bilateral lower extremities while the patient self-dialogued and yelled at times. Per outside records, he was observed to be persistently agitated, engaging in self-injurious behaviors such as hitting himself, banging his head, and refusing to eat or drink for a week.\nThree weeks prior, he was admitted to the medicine service with early signs of NMS that resolved over the course of a few days with discontinuation of neuroleptics and treatment with parenteral lorazepam. He was subsequently transferred back to the outside hospital psychiatric unit for further stabilization and optimization of his psychotropic regimen, with a recommendation to avoid high-potency neuroleptics. There, he was started on fluphenazine, a high-potency first generation antipsychotic, after a washout period of one week. His religious delusions with disorganized thought process showed minimal improvement. He was subsequently switched to haloperidol, which was rapidly increased to 35 mg per day. Clonazepam 1.5 mg per day and lorazepam 1 mg per day were also utilized over this time frame. The patient, however, became increasingly agitated, with self-injurious behavior and some posturing that was attributed to “refractory psychosis.” This prompted further antipsychotic dose escalation. He had stopped eating or drinking by this time with associated worsening of behavioral dysregulation. 75 mg of chlorpromazine was given the same day after 35 mg of haloperidol showed minimal benefit. While chlorpromazine temporarily decreased his behavioral dysregulation, his agitation continued unabated the following morning. He was given additional chlorpromazine 25 mg with fluid resuscitation in urgent care before his transfer to our facility for a delirium work-up.\nOn arrival, standard treatment was implemented, including antipsychotic discontinuation, supportive care, and initiation of parenteral benzodiazepines consisting of lorazepam 2 mg intravenously (IV) every 8 hours. His complete blood count, electrolytes, vitamin B12, thyroid function, and liver function tests were within normal limits, except for a CK of 1090 IU/L, mildly elevated 10,680 white blood cells per mcL, low iron level of 26 ug/dL, and creatinine value of 1.26 mg/dL thought to be prerenal in nature secondary to dehydration. He tested negative for syphilis on the rapid plasma reagin test. Urinalysis did not reveal an infection. Urine drug toxicology was positive for benzodiazepines only, which the patient had been receiving on the inpatient unit. A head CT revealed no acute intracranial pathology. An electroencephalogram performed on day 4 of admission was notable only for increased beta waves, reflecting the high-dose benzodiazepines he was receiving at the time.\nThe patient's hospital course was notable for continued fluctuating motor symptoms with episodes of severe rigidity in both upper and lower extremities, intermittent droning vocalizations, waxy flexibility, posturing, negativism, automatic obedience, and presence of mitgehen. Such periods were accompanied by diaphoresis and autonomic hyperactivity. On the fifth day, lorazepam was increased to 4 mg IV every 4 hours and amantadine 100 mg (by mouth) PO twice a day was started the following day. The doses were held past midnight due to planned ECT on the seventh day of admission. Lorazepam was resumed post-ECT but amantadine was held until after the third session. Prior to each subsequent ECT treatment, benzodiazepines were held to prevent increasing the patient's seizure threshold. He received a total of three ECT sessions, each three days apart. In between the second and the third ECT sessions, the patient became overtly delirious secondary to a urinary tract infection, which was treated with antibiotics. After the second ECT session, lorazepam was decreased to 2 mg IV every 6 hours and then switched to a longer acting diazepam 20 mg IV every 6 hours after the third ECT session. Amantadine was restarted at the same dose and was increased to 100 mg PO three times a day, four days after the last ECT session. After a three-week washout period, Clozapine 12.5 mg PO twice a day was introduced for underlying psychotic symptoms and titrated to 25 mg twice a day without recurrence of NMS or catatonia. With gradual lysing of catatonia and normalization of lab values, the patient was discharged back to the inpatient psychiatric unit after a month-long hospitalization.","69-year-old man was admitted to the hospital after experiencing agitation, self-harm, and difficulty communicating due to a worsening of his schizophrenia. He had a history of catatonia and had previously struggled with side effects from medications. After trying several medications, doctors started him on Clozapine, which helped to calm him down and reduce his symptoms. He also received ECT treatments and antibiotics for a urinary tract infection, and his condition gradually improved, allowing him to return to a psychiatric unit for continued care."
659,"A 67-year-old woman, gravida 1, para 1, with a medical history of psoriasis and bipolar affective disorder, presented with postmenopausal vaginal bleeding. Physical examination found an 18-week sized uterus without palpable groin lymph nodes. Both adnexa were unremarkable. Magnetic resonance imaging (MRI) of the pelvis and computed tomography (CT) with contrast of the abdomen and thorax demonstrated a localized anterior intrauterine mass with deep myometrial invasion. There was no pelvic or inguinal lymphadenopathy []. The liver was normal in size and outline, with no mass lesion demonstrated on contrast CT. All other intra-abdominal organs were unremarkable. Histologically, the uterine curettage showed carcinosarcoma composed of mixed endometrioid adenocarcinoma, chondrosarcoma, and a hepatoid component. The hepatoid component consisted of trabeculae of polygonal cells with moderate amount of eosinophilic cytoplasm, round to oval nuclei and distinct nucleoli, histologically reminiscent of hepatocellular carcinoma. Immunohistochemically (IHC), the hepatoid tumor cells are positive for AFP, HepPar-1, and arginase-1. Preoperative hepatitis B virus surface antigen was negative and liver function was normal. The patient underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. Serum alpha-fetoprotein (AFP) dropped from 31896 ug/l preoperatively to 2063 ug/l postoperatively []. Carbohydrate antigen 125 (CA125) level was normal.\nThe resected specimen weighted 575 g and measured 11.0 x 9.5 x 8.0 cm with an anterior exophytic tumor measuring 7.5 x 6.0 x 4.0 cm with a tan cut surface [] and detached hemorrhagic fragments. Microscopically, the tumor involved the outer half of the myometrium without extension to the cervix or the vagina. Bilateral ovaries were involved. Extensive lymphovascular permeation was seen. Histologic findings were those of a carcinosarcoma with endometrioid adenocarcinoma (20%), hepatoid adenocarcinoma (20%), and sarcomatous components consisting of chondroid (10%) and spindle cell components (50%) []. IHC staining was repeated and the profile was the same as the curettage specimen [].\nThe postoperative clinical course was uneventful, and she was planned to receive taxotere and cyclophosphamide (TC) as adjuvant chemotherapy. Before adjuvant therapy was initiated, a tumor was found in the residual vaginal canal on 8-week postoperative follow-up clinical examination, and biopsy confirming histological recurrence. The patient proceeded to chemotherapy (TC), and the serum AFP level showed a decrease from 6152 ug/l to 4288 ug/l after the first cycle. Serological response was sustained throughout the six cycles of chemotherapy, with a postchemotherapy serum AFP level of 4770 ug/l []. Follow-up CT of the thorax, abdomen, and pelvis did not show any radiological evidence of disease. A subsequent rise of serum AFP to 13409 ug/l was detected 1 month after completion of chemotherapy and the patient was started on Adriamycin. Serum AFP did not show response and was further elevated to 41826 ug/l after cycle two [], accompanied by the development of ureteric obstruction, ascites, and radiological evidence of liver and peritoneal metastases []. The patient succumbed 11 months postoperation.","A 67-year-old woman with a history of psoriasis and bipolar disorder was admitted after experiencing vaginal bleeding. Imaging revealed a large, invasive tumor in her uterus, which was later determined to be a rare type of cancer called carcinosarcoma. She underwent surgery to remove the uterus and ovaries, and initially showed a positive response to chemotherapy. However, the cancer unfortunately returned, and despite further treatment, she passed away 11 months after her initial diagnosis."
660,"A 62-year-old Japanese female patient presented with a left abdominal mass. She was referred to our surgical outpatient clinic to undergo a detailed examination and treatment for the left abdominal mass. A clinical examination revealed an elastic soft, smooth-surfaced, painless, child-head-sized tumor with poor mobility, which was located in the left upper abdomen. Abdominal computed tomography (CT) demonstrated a child-head-sized mass with heterogeneous contrast at the left upper abdomen around the stomach, spleen, pancreas, and left kidney on a horizontal image () and coronal image ().\nMagnetic resonance imaging (MRI) revealed a heterogeneously hyperintense mass on T1-weighted imaging (), a relatively uniform and hyperintense mass on T2-weighted imaging (), and a hypointense mass with an enhanced border on gadolinium- (Gd-) enhanced imaging (). A retroperitoneal tumor was diagnosed. Her laboratory data were white blood cell count, 4600/mm3; hemoglobin, 12.8 g/dl; hematocrit, 36.5%; and platelet count, 182,000/mm3, with normal electrolytes, as well as normal blood urea nitrogen levels, but slight liver dysfunction. Her serum levels of corticosteroid and/or androgen were 13.3 ng/ml (10.4–35.0 in female) and 173 pg/dl (35.7–240.0), respectively, which are within the normal ranges; however, her serum level of ACTH was elevated at 138.70 pg/ml (7.2–63.3).\nThe retroperitoneal tumor was resected (). The tumor was located at the left side of the stomach, posteriorly to the transverse mesocolon and pancreas, on the cranial side of the left kidney (Figures and ), but has not invaded the surrounding organs (Figures and ). The right adrenal gland was normal in size. The resected tumor was 20 × 18 × 10 cm in diameter and weighted 1500 g. An examination of the cut surface of the tumor revealed a multilobular yellow mass with bleeding in places ().\nA histopathological examination with hematoxylin and eosin staining revealed that the tumor was composed of a proliferation of mature and variable-sized adipocytes admixed with aggregates of hematopoietic elements, associated with adrenal gland tissue in the peripheral region within the tumor (). These findings were compatible with AML.\nThe patient had an uneventful recovery and was discharged from the hospital on the 6th day after the operation. She has been followed up in our outpatient clinic without recurrence for approximately 12 years since undergoing the operation.","A 62-year-old woman came to the clinic with a large mass in her left abdomen. Tests, including CT scans and MRIs, showed a tumor near her stomach, spleen, pancreas, and kidney. The tumor was surgically removed, and a biopsy revealed it was a type of leukemia. The patient had a successful recovery and has been monitored closely for over 12 years without any signs of recurrence."
661,"A 41-year-old man presented to the emergency department following an automotive accident where he was thrown from his motorcycle traveling approximately 35 mph. The patient denied loss-of-consciousness and chiefly complained of left wrist pain at presentation with exam demonstrating tenderness and swelling. Radiographs () revealed a volar dislocation and rotatory deformity of the proximal pole of the scaphoid. The distal pole remained properly located, articulating with the trapezium and trapezoid. No other injuries were identified. Closed reduction failed in the emergency department; therefore, the patient elected to proceed with operative management. A dorsal approach was used to access the radiocarpal joint. The scapholunate joint was completely disrupted and the lunotriquetral joint was found to be unstable as well. The distal pole of the scaphoid appeared to be appropriately located without ligamentous disruption. No fractures or chondral injuries were seen. Three 0.045-inch K-wires were placed into the proximal pole and used as a joystick in concert with dorsally directed manual pressure over the distal pole to reduce the scaphoid dislocation. These were then advanced across the scapholunate articulation to hold the reduction. Three more K-wires were passed across the lunotriquetral joint to address the instability. The distal pole was once again examined but did not demonstrate any instability. The capsule was closed with 0 VICRYL suture, and the K-wires were cut just below the skin. The subcutaneous layer was closed with buried 2–0 VICRYL suture, the skin was closed with 4–0 NOVAFIL suture, and the wrist was splinted (). The patient did well postoperatively and was brought back to the operating room eight weeks later for hardware removal. He went on to heal well and regained his wrist range of motion with occupational therapy. At most recent follow-up (), five months since injury, the patient had no complaints and had returned to work and all desired activities.","A 41-year-old man was brought to the hospital after a motorcycle accident, resulting in a dislocated wrist. Initial attempts to fix the dislocation in the emergency room were unsuccessful, so surgery was performed to realign the bones. During the surgery, the doctors addressed instability in the joints around the wrist and secured the bones with K-wires. The patient recovered well and was able to return to work and his normal activities after a few months of physical therapy."
662,"A 56-year-old female presented with repeated fevers in a span of four months. Her medical history was significant for systemic lupus erythematosus (SLE) with nephritis and hypothyroidism. Her medications were methylprednisolone and levothyroxine. She was allergic to cephalexin, penicillin, and levofloxacin. She was a nonsmoker, denied alcohol, and illicit drug use.\nIn her first hospital admission, she presented with fever and chills after traveling to Jamaica. She had no other symptoms. Her physical examination was only remarkable for fever of 38°C. Diagnostics revealed blood cultures positive for Salmonella enteritidis. Urine culture was negative. Due to her complicated history of antibiotic allergies, she was treated with aztreonam and discharged on trimethoprim/sulfamethoxazole (TMP/SMX) for two weeks.\nShe was well in the interim until one month later when she returned with fever (39°C), nausea, and dysuria. Physical examination identified costovertebral angle tenderness. Blood culture again grew the same organism, and urine culture was also positive. She was treated with aztreonam and discharged home with TMP/SMX for two weeks. Repeat urine culture as outpatient was negative for Salmonella.\nWithin the next month, she was readmitted with generalized body and joint pains. She was then treated with steroids for a lupus flare. During this admission, there were incidental findings of elevated lipase 668 U/L (reference range 4–66), and CT scan showed pancreatitis. She did not have any abdominal pain or symptoms to suggest acute pancreatitis. Her blood and urine cultures were negative. Gastroenterology service recommended that she be managed supportively. After clinical improvement, she was discharged to a rehabilitation facility.\nIn a week, she again developed fever accompanied by chills, nausea, and vomiting. There were no other symptoms. Physical examination was only remarkable for temperature of 38.5°C and blood pressure of 144/63 mm Hg. The examination was otherwise normal.\nThe serum white blood cell count was 13.1 × 103 µL (ref 4.8–10.8), and hemoglobin was of 7.9 (ref 12–16). Lipase was 22 U/L (ref 4–66). Bilirubins, transaminases, and the remainder of the metabolic profile were unremarkable. Blood and stool culture grew Salmonella enteritidis sensitive to ampicillin, aztreonam, ceftriaxone, ciprofloxacin, piperacillin/tazobactam, and trimethoprim/sulfamethoxazole.\nComputed tomography (CT) scan of the abdomen showed enlargement of the pancreatic head with adjacent stranding consistent with pancreatitis along with adjacent low-density regions anteriorly and inferiorly consistent with inflammatory liquefaction (). Other abdominal structures were normal. Magnetic resonance imaging (MRI) showed an 8 cm mass below the level of the pancreas.\nShe was initially given aztreonam for two weeks pending sampling of the pancreatic mass. The patient then changed her mind and refused the procedure. Since she was clinically doing well and repeat cultures were negative, she was monitored off antibiotics with a plan to do the procedure if she worsened.\nWhile awaiting discharge, she had a fever of 38°C. Blood and stool cultures were again positive. This time, she was given intravenous ceftriaxone. Repeat CT and MRI demonstrated multiple complex fluid collections around the pancreas: a 9 × 6 cm mass inferior to the pancreas, a 7 × 4 cm mass superiorly, and two smaller masses abutting the head and body.\nCT-guided drainage was performed with purulent fluid identified. A Jackson-Pratt drain was then placed (). Fluid culture grew Salmonella enteritidis. The patient was transitioned to 4 weeks of oral ciprofloxacin, which the patient tolerated despite the reported levofloxacin allergy. She was followed up as outpatient and was doing well.","This 56-year-old woman has been experiencing recurring fevers for several months, likely due to a Salmonella infection. She has a history of lupus and other health issues, which made it challenging to find effective antibiotics. After multiple hospital visits and tests, doctors discovered a mass in her pancreas and performed drainage to remove the infection. She is now taking oral antibiotics and is being closely monitored as an outpatient."
663,"A 30-year-old woman was transferred to our emergency department five hours after delivering her baby at a clinic. She was a primipara at 41 weeks of gestation. She delivered a baby with vertex presentation vaginally, without dystocia. Massive vaginal bleeding started 2 hours after delivery. After excluding birth canal laceration and retaining placental tissue, the obstetrician began IV fluid and uterotonic treatment, but the bleeding continued. She was then transferred to our hospital due to PPH. However, when she arrived, she had severe tachycardia (heart rate, 160 bpm) and hypotension (BP, 44/34 mmHg). Her consciousness was clear, but she was agitated. We immediately began transfusion of packed red blood cells (6 units), fresh frozen plasma (4 units), apheresis platelets (2 units), and whole blood (2 units) as we simultaneously examined the patient. Signs of DIC developed with continuous blood loss (), and her consciousness deteriorated within 30 minutes after arriving at the emergency department.\nUterine atony and an ischemic uterus were found during emergency laparotomy. A subtotal hysterectomy was completed. Intraoperative blood loss was 800 mL. The patient was transferred to the ICU after surgery. Her postoperative fibrinogen level was 54.6 mg/dL (normal: 200–400 mg/dL). We transfused fresh frozen plasma and cryoprecipitate to achieve a fibrinogen level greater than 100 mg/dL. However, unstable blood pressure and progressive abdominal distension were found 4 hours after the primary surgery. We rushed the patient back into surgery due to suspicion of internal bleeding. Hemoperitoneum of 2000 mL and active bleeding from ruptured pararectal vessels were identified. After the secondary surgery for ligation of the bleeding vessels, the patient had acute kidney injury with anuria, intractable hyperkalemia, and metabolic acidosis. Thus she underwent continuous venovenous hemofiltration (CVVH).\nThe patient's hemodynamic status and ventilation function gradually improved after hemostasis. CVVH was shifted to intermittent hemodialysis on postoperative day 10. She was extubated on the same day.\nUnfortunately, hyperbilirubinemia progressed and became the main problem (). Liver enzyme levels peaked on postoperative day 3 and then settled to about 100–200 IU/L (). Thrombocytopenia continued, along with prolonged prothrombin time (INR, 1.2–1.5) and activated partial thromboplastin time (1.5–2.5 times of normal control) (). Abdominal ultrasonography revealed no biliary tract obstruction but an ill-defined hypoechoic lesion in the right lobe of the liver, about 6.5 × 6.5 cm, probably due to an inflammatory process or tumor growth. Abdominal CT was indicated to confirm the characteristic of the lesion but was postponed due to the patient's poor renal function. After consultation with a gastrointestinal expert, the lesion was thought to be a liver abscess or focal necrosis due to ischemic change. Her consciousness had been deteriorating since post-PPH day 20 in combination with intractable fever. Brain CT showed no intracranial lesion. Worsening cognition was likely due to metabolic encephalopathy of hepatic or infectious origin.\nA liver transplantation was indicated, and a pretransplantation CT scan showed poor enhancement of the right hepatic lobe on the periphery covering about 50% of the total area, which was suggestive of liver infarction (). Living donor liver transplantation was performed 28 days after PPH. The donor was her younger brother, and the graft liver weight was 840 gm. The graft-to-recipient weight ratio was 1.24%. Intraoperatively, there were multiple micronodular lesions on the liver surface with marked swelling of the liver parenchyma and diffuse devitalized liver tissue in the right lobe of liver with necrosis and in the left lobe peripheral zone accounting for about 70%–80 % of the total liver volume (Figures and ).\nThe patient's bilirubin level improved on the first three days but increased again on the 4th day after transplantation. The coagulation parameters did not change significantly after liver transplantation. Serial liver ultrasonography showed acceptable vascular status of the transplanted liver, without thrombosis or biliary obstruction. Fever, tachycardia, and hypotension occurred on the 6th day after transplantation as well as vaginal and anal stool leakage. An emergency colostomy and perianal debridement were done under the suspicion of a perianal abscess and rectovaginal fistula. Unfortunately, the patient died of an intractable infection the next day.","This woman experienced a very difficult postpartum recovery after giving birth to her baby. She began bleeding heavily after delivery, and despite treatment, her condition worsened rapidly, leading to shock and the need for a hysterectomy. Following the surgery, she developed complications including kidney failure, high potassium levels, and a serious infection in her liver. Ultimately, she required a liver transplant from her brother, but sadly, she passed away due to an infection shortly after the transplant."
664,"A 55-year-old gentleman, ex-smoker, presented to our hospital complaining of mild epigastric pain, regurgitation, and heartburn. On top of that, he has a long-standing history of gastroesophageal reflux disease (GERD), which was managed by proton pump inhibitors. His past medical history was significant for hypertension. He was previously diagnosed with a liver hemangioma based on abdominal ultrasound two years before the presentation. He had no relevant family history. Physical examination revealed mild epigastric tenderness with no palpable abdominal mass. Laboratory data showed no anemia but positive stool occult blood test. Tumor markers including AFP, CEA, and CA 19-9 were all within normal range. Upper GI endoscopy revealed mild esophagitis, Los Angles grade A along with Barrett's esophagus without dysplasia and a 1 cm polyp at the GEJ. A sample was sent for histopathology; the rest of the stomach and duodenum were normal. The patient did not have a previous endoscopy prior to this one.\nInfused computed tomography (CT) of the abdomen and chest showed mild GEJ thickness with no evidence of mediastinal or celiac lymphadenopathy and no signs of metastasis. It also demonstrated a large heterogeneously enhancing mass about 6 × 9.5 cm with central necrosis in the upper abdomen that appears to be originating from the gastric antrum (greater curve). The mass was highly suggestive of GIST based on CT; it was the same mass that was previously misdiagnosed as a liver hemangioma (). Endoscopic ultrasound confirmed the previous findings. However, no biopsy was attempted due to the risk of bleeding.\nHistopathological examination of the GEJ polyp revealed tubulovillous adenoma with elements of adenocarcinoma in situ. The patient was admitted with a provisional diagnosis of early-stage adenocarcinoma of GEJ along with the incidental finding of enlarging gastric GIST. A trial of endoscopic mucosal resection of GEJ polyp was attempted but failed because of the polyp location that created a technical difficulty. Therefore, the patient was taken to the operating room with a plan to perform a wedge resection of the gastric mass and a submucosal resection of GEJ polyp through the same gastric opening. We planned to use frozen section (FS) to document negative margin resection and determine the need for a formal esophagectomy. Intraoperatively; a large (10 × 7 × 6 cm), extraluminal pedunculated mass was found at the posterior wall of the greater curvature of the stomach (). Wedge resection of the gastric mass with negative margins was achieved along with a transgastric submucosal resection of the GEJ polyp. Fortunately, the FS examination of the polyp showed negative margins as well with no evidence of deep invasion. Postoperatively, the patient had a smooth course and was discharged home in a stable condition. The final pathological examination revealed a GEJ polyp around 1.7 × 1.4 × 0.6 cm. Microscopically, there was a focus of invasive adenocarcinoma involving the superficial submucosa of the polypoid lesion, negative margins, and no lymphovascular invasion (T1a NxM0). Furthermore, the gastric wall mass measured around 10 × 7 × 6 cm with a 2 × 1.5 cm stalk. Histopathology revealed encapsulated high-grade epithelioid GIST tumor with negative margins (pT3). The mitotic rate of 6/50 HPF and immunohistochemical stains were positive for DOG1 and CD34 but negative for CD117 (c-Kit) ().\nThe final diagnosis was synchronous early-stage GEJ adenocarcinoma and a high-grade gastric GIST. Therefore, the patient was started on adjuvant imatinib treatment, along with endoscopic surveillance every six months and proton pump inhibitors.","This 55-year-old man came to the hospital with stomach pain and heartburn, which he’s had for a while due to GERD. During the visit, doctors found a polyp in his esophagus and a large mass in his stomach that turned out to be a type of cancer. Further tests revealed that he also had early-stage cancer in his esophagus. Fortunately, the doctors were able to remove the mass and the polyp with clear margins, and he will now receive medication to help prevent the cancer from returning."
665,"A 25-year-old G3 P0020 at 36 3/7 weeks of gestational age with a singleton pregnancy presents with acute-onset, severe back pain and fever. Pain was described as constant, aching, and sharp. Her prenatal course was significant for multiple left antecubital abscesses requiring drainage (culture positive for methicillin-resistant staphylococcus aureus, MRSA) at 34 weeks and she was treated with clindamycin. On initial questioning, she admitted daily tobacco use but denied intravenous drug use. She was afebrile on presentation, but nodularity was appreciated at the left antecubital fossa and she had lower back tenderness to palpation. Physical exam was otherwise unremarkable. Biophysical profile and nonstress test confirmed a reassuring fetal status.\nInitial white blood cell count (WBC) was 21 [K/uL], C-reactive protein (CRP) was 27 [mg/L], and erythrocyte sedimentation rate (ESR) was 63 [mm/hour]. Urine toxicology screening was negative. Empiric treatment with vancomycin and piperacillin/tazobactam was initiated after she developed hypotension and a fever. Preliminary blood cultures were positive for gram positive cocci, later found to be positive for MRSA. Magnetic resonance imaging (MRI) of lumbar spine was obtained because of severe lower back pain that did not resolve with analgesia. This study revealed a small dorsal spinal collection with edema in the left psoas muscle. Neurologic reflexes were intact and serial neurologic exams were normal.\nBack pain continued to increase, and the patient developed weakness of bilateral lower extremities. Given the concern for acute structural damage to the spinal cord, the patient was counseled regarding risks, benefits, and alternatives to contrast imaging during pregnancy and opted for MRI with gadolinium intravenous contrast []. This was repeated after two days of antibiotic therapy to assess for further progression of abscess. MRI revealed an epidural abscess from T5-6 to T8-9 causing mild thecal cord compression and a collection from L4 to S1 (see Figures and ). Because of concern for worsening neurologic symptoms of lower extremity weakness and difficulty ambulating, cesarean delivery was performed followed by laminectomy (T5-10) and decompression of the epidural abscess. Intraoperative cultures were obtained and were positive for MRSA. Cesarean delivery was uncomplicated. Neonate weighed 2690 grams with Apgar score of 8 and 9 and was treated with vancomycin and gentamycin for suspected sepsis. Placental pathology and cultures were negative.\nPostoperative course was complicated by persistent MRSA bacteremia. Repeat MRI revealed residual abscess from T6-T9, with cord compression at T6-T7, and a left psoas muscle collection (see ). Three days later, reexploration and laminectomy from T4-T7 were performed and antibiotic treatment was continued with vancomycin and ceftaroline fosamil. During her extended hospital course, she admitted to previous illicit drug use but denied current use. However, there was a high suspicion for intravenous drug use due to her poor pain tolerance and history of antecubital abscesses. Later in her clinical course she endorsed a history of intravenous drug use. Once blood cultures were negative, she was discharged home in stable condition to continue long-term antibiotic therapy. Lower extremity weakness had resolved at time of discharge; however, she continues to have residual weakness, pain, and incontinence.","This 25-year-old woman, who had previously experienced MRSA infections, was admitted to the hospital with severe back pain and a fever. She had a history of abscesses in her arm and was treated with antibiotics. Tests showed she had a serious infection in her spine, causing weakness in her legs, and an MRI revealed an abscess. She underwent surgery to remove the abscess and relieve pressure on her spinal cord, and a baby was delivered safely via C-section. Despite the surgery, she continues to experience some weakness and incontinence, and will need to continue taking antibiotics."
666,"A 32-year-old female with a past medical history significant for schizophrenia, bipolar disorder, and hepatitis C antibody positive presented from the behavioral health center for a 2-day history of a diffuse rash. The rash had started on her upper extremities and then spread to her face, chest, and thighs 2 days prior to admission. At times, the rash had been itchy and the patient had reported chills. She had been started on clozapine 10 days prior to admission. On admission she was febrile to 38.1°C, tachycardic to 113 bpm, and hypotensive to 96/63. On exam, she had a maculopapular rash that was nonblanching over her entire body except her lower legs. There was no mucosal involvement, but she had mild facial edema. Pertinent admission labs included WBC 6.9 (3.7–11.4 103/µL), Hgb 10.6 (10.8–15.3 g/dL), platelet 196 (140–393 K/µL), eosinophils 10 (0–6%), eosinophils absolute 0.7 (0.0–0.5 103/µL), aspartate aminotransferase 81 (14–36 U/L), alanine aminotransferase 125 (9–52 U/L), alkaline phosphatase 155 (38–126 U/L), and hepatitis C antibody positive. Her urinalysis showed moderate leukocyte esterase with white blood cells, squamous cells, and few bacteria. Imaging on admission included chest X-ray, which showed a small left sided pleural effusion. A CT chest was done which showed minimal bilateral atelectasis with trace pleural effusions and cholelithiasis with contracted gallbladder and pericholecystic fluid.\nAt this point, there was concern for infectious etiology, and blood cultures were drawn. The patient was then started on broad-spectrum antibiotics with cefepime and vancomycin. Clozapine was stopped and her benztropine and lithium were initially continued as she had been on these for many years. After being seen by psychiatry, they were also discontinued. She was given Benadryl for her rash. Hepatitis C viral PCR pathogens were found to be negative. Blood cultures grew staphylococcus coagulase negative and urine culture showed mixed flora with no infection. The blood cultures were thought to be a contaminant, so they were redrawn and the second set did not grow anything. Antibiotics were discontinued, as there was no further concern for infectious etiology. Eosinophils continued to be elevated and she developed a leukocytosis. The next three days, her white blood cell count continued to rise and peaked at 26.5 103/µL. She had worsening fever and became more tachycardic. Liver enzymes continued to rise, so an ultrasound abdomen was performed due to concern for cholecystitis, which was negative. Antibiotics were restarted due to concern for infection. Her eosinophil count rose and peaked at 14%. She had an echocardiogram to rule out endocarditis, which was within normal limits. CT abdomen and pelvis with contrast was done to rule out an abdominal infection. It showed colon thickening, cholelithiasis, trace pleural effusions, and porta hepatis lymphadenopathy. Antibiotics were all discontinued again as infectious etiologies had been ruled out. At this point, there was concern for drug reaction with eosinophilia and systemic symptoms (DRESS syndrome) as she had the classic signs of DRESS including eosinophilia, skin rash, lymphadenopathy, and elevated liver enzymes. Other viral exanthema reactivations that are associated with DRESS were also considered. EBV, HHV-6, and HHV-7 were drawn, and all came back negative. Mononucleosis testing was not done on presentation although this can present as DRESS syndrome.\nDermatology was also consulted and agreed that this was DRESS syndrome. Methylprednisone IV 125 mg three times daily was started along with clobetasol twice a day. After starting prednisone, fever and white blood cell count improved. Eosinophils trended back down to normal limits and liver enzymes started trending down. She was discharged on an oral prednisone taper for 12 days. Outpatient follow-up did not show any further eosinophilia, white blood cell elevation, or elevated liver enzymes. Rash improved and disintegrated.","This 32-year-old woman with a history of mental health conditions and hepatitis C was admitted to the hospital after developing a widespread rash. She was also experiencing fever, chills, and low blood pressure. Initial tests ruled out many possible infections, but her symptoms continued to worsen, and her white blood cell count rose significantly. After consulting with specialists, doctors suspected a drug reaction called DRESS syndrome and started her on medication to help her recover. Thankfully, her condition improved, and she was discharged home with a plan to continue taking medication and follow up with her doctors."
667,"A 23-year-old woman presented with progressive headache, nausea, and vomiting for 1 week. Right-side weakness, ptosis, and diplopia were also found. Due to acute onset conscious disturbance (Glasgow Coma Scale of E3VaM5) in the hospital, brain computed tomography was arranged and revealed an enhanced brain tumor with necrotic cystic change. This tumor was located at the left temporal lobe with upward extension to the left basal ganglion and periventricular region, causing perifocal edema and midline shift (). We performed emergent craniectomy for tumor removal in December 2006.\nPathology revealed pleomorphic, hyperchromatic cells with glassy, astrocytic cytoplasm, as well as hypercellularity, microvascular proliferation, and necrosis, consistent with the diagnosis of classic GBM ().\nThe patient underwent radiotherapy 1 month later and followed by chemotherapy with temozolomide for 6 months. Her performance status improved to a Karnofsky Grade of 70, and her clinical condition was stable thereafter. However, follow-up brain magnetic resonance imaging (MRI) in June 2014 revealed a new enhanced nodular lesion, approximately 1.1 cm in diameter, at the left temporal base. The brain MRI in October 2014 revealed a progressive change of lesions, maximum 3.0 cm in diameter (). Thus, she again received surgery for gross tumor removal.\nHistologically, except for the necrosis feature of GBM, the tumors showed the oligodendroglial component. Neoplastic cells also showed isocitrate dehydrogenase 1(IDH1)(+), p53(diffuse +), and O6-methylguanine-DNA methyltransferase (MGMT)(−) as revealed by immunostaining ().\nDuring follow-up, signs of increased intracranial pressure were noted in May 2015. Therefore, she received a third debulking surgery. The third pathology revealed both GBM- and PNET-like components. In immunohistochemistry, the PNET-like component exhibited positivity for synaptophysin and CD56 and focal weak positivity for glial fibrillary acidic protein (GFAP) ().\nOne month after the surgery, her condition rapidly deteriorated. The patient and her family chose to pursue hospice care. She passed away with a total 9-year survival since diagnosis ().","This 23-year-old woman started experiencing headaches, nausea, and vomiting, which led to weakness and vision problems. Doctors discovered a large, aggressive brain tumor causing pressure on her brain. She underwent surgery to remove the tumor, followed by radiation and chemotherapy, which initially helped her condition. However, the tumor returned, requiring further surgery and ultimately leading to her passing away nine years after her initial diagnosis."
668,"A 4-year-old boy was admitted to pediatric department because of newly occurred hypertension as status posterior right heminephrectomy of neuroblastoma. Computed tomography angiography (CTA) scan revealed renal artery severe stenosis and right kidney atrophy. He had undergone Transcatheter Arterial Embolization of right renal artery 4 days ago because of the refractory hypertension. He was stable after the surgery and transfused 1 unit of packed red blood cells due to anemia. Five hours later, he became anxious and breathless, spitted frothy sputum, and then suffered an episode of cardiac arrest. After being intubated and 20 minutes' CPR, he underwent restoration of spontaneous circulation (ROSC). The attending physician treated him with cortisone as transfusion related acute lung injury (TRALI) was suspected. Then the patient was transferred to the ICU to receive respiratory support and further treatment. At presentation, he had a heart rate of 160 times/min and blood pressure of 150/111mmHg without any vasoactive drugs. A lot of flesh-colored aqueous sputum was spurred out of endotracheal tube. Tidal volume is only about 30ml on invasive ventilation with PI 15cmH2O and PEEP 10 cmH2O (PCV mode). Before he arrived to the ICU, the patient received manual ventilation with balloon and sputum suction constantly for 1 hour. The lung was very stiff and hard to inflate by balloon. Arterial blood analysis showed pH 6.7, PO2 56mmHg, PCO2 28mmHg, lactate 16 mmol/l, and BE -30. The FiO2 was 100%. There was no urine output in the first hour. We performed critical care ultrasound using the 7-step approach workflow at that time to make the puzzle clear ().","This 4-year-old boy was admitted to the hospital because of high blood pressure after a surgery to remove a tumor in his kidney. Doctors used a special procedure to try to control the high blood pressure, but he then became very sick and had a cardiac arrest. After being revived with CPR and receiving treatment for a lung problem, he was transferred to the intensive care unit for breathing support. He is currently receiving oxygen and fluids, and doctors are monitoring his condition closely."
669,"A 61-year-old male patient was admitted to the liver surgery department because of discovering liver mass for 6 days. The alpha-fetoprotein (AFP) was 1009 ng/ml, and liver contrast CT scan indicated hepatic cell cancer in the right lobe. As a generally healthy status before surgery, the patient received ALTPS surgery. 20 days later, he got fever and abdominal pain and developed shock as well as hypoxia in hours. He was intubated and treated with fluid resuscitation and norepinephrine (1.8 mcg/Kg.min) and then transferred to the ICU. Auxiliary examination showed WBC 0.63×109/L, PLT 7×109/L, and PCT 45.88 ng/ml; bedside ultrasound was ordered and ascites were found. The doctors cultured and drained the ascites and treated him with Imipenem and Vancomycin. Then they ordered abdominal CT and it reveals signs of necrosis of right lobe of the liver. Later, the patient suffered the second surgery to remove the right half of the liver. Culture of ascites reports Escherichia coli. After three days, the patient got better. No fever existed and the norepinephrine had been decreased to 0.4 mcg/Kg.min, and urine output had been maintained at 2000–2500ml per day. Two days later the patient had fever again, with the highest temperature of 38.8°C, as well as an increase of norepinephrine from 0.4 mcg/Kg.min to 2.0 mcg/Kg.min, deterioration of liver function, coagulation, and oxygenation. Arterial blood gas test showed pH 6.988, PaO2/FIO2 154, PaCO2 147.7mmHg, BE -19 mmol/L, and lactate 9.7 mmol/L. We performed critical care ultrasound using the 7-step approach workflow at that time to make the puzzle clear ().","A 61-year-old man was admitted after finding a mass on his liver, which was later diagnosed as liver cancer. Following surgery, he developed a serious infection and complications, including shock and difficulty breathing, requiring intensive care and multiple medications. Doctors treated the infection with antibiotics and drained fluid from his abdomen, but he experienced a recurrence of fever and worsening symptoms. A second surgery to remove more of the liver was performed, and he gradually improved with supportive care, but his condition remained unstable. Ultimately, the patient’s condition deteriorated significantly, leading to critical care intervention and monitoring."
670,"A 25-year-old previously healthy male patient from Yunnan province in Southern China was airlifted to the First Affiliated Hospital of Zhejiang University for “fever of unknown origin” and respiratory failure on October 29, 2017. Ten days before, he started having a fever of 38°C and mild diarrhea without an obvious etiology. A few days later, he started feeling chest tightness and shortness of breath and having cough with yellow purulent sputum. He was admitted to a local hospital, where a thoracic computed tomography (CT) scan revealed pneumonia with a small amount of pleural effusion in the right lung. He was diagnosed with “lobar pneumonia” and treated with moxifloxacin plus cefoperazone sulbactam for 5 days. However, the symptoms worsened, and the patient continued having a high fever (40°C). Another CT scan showed significant progress of consolidation in the right lung and multiple nodules and pleural effusion in the left lung. The treatment regimen was changed to imipenem, linezolid, caspofungin, and ganciclovir. Methylprednisolone was given as well. However, the patient’s condition rapidly deteriorated to respiratory failure, which required mechanical ventilation, thoracic drainage, and drug sedation, before he was transferred to our hospital. No laboratory results were available from the outside hospital. Personal history revealed the patient to be a heavy smoker.\nUpon admission, he was febrile (38.4°C), tachycardic (109 bpm), and hypotensive (62/51 mmHg) with leukocytosis (white blood cell count [WBC] 18.6 X10E9/L). His C-reactive protein (CRP) was 146.42 ng/L, but procalcitonin (PCT) was only 0.49 ng/L. His troponin (0.3 ng/mL) level and his liver enzyme (aspartate aminotransferase [AST] 140 U/L) were both elevated. Bronchoscopy showed profound congestion and edema in the airway mucosa accompanied by multiple bleeding ulcers. Microbiology work-ups were initiated, and the patient received daptomycin (for suspected endocarditis after bedside echocardiogram showed hyperechoic response in the right ventricle) and piperacillin/tazobactam. On hospital day (HD) 2, the respiratory viral polymerase chain reaction (PCR) panel detected adenovirus in the sputum. Ganciclovir (0.3 g qd) was started again, and immunoglobulin was added. However, the exact origin of the fever was unclear, and the severity of the condition didn’t seem to match adenovirus respiratory infection alone. A transesophageal echocardiography (negative results) was performed to rule out infective endocarditis. Bone marrow puncture and lymph node ultrasonography (negative results) were performed to rule out lymphoma. These revealed T-cell subpopulations in the bone marrow suggestive of suppressed immune status, and therefore thymosins was given. On HD 3, BAL and blood were sent for shotgun metagenomics testing to identify the source of the fever. On HD 4, BAL and sputum culture came back positive with Aspergillus fumigatus; thus voriconazole was started. At this point, the diagnosis of the unknown fever was starting to shift to a fungal pneumonia. On HD 5, the shotgun metagenomics results came back positive with large amount of adenovirus in both BAL (1301 reads) and blood (795 reads). No other pathogens, including bacteria, fungus, and parasites, were found. Aspergillus fumigatus, which was isolated in the initial sputum culture, was not detected by the shotgun metagenomics. Because the subsequent respiratory cultures also did not yield any fungus, combined with the shotgun metagenomics results, the 1-time finding of A. fumigatus in the sputum was deemed to be a result of environment contamination. The high number of adenovirus reads were sufficient for de novo assembly into several large contigs (max size, 4800 bp), which were closely matched to human adenovirus B55 (HAdV-B55). Of note, this is the same strain that has been reported to cause numerous outbreaks in military units, hospitals, and schools in China, and that often causes deaths in immunocompetent young patients []. With this information, it was very clear that this patient was suffering an invasive HAdV-B55 infection that started from pneumonia and progressed into viremia. All clinical efforts were refocused to antiviral therapy. Because cidofovir, the firstline drug for adenovirus infection, is not available in China, ganciclovir was replaced by ribavirin (0.5 g q12h) administered intravenously on the basis of more successful treatment cases reported on the literature [].\nOn HD 8, the patient’s inflammatory markers, including WBC, CRP, and PCT, all increased significantly. Bronchoscopy showed an increased amount of purulent sputum, and the culture grew pan-drug-resistant (only susceptible to tigecycline) Acinetobacter baumannii, a common nosocomial pathogen circulating in many ICUs in China, including ours []. Piperacillin/tazobactam was then replaced by cefoperazone/sulbactam (1 g q6h) plus tigecycline (50 mg q12h). To rule out other infections and monitor the antiviral therapy, a second set of BAL and blood samples was sent for shotgun metagenomics on HD 9. The results were negative in the blood but positive in the BAL, with a reduced load of adenovirus (10 reads) and a high load of A. baumannii (1666 reads). No other pathogens were detected in the BAL. The absence of adenovirus in the blood (confirmed by PCR) and the significant decrease of adenovirus load (1 log reduction by a quantitative PCR) in the BAL suggested that the patient was responding to the ribavirin treatment. The detection of A. baumannii was consistent with the culture results. The absence of any fungus in the BAL by metagenomics testing again, supported by repetitive negative fungal culture in the respiratory samples, prompted the discontinuation of voriconazole. Ribavirin treatment was continued, and a qualitative adenovirus PCR test on the sputum was done daily to monitor viral infections in the lung. On HD 10, the patient’s liver enzymes, troponin level, and ejection fraction had all returned to their normal ranges. On HD 18, due to repeated positive cultures of A. baumannii in the sputum, the dose of cefoperazone/sulbactam was increased from 1 g q6h to 3 g q6h. On HD 21, the adenovirus PCR in the sputum finally turned negative, the amount of A. baumannii grown in sputum culture also turned from heavy to rare, and the inflammatory markers (WBC/CRP/PCT) had all dropped significantly. On HD 24, bronchoscopy showed significant improvement in the trachea and the airways; thus, the chest drainage tube was removed, and the antibiotics were discontinued. On HD 26, the patient woke up after sedation was discontinued. On HD 28, due to the concern for drug-induced liver damage (AST increased to 161 U/L), ribavirin was discontinued. His condition began to stabilize, and ventilator support was gradually reduced.\nHowever, on HD 28, the patient developed altered mental status, seizures, and muscle weakness. Head CT showed widening in the sulci and cisterns, concerning for encephalitis. Meanwhile, the patient started having fever again (38.6°C). On HD 30, the sputum adenovirus PCR test was positive again, raising concern for neurological adenovirus infection. A lumbar puncture was performed and the CSF, which surprisingly had unremarkable biochemistry (glucose 4.3 mmol/L, protein 0.331 g/L) and cellular (RBC 170/uL, WBC 33/uL, 12% neutrophils, 88% lymphocytes) results, was sent for shotgun metagenomics testing. Two days later, the metagenomics results came back positive for herpes simplex virus–1 (HSV-1; 844 reads), which was subsequently confirmed by HSV-1 PCR. Neither adenovirus nor any other pathogens was detected. A diagnosis of HSV-1 encephalitis was made, and high-dose acyclovir (500 mg Q8h) was given to the patient. After 3 days of acyclovir treatment, the fever and seizures were gone, and the limb muscle strength was improved. On HD 40, the patient showed substantial improvement and could eat unassisted; therefore, the nasogastric intubation was removed. On HD 44, ventilation support was finally withdrawn. Another lumbar puncture was done, and the CSF was sent for shotgun metagenomics testing, which came back negative (confirmed by HSV-1 PCR). The patient was discharged on HD 52. Blood cultures were never positive, and the patient’s adenovirus PCR in the sputum was intermittently positive until HD 48. The hospitalization history, including major events, treatment courses, inflammatory markers, and test results, is summarized and illustrated in .","This patient, a 25-year-old man from China, was admitted to the hospital after developing a high fever and shortness of breath. Initially, doctors suspected pneumonia, but tests revealed a complicated infection with multiple viruses and bacteria. He was treated with several different medications, including antibiotics, antivirals, and steroids, but his condition worsened and he required a breathing machine. Eventually, tests showed he was infected with adenovirus, a common virus, and then with herpes simplex virus, leading to encephalitis. After receiving antiviral treatment, he gradually improved and was eventually discharged home."
671,"A 38-year-old male was referred to the outpatient clinic at the Department of Nephrology with treatment-resistant hypertension, rapidly developing edema and overt proteinuria (week 11, Fig. A). The patient was initially followed at the outpatient clinic at the Department of Endocrinology with poorly controlled type 1 diabetes for 15 years with microvascular complications including retinopathy and albuminuria, thus presenting with urinary albumin/creatinine ratios over 1000 mg/g for at least 3 years. There were no clinical signs of neuropathy. Plasma creatinine had previously been normal, in the range 60–90 μmol/L. Through several years, the patient had hypertension that was well-controlled with ACE inhibitors. One year prior to the presentation, blood pressure increased progressively concomitant with development of edema. The patient presented with severe hypertension (200/140 mmHg, week 0, Fig. B), edema and urinary protein excretion at 18.5 g/24 h (week 1, Fig. D). Despite increasing doses and numbers of antihypertensive agents and diuretics (Fig. A), blood pressure continued to be severely elevated combined with progressive fluid overload and proteinuria (Fig. B and D). The patient was referred to the Department of Nephrology (week 11, Fig. A–D) with NS. At this time, a renography performed on treatment with an ARB revealed no perfusion of the right kidney, and ultrasound confirmed the presence of a 4 cm long, hypoechoic structure in the right retroperitoneal space believed to be a rudimentary right kidney. The left kidney was morphologically and scintigraphically normal. The antihypertensive medication at referral was thiazide, beta-blocker, calcium channel antagonist, ACE-inhibitor and mineralocorticoid receptor antagonist spironolactone with no suspicion of noncompliance (Fig. A). At presentation, the patient was alert but complained of headache, fatigue, and recent weight gain of 10 kg. On physical examination, blood pressure was 161/102 mmHg, and the patient revealed periorbital and universal pitting edema, no signs of ascites and otherwise normal examination. Based on edema, proteinuria, and hypoalbuminemia (25 g/L), he was diagnosed with NS assumed to be related to diabetes. There was negative test for M-component and no detectable autoantibodies (ANA, ANCA, anti-GBA) nor phospholipase A2 antibody. Hepatitis B and C as well as HIV serology were negative. To reduce blood pressure, treatment with a loop diuretic was initiated (furosemide 80 mg/day, Fig. A). After 2 weeks without effect on edema and blood pressure (Fig. B and D), with a decrease in eGFR and no change in plasma potassium (Fig. C), furosemide was stepped up to 160 mg/day (Fig. A). Despite treatment with five combined antihypertensive/diuretic agents, there was only a minor weight loss (~1 kg) and reduction in blood pressure. Therefore, a low dose of the ENaC blocker amiloride 5 mg/day was initiated (Fig. A) and a 7-day follow-up was scheduled, which the patient missed. At next contact after 2 weeks, this treatment resulted in effective resolution of edema, concomitant weight loss of 7 kg and reduction in blood pressure from 150/100 mmHg to 125/81 mmHg (Fig. B). The patient continued his normal diet and 24 h urinary sodium excretion increased from 127 mmol/day to 165 mmol/day. Proteinuria decreased from 8 g/day to 4.1 g/day (Fig. D), eGFR decreased from 41 mL/min to 29 mL/min and plasma potassium concentration increased from 4.6 to 7.8 mmol/L (Fig. C). The patient was immediately hospitalized for cardiac monitoring and treatment of hyperkalemia. Amiloride and spironolactone were both discontinued. At follow-up 5 weeks later, a combination-drug containing 2.5 mg amiloride and 25 mg hydrochlorthiazide was successfully reinitiated due to increased blood pressure 141/96 and edema. At the last visit to the outpatient clinic, the patient received the following antihypertensive /diuretic drugs; amiloride/hydrochlorthiazide 2.5 + 25 mg/day, metoprolol 50 mg/day, furosemide 125 mg/day, lercanidipine 10 mg/day, and lisinopril 20 mg/day and his blood pressure was 112/80, body weight was stable at 90 kg (15 kg weight loss), plasma potassium was 4.6 mmol/L, and plasma creatinine was 203 μmol/L with eGFR at 35 mL/min.","This man has been struggling with high blood pressure and swelling for a while, and it’s getting worse. He also has diabetes and has been experiencing a lot of protein in his urine. After trying many different medications, doctors discovered a small, non-functioning kidney on the right side. Despite taking multiple medications, his blood pressure and swelling didn’t improve, and he developed a potassium imbalance. However, by adjusting his medications and adding a new one, his blood pressure and swelling finally went down, and his kidney function improved."
672,"A 67-year-old Caucasian man came to our attention after a fixed oral prosthesis surgery. His past medical history was significant for a paroxysmal atrial fibrillation for which he was taking Amiodarone and Acenocumarol with stable International Normalized Ratio (INR) values. Following orthopantomography and CT scan, the procedure of implant insertion was performed with local anesthesia (Mepivacaine and vasoconstrictor) in area 36 [left lower jaw, designated according to the ISO system] [] without any complication. Acenocumarol had been discontinued 2 days before, switching to LMW heparin. The patient was discharged under antibiotic therapy (Amoxicillin and Clavulanic acid) and instructed to restart oral anticoagulant therapy after 2 days. About 48 h after the procedure, hiccup abruptly presented and failed to cease.\nThe patient did not report any further symptom, and no complications were found at the revision of the surgical area. Three days after the onset, the patient came to the emergency department due to this persisting symptom: Baclofen, Metoclopramide and Bromazepam were administered without significant clinical improvement. Both ENT, neurological examinations, blood tests and a brain CT scan failed to show any abnormality. Three days later, Chlorpromazine 25 mg b.i.d. was administered for 2 weeks. Furthermore, the patient was advised to program in the next few days a brain MR scan and chest imaging that were negative. Seven days following the surgical procedure, the stitches were removed and the wound did not show any problem. The hiccup continued resulting in significant distress and sleep deprivation. Providentially, it spontaneously ceased after 18 days. Neither relapses or neurological symptoms were reported in the later months.","This 67-year-old man had a procedure to insert a new dental implant. He was already taking medications for a heart condition and had to temporarily stop one of them before the surgery. After the procedure, he developed persistent hiccups that didn't go away despite trying several medications. Fortunately, the hiccups eventually stopped on their own after about three weeks, and further tests ruled out any underlying problems."
673,"The patient, a 32-year-old Caucasian woman, presented to the West Virginia University Hospital Emergency Department via Emergency Medical Services. The patient had been at her usual baseline state of health with no significant past medical history prior to visiting the chiropractor for neck adjustment earlier that day for tension like soreness. The patient underwent neck manipulation after which she immediately complained of neck pain, diaphoresis, and proceeded to experience cardiac and respiratory arrest. Emergency Medical Services was called, and cardiopulmonary resuscitation was performed with one round of epinephrine administered. It was reported that the patient was pulseless and apneic for 3 minutes prior to EMS arrival. The patient was intubated on transport and her Glasgow Coma Scale score was 3T prior to arrival. Mean arterial blood pressure was 80 with palpable femoral pulses at arrival to the emergency department. Upon arrival in the emergency department, a CT stroke protocol was performed which demonstrated bilateral severe distal cervical vertebral artery dissections with acute thrombotic emboli seen in the left cervical vertebral artery ( and ). This was accompanied by complete occlusion of the basilar tip including the proximal posterior cervical arteries.\nThe patient received an initial bolus of intravenous tissue plasminogen activator (IV rtPA) at this time and the decision was made to proceed with endovascular intervention given the recent onset of occlusion. The patient was brought to the neurovascular angiography suite and femoral access obtained. Angiography of the left vertebral artery demonstrated severe dissection involving the distal cervical vertebral artery segments at the C1-C2 level with presence of sub occlusive thrombi. There was an occlusive clot in the left Posterior Inferior Cerebellar Artery (PICA). Intracranial imaging demonstrated occlusion at the basilar apex with absent filling into the right Posterior Cerebral Artery (PCA). There was occlusion of the distal left PCA. Angiography of the right vertebral artery demonstrated severe dissection of the distal cervical vertebral artery at C1-C2 with the presence of trickle-like flow into the vertebrobasilar junction. No filling was observed in the right PICA territory (). At this point, it was decided that the left vertebral artery offered the best access to the basilar trunk.\nSubsequently, distal aspiration was begun with a Penumbra 5 Max ACE distal aspiration catheter which initially demonstrated slow flow through the suction tubing. The 5 Max ACE was withdrawn into the proximal basilar artery until flow was seen within the suction tubing. Repeat angiography at this time demonstrated recanalization of the basilar apex and proximal PCAs. TICI3 perfusion was seen in the right PCA. Occlusive clot remained in the left distal P2 segment. Given the large size of the PCA, timing of events, and patient’s age, the decision was made to attempt clot retrieval of this. At this time, the Trevo ProVue microcatheter was navigated into the left distal PCA distal to the clot. The Trevo 4 mm × 30 mm stent retriever was deployed for approximately 3 minutes. The suction canister was attached, and the stent retriever was pulled with distal aspiration. No significant recanalization was achieved with what amounted to TICI0 perfusion to the left PCA territory. No further attempts were made as it was believed that the left PCA territory had completed its infarction.\nFollowing completion of endovascular therapy, the patient was taken for immediate MRI Brain with and without contrast for assessment of brainstem integrity and cerebrovascular status prior to transport to the intensive care unit. MRI demonstrated extensive areas of restricted diffusion accompanied by perfusion abnormalities consistent with acute infarction of the posterior circulation, specifically within the bilateral cerebellar hemispheres, right medulla, pons bilaterally, midbrain, thalami, and left occipital lobe (). The following day, additional CT Brain imaging was acquired and demonstrated signs of elevation of intracranial pressure with hydrocephalus, worsening of cerebral edema diffusely, hemorrhagic transformation of the left occipital lobe, continued infarct evolution within the posterior circulation, and cerebellar tonsillar herniation.","This 32-year-old woman experienced a sudden medical emergency after a neck adjustment, leading to cardiac and respiratory arrest. Emergency responders quickly intervened, and she was stabilized with intubation and medication. Imaging revealed severe dissections in her neck arteries, causing a blockage in her brain, leading to a stroke. Doctors successfully used a specialized procedure to open the blocked arteries, but unfortunately, some brain damage had already occurred."
674,"A 51-year-old woman with a history of morbid obesity, obstructive sleep apnea on nocturnal continuous positive airway pressure ventilation, and non-insulin dependent diabetes mellitus presented to the urgent care clinic with an unresponsive intractable chronic headache for almost a year. The headache was 7/10, intermittent, non-radiating, throbbing, the frontal headache lasting for almost a year and was thought to be secondary to post-concussion syndrome given a history of head trauma one year ago with no loss of consciousness. At the time, computed tomography (CT) scan of the head was unrevealing (Figure ). Over the past year, the patient had visited the emergency department multiple times with a severe headache. Secondary causes of headache such as severe hypertension, pharyngitis, sinusitis, meningitis as well as tumor, subdural hematoma, and hydrocephalus were ruled out. Laboratory work up was unrevealing with a normal ESR and CRP. Magnetic resonance imaging (MRI) scan and lumbar puncture were both negative. In addition, the patient’s headache persisted despite a trial of NSAIDs, acetaminophen, tramadol, and Fiorecet. Upon reconciliation of the patient's medications, it was found that she was switched from metformin 500 mg twice daily to metformin-sitagliptin 50-1000 almost a year ago, about a week before the onset of headache. Considering the temporal association of the medication and symptom presentation, sitagliptin was discontinued as a trial treatment and the patient was switched back to metformin. The patient reported resolution of her headache two days after discontinuation of sitagliptin.","6 This 51-year-old woman has been dealing with a persistent, severe headache for almost a year. She has a history of obesity, sleep apnea, and diabetes, and she also had a head injury a year ago. After many visits to the emergency room and tests to rule out serious causes like tumors or infections, doctors found no underlying medical problems. It turned out that a recent change in her diabetes medication was causing the headache, and stopping the new medication resolved the issue."
675,"A 57-year-old Caucasian female presented to the hospital with a worsening, diffuse, bullous eruption. The eruption started four weeks prior and was distributed mainly on her lower extremities. The patient went to her primary care physician, who prescribed doxycycline and sulfamethoxazole/trimethoprim and told the patient that she had cellulitis. The patient took the antibiotics but the rash continued to worsen. After completing the antibiotic course without improvement, the patient presented with diffuse and erythematous tense bullae ranging from 1.5 to 2 centimeters in diameter. The lesions can be appreciated on the patient’s face, neck, back, chest, abdomen, and extremities (Figure ).\nSome of the lesions had ruptured and were both pruritic and painful.\nThe patient was afebrile and without leukocytosis, yet C-reactive protein was elevated at 97.8 mg/L. An initial punch biopsy was performed and returned negative for a definitive diagnosis. A repeat punch biopsy four days later showed a subepidermal blister with eosinophils and neutrophils. The underlying dermis demonstrated severe edema and infiltrate composed of eosinophils and lymphocytes (Figure ).\nDirect immunofluorescence (DIF) of the skin revealed the linear deposition of immunoglobulin G (IgG) and complement C3 along the dermo-epidermal junction (Figure ).\nThe patient was diagnosed with bullous pemphigoid and was treated with prednisone 60 mg daily. The patient responded well with a decreased number of bullae, as well as an improvement of the erythema and pruritus. The patient was discharged on a tapering regimen of prednisone 60-40-20 mg for one month per dose, along with oral sulfamethoxazole/trimethoprim 160 mg daily for prophylaxis of Pneumocystis carinii pneumonia. The patient was referred to a dermatologist to discuss adding a steroid-sparing agent such as methotrexate or azathioprine.","This 57-year-old woman came to the hospital because her skin rash was getting worse despite taking antibiotics. The rash started a few weeks ago and spread across her body, causing painful, blistering bumps. A skin biopsy revealed a specific type of inflammation called bullous pemphigoid, which is characterized by blisters under the skin. She is now being treated with steroids and will continue to take medication to prevent the rash from returning."
676,"The patient is a 49-year-old male with longstanding back and left leg pain resistant to pain management. He developed acute worsening of his left sciatic pain and suffered a fall with a left wrist fracture. He subsequently developed shortness of breath and was seen in the emergency room. A computerized tomography (CT) study revealed two right retroperitoneal masses, a right prevertebral lesion measuring 4.1 x 3.6 x 5.7 cm with anterior displacement of the inferior vena cava (IVC) and a right paraspinal lesion centered in the psoas measuring 4.0 X 3.5 x 6.6 cm (Figure ).\nThe percutaneous biopsy of these lesions was consistent with a benign nerve sheath tumor. He was sent for neurosurgical management and, during his evaluation, was noted to have an 8 x 5 x 5 cm left sciatic tumor (Figure , Figure ).\nThis was excised uneventfully and found to be a Grade I neurofibroma. His chronic left sciatica resolved although his back discomfort persisted.\nDespite the multiple neurofibromas, the patient did not meet the criteria for NF1. He had a family history of multiple melanomas and other malignancies and was sent for genetic evaluation. He was found to have a large, contiguous genetic deletion of chromosome 9p21.3 extending beyond the cyclin-dependent kinase inhibitor 2A (CDKN2A) gene and spanning approximately 25 genes [].\nHis medical comorbidities included uncontrolled insulin-dependent diabetes mellitus with a HgA1C of 11.0, complicated by neuropathy and renal insufficiency, a cerebrovascular disease with two prior cerebral vascular accidents (CVA), and mild residual left hemiparesis, a peripheral vascular disease involving iliac stenting, tobacco abuse, and obesity.\nThe retroperitoneal masses were followed with imaging and were stable for three years. He then developed progressive back and radicular abdominal pain. Imaging revealed the growth of the right prevertebral lesion to 4.4 x 4.0 x 6.2 cm and the growth of the right paraspinal lesion to 4.7 x 4.0 x 7.5 cm (Figure ). Neither tumor had changes concerning for malignant degeneration. Given the tumor locations, the progression of the patient’s comorbidities, and his predisposition to develop malignancy, he was not felt to be a good candidate for surgical excision or radiosurgery. For these reasons, he was referred to interventional radiology for image-guided RFA. The procedure was performed with the aid of anesthesia. Under CT guidance, a 17G 3-cm burn radius ablation needle (Cool-tip, Covidien, Boulder, CO, USA) was advanced to each of the lesions, with a 12-minute burn cycle performed at each site (Figure , Figure ). He had no complications.\nAt six weeks post-ablation, the right prevertebral lesion had decreased in size to 3.4 x 3.5 x 5.4 cm and the right paraspinal lesion now measured 3.3 x 4.2 x 6.3 cm (figure not shown). Six months after the procedure, he reported his pain had improved. He no longer required methadone for pain control. On the most recent imaging performed four years after the ablation, the anterior prevertebral lesion shows stable regression at 2.9 x 3.3 x 3.5 cm with the posterior paraspinal lesion further decreasing in size to 2.6 x 3.6 x 5.1 cm (Figure ). Table shows tumor measurements over time.","This 49-year-old man has been dealing with chronic back and leg pain for a long time. Recently, he experienced a fall and developed shortness of breath, leading to scans that revealed two tumors in his back and abdomen. These tumors were found to be benign nerve sheath tumors, and one in his leg was removed. Despite these procedures, he was found to have a genetic deletion that increases the risk of cancer, and he is being monitored closely. His pain has improved significantly after a new treatment called RFA, and he no longer needs strong pain medication."
677,"A 19-year-old healthy male with no significant medical history presented with complaints of palpitations for one day along with chest pain and shortness of breath. It started when he was working on a Christmas tree farm. The patient reported a history of similar episodes for last six years usually triggered with mild exertion. He had noticed increased frequency and severity of symptoms for last six months and it started happening at least twice per week. He denied smoking, drinking alcohol, excessive caffeine consumption, substance use, recent travel, cough, fever, chills, night sweats, hemoptysis, weight loss, joint pain, rash, nausea, vomiting, abdominal pain and syncopal episodes. The patient has been feeling more fatigued and tired for last few months. On admission, vitals were normal. On physical examination, he was anxious and appeared to be in distress, rest of the systemic examination was unremarkable. Laboratory results showed normal liver, kidney functions and troponins. Urine drug screen was negative. Chest X-ray showed a right perihilar mass of 3.1 x 3.4 cm (Figure ).\nComputed tomography scan of the chest showed stippled-type calcified mediastinal lymphadenopathy of 1.6 cm x 2.1 cm, and right hilar mass of 3.8 x 4.8 cm (Figure ).\nThe patient had a bronchoscopy with fine needle aspiration (FNA) of mediastinal mass and lymph nodes. FNA results came back negative for malignancy, gram stain and cultures, acid-fast bacilli (AFB) stain and cultures, fungal stain and cultures were all negative. Histological examination showed predominant necrosis with rare benign lymphoid tissue. Urine histoplasma antigen and serum Aspergillus galactomannan antigens were negative. The patient continued to have chest discomfort and dyspnea after bronchoscopy. He had a mediastinoscopy with lymph node biopsy which showed an inflammatory granulomatous process. Pathology report showed lymphoid tissue with necrotizing granulomatous inflammation and positive fungal stain and morphology consistent with histoplasma species. Fungal and AFB cultures remained negative. The patient was started on oral itraconazole 200 mg twice a day with a plan for regular outpatient follow-up. He had improvement in chest symptoms during routine infectious disease clinic follow-up. He could not tolerate itraconazole due to diffuse rash after two weeks of treatment. He was switched to oral fluconazole 400 mg once daily which he tolerated without any problems.","A 19-year-old man came to the hospital because he was experiencing chest pain, shortness of breath, and palpitations. Tests revealed a mass in his chest and enlarged lymph nodes, which were initially suspected to be cancerous but were later found to be caused by an inflammatory infection with Histoplasma. After trying itraconazole, he developed a rash and was switched to fluconazole, which he tolerated well. He is now following up with infectious disease specialists and his symptoms have improved."
678,"A 69-year-old man presented with chief complaints of a growing mass on his nose, concomitant nasal airway obstruction, visual field impairment, and an inability to wear glasses. The patient also described the recent appearance of a glabellar lesion. Several weeks prior to our consultation, the patient sought the care of his primary care physician, who incised this newer lesion hoping to drain it. When this procedure was unsuccessful, the patient was referred for further treatment.\nUpon physical examination, the patient was found to have an extensive rhinophyma and additional lesions of the nasal glabella and right upper forehead regions (Figures , ). The rhinophyma measured 6.7 centimeters (cm) in diameter. The patient reported that it had been enlarging for six years. Observation revealed an irregular, nodular tumor with telangiectasia and sebum inspissation. The glabellar lesion measured 3.0 cm in diameter and appeared as a discrete erythematous tumor with central ulceration and necrosis. The location of this lesion on non-sebaceous skin suggested a non-rhinophymatous lesion and raised our suspicion for malignancy. An additional 1.5 cm lesion of the right forehead region appeared as a round, telangiectatic nodule with a waxy border. A preoperative diagnosis of basal cell carcinoma for this separate lesion was confirmed by the pathological report.\nSurgery was performed on the nasal lesions. The rhinophyma was excised and debulked, then closed with adjacent cheek flaps and skin graft. The glabellar lesion extended to the left medial canthal area. It was treated with wide local excision and was closed with forehead flaps. Frozen section analysis of the glabellar specimen revealed a diagnosis of diffuse large B-cell lymphoma. The area of rhinophyma on the lower portion of the nose displayed no evidence of lymphoma. After a three-week interval, the basal cell carcinoma of the right forehead was removed, and closure was accomplished with double opposing V-Y advancement flaps (Figures , ).\nThe final pathology report confirmed the diagnosis of diffuse large B-cell lymphoma (DLBCL) with BCL6 positivity. The patient was referred to an oncologist for further treatment. Subsequent oncological workup revealed several positive lymph nodes in the anterior cervical chain. One such node was biopsied by a general surgeon and was found to be positive for diffuse large B-cell lymphoma. Intensive combination chemotherapy, including Rituxan®, Cytoxan®, Oncovin®, Adriamycin®, and prednisone, was initiated.","A 69-year-old man came to the hospital because he had a growing mass on his nose, difficulty breathing through his nose, and trouble seeing clearly. He also noticed a new, concerning spot on his forehead. Doctors found that his nose had thickened significantly (rhinophyma) and discovered two other suspicious lesions, one on his forehead and one near his eye. Tests revealed that the forehead lesion was actually a type of cancer called diffuse large B-cell lymphoma, and the nose mass was also removed. After the growths were removed, the patient was referred to an oncologist to begin chemotherapy to treat the lymphoma. Further testing revealed additional lymphoma involvement in the neck, and the patient is now receiving intensive chemotherapy treatment."
679,"A 64-year-old African American female with a past medical history of insulin-dependent diabetes mellitus, hypertension, hyperlipidemia, prior history of stroke, hypothyroidism, and family history of coronary artery disease presented to the emergency department with complaints of typical chest pain. The patient's chest pain was associated with nausea and vomiting. The physical examination and initial electrocardiogram were unremarkable. Cardiac enzymes were negative. The patient was started on aspirin, statin, and nitroglycerin. Cardiology was consulted and they decided to do left heart catheterization through right radial access and an echocardiogram, as the patient was having unstable angina. The echocardiogram showed a normal ejection fraction with no wall motion abnormalities.\nLeft heart catheterization showed anomalous coronaries, with all three coronaries arising from the right coronary cusp with a separate ostium, as shown in Figures -.\nThe left anterior descending artery (LAD) had an anomalous origin with a separate ostium from the right coronary cusp. There was focal moderate to severe 70%-80% disease in the mid vessel. The LAD was a small vessel. The left circumflex artery had an anomalous origin with a separate ostium from the right coronary cusp. Mild luminal irregularities were present. The right coronary artery was a large dominant vessel with mild luminal irregularities. It was decided to treat the patient with medical management.","64-year-old woman with diabetes, high blood pressure, and a history of stroke came to the hospital because of chest pain, nausea, and vomiting. Tests showed her heart was generally healthy, but she had a blockage in one of her arteries. The doctors discovered that her coronary arteries had an unusual branching pattern. They decided to manage her condition with medication and will continue to monitor her heart health."
680,"A 13-year-old boy presented with a history of headache, nausea and vomiting with an acute onset 2 weeks earlier. Magnetic Resonance Imaging (MRI) of the brain and spinal cord revealed left cerebellar expansive lesion with no evidence of metastasis. Cerebrospinal fluid (CSF) examination revealed no evidence of dissemination. He underwent complete surgical resection as confirmed by postoperative imaging. Histopathological analysis including reticulin staining revealed a desmoplastic/nodular MB (confirmed by a central review by T.P.) as shown in Figure . Diffuse severe cytological anaplasia was not present. Complementary immunophenotypic characterization as described (, ) suggested a MB with SHH activation, TP53 wild-type (Figure ). Of note, nuclear INI-1 staining was preserved (Figure ) while P53 immunostaining showed nuclear positivity only in a small proportion of the tumor cells (data not shown). There was no evidence of MYCN or MYCC amplification by fluorescence in-situ hybridization (FISH). Next generation sequencing (NGS) over a panel of 50 genes (Ion AmpliSeq™ Cancer Hotspot Panel v2) revealed IDH1 R132C mutation in 46% of cells. NGS was repeated over a panel of 400 genes (Ion AmpliSeq™ Comprehensive Cancer Panel)1 and it revealed IDH1 R132C mutation in 24% of cells as well as SMARCB1-R201G in 30% of cells and CDH11-L625T in 26% of cells (Table A1). The panel was tested on both tumor and normal tissue to confirm the somatic nature of the mutations. Of note, mutations in SMO, PTCH1, SUFU and TP53 were not detected. Infinium Methylation EPIC BeadChip (850k) array revealed highest resemblance to the methylation class MB, subclass SHH A (children and adult). However, the calibrated score was 0.44 so that a clear subgroup assignment could not be done (). Calculation of a low density copy number profile from the array data indicated a flat genome without relevant chromosomal aberration (Figure ). Deletions of chromosome 9q (PTCH1) were absent. Assessment of overall CpG methylation and CpG island methylation levels of the tumor showed relative CpG hypermethylation compared to other reference classes of MB () (Figure ). There was no family history indicating a tumor predisposition syndrome.\nThe patient started treatment according to the HIT-SIOP PNET 4 protocol on the standard radiotherapy (RT) arm () where he received cranio-spinal RT (23.4 Gy) with boost to the posterior fossa up to 55.8 Gy in 30 fractions over 42 days. Adjuvant chemotherapy started 6 weeks after the end of RT and it includes eight cycles of maintenance chemotherapy (). Treatment was well tolerated with no major life-threatening adverse events or dose limiting toxicities. To date, the patient is in complete remission more than 2 years since diagnosis.\nSeveral mutation databases were searched, namely; the catalog of somatic mutations in cancer (COSMIC) database (), the cBioPortal for cancer genomics database (), the integrative onco genomics (IntOGen) database for mutational cancer drivers (), the cancer genetics web database () as well as the medulloblastoma advanced genomics international consortium (MAGIC) project (). We checked for reported IDH1 and SMARCB1 mutations in MB samples.\nThe COSMIC database reports on IDH1 mutation in only 5 tumor samples from 4 patients with MB; three samples from two adult cases (, ) and two samples from two pediatric patients [(), ICGC PedBrain Tumor Project; unpublished data]. Searching other databases didn't reveal any additional reported cases.\nAs for the SMARCB1 mutation, the COSMIC database reports only on 9 mutated samples of MB from four different studies (, –). Similarly, we couldn't find any additional reported cases in other mutation databases. The specific mutation (SMARCB1-R201G) was only reported in a single case of ovarian cancer, and wasn't previously reported in any central nervous system (CNS) tumor.\nThere was no reported case of a MB harboring both mutations of IDH1 and SMARCB1 in any of the above mentioned databases.","This 13-year-old boy was diagnosed with a rare and unusual growth in his brain, which doctors called a desmoplastic nodular MB. After surgery to remove the growth, tests revealed specific genetic changes (mutations) in the tumor cells, including IDH1 and SMARCB1, which are not commonly found in other types of tumors. He received a combination of radiation therapy and chemotherapy, and so far, he is in complete remission and doing well. Doctors found no other similar cases in existing databases, making his case unique."
681,"A 10 year old male was referred to endocrinology clinic for evaluation of obesity, rapid weight gain, and growth deceleration. His mother noted he was previously one of the tallest children in his class, but now was one of the shortest. Review of previous growth charts revealed growth at the 90th percentile for height at 8 years of age with decrease to the 75th percentile at 9 years, and the 50th percentile by 10 years. His weight was consistently at the 95th percentile, but he had gained 5.5 kg (12 lbs) in the past year with weight now at the 97th percentile and body mass index (BMI) 27.5 kg/m2 at the 99th percentile, meeting criteria for extreme obesity.\nHis mother noted he had been markedly hyperactive as a child and that this behavior had decreased over the past 1–2 years with great improvement in his grades over the past year. His medical history was unremarkable and he did not take medication. Review of systems was unremarkable and he denied fatigue, muscle weakness, constipation, or cold intolerance. He had a good energy level and there were no recent changes in appetite or concentration. He had occasional dry skin. Family history was remarkable for maternal grandmother and mother with hypothyroidism. His midparental target height was 176.5 cm (69.5 inches) at the 50th percentile for height.\nOn physical examination, the patient measured 134.9 cm (26th percentile) and weighed 50.2 kg (97th percentile) with BMI 27.5 kg/m2 (99th percentile). He had a normal blood pressure 104/55 mm Hg and heart rate of 84 bpm. The patient was well appearing without dysmorphic features and had a normal affect. He had cherubic facies and fundi appeared normal. His thyroid was palpable and smooth with right and left lobe each measuring 4 cm with no lymphadenopathy. His chest, heart, and abdomen were normal. He had Tanner stage 1 genital development with 3 cc testes and no pubic hair. Skin examination was negative for rash, acanthosis, or striae.","This 10-year-old boy is being evaluated because he’s gained a lot of weight and is growing more slowly than before. His weight and height are now in the highest percentiles, indicating he’s obese. He used to be a very active child, but has become less hyperactive and his grades have improved. The doctor will be checking his thyroid and looking for any other possible reasons for his weight gain and growth changes."
682,"A 20 years old woman was referred to Gazi University Department of Prosthodontics with a chief complaint of tooth discoloration, diastema, unsatisfactory esthetics and slight tooth sensitivity. The medical and dental history revealed that the patient’s family was not affected by AI. A renal ultrasound scan was normal, and it showed no evidence of nephrocalcinosis. Laboratory findings, including serum electrolytes, calcium, phosphate, urea, creatinine, alkaline phosphatase and parathormone levels were all normal. Clinical examination of the patient showed the insufficient enamel thickness, and the patient’s anterior and posterior teeth were discolored (Fig. ). The panoramic radiography also showed that the thin enamel layer could not be distinguished from the underlying dentin (Fig. ). There were no anterior open bite and missing teeth. However, short crowns, multiple diastema, occlusal wear with exposed dentin in the posterior areas, poor contact points and dental caries are the additional clinical findings (Figs. and ). The roots showed normal length and form. The pulp chambers were regular in size. Her oral hygiene was acceptable with no signs of gingivitis (Fig. ).\nThe maxillary and mandibular left third molar teeth were extracted to perform SEM and histologic analyzes. These teeth were totally covered by mucosa (Fig. ). Therefore, they were selected for SEM and histologic analyzes by the purpose of examining the tooth structure of the patient which had not been exposed to the oral environment. SEM and histologic analyzes were performed on the extracted mandibular and maxillary third molar teeth (Figs. and ). One of the third molar teeth was fixed in 4% glutaraldehyde. The tooth was then cut longitudinally, and the sections were coated with gold (Sputter Coater SC7620, Polaron, VG Microtech, England). The analysis was done via SEM (JEOL, JSM-6060LV, Tokyo, Japan). SEM analysis showed that there was an insufficient enamel layer (Fig. ). Additionally, the other third molar tooth was demineralized with 10% formaldehyde for 3 weeks. The tooth was then cut longitudinally, and it was stained with hematoxylin-eosin stain. Histological findings revealed that dentin structure was intact and there was no irregularity in tubular structure. These findings confirmed that there was no defect related to dentin (Fig. ).\nDiagnostic casts were attached on a semi-adjustable articulator (Stratos 200; Ivoclar Vivadent Ag, Pforzeim, Germany) in centric occlusion. The occlusal vertical dimension was determined by the Niswonger method [] and verified with the closest speaking space method. The interocclusal distance at the physiologic rest position was 6 mm. In order to ensure proper vertical dimension and to create enough space for a restorative reconstruction, the bite was opened 5 mm in the anterior region. Furthermore, to predict the last appearance of the restoration, diagnostic wax-up was prepared at the determined vertical dimension (Fig. ), and expected treatment outcome was performed with the digital smile design software (Romexis 4.5, Planmeca USA, Inc).\nUnder local anesthesia, all teeth were prepared with chamfer margins. The crown length of maxillary and mandibular posterior teeth was insufficient for crown retention. In addition, there was an asymmetry of the gingival contours in the anterior maxillary teeth (Fig. ). After periodontal evaluation, because of insufficient crown length, ostectomy procedure was performed for maxillary and mandibular posterior teeth. In addition, gingivectomy was applied for maxillary anterior teeth. All periodontal surgery procedures were performed by the periodontologist. The impressions for provisional casts were made with a condensation silicone impression material (Zetaflow; Zhermack, Italy). Then, an interocclusal record was prepared with a hard addition-type A-silicon material (Imprint™ Bite, 3M ESPE) at the increased vertical dimension (5 mm in anterior region) which was determined in diagnostic wax-up stage. The autopolymerizing acrylic resin (ALIKETM; GC America, Alsip, IL, USA) provisional crowns were fabricated at the determined vertical dimension extraorally by an indirect method, then they were cemented with temporary cement (Temp Bond NETM; Kerr) (Fig. ). These restorations were assessed in terms of esthetic and phonetics. 1, 2 and 3 month regular checkups were performed. The speech, swallowing, anterior and posterior speaking space, muscle sensitivity, mastication, TMJ discomfort, were assessed during this period. The patient was asymptomatic. The criteria of the success for increased vertical dimension were the absence of pain, no sensitivity in facial and masticatory muscles, phonetic and swallowing satisfaction. At the end of the 3 months follow up period, definitive impressions were made with a condensation silicone impression material (Zetaflow; Zhermack, Italy). Occlusal registration was obtained with a hard addition-type A-silicon material (Imprint™ Bite, 3M ESPE) with a slightly reduced vertical dimension from the provisional restoration. After that, the working casts were obtained and mounted on the semi-adjustable articulator (Stratos 200) using a face-bow transfer (Facebow UTS-3D, Ivoclar Vivadent) (Fig. ).\nThe patient was rehabilitated with metal alloy (Mıcrolit Isı, Schütz Dental Group) - ceramic (Cerabien ZR Noritake) fixed partial dentures in the posterior regions. In order to achieve better esthetic appearance and to camouflage the AI affected tooth color, zirconia ceramic (Katana, Kuraray Noritake Dental Inc) based crowns were used in the anterior regions of low and upper jaws (Fig. ). With this prosthodontic rehabilitation, maximum interdigitation and a canine guidance could be achieved. Metal-ceramic fixed partial dentures were cemented with zinc polycarboxylate cement (Adhesor® Carbofine; Kerr) and zirconia ceramic crowns cemented with self-etch dual cured resin cement (Maxcem Elite, MXE; Kerr). Following cementation, a maxillary protective occlusal splint was manufactured to protect the restorations from chipping or fracture due to the bruxism. The patient was instructed about oral hygiene maintenance.","The patient came in for help with tooth discoloration, gaps between her teeth, and some sensitivity. Tests showed her teeth were thin and her enamel wasn’t as strong as it should be, and she had some issues with her bite. After careful planning and adjustments to her bite, the dentist created new crowns to improve the appearance and function of her teeth, ensuring a comfortable and healthy smile. The dentist also performed some minor procedures to prepare the teeth for the crowns and monitored the patient’s comfort and speech throughout the process."
683,"In March of 2017, a 20-year-old virgin female with \nachronic pelvic pain was referred to our center. The patient \ncomplained of severe pelvic pain with verbal numerical \nrating scale (VNRS) of 9 during the menstrual \ncycle. This chronic pain had lasted for almost one year. \nThe patient did not mention dyschezia, pain during or afterurination, \norother symptoms associated with diaphragmatic \nendometriosis, such as chest pain, shoulder pain, or \nright upper abdominal pain. Furthermore, she had used no \nhormone replacement therapy.\nIn abdominal examination, there was fullness on the \nleft side, while in both rectal examination and abdominal \nexamination, there was fullness in the posterior cul-de-\nsac. An immobile 10-cm mass wasfelt on the left side, \nwhereas another immobile 5-6-cm mass was on the right \nside that was fixed to the uterus.\nPelvic ultrasonography results indicated a cyst with an \napproximate size of 12×7 cm consisting of thick contents \nin the left ovary with internal septae, raising suspicion \nregarding formation of the tubo ovarian complex in endometrial \ncavity. Furthermore, the ultrasound findings \nshowed an endometrium a cyst with an approximate dimension \nof 4 cm on the right side with adhesion and endometrial \nnodule of the posterior fundus with moderate \nadhesion to the rectosigmoid. Therefore, magnetic resonance \nimaging (MRI) was performed to exclude the left \nmass from adenocarcinoma, while the results showed normal \nupper abdominal organs, including liver, spleen, pancreas, \nkidneys, adrenal, as well asthe lungs. In pelvic MRI \nfindings, there was endometrium in both adnexae along \nwith hydrosalpinx on the left side, whereas enhancement \nwas not reported in the left adnexal masses.\nIn addition, the blood test showed an anti mullerian hormone \n(AMH) of 1.82 and CA-125 of 125.1, while other \ntumor markers, including risk of ovarian malignancy algorithm \n(ROMA) and HE4 were normal.\nDuring laparoscopy, we noticed extensive endometriosis \nthat involved the anterior and posteriorcul-de-sac, both pelvic \nside walls, both ovaries, and sigmoid colon. The left \novary contained a cyst measured 10-12 cm with severe adhesion \nto the rectum, while the right ovary contained a cyst \nmeasured approximately 6 cm with moderate adhesion to \nthe tube and the right ovary. There was also no evidence \nof endometriosis in ureters. Anatomy of pelvis restored, \npelvic Die corrected and a 2-cm endometriotic nodule attached \nto the rectovaginal septum (RVS) was shaved.\nOn exploring the upper abdomen, 5 to 6 areas of superficial \nendometriosis were discovered in the, anterior and center \nof the right hemi-diaphragm (Figes, ), but the left hemi-\ndiaphragm was intact. The tota Redwine l surface area of the \ndiaphragm (left side, center, and right side) was thoroughly \ninvestigated when the patient was put into reverse Trendelenburg \nposition. The fulguration was performed using bipolar \nenergy for endometriotic lesions of the diaphragm. The \nendoscopic exploration of thoracic cavity was not performed \nbecause the patient had no symptoms of shoulder or chest \npain, no history of catamenial hemothorax or pneumothorax \nand diaphragmatic involvement was superficial.","This 20-year-old woman has been experiencing severe pelvic pain for almost a year. During her examination, doctors found two large masses in her pelvis, and a laparoscopy revealed extensive endometriosis affecting her ovaries, bowel, and diaphragm. The endometriosis was surgically removed, and the patient’s blood tests showed elevated levels of certain markers, suggesting the presence of endometriosis. Further testing ruled out other serious conditions, and the patient is now being monitored to ensure the endometriosis is fully resolved."
684,"A 50-year-old male with HIV, ESRD on PD, HTN, and recently diagnosed DLBCL presented to the Emergency Department with RUE pain and swelling ten days after receiving his first cycle of R-CHOP (cyclophosphamide dose reduced for PD) through a peripheral IV in the right hand. The patient reported that the swelling began in his right hand six days after the chemotherapy infusion, and was associated with tender, itchy, and notably darkened forearm veins. He also described shooting pains coming from the darkened veins. He denied any other rash or erythema, and endorsed chills but no fever. Physical examination was remarkable for mildly tender, deeply hyperpigmented veins originating at the site of his chemotherapy infusion site, and edema of the dorsum of the right hand without warmth or erythema (). Labs were significant only for neutropenia (ANC 900). An upper extremity ultrasound was performed to rule out a DVT associated with this possible thrombophlebitis which was negative.\nThe patient was diagnosed with serpentine supravenous hyperpigmentation (SSH) and discharged home with supportive care on hospital day 2. Upon follow-up in the office four months later, the original SSH in his right arm had improved significantly (), but he developed new SSH in his left arm where he was now getting his chemotherapy infusions ().\nIn this instance, however, he did not feel any pain or discomfort from the darkened veins.","A 50-year-old man with HIV and kidney problems was admitted to the hospital after experiencing pain and swelling in his right hand ten days after starting chemotherapy for lymphoma. The swelling was caused by a condition called serpentine supravenous hyperpigmentation (SSH), which is a harmless darkening of the veins. Although the initial swelling in his right hand improved over time, new darkening appeared in his left arm after receiving more chemotherapy. He was treated with supportive care and continues to be monitored."
685,"Brown-skinned patient, 20 years old, female, presented with good general health and excellent oral health. Her main complaint concerned the projection of the lower incisors out of the mouth. She presented a Class III skeletal pattern, aggravated by the lack of space for correct alignment of the lower arch, due to the presence of two supernumerary lower incisors.\nFrontal facial examination revealed mandibular asymmetry to the right side. In sagittal view, the lower facial third was increased in comparison to the upper and middle thirds. The facial profile was concave due to mandibular projection, with passive lip seal. The aesthetics of smile was impaired due to the anterior crossbite.\nThe patient had a Class III skeletal pattern, and facial growth was predominantly horizontal. Occlusal analysis revealed Angle Class I malocclusion with 1-mm overbite and anterior crossbite. The mandibular arch presented moderate anterior crowding and the maxillary arch exhibited anterior contraction on the right side ().\nBolton’s analysis revealed inferior excess of 7.5 mm, considering the proportion between the sum of the mesiodistal widths of the fourteen lower and twelve upper teeth; and inferior excess of 9.2 mm, considering the proportion between the anterior lower teeth with the anterior upper teeth.\nThe periapical and panoramic radiographs revealed intact roots, absence of the upper and lower third molars on the right side, presence of two fully erupted supernumerary incisors, as well as light horizontal bone loss in the lower arch (Figs 2 and 3).\nCephalometry confirmed the Class III skeletal pattern with ANB = -3o, horizontal growth pattern (SN.GoGn = 22o and FMA = 12o) and compensatory inclinations of the incisors (1.NA = 29o, 1.NB = 37o and IMPA = 107o).This positioning of incisors contributed to an unfavorable tegumentary relationship that impaired the patient’s facial aesthetics (Upper lip - S line = 0mm, Lower lip - S line = 4mm) ( and ).","This 20-year-old woman came in because her lower teeth were jutting out slightly. She has a noticeable imbalance in her jaw and face, which is causing her smile to look less appealing. X-rays showed she has two extra teeth in the lower jaw and a slight bone loss in the lower arch. Overall, she has a significant skeletal imbalance that needs to be addressed to improve her facial appearance and bite."
686,"Case 1: A 53-year-old woman presented with a recurrent headache, blurred vision, and progressive memory loss. The headache first appeared 5 years earlier and worsened gradually. One and a half years ago, she developed blurred vision. Brain magnetic resonance imaging (MRI) at that time showed hydrocephalus. Repeated lumbar punctures revealed increased opening pressure, elevated protein and pleocytosis without identifing the etiology. Two months ago, she developed progressive memory loss. She also had recurrent grand mal seizures about 20 years ago. On admission, head computed tomography (CT) showed scattered parenchymal calcified lesions in the right frontal lobe, right parietal lobe, right thalamus, left temporal lobe, left occipital lobe, and bilateral basal ganglia area (Figure ). Brain MRI showed hydrocephalus and diffuse T2-weighted hyperintensity in the juxta-ventricular white matter, together with enhancement of the meninges, especially the basal meninges, and multiple cystic lesions in the prepontine cistern, ambient cistern, and suprasellar cistern (Figures ). CSF cytology revealed increased eosinophils. NGS of CSF identified T. solium DNA sequences (Figures ). Therefore, the serum and CSF samples were sent for C. cellulosae IgG testing; and both were positive. Plain x-rays showed scattered “cigar-shaped” calcified lesions in the legs. She was diagnosed with NCC (basal subarachnoid NCC and parenchymal NCC with calcified cysts) and was treated with albendazole and dexamethasone. She also underwent an endoscopic third ventriculostomy (ETV) because of the severe hydrocephalus. The patient's symptoms, neuroimaging and CSF findings improved markedly after treatment.","6 This 53-year-old woman has been experiencing headaches, blurry vision, and memory problems for several years. Brain scans revealed fluid buildup and calcium deposits in her brain, along with inflammation and fluid-filled cysts. Tests showed she had an infection with a parasite called Toxoplasma gondii, which caused the inflammation. She is now being treated with medication and a surgical procedure to relieve the pressure on her brain, and her symptoms are improving."
687,"Case 2: A 57-year-old man presented with paroxysmal blurred vision for 2 months. When he was admitted 1 month ago, lumbar puncture revealed increased opening pressure, pleocytosis, elevated protein level, and reduced glucose level. CSF cytology showed lymphocytic inflammation. Cryptococcus antigen test and Mycobacterium PCR of the CSF were negative. He was diagnosed with possible tuberculous meningitis and started on empirical anti-tuberculous treatment. However, he was readmitted after 1 month when his symptoms were not relieved. Lumbar puncture was repeated and NGS of CSF was negative. Serum and CSF samples were both positive for C. cellulosae IgG. Head CT revealed a single calcified lesion in the left frontal lobe (Figure ). Brain MRI revealed no obvious abnormalities, including hydrocephalus (Figure , Supplementary Figure ). Spine MRI was not performed. He was diagnosed with parenchymal NCC (calcified cyst), and possibly extraparenchymal NCC or spinal NCC without radiological evidence. Treatment with albendazole and dexamethasone was started. However, the symptoms and CSF findings worsened initially. To validate the diagnosis and rule out other possibilities, NGS of CSF was repeated 1.5 months later and identified T. solium DNA sequences (Figures ). The albendazole and dexamethasone were continued and the patient's symptoms and CSF findings improved. Note that the diagnosis of extraparenchymal NCC or spinal NCC in Case 2 was not very convincing without radiological proof. A false-positive result was not completely ruled out in this patient.","6 This 57-year-old man has been experiencing blurry vision and headaches for several months. After several tests, including spinal taps and scans, doctors initially suspected tuberculosis but ruled it out. Further testing revealed a small, calcified cyst in his brain, which is likely the cause of his symptoms. He is now being treated with medication, and his condition is improving."
688,"Case 3: A 58-year-old man presented with recurrent headache, transient loss of consciousness (LOC), and progressive memory loss. Eight years before admission, his symptoms began with recurrent headache and transient LOC. Lumbar puncture revealed increased opening pressure, pleocytosis, elevated protein level, and reduced glucose level. He was diagnosed with possible tuberculous meningitis and given empirical anti-tuberculous treatment for more than 1 year. Six years ago, he was admitted with the same symptoms and diagnosed with possible cryptococcal meningitis, for which he received fluconazole for more than 6 months and amphotericin B for 1 month. Three months before admission, he developed progressive memory loss. On admission, brain MRI showed an enhanced lesion posterior to the medulla (Figures ) and hydrocephalus (Figure ). NGS of CSF identified T. solium DNA sequence (Figures ). Plain x-rays showed scattered “cigar-shaped” calcified lesions in the legs and thoracic wall. Serum and CSF samples were both positive for C. cellulosae IgG antibodies. He was diagnosed with intraventricular NCC and treated with albendazole and dexamethasone. The symptoms and CSF findings subsequently improved.","This man has a long history of recurring headaches and brief periods of losing consciousness, which started about eight years ago. After several tests and treatments for possible meningitis caused by tuberculosis and cryptococcus, he was recently diagnosed with a rare type of meningitis called intraventricular neurocryptococcosis (NCC). Imaging showed a problem in his brain, and tests revealed the presence of the bacteria causing the infection. Fortunately, treatment with albendazole and dexamethasone helped to improve his symptoms, and his condition is now stable."
689,"Case 4: A 31-year-old man presented with progressive blurred vision for 3 weeks. On admission, brain MRI showed multiple cystic lesions in the suprasellar cistern (Figures ). Lumbar puncture revealed increased opening pressure, an elevated white blood cell count, elevated protein level, and reduced glucose level. CSF cytology revealed increased eosinophils. Cryptococcus antigen tests and an Xpert-MTB assay of the CSF were negative. NGS of CSF identified T. solium DNA sequences (Figures ). Serum and CSF were positive for C. cellulosae IgG antibodies. He was diagnosed with basal subarachnoid NCC and was treated with albendazole, dexamethasone, and ETV. His symptoms and CSF findings improved significantly after treatment.","A 31-year-old man came to the hospital because he was experiencing blurry vision that had been getting worse over three weeks. Brain scans showed several fluid-filled cysts in his brain, and a spinal tap revealed signs of infection, including high pressure and an increased number of white blood cells. Tests showed that he had a parasitic infection called Taenia solium in his cerebrospinal fluid. He was treated with medication, and his vision and symptoms improved significantly after starting treatment."
690,"A 46-year-old gentleman, morbidly obese (BMI 57.4 kg/m2), was referred to our institute 20 days after a laparoscopic sleeve gastrectomy, complicated by gastric leak. On presentation, he was septic and in distress, tachycardic, and tachypneic. He was febrile and complaining of abdominal pain. Examination revealed a distended abdomen with diffuse tenderness and left basilar crackles on lung examination. Initial laboratory tests revealed elevated WBC and CRP. Upper GI series and CT scan of the abdomen showed evidence of contained gastric fistula with perigastric fluid collection (Figs. and ).\nThe patient was kept NPO, started on parenteral nutrition, intravenous antibiotics and was well-hydrated to control the sepsis. He underwent CT-guided drainage of the collection. One week post-drainage, upper GI series was repeated showed a well-drained gastric leak.\nAfter 10 days of stabilization, the patient showed marked improvement, became afebrile, and his WBC and CRP normalized, so a decision to undergo a Baltazar procedure was taken (Fig. ).\nAfter insufflation of the abdomen and insertion of trocars, lysis of loose adhesions was successfully done, in aim to uncover the gastric tube, which was covered with omental adhesions. The perigastric cavity was opened and well-irrigated, and with careful dissection, we unexpectedly identified two leak sites along the staple line, the first one was located 4 cm below the gastroesophageal junction, and the second one was located 6 cm away from the first fistula site. Unfortunately, stenting was not available at our institution.\nAfter careful assessment, intra-op decision was made to attempt a new surgical technique: double Baltazar procedure. Two fistulo-jejunostomies were done with the same jejunal limb. The first fistulo-jejunostomy was done at the cephalic gastric fistula site with handsewn double-running 2/0 PDS suture. More distally to the created fistulo-jejunostomy, along the same jejunal loop, the second fistulo-jejunostomy was done using the same technique. Methylene blue test was done and showed no residual leak. Jejuno-jejunostomy (Brown anastomosis) was done after that to divert the biliary secretions and to protect the anastomotic sites. All quadrants of the abdomen were drained (Fig. ).","A 46-year-old man had complications after surgery to help him lose weight, resulting in a leak in his stomach. He became seriously ill with a severe infection and needed intensive care, including drainage of fluid from his abdomen. After several weeks of treatment and further surgery, the leak was finally repaired with a new technique. The patient is now recovering well and is being monitored closely to ensure the repair is successful."
691,"This is a 50-year-old right-handed male, with 33-year history of T6 AIS A SCI from a gunshot wound complicated by chronic pain, left hip and knee heterotophic ossification, and a chronic dislocation of his right hip, who initially presented to the emergency room with a right shoulder mass in September 2014. While he initially noticed the mass about 2 months earlier, he presented for evaluation now because of acute onset of pain, weakness and paresthesias in the right arm. He was admitted to the general medicine service for pain management and underwent an initial work up for his right shoulder mass, including advanced imaging and a core biopsy. Physiatry was consulted due to his functional deterioration that precluded him from returning to his previous independent living arrangement. He demonstrated diffuse, mild weakness throughout the right arm that was variable and seemed to be correlated with his reported pain level, but his most consistent and weakest movement patterns were his grade 4/5 weakness in finger abduction and distal interphalangeal joint flexion. He had reduced pin prick sensation over the volar surface of digits 3–5, palm and forearm of the right arm and hand. He was not able to perform transfers to or from his manual wheelchair due to his level of pain. The magnetic resonance imaging (MRI) revealed a heterogeneously enhancing mass with a maximum diameter of 6.9 cm (Fig. ) that involved the right deltoid and pectoralis major muscles. His core biopsy demonstrated a STS that was classified as a high grade (III) spindle cell sarcoma.\nOncology recommended treatment for his STS with a course of outpatient neo-adjuvant radiation therapy followed by gross total resection with wide margins. Physiatry pre-operative consult focused heavily on functional prognostication. The patient expressed multiple times that he placed the highest priority on return to his previous modified independent living arrangement and not only survival after his STS treatment course. The patient’s personal values combined with the physiatric assessment informed the pre-surgical planning. In particular a decision was made to take a narrower surgical margin around key muscle group (pectoralis major and deltoids) in order to help maintain the man’s manual wheelchair mobility and his ability to independently transfer himself.\nHe completed neo-adjuvant radiation therapy in November of 2014 and underwent radical excision of his right shoulder mass with flap closure that December. He began intensive inpatient rehabilitation after he was given clearance to weight bear through his arm about 8 weeks later. Initially he required total assistance for most ADL’s, including transfers, manual wheelchair propulsion, dressing and toileting. Despite the extensive surgery and radiation treatments, the gentleman was able to return to a functional level, approaching his pre-morbid status (modified independence). He was successfully discharged home to live alone in his accessible apartment complex.","A 50-year-old man with a long-term spinal injury and a history of chronic pain was admitted to the hospital because of a new painful mass in his shoulder. Tests revealed he had a type of cancer in his shoulder muscle, and doctors planned a treatment plan including radiation therapy and surgery. The surgery was carefully planned to preserve his ability to use his arm and wheelchair, as he strongly wanted to return to living independently. After completing his treatment and rehabilitation, he was able to return home and live alone, regaining a good level of function."
692,"A 70-year-old woman was diagnosed with DLBCL (Ann Arbor Stage: IIIA) and received chemotherapy but relapsed. She was admitted to our hospital in 2017 to undergo auto-PBSCT after salvage chemotherapy. She suddenly developed grade 3 haematuria on the day of transplantation. We detected a BKV DNA load of 5.0 × 107 copies/mL and adenovirus (ADV) type 11 DNA load of 5.0 × 107 copies/mL in the urine and diagnosed her with haemorrhagic cystitis (HC) associated with BKV and ADV. Although she received immunoglobulin and adenine arabinoside, the HC symptoms did not improve. Moreover, we detected a BKV DNA load of 2.2 × 102 copies/mL in the blood and diagnosed the patient with BK viraemia with complications. Although we administered cidofovir (1 mg/kg, three times a week) from days 8 to 26 post-auto-PBSCT, the HC symptoms persisted. The ADV DNA load in urine became negative, but the BKV DNA load in urine did not decrease. Overall, the BKV infection did not stabilize adequately.\nShe exhibited respiratory failure and elevated serum C-reactive protein levels at day 32 (Table ). Chest computed tomography (CT) showed ground-glass opacity (GGO) in the bilateral upper lobe, and we performed BAL at day 34. Although BAL fluid (BALF) was not macroscopically reddish, BAL slightly detected red blood cells on cytology. In BALF, the BKV DNA load was 1.5 × 102 copies/mL, although the ADV and cytomegalovirus DNA loads were not elevated. Although we could not perform lung biopsy because the blood platelet count was low, we diagnosed the patient with BKV pneumonia. After re-administering cidofovir, respiratory symptoms and GGO in CT abated, although HC symptoms persisted (Fig. ). The patient has not experienced a relapse of BKV pneumonia and DLBCL even after 11 months.","This 70-year-old woman was initially diagnosed with a type of lymphoma and received chemotherapy, but the cancer returned. After a stem cell transplant, she developed a serious bladder infection caused by viruses (BKV and ADV) that led to significant bleeding. Despite treatment, the infection didn't improve, and she developed pneumonia related to the same viruses. Thankfully, with further medication, her pneumonia cleared up, and she has remained in remission for over 11 months."
693,"A 6-month old exclusively breastfed, African boy presented to the emergency department (ED) with an out-of-hospital cardiac arrest. In the weeks prior to presentation, he had 3 brief episodes of peri-oral cyanosis and pallor and presented twice to ED with increased work of breathing. On initial assessment by paramedics he showed no signs of life and was in asystole. He was resuscitated until spontaneous circulation was restored at 36 min. Investigations revealed low ionised calcium (0.72 mmol/L), warranting repeated intravenous calcium boluses followed by continuous infusion. Cefotaxime was commenced for presumed sepsis, and oseltamivir was added after isolating influenza A on a nasal swab. Intravenous fluids and inotropes were administered. In the intensive care unit, an echocardiogram showed severe dilated cardiomyopathy with poor left ventricular ejection fraction (LVEF) of 25–30% [normal 55–70%]), fractional shortening (FS) of 7% [normal 29–40%], dyskinetic septal motion, global hypokinesia, and moderate to severe mitral regurgitation with a structurally normal heart. Rickets was confirmed radiographically (Fig. ), with elevated serum ALP and PTH concentrations, and low 25OHD < 15 nmol/L (Table ). Cholecalciferol (6000 IU daily) was commenced, and intravenous calcium was continued until serum calcium normalised (72 h). Cardiac failure was managed with diuretics and vasodilators. Brain Magnetic resonance imaging (MRI) revealed severe hypoxic-ischaemic encephalopathy, correlating with the clinical finding of unresponsiveness to external stimuli. The care team and family elected to withdraw life support, and the infant died 6 days after presentation.\nPost-mortem examination confirmed severe nutritional rickets with rachitic rosary (enlarged rib growth plates) (Fig. ), craniotabes, soft ribs, dilated cardiomyopathy (heart weight 71 g [>95th centile], with multifocal myocardial necrosis) and massive ischaemic brain injury. Histological analysis of a 7th rib growth plate showed extreme disarray, widening and lengthening, with islands of hypertrophic chondrocytes reaching far into the primary spongiosa and mature bone, typical of rickets (Fig. ). Histomorphometric analysis of a transiliac bone sample identified severe osteomalacia with increased osteoid thickness (23.2 μm [normal 6.4 +/− 1.4]), osteoid surface/bone surface (76.3% [normal 24.9 +/− 10]) and osteoid volume/bone volume (40.5% [normal 2.4 +/− 1.22]). Specifically, osteoid thickness was + 262% and osteoid volume/bone volume + 1573% of normal reference values [9]. Since Goldner’s Trichrome staining does not discriminate well between non-mineralized and poorly mineralized matrix, we also performed quantitative backscattered electron imaging, which confirmed the extremely low bone mineralization density (Fig. ).\nThe mother had received antenatal multivitamin supplementation and attended all post-natal child surveillance and vaccination appointments. She was not informed of the need for infant vitamin D supplementation. Mother (Table ) and a 9-year old sibling had suboptimal 25OHD concentrations.","This baby boy had a sudden cardiac arrest while at home, and paramedics were able to revive him. Tests revealed he had a very weak heart and severe rickets, likely due to a lack of vitamin D. He also had a brain injury from the lack of oxygen. Sadly, despite the best efforts of the medical team, he passed away, and a post-mortem examination showed the cause of death was related to severe nutritional problems and brain damage."
694,"A 6-month old, partially breastfed and previously well Somali boy presented to the ED following respiratory arrest and seizure. He was found pale, floppy and not breathing while held by his sibling. Following emergency telephone advice, his mother, a nurse, commenced Cardio-pulmonary resuscitation (CPR) at home. Two minutes later he had a 2-min tonic-clonic seizure. With continued CPR, spontaneous breathing was established at 4 min. Paramedics found him drowsy with normal blood glucose. In the ED, he responded to pain, respiratory rate was 40/min, heart rate was 112/min with normal capillary refill. A grade 2/6 systolic ejection murmur was present. A venous blood gas was normal except for low ionised calcium (0.66 mmol/L). A chest radiograph showed cardiomegaly (Fig. ), and echocardiogram demonstrated a structurally normal heart with severely dilated left ventricle with reduced LVEF of 29%, FS of 7%, global hypokinesia and moderate mitral regurgitation, confirming hypocalcemic dilated cardiomyopathy. Diuretic and ACE (Angiotensin converting enzyme) inhibitor therapy was commenced. Nutritional rickets due to vitamin D deficiency was confirmed with knee radiographs (Fig. ), elevated serum ALP and PTH, and low 25OHD of < 5.2 nmol/L (Table ). He received intravenous calcium and oral cholecalciferol (6000 IU daily). Alfacalcidol (1-hydroxycholecalciferol) was temporarily administered to improve calcium absorption. On day 3, following a switch from intravenous to oral calcium, he had another seizure with respiratory arrest in hospital, requiring mechanical ventilation and intensive care. Intravenous calcium was recommenced, and a head computed tomography was normal. He was extubated 24 h later and continued intravenous calcium for 5 more days. He was discharged home on day 17 and 3 months later showed slow recovery (LVEF 35%; FS 16%; Left ventricle diameter 42 mm [Z-score + 4.7], marked reduction in mitral regurgitation).\nThe mother had been provided with one bottle of vitamin D for the baby at birth but was not informed to continue supplementation, and adherence was not assessed. She (Table ) and three of the infant’s four siblings (aged 3, 6, 7, 9 years) were vitamin D deficient, with elevated ALP and PTH.","This 6-month-old boy was rushed to the hospital after collapsing and having a seizure. He was found to have a very weak heart and low calcium levels, which caused the problems. Doctors treated him with medications and vitamin D to help his heart and calcium levels improve, and he gradually recovered over several weeks."
695,"A five-month old British Pakistani girl presented to ED with cough, difficulty in breathing and poor feeding. She was born at 35 weeks with a birth weight of 1.75 Kg (9th centile) and required admission to the neonatal unit for 6 days to establish oral feeding. At presentation, she was found to be pale, irritable, tachypnoeic and tachycardic. She had faltering growth (fall across ≥2 weight centiles) with a weight of 4.5 kg (< 0.4th centile) and length 58 cm (on 0.4th centile). She was diagnosed with bronchiolitis. Only the faltering growth triggered further investigations which identified hypocalcemia (1.96 mmol/L). Further evaluation of hypocalcemia revealed raised ALP and PTH, and low 25OHD of 12.5 nmol/L (Table ) and rickets on knee radiograph (Fig. ). An echocardiogram performed in view of persistent tachycardia, systolic murmur and cardiomegaly on chest radiograph (Fig. ) revealed a structurally normal heart with a severely dilated left ventricle (LVEF of 25%, FS of 15%, global hypokinesia and severe mitral regurgitation), confirming hypocalcemic dilated cardiomyopathy. She was commenced on oral calcium supplements (500 mg/day in divided doses) and cholecalciferol (initially 3000 IU daily, later increased to 6000 IU daily) and transferred to our tertiary center for specialist cardiology care. She was commenced on diuretics and ACE inhibitors.\nNobody had informed mother of the need for vitamin D supplementation during pregnancy and infancy. Her 3 year old sibling had normal 25OHD levels, however mum was deficient with a raised PTH (Table ).","This five-month-old girl came to the hospital with a cough, trouble breathing, and not wanting to eat. She was born early and had some difficulties feeding as a baby. Doctors found she was very pale and breathing fast, and her growth had slowed down. After further tests, they discovered she had a heart problem called hypocalcemic dilated cardiomyopathy, which was likely caused by low calcium levels, and she was started on medication and vitamin supplements to help her heart."
696,"A 45-year-old previously healthy Asian man presented with a history of intermittent fever with chills and rigors over 2 months’ duration. There were associated night sweats, loss of appetite, and loss of weight. There was a history of transient macular rash at the onset of the fever which spontaneously resolved without treatment. Generalized lymphadenopathy was noted by our patient mainly involving cervical, axillary, and inguinal regions over 1 month which became extremely painful a few days prior to his presentation. He had synovitis involving lower limb small joints following the presentation, progressing to lower limb large joints and ultimately upper limb small and large joints over 3 days. He did not have past history or family history of arthritis and he had an unremarkable past medical history. He worked as a mason but had never been exposed to toxic environmental conditions to his knowledge and there was no promiscuous sexual behavior. He did not consume alcohol and he did not smoke tobacco.\nOn examination at the initial presentation he was emaciated, febrile, and pale. There were bilateral, firm, matted lymph nodes of varying sizes of 2–3 cm in the cervical, axillary, and inguinal regions which were tender. There was tender hepatosplenomegaly. The rest of the examination was normal. However, a few days following admission there was bilateral symmetrical polyarthritis involving both small and large joints of upper and lower limbs with lower limb predominance. There was marked synovitis of distal and proximal interphalangeal joints of lower limbs compared to the rest of his joints.\nLaboratory investigations revealed high white cell counts with normocytic anemia. Platelets were within the normal range. His inflammatory markers were high and they were in a rising trend following the onset of arthritis. His liver and renal functions were normal. Rheumatoid factor, anti-cyclic citrullinated peptide, anti-nuclear antibodies, human immunodeficiency virus (HIV) 1 and 2 antibodies, Epstein–Barr virus immunoglubulin G (IgG) and immunoglubulin M (IgM), cytomegalovirus IgG and IgM, and Toxoplasma antibodies were all negative or within normal limits. His serum uric acid was marginally elevated. An X-ray of his hands and feet showed soft tissue swelling without evidence of erosions or osteopenia.\nHis first lymph node biopsy showed a reactive lymph node. The biopsy was repeated due to a strong suspicion of lymphoma. The second lymph node biopsy with immunohistochemistry showed large pleomorphic cells with CD20 positivity and small lymphoid cells with CD3 positivity. The population of T cells was high, but the presence of B cells arranged in cohesive clusters and sheets favored a high grade DLBCL. Contrast-enhanced computed tomography (CT) of his neck, chest, and abdomen staged him at Ann Arbor stage IV.\nHe was started on non-steroidal anti-inflammatory agents and colchicine for the arthritis but there was no response to treatment. Treatment with rituximab, cyclophosphamide, vincristine, doxorubicin, and prednisolone (RCHOP) commenced; following the first cycle of therapy, his arthritis showed marked response with complete resolution of the synovitis. He completed his chemotherapy but intermittently succumbed to infections. His synovitis completely resolved following chemotherapy.","This 45-year-old man experienced a prolonged illness with fever, chills, and weight loss, accompanied by swollen lymph nodes and joint pain. Initial tests showed high white blood cell counts and inflammation, but blood tests for common infections and autoimmune diseases were negative. As the illness progressed, he developed widespread arthritis, and a lymph node biopsy revealed a type of aggressive lymphoma. Fortunately, he responded well to chemotherapy, and his arthritis completely resolved after treatment."
697,"A 33-year-old nulliparous woman was referred to our institution from a private infertility clinic complaining of lower abdominal pain. She reported a history of 5 weeks and 4 days of amenorrhea and had undergone intrauterine insemination (IUI) 27 days previously. Ovarian hyperstimulation for IUI was started with Clomiphene citrate 100 mg daily during the 3rd–7th days of the menstrual cycle, followed by 75 IU hMG (IVF-M HP, LG life science, Seoul, Korea) daily on the 7th–9th days of the menstrual cycle. Transvaginal ultrasound had revealed four dominant follicles in the left ovary after ovarian stimulation.\nOn physical examination, she had normal vital signs and diffuse lower abdominal tenderness. The serum beta-chorionic gonadotropin level was 3154 mUI/mL. Transvaginal ultrasound performed in the gynecology department revealed a large hyperechoic mass, a suspected hematoma, in the cul-de-sac. It also revealed a normal-sized uterus without an intrauterine gestational sac, and endometrial thickening of 20 mm. Both right and left adnexa were normal on the ultrasound. The initial complete blood count was as follows: hematocrit 35.9%, hemoglobin 11.9 g/dL, white blood cells 9.3 × 109/L and platelets 252 × 109/L. The provisional diagnosis was ruptured ectopic pregnancy with hemoperitoneum, and emergency laparoscopy was performed. Intraoperatively, a dark blood clot of about 800 ml was seen along with a small amount of fresh blood (Fig. ). An approximately 2 × 2 × 1.5 cm unruptured ectopic pregnancy was found in the right fallopian tube (Fig. ), while the left fallopian tube appeared to be normal. While examining the ovaries to locate the cause of the bleeding, we observed minimal bleeding from the proximal pole of the left ovary, where there was a 1.0 × 0.5 × 0.5 cm hemorrhagic mass with surrounding blood clot suggestive of a ruptured ectopic pregnancy (Fig. ). We resected the mass from the left ovary and performed a right salpingectomy using monopolar electrocautery and 5 mm multi-functional bipolar electrocoagulation forceps (LiNA PowerBlade; LiNA Medical, Glostrup, Hovedstaden DK-2600, Denmark) (Fig. ). After intraperitoneal irrigation with 3 L of saline, we ascertained that there were no abnormal findings. We therefore placed drains into the cul-de sac and finished the operation (Additional file ). Histopathological analysis demonstrated a left ovarian pregnancy with presence of chorionic villi within the ovarian tissue, along with a right tubal pregnancy (Fig. ).","This 33-year-old woman was referred to us because she hadn't had a period in several weeks and was experiencing lower abdominal pain. After trying fertility treatments, an ultrasound revealed a large blood clot in her abdomen, which led to an emergency surgery. During the surgery, doctors found two pregnancies – one in her right fallopian tube and one in her left ovary – both causing bleeding. The doctors removed the affected tubes and ovary to stop the bleeding and ensure her safety."
698,"A 25-year-old female with a two-year history of erythema, papules, nodules, and scales on her sole of left foot was presented to our outpatient center. She has no history of autoimmue disease and untreated with immunosuppressive therapy. Considering her pregnancy, she was not given treatments for 1 year. The left foot skin lesion on the medial and lateral margins and on the fourth toe dorsum became enlarged with evident pain after more than 1 year (Fig. ). Approximately 1 month before visiting our department, she received treatment ineffectively in a local clinic, and the diagnosis was unclear.\nSamples were obtained by the scraping of lesion and for light microscopy. Hyphae were observed by microscopic examination (Fig. ).\nWe carried out a skin tissue biopsy, which showed multiple granulomatous nodules (Fig. ). The Ziehl–Neelsen stain was negative. Periodic acid–Schiff (PAS) and Grocott methenamine silver (GMS) staining were carried out two times. Results were also negative. Biopsy specimens were also inoculated onto two kinds of media: Sabouraud’s dextrose agar (SDA), where one of which contained chloramphenicol and cycloheximide, and the other one contained chloramphenicol only. After being cultured on SDA at 27 °C for 7 days, spreading-woolly-white colonies grew on the inoculation sites of media containing chloramphenicol only and there,s no other colonies grew (Fig. ). The colonies produced an unpleasant smell like biogas. No colony was observed on the media with chloramphenicol and cycloheximide. Clamp connections, spicules, tear-like secretions, and medusa-like isomers were observed on the slide culture at 27 °C after 3 days (Fig. ). Urease activity tests were also performed. Trichophyton rubrum standard strain and the isolated strain were cultured on urease media at 27 °C for 7 days. The T. rubrum standard strain was negative, whereas the isolated strain turned red (Fig. ).\nSequencing of large subunit rDNA was performed by using the E.Z.N.A.™ Fungal DNA Mini Kit (Omega Biotek, USA). We utilized set primers for the region of internal transcribed spacer (ITS) and performed PCR. The PCR primers were ITS1: 5′-TCCGTAGGTGAACCTGCGG-3′ and ITS4: 5′-TCCTCCGCTTATTGATATGC-3′. The PCR-amplified DNA was matched with that of S. commune (Nos. KP 326677.1 and KP 004975.1) with a homology of 100%. After identification, the sequence was submitted to the GenBank (MF 495704).\nPathological finding and mycological examination indicated a cutaneous granuloma caused by S. commune. Oral itraconazole (100 mg) was applied twice a day. The rashes on the left foot and the pain regressed after 1 month of treatment (Fig. ). Follow-up is currently under way.","A 25-year-old woman with a long-standing skin problem on the sole of her left foot was seen in the clinic. Tests revealed a fungal infection, specifically Stachybotrys commune, causing a growth resembling a granuloma. The infection was treated with oral itraconazole, and the skin rash and pain improved significantly after one month. The doctor is continuing to monitor the patient to ensure the infection is completely cleared."
699,"This was a 69-year-old man diagnosed with metastatic colorectal adenocarcinoma to liver, lung, and skeletal. He underwent previous treatments with schemes based on fluoropyrimidine, platinum, antiangiogenics, and irinotecan and cetuximab, however, the patient's disease had progressed with all these therapies. Treatment was initiated with pembrolizumabe 10mg/kg every 2 weeks, although this medicines use to the patient's disease is not approved in Brazil. After second administration, the patient reported fatigue and dyspnea. Upon physical examination, he had saturation of 83% in an open environment. Chest tomography evidenced infiltrated interstitial to left and bilateral pleural effusion without signs of pulmonary thromboembolism. Blood count showed leukocytosis with 21,580 leukocytes, 69% of them were segmented, 11% rod cells and 3% metamyelocyte. After thoracocentesis, antibiotic therapy with ceftriaxone and clarithromycin was initiated, and oxygen intake with nasal catheter was maintained, however, no improvement was observed. Reassessment of chest computed tomography showed increase of ground-glass infiltrate () that suggested drug reaction (acute interstitial pneumonitis pattern); a lung biopsy was not performed for histological confirmation. Because of worsening in patients’ conscious level and respiratory pattern, after discussion with his family, the sedation was initiated for patient's comfort.","This 69-year-old man has advanced cancer that has spread to his liver, lungs, and bones. He had previously tried several cancer treatments, but his disease continued to grow. He recently started a new medication, pembrolizumabe, but experienced side effects like fatigue and difficulty breathing. Tests revealed fluid in his lungs and an increase in white blood cells, and he needed supplemental oxygen. Due to his worsening condition, he was given medication to help him relax and be more comfortable."
700,"This was a 73-year-old man diagnosed with melanoma on his right thigh. He underwent resection and clinical follow-up. After 8 years, he had untreatable metastatic lung melanoma without mutations. The patient was treated with dacarbazine followed by ipilimumab, but disease had progressed. After, we opted to begin pembrolizumab 2mg/kg administration every 3 weeks.\nFourteen days after first cycle, the patient had a dry cough but without fever or other symptoms, no changes in blood count was observed. Chest computed tomography showed opacities in ground-glass in both lungs (). The hypothesis raised was pembrolizumab-induced pneumonitis, although lung biopsy was not performed for histological confirmation. A treatment with 1mg/kg prednisone associated with antibiotic therapy and the patient had a rapid and important improvement in symptoms. Two months later, a staging computed tomography showed complete resolution of clinical feature (). Patient maintained treatment with pembrolizumab, and showed good tolerance.","A 73-year-old man was diagnosed with melanoma on his leg and had it surgically removed. Over time, the cancer spread to his lungs, and despite treatment, it continued to grow. He then started a new medication, pembrolizumab, which initially caused a cough, but was quickly treated with steroids and antibiotics. Fortunately, the cough resolved, and scans showed the cancer had completely disappeared, allowing him to continue with pembrolizumab treatment."
701,"This was an 81-year-old man diagnosed with untreatable stage IIIA lung adenocarcinoma without mutation. He underwent surgery followed by radiotherapy and adjuvant chemotherapy with carboplatin and pemetrexed. After 4 months of follow-up, the patient evolved with local recurrence. The affected site was irradiated but no response was seen, therefore, we opted for palliative chemotherapy with carboplatin and paclitaxel. A progression of the disease was also observed. Subsequently, we decided to begin immunotherapy with pembrolizumab 2mg/kg every 3 weeks. After four cycles, the patient had dyspnea and dry cough with oxygen saturation of 80%. Chest tomography showed extensive bilateral pulmonary infiltration (), and blood count showed leukocytosis. No lung biopsy was performed to confirm pathology. Corticosteroid therapy was introduced with metilprednisolone 2mg/kg and antibiotic therapy. An important clinical improvement was seen and resolution of findings from controlled computed tomography ().","6 This 81-year-old man was diagnosed with an advanced lung cancer that couldn't be treated with standard methods. He received surgery, radiation, and chemotherapy, but the cancer returned. To help manage the cancer’s progression, doctors started immunotherapy, but it caused some breathing problems. After treatment with steroids and antibiotics, the patient’s condition improved significantly, and scans showed a reduction in the cancer’s spread."
702,"This was a 54-year-old man diagnosed with pulmonary large-cell neuroendocrine carcinoma located and resected that evolved for metastatic disease. Initially the patient was treated with carboplatin and paclitaxel followed by cisplatin and etoposide, and radiotherapy for controlling specific injuries. The disease progressed to central nervous system and liver. We opted for immunotherapy with pembrolizumab 2mg/kg every 3 weeks.\nAfter 5 cycles of treatment, patients’ clinical feature evolved with dyspnea and cough, but no fever. Upon clinical examination his oxygen saturation was 84% in an open environment. In thorax angiotomography the possibility of pulmonary thromboembolism was discarded and it identified opacities in bilateral ground-glass. Thus, we opted for treatment with metilprednisolone 2mg/kg associated with piperaciline-tazobactam 4.5g every 6 hours for the hypothesis of pneumonitis, although no histological confirmation was performed. An important clinical improvement was seen within 24 hours. The controlled computed tomography performed 1 week after treatment showed almost full resolution of pulmonary opacities ().","This 54-year-old man was diagnosed with lung cancer that had spread to his brain and liver. He received chemotherapy and radiation to try to control the cancer, but it continued to grow. After trying immunotherapy, he developed breathing problems and low oxygen levels. Doctors treated him with medications to reduce inflammation in his lungs, which quickly improved his condition. Further tests ruled out a blood clot, and the lung opacities resolved with treatment."
703,"This was a 70-year-old man with metastatic lung epidermoid carcinoma with multiple liver injuries. The first-line treatment adopted included nivolumab 3mg/kg every 2 weeks, even without these agents approval in Brazil and in the United States considering the patient's status because he had contraindication for platinum-based chemotherapy.\nAfter 4 cycles, the patient had mental confusion, dyspnea and dry cough, without fever and oxygen saturation of 74% in an open environment. Blood count result was normal. We performed a chest tomography with appearance of infiltrated areas in ground-glass (). The hypothesis raised was pembrolizumab-induced pneumonitis, although the lung biopsy was not performed to confirm the disease. We opted for treatment with methylprednisolone 60mg every 8 hours and also antibiotic therapy. The patient improved clinically within few hours and he was discharged asymptomatic after 3 days of hospitalization.","This 70-year-old man was diagnosed with lung cancer that had spread to his liver. He started treatment with an immunotherapy drug, but after a few cycles, he developed symptoms like confusion, shortness of breath, and a dry cough. Tests showed signs of inflammation in his lungs, and doctors suspected it was related to the immunotherapy. He was treated with steroids and antibiotics, and his symptoms quickly improved, allowing him to go home without problems."
704,"We present a case of a 49-year-old male with a lung metastasis from hepatocellular carcinoma in the upper lobe of the left lung. He received radiotherapy to the lung metastasis according to the method described hereinafter. The proposed workflow started with acquiring planning CT images under deep inspiration breath-hold condition with a commercial gold coil marker, Visicoil 21G slim line (IBA Dosimetry, Schwarzenbruck, Germany) of diameter 0.5 mm and length 10 mm, implanted using CT guidance as close as possible to a tumor as shown in Figure . Then the CT images were exported to a treatment planning system, Monaco (Elekta AB, Stockholm, Sweden). A single-arc coplanar VMAT plan (gantry rotation from 320° to 100° ) was created with an isotropic planning target volume (PTV) margin of 5 mm and a prescribed dose of 60 Gy in 20 fractions as indicated in Figure . The plan was exported to a linac, Synergy (Elekta AB, Stockholm, Sweden), equipped with a kV fluoroscopic and cone-beam CT (CBCT) imager, Xray Volume Imaging (XVI).\nBecause VMAT beam-on-time typically exceeds 60 seconds, multiple breath-holds were required to complete the delivery. In other words, the single-arc VMAT beam was divided into several segmented VMAT beams each having different gantry start and stop angles. After performing CT imaging for the treatment planning, breath-hold training was given to each patient for optimizing the breath-hold and the following free breathing periods so that each segmented breath-hold VMAT delivery could be successfully completed.\nIn order to deliver segmented VMAT beams while the implanted marker stays at the planned breath-hold position, a DRR image at the gantry start angle was created in the Monaco TPS and transferred to the XVI. Subsequently, two lateral lines were drawn 2.5 mm above and below the center of the planned breath-hold marker position on the DRR image. Those lines were manually copied onto a fluoroscopic image window of the XVI display using a transparent sheet, each line being used as a tolerance limit for the breath-hold beam delivery.\nPrior to the beam delivery, CBCT imaging under free-breathing condition was performed to adjust the position of the patient couch by matching bone anatomy between the planning CT and the CBCT images. Subsequently, the patient was asked to breathe in slowly under fluoroscopy. Immediately after the marker position on the fluoroscopic image moved inside the tolerance range, the patient was asked to hold the breath and the VMAT beam was delivered. During the beam delivery, the breath-hold status was continuously monitored by checking if the deviation of the marker position exceeded the tolerance limit. As long as the marker stayed within the tolerance range, a segmented VMAT delivery continued for a preset period of 15 to 30 seconds depending on the breath-hold capability of each patient. As soon as each segmented delivery was completed, the beam interrupt button was pushed; and then, the patient was asked for free breathing. This procedure was repeated until all the segmented VMAT beams were delivered. Even when an intermediate beam interrupt due to a breath-hold failure during each segmented beam delivery was observed, the remaining beam delivery can be safely performed by referring to the two lateral tolerance lines for reproducing the breath-hold status for any gantry angles. It was decided that patients unable to hold the breath at least for15 seconds were considered not applicable. The patient who could hold the breath for 20 seconds was selected for this study after written informed consent was obtained. In order to confirm that the marker position relative to the tumor remained unchanged, multiple breath-hold CBCT imaging was also performed with the marker being inside the tolerance limit, thereby allowing comparison of the marker positions between planning CT and the breath-hold CBCT images.\nVideo shows a fluoroscopic movie showing the movement of the coil marker during the first coplanar segmented VMAT delivery (gantry rotation from 320° to 0° ) on the patient in reference to the two lateral lines (green color) giving a tolerance limit of 2.5 mm above and below the projected center of the marker. As long as the marker center stayed within the tolerance range, the segmented VMAT delivery continued for a preset period of about 25 seconds depending on the breath-hold capability of the patient. Unexpected intermediate beam interrupts due to a breath-hold failure during the segmented VMAT delivery can be well managed because the remaining beam delivery can be restarted at any gantry angle once the marker comes back within the tolerance range. In this lung tumor case, the total VMAT delivery time for a prescribed fraction dose of 3 Gy was approximately 115 sec with three beam interrupts and a 25 sec segmented beam delivery followed by 20 sec free breathing.","We’re treating a 49-year-old man with lung cancer that has spread from his liver. To shrink the tumor in his lung, we’ll be using radiation therapy. We’ll place a small marker in his lung to help guide the radiation and ensure it reaches the tumor accurately. The treatment will be done in small segments, requiring him to hold his breath during each part, and we’ll use X-ray imaging to make sure the marker stays in the right position throughout the process. We’ll carefully monitor his breathing and adjust the treatment as needed to ensure the radiation is delivered safely and effectively."
705,"A 19-year-old female was admitted to the emergency department with complaints of pain and swelling on the lateral side of the ankle after sustaining an ankle sprain. The patient was unable to bear weight upon admission. On physical examination, there was prompt swelling over the lateral side of the ankle and the tip of the fibula was tender on palpation. The ankle's range of motion was limited. The neurovascular examination was normal and the direct radiographic examination revealed a displaced distal fibular fracture (Weber type A) (Figure ). As the fracture was intra-articular and there was considerable displacement (>4 mm), fixation of the fracture was mandatory.\nUnder spinal anesthesia and tourniquet control, a small longitudinal incision was made over the distal fibula. The fracture was reduced and fixed with a single, 3.2 mm, intramedullary, magnesium headless compression screw (MAGNEZIX CS, Syntellix AG, Hannover, Germany) in a retrograde manner from the tip of the fibula. A short-leg plaster cast was applied to the patient for four weeks. After the removal of the cast, full weight-bearing was encouraged and ankle joint exercises were started. During the follow-up, fracture union was achieved without any complications within eight weeks (Figure ).\nAt the final follow-up examination, two years after the operation, the American Orthopaedic Foot & Ankle Society (AOFAS) score was 100 points and the patient had returned to the pre-injury level of activity. During the serial radiographic follow-up, a radiolucent zone was seen around the screw, but on the final follow-up radiograph, this radiolucency had almost completely disappeared (Figure ).","This 19-year-old woman was seen in the emergency room after twisting her ankle and experiencing pain and swelling. Examination revealed a broken fibula bone and limited movement in her ankle. To fix the fracture, doctors placed a small screw inside the bone and put a cast on her ankle for four weeks. After the cast came off, she was able to return to her normal activities, and the bone healed completely without any problems."
706,"The patient is a 16-year-old male without a significant past medical history who was transferred to our institution after a gunshot injury to the right lower extremity. On physical examination, two bullet entry points were evident at the right popliteal fossa and dorsal soft tissues of the distal right leg. Initial radiographs were negative for fractures or dislocation.\nComputed tomography angiography (CTA) demonstrated a retained bullet fragment within the popliteal fossa abutting the dorsal aspect of the popliteal artery. An 8 millimeter (mm) soft tissue density abutting the medial aspect of the popliteal artery was also identified, concerning for either a small pseudoaneurysm or short segment intramural hematoma. Streak artifact from the retained bullet precluded adequate assessment of this region. The peroneal artery demonstrated a 10 centimeter (cm) occlusion 2.5 cm distal to its origin but reconstituted distally at the level of the mid-tibia. The anterior and posterior tibial arteries were both normal in appearance and patent. The dorsalis pedis artery was unremarkable. There was subcutaneous emphysema throughout the deep and superficial posterior compartment of the knee and throughout the medial aspect of the leg.\nThe patient was taken to the interventional radiology suite and a right lower extremity diagnostic runoff angiogram was performed. Initial images obtained with the patient’s leg held in extension demonstrated abrupt cutoff of the popliteal artery immediately adjacent to the bullet fragment (Figure ). We then proceeded to reposition the patient’s right leg in the “frog-leg position”. A second diagnostic runoff angiogram was then performed demonstrating mild short segment narrowing of the popliteal artery immediately adjacent to the bullet fragment but with reconstitution of flow down to the level of the tibial-peroneal trunk (Figure ). The anterior and posterior tibial arteries demonstrated patency on both the extension and frog-leg positions. It was concluded that leg straightening/extension was contributing to extrinsic compression and subsequent dynamic occlusion of the popliteal artery secondary to the bullets close proximity to the vessel.\nThe patient was subsequently taken to the operating room for exploration and removal of the bullet fragment. An intraoperative angiogram was negative for popliteal artery vascular injury, and the anterior and posterior tibial arteries were both patent without evidence of injury or flow-limiting stenosis.","This 16-year-old male was brought to the hospital after being shot in his leg. Doctors found a bullet fragment in his leg and determined that straightening his leg was causing a blockage in the artery. They performed surgery to remove the bullet and found that the artery was not damaged. The arteries in his leg are now open and healthy, and he is recovering well."
707,"A previously healthy 3-year-old Moroccan boy was admitted with anemia and thrombocytopenia. He had been well until 3 weeks prior to presentation, when he developed a febrile erythematous rash. Fever recurred a week before admission, associated with lethargy, vomiting, and non-bloody diarrhea. Family history is negative for kidney or hematological disorders; the non-consanguineous parents and the boy’s three siblings are healthy.\nThe patient appeared pale, with bruises on abdomen, back, and lower extremities. The clinical exam was otherwise unremarkable. Laboratory work-up revealed hemolytic anemia with marked reticulocytosis, presence of schistocytes, profound thrombocytopenia, elevated uric acid, and normal serum creatinine concentrations. Plasma haptoglobin was undetectable, lactate dehydrogenase (LDH) elevated, and direct Coombs test negative. A stool sample was negative for E. coli O157:H7. Anti-streptolysin titers were only marginally elevated. D-dimers were increased to 2.49 μg/mL fibrinogen-equivalent units (N 0.02–0.47 μg/mL). Prothrombin, international normalized ratio (INR), partial thromboplastin time, fibrinogen, and C3 and C4 concentrations were normal, and sC5b-9 was increased to 653 ng/mL (normal < 300 ng/mL; SC5b-9 Plus MicroVue, ELISA, TECOmedical/Quidel, San Diego, CA). Urinalysis revealed microscopic erythrocyturia and mild proteinuria. On Day 2, the patient received transfusions of red blood cells and platelets. Hemoglobin (Hb) continued to fall to 48 g/L, and platelets dropped to 5 × 109/L within 2 days of the transfusions (Table ).\nA tentative diagnosis of aHUS was made, and a single dose of eculizumab (~ 900 mg/m2) was given 2 days after admission. The patient was vaccinated against N. meningitidis and started amoxicillin prophylaxis. Platelet count, Hb, and LDH started to improve after 4 days and normalized within 17 days. The diagnosis was corrected to TTP several days after discharge from hospital, when the ADAMTS13 activity in the pre-treatment plasma sample was found to be unmeasurably low using the fluorescence resonance energy transfer (FRETS-VWF73 substrate) assay (Peptide International Inc. Louisville KY) []. The patient also had anti-ADAMTS13 IgG antibodies (1:64) (in-house titration ELISA with recombinant ADAMTS-13 (Baxter, Mississauga, Canada) as target antigen and serial plasma dilutions). Incubating patient and reference plasma (ADAMTS13 activity 0 and 100%, respectively) in equal volumes at 37 °C for 30 min reduced the ADAMTS13 activity in the mixture to 0%, indicating the presence of an inhibitor in an assay analogous to the Bethesda assay using the FRETS-VWF73 substrate. Due to rapid clinical and laboratory improvement following treatment with eculizumab, we refrained from PLEX and immunosuppressive therapy. ADAMTS13 activity normalized completely after 15 months (Fig. and Table ).\nThe initial mutation screen for ADAMTS13 and genes encoding complement factors CFH, CFI, and CFB, CD46/membrane cofactor protein (MCP), factor H-related protein (CFHR) 5, C3, apelin and thrombomodulin was negative. CFH protein concentration was normal. Comprehensive re-testing confirmed the previous results and excluded mutations of diacylglycerol kinase-epsilon (DGKE), plasminogen (PLG) and methylmalonic aciduria and homocystinuria, cblC complementation type (MMACHC), but identified a homozygous deletion of CFHR3/1. No anti-CFH antibodies were demonstrated during active disease and follow-up, and there was no documented relapse of TTP or TMA over the 4 years of observation. A moderate, temporary increase of plasmatic sC5b-9 was noted > 2½ years after presentation in the absence of clinical symptoms or hematological evidence of TMA. At the time, ADAMTS13 activity had normalized, and anti-CFH antibodies were undetectable (Table and Fig. ).","This 3-year-old boy was admitted to the hospital because he developed a fever and rash, followed by fatigue and bruising. Tests showed he had a serious type of anemia and low platelet count, and his body was breaking down red blood cells. After trying different treatments, doctors discovered he had a rare condition called TTP, which was successfully treated with a medication called eculizumab. The boy is now doing well and has been closely monitored for any signs of recurrence."
708,"A 57-year-old man presented with neutropenia, since May 2016 due to a myelodysplastic syndrome. The revised international prognostic scoring system was 0, and no specific treatment was undertaken. Other significant past medical history included well-controlled hypertension treated with quinapril and type 2 diabetes mellitus without medication.\nIn November 2016, he experienced severe asthenia and excessive sweating. Laboratory tests revealed leukocyte count of 8,000 per cubic millimeter with hyperblastosis (23%), anemia, and thrombocytopenia. The results of the bone marrow aspiration confirmed AML (M4 type) according to the French–American–British classification, without extramedullary manifestations. FLT3, CEBPα, and NPM1 were not mutated and no cytogenetic abnormalities were found. This AML was secondary to a myelodysplastic syndrome with single lineage dysplasia. For these reasons, the patient was eligible for a hematopoietic stem cell allograft.\nIn the Hematology department, an asymptomatic hypoglycemia that persists despite glucose infusion was found. Laboratory tests showed type B LA with an elevated blood lactate of 14 mmol/L (normal range, 0.5–2 mmol/L) associated with a slightly decreased pH of 7.35 (normal range, 7.38–7.42). Serum bicarbonate was low at 13 mmol/L (normal range, 24–32 mmol/L) with normal renal function tests and an elevated anion gap of 28 mmol/L. Liver function tests were normal.\nThe patient was transferred to the Intensive Care unit. His temperature was 37.7°C, his blood pressure was normal at 149/82 mmHg, his pulse was 119 bpm, and the respiratory rate was 28 per minute without respiratory distress which indicated Kussmaul breathing. The patient did not present any signs of hypoperfusion (he had normal blood pressure, absence of mottling, normal capillary refilling test). The palpation of the abdomen was normal without diarrhea. In the absence of a type A LA etiology, and in the context of AML, we suspected WE. Chemotherapy by doxorubicin 60 mg/m2 and cytarabine 100 mg/m2 was initiated at day 1. A slight tumor lysis syndrome was observed without kidney injury. We noted no recurrence of hypoglycemia, a rapid normalization of pH, and a decrease in the blood lactate level: 12.1 mmol/L at day 2, 9.2 mmol/L at day 3, 2.6 mmol/L at day 5, and 1.6 mmol/L at day 7 (Figure ). We observed no further complications, allowing ICU discharge at day 8.\nUnfortunately, a relapse of AL occurred at day 43. This relapse was associated with a new hyperlactatemia (pH: 7.37 and blood lactate level: 12.8 mmol/L) and asymptomatic hypoglycemia related to a recurrence of WE (Figure ). Salvage chemotherapy with cytarabine 1,500 mg/m2 and gemtuzumab 3 mg/m2 was initiated, resulting in the disappearance of WE. However, the patient later developed septic shock as a complication of chemotherapy-induced aplasia and digestive infection. We suspected infectious colitis because of diarrhea and abdominal guarding. Unfortunately, the CT scan was not contributory and we could not perform a colonoscopy to confirm the diagnosis. Empirical broad-spectrum antibiotic therapy with imipenem, vancomycin, and gentamycin was administered. He eventually died in the Intensive Care unit in a state of multiple organ dysfunction due to the septic shock.","This 57-year-old man had a long-standing condition called myelodysplastic syndrome, which led to a drop in his white blood cell count. In November 2016, he developed signs of leukemia, and tests confirmed he had acute myeloid leukemia (AML). Despite this, he was eligible for a bone marrow transplant. During treatment, he experienced a recurrence of lactic acidosis, a dangerous buildup of lactic acid in his blood, which led to septic shock and ultimately his death."
709,"A 54 yr old man from Southwest of Iran (Yasuj) presented to the Emergency Ward with a 3-wk history of headache (continuous, throbbing, and general), fever, chills, weakness, anorexia, and weight loss. He also had a history of benign prostatic hyperplasia, gastroesophageal reflux disease, and hemorrhoid. Medications were tamsulosin, propranolol, rabeprazole, and cathartic syrup. His parents had no any congenital or infectious diseases.\nOn examination, the body temperature and blood pressure were 38 °C and 130/82 mm Hg, respectively. Abdominal examination revealed mild tenderness in right upper quadrant and moderate splenomegaly. All other examinations were normal.\nThe hemoglobin was 8.1 (gr/dl), the white blood cell count 1900, retic count 0.5%, and the platelet count 20000. The ESR was 56 (mm/h), alanine aminotransferase 84 (Iu/l), aspartate aminotransferase 67 (Iu/l), alkaline phosphatase 401 (Iu/l), albumin 3.4 (gr/dl), conjugated bilirubin 0.6 (mg/dl), ferritin 658.6 (mcg/dl), serum iron 23 (mcg/dl), total iron binding capacity (TIBC) 116 (mcg/dl). Other laboratory tests such as creatinine, blood sugar, partial thromboplastin time, prothrombin time, urinalysis, stool exam, sputum exam, wright test, 2ME, calcium, phosphorus, magnesium, and prostate-specific antigen were normal. Serologic tests for HBV, HCV, and HIV were negative.\nAbdominal ultrasonography revealed mild hepatomegaly and moderate splenomegaly. Doppler ultrasound of abdomen showed dilation of splenic veins such as superior mesenteric vein (15 mm) and portal vein (15 mm).\nA computerized tomography (CT) scan of brain revealed no abnormal finding. Axial fluid-attenuated inversion recovery MRI image (FLAIR) revealed an increase in signal intensity of central part of right side of pons (). Bone marrow aspiration and biopsy revealed macrophages with numerous Leishmania amastigotes (). Treatment with amphotericin B was initiated and the patient symptoms resolved completely. In second bone marrow exam, no amastigote was observed.\nFive months after initial presentation and treatment with amphotericin B our patient has no any neurological morbidity and disability.","This 54-year-old man came to the hospital with a three-week history of persistent headaches, fever, and weakness. Initial tests revealed low blood counts, high inflammation, and some liver abnormalities, along with enlarged spleen. Further investigation, including a bone marrow biopsy, showed he had a rare parasitic infection called Leishmania. Treatment with medication successfully cleared the infection, and he has been doing well for five months."
710,"A 78 yr old man from a rural area at the western of Iran referred to Razi Hospital Dermatology Clinic, Tehran for multiple ulcerative and exudative lesions on mid face, dorsal aspect of hands and the posterior aspect of heels (, ).\nInformed consent was taken from the patient.\nLesions initiated three years before with papules on the dorsal aspect of the hands then progressively enlarged above the upper lip, anterior portion of the nasal fossa, above the eyebrows and heels and became ulcerative.\nDuring the past 3 yr, the lesions of hands were so developed that destroyed the tendons and soft tissue of fifth finger in the right hand so led to amputation of this finger. There was no history of comorbid condition, drug consumption, systemic symptoms, weight loss, fever, lymphadenopathy, hepatosplenomegaly or any signs of systemic involvement in physical examination and laboratory survey.\nMultiple treatments in order to some heterogenic diagnosis such as pyoderma gangrenosum, sarcoidosis, and leishmaniasis were tried without any improvement in lesions.\nIn Razi Hospital Dermatology Clinic, initial Skin biopsy revealed necrotizing and palisading granulomatous tissue pattern that suggested infections etiology but the smear of lesions for fungal and mycobacteria and Leishmania was negative. In order to result of PPD test with 27 mm induration, anti-tuberculosis treatment including isoniazid, rifampin, ethambutol, and pyrazinamide was started.\nA month after initiating drugs for tuberculosis, the smear of leishmaniasis repeated that was positive this time, the second biopsy revealed pseudoepitheliomatous hyperplasia and infiltration of the dermis by mixed inflammatory cells and Leishman bodies compatible with leishmaniasis.\nRestriction fragment length polymorphism (RFLP) PCR was carried out on DNA extraction was carried out with QIAGEN Kit according to the manufacturer’s instruction using two primers, LITSR (5-GTG CAG GAT CAT TTT CCG ATG) and L5.8s: 5-TGA TAC CAC TTA TCG CAC TT was designed for LTS1–PCR and cutting with and HaeIII enzyme. Positive controls containing DNA of L. major, L. tropica and a negative control containing distilled water were included. The PCR–RFLP on specimens of lesions proved L. major as the pathogenic agent (, )().\nFrom the left side, the 2 bands show L. major, the second 2 bands show L. tropica, the 5th one is the specimens of lesions that compatibles with L. major and the last one show L. tropica.\nTreatment initiated with 3 gr per day of meglumine antimoniate (Glucantime®) until 1 wk followed by 4.5 gr per day for another week, in spite of good response to meglumine antimoniate after 2 wk of treatment we forced to discontinue of drug because of cardiac toxicity (, ).\nThe patient was informed about publishing his figures whit covering eyes.","This 78-year-old man from Iran has been struggling with unusual sores on his face, hands, and heels for the past three years. Doctors initially suspected different infections, but after several tests, they discovered he had leishmaniasis, a parasitic infection. Further testing confirmed the parasite causing the sores, and he started treatment with medication. Despite the treatment, the patient experienced cardiac toxicity and had to discontinue the medication."
711,"A 24-year-old male presented with photophobia since birth. No family history for colour vision defects or retinal dystrophies was reported. Myopia with an refractive error of − 5.50 D (right eye) and − 6.50 D (left eye) and astigmatism were found in the patient (III:2) at the age of 8 months along with nystagmus but devoid of strabismus. Glasses were given at the age of 1 year. Difficulties distinguishing colours were noticed by his parents at the age of 3 years. Achromatopsia was the first suspected diagnosis. At the age of 4 years occlusion therapy alternating in both eyes for 2 months was attempted to treat amblyopia but was unsuccessfull. A best-corrected visual acuity of 20/200 was measured with Snellen charts at the age of 6 years. No brain injuries were detected by magnetic resonance imaging. Visual evoked potential flash and B-scan ultrasonography performed normal for both eyes. At the age of 11 years a visual acuity of 20/250 was measured. At the latest exam at the age of 24 years, full-field light- and dark-adapted electroretinogram (ERG) recordings were performed according to the International Society for Clinical Electrophysiology of Vision (ISCEV) standard protocol []. Subnormal amplitudes under scotopic conditions and extinct responses under photopic conditions were observed in both eyes of the patient (III:2) in comparison to normal controls (Fig. ). A visual acuity of 20/400 was measured for both eyes with a myopic correction of − 12.00 D (right eye) and − 11.50 D (left eye). Anterior segment, pupillary reflexes and intraocular pressure revealed no abnormalities. Eye fundus examination revealed normal retinal vessels, optic nerve heads showing tilted optic discs with myopic conus and the maculae had elapsed reflex without waxy reflex (Fig. ). Spectral domain optical coherence tomography (SD-OCT) performed with Spectralis OCT (Heidelberg Engineering) showed normal retinal architecture with thinned photoreceptor layer (Fig. ). Colour vision test evaluated with the Farnsworth D-15 Colour Test revealed protan-deutan confusion errors (Fig. ).\nMutation screening of OPN1LW/OPN1MW gene cluster in the patient (III:2) was performed as previously described []. Genotyping PCRs with genomic DNA from the patient (III:2, see Fig. for a pedigree of the genetically investigated family members) revealed absence of the LCR and promoter regions of both OPN1LW and OPN1MW genes – indicative for a large deletion – but presence of the 3′ parts of the OPN1MW gene, namely exons 4, 5 and 6. Upon fine sequence tag site content mapping, the deletion was finally bridged with a PCR amplicon performed with primers BCM#27_F (5′- TCGACCCAGAATTAACCTCTCT -3′) and BCM#27BPR (5’-TCTAAAAATGGACAAGGATTAACCA -3′) which was sequenced with Sanger to determine the exact breakpoints in patient III:2 (Fig. and Fig. ). The deletion, NC_000023.11:g.154,118,184_154,191,311del, encompasses 73,128 bp with the centromeric breakpoint located in the intergenic region between MECP2 and OPN1LW and the telomeric breakpoint within intron 3 of OPN1MW (Fig. ).\nA sequence alignment of the breakpoint junction sequence in the patient (III:2) with the corresponding non-mutant sequence sections from his grandfather (I:1) revealed an overlapping stretch of 13 bp between the centromeric and the telomeric breakpoint sequences (Fig. ) shared by two Alu elements. The sequence remnants embedded within the deletion breakpoints resembled the junction of two Alu elements; (1) a fossil right Alu momomer (FRAM) element at the centromeric breakpoint of the deletion, and (2) an AluJo element in intron 3 of OPN1MW at the telomeric breakpoint of the deletion (Fig. ). Microsatellite marker analysis revealed that the X-chromosome present in the patient (III:2) had been transmitted from his maternal grandfather (I:1, Fig. ). Segregation analysis performed by means of breakpoint PCR amplification showed that neither the patient’s grandfather (I:1) nor the mother (II:1) carry the deletion (Fig. ). The CARE guidelines were followed in reporting this case.","This 24-year-old man has had difficulty seeing colors since he was a baby, and his vision has continued to decline. He was diagnosed with a rare condition called achromatopsia, which affects the ability to distinguish colors. Tests revealed abnormalities in his retinal cells, and genetic testing identified a large deletion in two genes that are important for vision. This deletion was inherited from his grandfather. Despite these findings, his eye exam showed no other abnormalities, and his vision remains significantly impaired."
712,"A 52-year-old Japanese man with lower abdominal pain underwent lower endoscopy, revealing a type 2 lesion with the entire circumference raised in the rectosigmoid colon. He was diagnosed with rectosigmoid colon cancer and underwent endoscopic stent placement as a bridge to surgery for large bowel obstruction. He was found to have multiple lung metastases and a horseshoe kidney on CT scan (Fig. ). 3D-CT angiography showed an aberrant renal artery at the isthmus from 3 cm under the inferior mesenteric artery (IMA) branch of the aorta (Fig. ). Laparoscopic anterior rectal resection was performed with a five-port conventional technique in which sigmoid colon and upper rectum were mobilized via a medial approach. During the operation, the IMA, left ureter, left gonadal vessels, and hypogastric nerve plexus were identified and preserved (Fig. , ). The root of aberrant renal artery was not visualized. The root of the IMA was located considerably cephalad to the renal isthmus, and the left branch of aberrant renal artery that was close to IMA was detected and preserved (Fig. ). The specimen was removed through a small laparotomy wound, and intraperitoneal reconstruction was performed according to the standard double stapling technique. The patient recovered uneventfully and was discharged on postoperative day 16. Pathological examination demonstrated no metastasis of the lymph node.\nHorseshoe kidney is rare, with an incidence of 0.25%, and the incidence is higher in males than in females at a ratio of 2:1. Horseshoe kidneys are fused at the lower pole in 95% of cases, and the isthmus is composed of fibrous tissue alone or contains parenchyma. The horseshoe kidney is located at a level lower than the normal kidneys because elevation of the kidneys interferes with the isthmus at the origin of the IMA.\nA literature search revealed 23 patients who underwent surgery for colon cancer with concomitant horseshoe kidney including our patient, from 1983 to 2017 [–]. There were 14 men and 9 women, and the affected region was the sigmoid colon in 12, rectum in 8, descending colon in 2, and ascending colon in 1. Surgery was performed under laparoscopy in 20 patients. The positions of the anatomical structures necessary for surgery are summarized in Table .\nRegarding the ureteral distribution, it was present on the ventral side of the horseshoe kidney, i.e., almost the same as that in patients with normal kidneys, in all cases in which the distribution was described. The ureters were present on the ventral side of the horseshoe kidney in many reported cases, suggesting that it was conserved on dissection similarly in patients with normal kidneys. However, there were some cases where the ureters were present on the dorsal side of the isthmus of the horseshoe kidney, where a ureter other than the bilateral ureters independently came out from the isthmus of the horseshoe kidney, where two ureters each came out from the bilateral sides, or where the ureters ectopically opened in regions other than the urinary bladder [], suggesting the necessity of preoperative imaging diagnosis and careful surgery.\nIn the cases described in the literature, the distribution of the gonadal artery and vein were usually normal. However, variations have been reported, such as the presence of two left ovarian veins and branching of the testicular artery from the renal artery and renal sinus [], again suggesting the necessity of preoperative imaging diagnosis and careful surgery.\nThe bilateral lumbar splanchnic and superior hypogastric nerves were distributed on the ventral side of the horseshoe kidney in six patients reported, including in our patient, but superior hypogastric nerves distributed from the dorsal side of the horseshoe kidney into the pelvis were noted in one patient. Although there were only a few reports on the distribution of the bilateral splanchnic and superior hypogastric nerves in patients with horseshoe kidney, the second lumbar splanchnic nerves were present on the ventral side of the kidneys and the third lumbar splanchnic nerves were present on the dorsal side of the kidneys, and both nerves formed superior hypogastric plexuses in a report of an autopsied patient [].\nArterial anomalies are reported in more than 70% of patients with horseshoe kidney, due to remain of branches from the common iliac artery, median sacral artery, and IMA. Excess renal arteries were observed in 16 of 23 patients, including patients with unclear details. Kölln classified the vascular supply in the horseshoe kidney into the following three types: (1) direct single branching from the aorta on the bilateral sides (15%), (2) several blood vessels on the bilateral sides and in the isthmus all directly branching from the aorta (65%), and (3) several blood vessels on the bilateral sides and in the isthmus that branch from common iliac artery, internal iliac artery, inferior mesenteric artery, or median sacral artery (20%) []. The second pattern was noted in our patient. 3D-CT angiography is useful in these cases [, ]. As the resolution of CT increased, the positional relationship among the IMA, kidneys, and excess renal arteries may be sufficiently identified using 3D-CT angiography.\nIn our patient, the IMA was easily detected during usual mobilization via median approach. We usually dissected the layers preserving the plexus, and the left branch of aberrant renal artery that was close to IMA was detected and preserved uneventfully, because the locations of the excess renal arteries and IMA were sufficiently identified using 3D-CT angiography. Injury to excess renal arteries by laparoscopic colectomy may be avoided by retaining accurate dissection layers, because they are present in the retroperitoneum. Even when an accurate dissection layer cannot be retained, laparoscopic colectomy may be safely performed by sufficiently evaluating images and confirming the anatomical structures before surgery.","The patient recently had surgery to remove colon cancer and discovered he has a rare condition called a horseshoe kidney, where his two kidneys are fused together. During the surgery, doctors carefully preserved important blood vessels and nerves, including the main artery that supplies blood to the legs. The surgery was successful, and the patient is recovering well, with no signs of cancer spread to the lymph nodes. The doctors used 3D imaging to help them navigate the complex anatomy during the procedure."
713,"A 69-year-old man developed a sudden epigastric pain. He was presented at this hospital as an emergency outpatient. Six years earlier, he underwent laryngoesophagopharyngectomy, bilateral lymph node dissection for hypopharyngeal cancer, and esophageal reconstruction with a free jejunum flap. On physical examination, the abdomen was flat and soft with tenderness in the epigastric region, but no sign of peritoneal irritation. Blood biochemistry findings revealed elevated values: creatinine, 1.16 mg/dl; lactate dehydrogenase, 364 U/l; and creatine phosphokinase, 622 U/l.\nAbdominal contrast computed tomography (CT) revealed twisted mesentery with the small intestine around the point of torsion (whirl sign) and the superior mesenteric artery as the axis. Contrast enhancement was weakened in the same area of the small bowel (Fig. ). Given this information, we suspected small bowel volvulus and performed emergency surgery on the same day.\nA 5-mm camera port was placed in the umbilicus and 5-mm ports in the lower and right lower abdomen. During laparoscopic examination, the upper jejunum adhered to the small bowel close to the terminal ileum with overlapping of the small bowel. The entire part from the upper jejunum to the terminal ileum was twisted clockwise with the superior mesenteric artery and vein as the axes and the adhesion site as the starting point. There were areas of poor color enhancement throughout the twisted section of the small bowel (Fig. ). We laparoscopically separated the adhesion between different sections of the intestinal tract and traced the bowel from the small bowel in the region of the ligament of Treitz toward the anus to confirm the absence of adhesions or torsion up to the terminal ileum. The color of the small bowel improved; hence, the surgery was completed without resecting any part of the intestine.\nPostoperatively, the patient made good postoperative recovery, resumed oral intake on day 2, and was discharged on day 5 after surgery. No recurrence has been reported 1 year postoperatively.","69-year-old man experienced sudden stomach pain and was rushed to the hospital. He had previously undergone surgery to remove cancer in his throat and esophagus, and now he had a twisted part of his small intestine. Tests showed high levels of certain enzymes, and a CT scan confirmed the diagnosis of small bowel volvulus. The doctors successfully repaired the twisted section of his intestine with laparoscopic surgery, and he made a good recovery and was discharged home after five days."
714,"A 7-month-old male, neutered Maine Coon cat was presented to the Small Animal Teaching Hospital at the University of Bern with acute neurological signs consistent with unilateral otitis media/interna. Six weeks earlier an inflammatory aural polyp had been removed by traction and flushing of the ear canal. The cat had fully recovered after three weeks of treatment with oral and topical glucocorticoids and topical ofloxacin (Floxal, Bausch & Lomb Swiss AG). Otoscopic and cytologic examinations revealed brown-colored fluid in the external canal with numerous extra- and intracellular rod-shaped bacteria and neutrophils. A deep ear swab was submitted for culture and subsequent antimicrobial susceptibility testing.\nThe ear swab was cultured on sheep blood agar at 37 °C for 2 days, yielding a pure culture of small white colonies (strain number 17KM38). Gram staining showed Gram-positive, polymorphic rods and the bacteria were catalase positive. Thus, the bacteria were classified as belonging to the genus Corynebacterium, however species identification was not possible with either Maldi-Tof MS (MALDI Biotyper, Bruker using the in-house database and MBT 6903 MSP Library, Bruker) or VITEK® 2 Compact (Biomérieux) (cards GP and CBC). Therefore, the 16S rRNA gene was amplified and Sanger sequenced using universal primers []. Sequence analysis and sequence comparison using the BLAST program (NCBI, ‘rRNA_typestrains/prokaryotic_16S_ribosomal_RNA’ database) revealed 98.6% identity to Corynebacterium variabile (NR_025314.1), 98.0% to Corynebacterium terpenotabidum (NR_121699.1) and 97.8% to Corynebacterium glyciniphilum (NR_121782.1), thus the strain 17KM38 could not be assigned to any species present in the database. However, the 16S rRNA sequence showed a 100% identity with the whole genome shotgun sequence of the recently described Corynebacterium provencense SN15 (GenBank accession no.: NZ_LT160593.1) isolated from faeces of an obesity patient [].\nIn order to select the appropriate antimicrobial therapy, the isolate was tested for antimicrobial resistance using broth microdilution (Sensititre EUST, Thermo Fisher Scientific) initially following in-house guidelines. Minimum inhibitory concentration (MIC) testing was however repeated according to the most recent EUCAST (European Committee on Antimicrobial Susceptibility Testing) guidelines [] using Streptococcus pneumoniae ATCC 49619 as a quality control. Briefly, the bacteria were grown on sheep blood agar at 37 °C for 24 h. They were then suspended in Mueller-Hinton broth with 5% lysed horse blood (Thermo Fisher Scientific) and 20 mg/l β-NAD to a concentration of 5 × 105 CFU/ml and used for plate inoculation. Our strain tested resistant to clindamycin, penicillin and ciprofloxacin but susceptible to tetracycline, gentamicin and vancomycin. In addition, high MIC values were found for cefoxitin (8 mg/l), mupirocin (≥256 mg/l) and trimethoprim (≥32 mg/l) while the MIC for chloramphenicol was low (≤4 mg/l) (Table ).\nTo better characterize the strain, whole genome sequencing was performed using the PacBio method. The strain was grown on sheep blood agar at 37 °C for 24 h and the harvested cells were used to extract gDNA according to a protocol published earlier []. Sequencing was performed by the Lausanne Genomic Technologies Facility (University of Lausanne, Switzerland). The genome was assembled from PacBio reads using Canu 1.4 []. A single contig was built after assembly of the obtained reads, and circularized using amos 3.1.0 []. Subsequently the genome was annotated using Prokka 1.12 [] and submitted to GenBank (Accession No. CP024988). The genome of 17KM38 has a size of 3.11 Mb and 2682 coding sequences were detected. Of these, 862 out of 2682 were annotated as hypothetical proteins. In order to confirm that 17KM38 belongs to the same species as SN15 and is indeed distinct from its closest relatives, average nucleotide identity (ANI) was calculated according to Goris et al. [] using an online tool (ANI calculator) developed by Rodriguez-R and Konstantinidis [] with default settings. The ANI of 17KM38 was found to be 81.5% with C. variabile (NC_015859.1), 81.0% with C. terpenotabidum (NC_021663.1), 79.1% with C. glyciniphilum (CP006842.1) and 99.1% with SN15 (FIZC01). Since the recommended cutoff point for species delineation is 95% ANI [], these results confirm that SN15 [] and 17KM38 indeed belong to the same species which is distinct from related species. Furthermore, a phylogenetic tree was constructed from the theoretical proteome according to Qi et al. [] applying the online tool CVTree [] (Fig. ).\nThe genome of 17KM38 was compared to the genome of the strain SN15 using mauve []. Gap regions were extracted and annotated resulting in 280 coding sequences (CDS), 113 of which could be assigned a putative function (Additional file ). Interestingly, 12 CDS unique to 17KM38 are related to iron acquisition (Fig. ), which is important for bacterial survival in the host and thus for virulence []. Most interesting is an approximately 17kbp region containing nine genes for siderophore synthesis and transport organized in two presumptive operons (Fig. ) []. Genes dhbBCEF are related to the corresponding genes in Bacillus subtilis (between 36 and 52% identity in blastx) where they form the biosynthetic pathway for the catecholic siderophore bacillibactin []. The whole gene cluster has similarity to the enterobactin gene cluster in E. coli which also includes fep genes and the fes gene []. A gene corresponding to dhbA/entA is missing from this cluster in 17KM38, but is located elsewhere on the genome (1,026,704 - > 1,027,528). C. variabile, the closest relative to C. provencense, also possesses a repertoire of iron acquisition genes, since its habitat is the iron-restricted environment found in cheese []. Interestingly the genome of C. variabile also encodes a pathway similar to that for bacillibactin. However, the dhbBCEF genes in 17KM38 are no closer related to those in C. variabile than those in B. subtilis.\nIn terms of virulence factors 17KM38 encodes a candidate adhesin (Csp1_16740) related to (82% query cover, 51% identity in blastx) a cell wall-associated hydrolase of C. resistens (encoded by cwlH) []. The exact function of this protein is unknown, however knockout of the homologous gene DIP1621 in C. diphtheriae led to reduced adherence to epithelial cells []. This gene is also present in strain SN15.\nAs mentioned above, 17KM38 showed resistance to several antibiotics. Quinolone resistance in corynebacteria has been described to rely on mutations in the quinolone resistance determining region (QRDR) of gyrA leading to changes in the SAIYD (aa 87–91 in C. glutamicum) motif of susceptible strains []. Mutations of Ser-87 to Arg in C. amycolatum and to Val in C. striatum have been shown to confer quinolone resistance []. 17KM38 showed a mutation in this region changing Ser to Ala, which could explain the quinolone resistance. A mechanism for resistance to ß-lactams in corynebacteria has been demonstrated for C. jeikeium where acquisition of the gene pbp2C encoding the low-affinity class B penicillin binding protein 2C was shown to confer resistance []. 17KM38 has four genes encoding penicillin binding proteins one of which is related to pbp2C (Csp1_14570, 96% query cover, 44% identity), however, if this gene is actually related to penicillin resistance remains to be shown.\nFollowing sensitivity testing results, the cat was treated with oral chloramphenicol palmitate solution (Cloropal, Dr. E. Gräub AG) 20 mg/kg twice daily for three weeks and an ear cleaner containing chlorhexidine digluconate (Otodine®, Ufamed AG) twice daily for two weeks. Re-examination after three weeks of therapy revealed that the head tilt was much improved and the cat was no longer ataxic and cytology of the ear canal did not show any bacteria or neutrophils.","The cat was diagnosed with an ear infection caused by bacteria, and a small polyp had been removed previously. Tests revealed the bacteria were resistant to several antibiotics, but the cat responded well to a new medication. After three weeks of treatment, the ear infection cleared up, and the cat’s head tilt improved significantly."
715,"A 70-year-old man underwent an endoscopic examination owing to epigastric pain and was diagnosed with esophageal cancer. The endoscopic examination revealed an irregular mucosa in the lower esophagus, and biopsies confirmed squamous cell carcinoma. Contrast-enhanced CT did not depict the esophageal lesion but showed enlarged lymph nodes in the tracheal bifurcation and bilateral hilum of the lung. FDG-PET/CT revealed abnormal accumulation in the main tumor in the lower esophagus (maximum standardized uptake value [SUV max]: 4.06) and higher accumulation in the hilar-mediastinal lymph nodes (SUV max: 15.0) and enlarged mediastinum lymph nodes (SUV max: 6.94) (Fig. ). The primary lesion of the esophagus was staged T1; nevertheless, it was still difficult to rule out metastasis in the lymph nodes. We selected chemotherapy as the first-line treatment. The patient was administered 2 cycles of 140 mg cisplatin and 1400 mg 5-fluorouracil over 2 months. In each cycle, 9.9 mg dexamethasone was administered to prevent side effects of chemotherapy. Subsequently, we observed a disappearance of the FDG uptake in the primary lesion, and a slightly reduced FDG uptake in the mediastinal and bilateral hilar lymph nodes (Fig. ). These non-identical responses to chemotherapy did not indicate cancer metastasis, but most likely a sarcoid-like reaction of the lymph nodes associated with squamous cell carcinoma of the esophagus. Therefore, the patient underwent video-assisted thoracoscopic surgery esophagectomy (VATSE) with gastric tube reconstruction via the retrosternal route. The pathological diagnosis was moderately to poorly differentiated squamous cell carcinoma of the lower thoracic esophagus. The resected lymph nodes demonstrated no tumor metastasis. However, some lymph nodes (#8a, #106RecR, #107, #108, #109) showed granulomatous reactions, histiocytes, multinucleated giant cells, and scar-like fibrosis (Fig. ), suggesting the presence of sarcoidosis or sarcoid-like reactions. In accordance with the Union for International Cancer Control (UICC) TNM staging system (7th edition), the tumor was classified as pT1N0M0, pStage IA. The patient was discharged 49 days after surgery. FDG-PET/CT performed 15 months after surgery showed bilateral FDG accumulation in the hilar lymph nodes without tumor recurrence.","A 70-year-old man was diagnosed with esophageal cancer after an examination revealed a growth in his esophagus. Imaging tests showed the cancer had spread to nearby lymph nodes in his chest. He received chemotherapy, which helped shrink the tumor, and then underwent surgery to remove the tumor and some of the affected lymph nodes. The surgery was successful, and follow-up scans showed no signs of the cancer returning."
716,"A 72-year-old woman with a past medical history of sarcoidosis underwent an endoscopic examination owing to dysphagia. The endoscopic examination revealed a circumferential tumor with ulceration in the cervical esophagus. Biopsies obtained during the endoscopy indicated squamous cell carcinoma. Contrast-enhanced CT showed extensive tumor growth with suspected tracheal invasion and enlarged lymph nodes extending from the cervical region to the upper mediastinum. FDG-PET/CT revealed abnormal FDG accumulation in the primary lesion (SUV max: 23.1) and lymph nodes (SUV max: 5.45) from the cervical to upper mediastinal region (Fig. ). However, it was difficult to determine whether the multiple lymphadenopathy was benign or metastatic because of her past medical history of sarcoidosis. Therefore, and also for the purpose of preserving the larynx, we initiated definitive chemoradiotherapy. The patient was administered 2 cycles of 45 mg cisplatin and 700 mg 5-fluorouracil with 60 Gy/30 fr radiation therapy over 2 months. In each cycle, 8 mg dexamethasone was administered to prevent side effects of chemotherapy. After completing the chemoradiotherapy, we observed complete disappearance of FDG uptake in the primary cancer in the esophagus, and only a slight reduction in FDG uptake in the mediastinal lymph nodes (SUV max: 3.26; Fig. ), which indicated that the lymph nodes were affected by sarcoidosis. The primary lesion of the esophagus relapsed 3 months later. Then, the patient underwent thoracoscopic and laparoscopic total laryngopharyngoesophagectomy with gastric tube reconstruction via the posterior mediastinal route. The pathological diagnosis was moderately differentiated squamous cell carcinoma in the cervical esophagus. The resected lymph nodes demonstrated no tumor metastasis. However, some lymph nodes showed granulomatous reactions and contained several small epithelioid cell granulomas (Fig. ), suggesting the presence of sarcoidosis. The final stage was determined as pT2N0M0, pStage IB (UICC 7th). The patient was discharged 27 days after surgery. Contrast-enhanced CT performed 6 months after surgery showed no tumor recurrence. However, the patient died of myocardial infarction 1 year after surgery.","This 72-year-old woman was referred to a specialist because she was having trouble swallowing. An examination revealed a tumor in her esophagus, which was confirmed by a biopsy to be squamous cell carcinoma. Imaging showed the tumor had spread to nearby lymph nodes, but it was difficult to determine if it was due to the patient’s history of sarcoidosis. To treat the tumor, she received chemotherapy and radiation therapy, which successfully shrank the tumor in her esophagus. Unfortunately, the tumor later returned, and she underwent a major surgery to remove it, but she passed away a year later due to a heart problem."
717,"A 75-year-old woman was admitted to our hospital complaining of itching around her anus. She had a history of sigmoidectomy for diverticulitis 6 years prior and a past history of Sjögren’s syndrome. She had noted a reddish skin lesion around her anus over the past several years. She reported no change in bowel habits, no gastrointestinal symptoms, no weight loss, and no family history of malignancy.\nPhysical examination revealed an erythematous, inflamed skin lesion in the perianal region (Fig. ), but a normal vagina and rectum. Neither colposcopy, cystoscopy, nor colonoscopy showed evidence of a visible lesion or any abnormality of the cervix, bladder, or rectum (Fig. a, b). Computed tomography and magnetic resonance imaging showed no evidence of malignancy in the genitourinary or gastrointestinal tracts. Histopathological examination of biopsy specimens showed many Paget’s cells within intraepithelial lesions of the perianal skin but no malignant cells in the rectal or vaginal mucosa. Therefore, primary extramammary Paget’s disease (EMPD) of the anogenital region was suspected. We performed anal-preserving wide local excision deep to the subcutaneous fat with 1-cm negative margin from the positive sites confirmed by frozen section examination and mucosal resection of the anal canal that was extended 1 cm proximal to the dentate line of the anal canal. Reconstruction was performed using a bilobed gluteal fold flap (Fig. a, b).\nHistopathological examination of the resected specimen showed Paget’s cells, characterized by clear cytoplasm and large pleomorphic nuclei, within the epidermis. Immunohistochemical analysis revealed that the Paget’s cells were positive for cytokeratin (CK) 7, CK20, and caudal-related homeobox gene nuclear transcription factor (CDX) 2 and negative for gross cystic disease fluid protein (GCDFP) 15 (Fig. a–e). These data suggested that her perianal skin lesion was secondary EMPD arising from the rectum, although there was no lesion found in the rectum. Additional histopathological examination of the resected specimen revealed well-differentiated adenocarcinoma in an anal gland, continuous with the Paget’s cells in the anoderm, but limited in the basement membrane without a desmoplastic change (Figs. a, b and a–e). Immunohistochemical staining of the resected specimen revealed secondary EMPD due to adenocarcinoma arising from the anal gland.\nBecause of the possibility of residual adenocarcinoma in the anal gland, possibly extending to the sphincter, we performed a radical laparoscopy-assisted abdominoperineal resection. Any reconstructive plastic surgery was not needed. Histopathological examination revealed no residual cancer cells in the resected specimen and no lymph node metastasis. The final diagnosis was anal gland adenocarcinoma in situ with pagetoid spread in the perianal skin.","A 75-year-old woman was admitted because of persistent itching around her anus, which she’d noticed for several years. After a series of tests, including scans and biopsies, doctors discovered she had a rare condition called extramammary Paget’s disease, where cancer cells spread to her skin around her anus. Further examination revealed that the Paget’s disease was actually linked to a more serious adenocarcinoma in her anal glands. To treat this, she underwent a complex surgery to remove the affected area and surrounding tissue, and thankfully, no further cancer was found."
718,"A 67-year-old man was admitted to our hospital because of liver dysfunction during a screening examination. Enhanced abdominal computed tomography (CT) revealed a hypervascular mass of 35 mm in diameter in the descending portion of the duodenum (Fig. ), and the left three sections of the liver were occupied by multiple cystic tumors with contrast enhancement of the cystic wall, 13 cm in diameter (Fig. ). A duodenal tumor was identified on gastrointestinal endoscopy (Fig. ), and a biopsy revealed a NET. The serum levels of insulin, gastrin, and glucagon were within normal ranges. CT did not initially reveal evidence of pancreatic invasion between the tumor and the pancreas; however, irregularities of the duodenal wall and swelling of the lymph nodes around the pancreatic parenchyma were observed. Thus, the patient was diagnosed with non-functional duodenal NET with multiple liver metastases, T2N1M1 stage IV (UICC 8th). In addition, CT revealed the anatomical variation of the CHA, which branched from the SMA and ran fully through the head of the pancreatic parenchyma (Fig. , Additional file Figure S1). The CHA branches into the left hepatic artery (LHA), the middle hepatic artery (MHA), and the right hepatic artery (RHA) (Fig. a, b). Furthermore, a developed gastric arterial arcade, 4 mm in diameter, was found between the left gastric artery (LGA) and the right gastric artery (RGA). The RGA was branched from a distal portion at a distance of 10 mm from the root of the LHA (Fig. ). Incidentally, we did not observe stenosis of the celiac axis due to compression by the median arcuate ligament. We planned PD and left trisectionectomy with caudate lobectomy combined resection of the tp-CHA with the preservation of the gastric arterial arcade in order to maintain arterial flow of the remnant liver, preserving the route of the celiac artery to the right posterior hepatic artery (RPHA) via the gastric arterial arcade from the LGA to the RGA, LHA, and RHA. If the hepatic arterial flow could not be maintained by this route, the preservation of the tp-CHA by separating from pancreatic parenchyma or arterial reconstruction using radial artery graft between CHA and RHA was planned. Four weeks after percutaneous transhepatic portal embolization, surgery was carried out.\nAfter laparotomy, the gastric arterial arcade was exposed and encircled, and the LHA, RHA, and proper hepatic artery (PHA) were encircled (Fig. ). The LHA was divided at the distal side of the origin of the RGA. The MHA and the right anterior hepatic artery (RAHA) were also divided. The left portal branch and the right anterior portal branch were divided (Fig. ). The liver was transected, and the left hepatic duct and right anterior hepatic duct were divided. The left trisections and caudate lobe were anatomically resected. After clamping the PHA, the hepatic arterial signals of the RPHA via the gastric arterial arcade were confirmed by intraoperative Doppler ultrasonography (Fig. ). After trisectionectomy and caudate lobectomy, PD was performed. The pancreatic head was dissected from the SMA after the upper jejunum was divided. The pancreas was divided in front of the SMV. Finally, the specimen was only connected by the tp-CHA and the common hepatic duct (CHD) (Fig. ). The hepatic arterial signals of the RPHA was maintained after clamping the PHA. The PHA and the origin of CHA were divided, and the tp-CHA was taken out with the pancreatic head (Fig. ). The CHD was divided, and the specimen was removed (Fig. ). Reconstruction was performed via modified Child’s method. The operative time was 1072 min and the intraoperative blood loss was 3052 ml, and red blood cell transfusion was performed (1680 ml).\nPostoperatively, the patient developed pancreatic fistula (Clavien-Dindo IIIa) and biliary leak (Clavien-Dindo IIIa), and these complications were treated conservatively. There were no signs of hepatic ischemia. The patient was discharged on postoperative day 39. The pathological diagnosis was duodenal neuroendocrine tumor G2 with multiple liver metastases. The Ki-67 labeling index was < 20%, and staining for chromogranin A and synaptophysin were positive. There was no evidence of invasion of the pancreatic parenchyma; however, the duodenal tumor was confined to the MP layer, and one of the 25 examined lymph nodes was positive, and moderate lymphovascular invasion was observed. The final diagnosis was pMP, med, INFa, ly1, v2, pPM0, pDM0, and pEM0. The patient has shown no recurrence in the 22 months since the operation. Enhanced abdominal CT at 4 months after surgery revealed the blood flow of the RPHA via the gastric arcade (Fig. ).\nOver the years, several authors have described variations in the hepatic arterial anatomy; a CHA arising from the SMA—called the hepatomesenteric type—is a rare clinical entity. Yang et al. and Hiatt et al. reported that this condition was observed in only 31 of 1324 patients and 15 of 1000 patients, respectively [, ]. A CHA passing through the pancreatic head parenchyma, tp-CHA, is even rarer; Yang et al. [] reported that among 31 patients with the hepatomesenteric type, only 3 had this condition.\nWhen PD is scheduled in such patients with tp-CHA, it is important to maintain the arterial supply to the liver. Surgeons should preoperatively determine whether to preserve or perform combined resection of the tp-CHA. Tp-CHA preservation was selected in several previous reports [, , ]. This surgical procedure is technically feasible; however, there is a risk of a positive surgical margin or insufficient lymph node dissection and a tendency for increased intraoperative blood loss during the separation of the pancreatic parenchyma. If the tp-CHA is resected, reconstruction is usually necessary in order to maintain the hepatic arterial flow. Previous reports [, , ] have described successful arterial reconstruction after CHA resection during PD; however, such procedures are associated with an increased risk of thromboembolism, which can lead to a fatal outcome, especially in HPD []. In contrast, when collateral circulation develops, surgeons can perform combined resection of the tp-CHA, preserving the collateral circulation without arterial reconstruction. Several reports have recommended preoperative embolization of CHA in order to maintain the hepatic arterial flow through enlarged collateral arteries []. Although preoperative embolization can increase the liver arterial flow through collateral arteries, it is not routinely recommended because of the risk of complications, which includes the migration of embolic material [, ].\nA developed gastric arcade or pancreaticoduodenal arcade is frequently seen in patients with the stenosis of the CHA due to factors such as compression by the median arcuate ligament []. There are only a few cases in which the hepatomesenteric trunk and the tp-CHA and the association between the tp-CHA and the development of a gastric arterial arcade have not been reported. On the other hand, Miyamoto et al. reported the case of a patient with pancreatic head cancer with a CHA arising from the SMA who underwent radical PD combined with the resection of the CHA, in which the hepatic arterial flow was maintained via the gastric arterial arcade []. In this report, the patient did not have a developed gastric arterial arcade; however, the hepatic arterial flow via the gastric arterial arcade was sufficient and hepatic ischemia was not detected after the operation. Considering this case, even if the patients with tp-CHA do not have a developed gastric arterial arcade, surgeons may be able to preserve hepatic arterial flow via the gastric arterial arcade alone. If the hepatic arterial flow via the gastric arterial arcade alone is adequate after clamping the PHA, the combined resection of the tp-CHA can be considered, even if the gastric arcade is not developed before surgery. In cases in which the hepatic arterial flow is not adequate, the preservation of the tp-CHA or arterial reconstruction should be considered.\nWhen performing HPD, a PD-first procedure before hepatectomy is generally performed, as this approach is anatomically rational []. However, in the present case, performing hepatectomy after PD carried a risk of the arterial supply to the liver being reduced during hepatectomy. Had we chosen a PD-first procedure and the hepatic arterial flow not been maintained after CHA resection, it would have been necessary to perform arterial reconstruction before liver transection. This method is associated with a risk of injury to the reconstructed artery and thrombosis during liver transection. Given the above, we opted to perform hepatectomy before PD in our patient with a tp-CHA undergoing HPD.\nIn the procedure for separating the tp-CHA from the pancreatic parenchyma entirely, the surgeon should be concerned about the increasing rate of hemorrhage, surgery time, and the risk of injury to the tp-CHA. The surgical reconstruction of the hepatic artery when performing HPD is also associated with a high degree of risk. The association between tp-CHA and gastric arterial arcade was recognized on preoperative CT scans; the development of this collateral circulation may have the potential to prevent ischemia-related liver complications. From these points of view, the preoperative identification of the developed arcade of the gastric arteries helps in planning an appropriate operative procedure, and this procedure seems to be a viable and simple option. To our knowledge, this is the first report of PD combined with resection of a tp-CHA without preoperative embolization. Furthermore, this is also the first report of HPD for a patient with a tp-CHA. The preoperative identification of the developed arcade of the gastric arteries helps in planning the appropriate operative procedure when PD is scheduled for patients with a tp-CHA.","This 67-year-old man was admitted to the hospital after tests revealed a tumor in his small intestine and several tumors in his liver. Doctors found that the tumor was spreading and that his blood flow was unusual, with a unique connection between his stomach and pancreas. To treat the tumor, he underwent a complex surgery to remove the tumor and part of his liver, while carefully preserving the blood supply to the remaining liver. After the surgery, he developed complications, but he is now recovering and has been discharged home."
719,"A 39-year-old Japanese man presented with a 3-month history of numbness on the left side of his face. His symptoms had gradually progressed and had become painful in the month before the initial visit. He also complained that sometimes he could not chew on the left side. An examination revealed decreased sensation over the distribution of the left trigeminal nerve that did not respond to nonsteroidal anti-inflammatory drugs or muscle relaxants and was only slightly responsive to carbamazepine. His symptoms were associated with dyskinesia of the left masticatory muscles but there was no clicking sound. His facial expression was symmetrical at rest.\nHis past medical history was significant for acute gastritis, duodenal ulcer, and depression, for which brotizolam, flunitrazepam, and paroxetine had been prescribed, respectively. He was reticent and had difficulty communicating his feelings and wishes, which appeared to be related to his history of depression. Panoramic radiography revealed no specific findings relevant to his symptoms (Fig. ) but did identify slight restriction of movement of the temporomandibular joint on the left (Fig. ). MRI of the temporomandibular joint region was inconclusive for temporomandibular disorder and his symptoms were nonspecific for trigeminal neuralgia. Therefore, we extended the scanning range into the brain region and found a tumor measuring 10 mm in diameter and a homogeneously high signal intensity on axial T1-weighted images compared with gray matter (Fig. ) and low signal on axial T2-weighted images (Fig. ) in Meckel’s cave. The tumor appeared to be exerting pressure on his trigeminal nerve. He was referred to the neurosurgery department where unenhanced computed tomography (CT) images demonstrated a localized well-defined mass lesion in Meckel’s cave, which was homogeneously hyperdense compared with gray matter. No calcification was present (Fig. ).\nEn bloc excision was subsequently performed. Immunohistochemistry was positive for melanocytic features of Melan A (MART1; melanoma antigen recognized by T cells-1), human melanoma black-45, vimentin, and S-100 protein and negative for cytokeratin AE1/AE3 and glia fibrillary acidic protein (Fig. ). Cellular proliferation was assessed by staining for Ki-67, which was positive, but the index was as low as 1–5%. These findings were associated with proliferation of tumor cells that contained abundant melanin pigment. Based on the above pathology results, a definitive diagnosis of melanocytoma was made.\nFollowing excision of the intracranial tumor, our patient underwent adjuvant gamma knife radiosurgery with 24 Gy in two fractions to the tumor bed in the epidural space of the middle cranial fossa. No chemotherapy was administered. His postoperative course was uneventful with progressive resolution of the neurologic deficits. At follow-up 6.5 years later, he remains well with no signs of recurrence.","This 39-year-old man has been experiencing numbness and pain on the left side of his face for three months, making it difficult to chew. After a thorough examination and scans, doctors discovered a small tumor in a part of his brain called Meckel’s cave that was pressing on his trigeminal nerve, causing these symptoms. Surgery to remove the tumor was successful, and he is now doing well with no signs of recurrence."
720,"An 84-year old man with chronic hepatitis C and liver cirrhosis was referred to our outpatient clinic for the evaluation of HCC, previously treated with transarterial chemoembolization (TACE) and radiofrequency ablation (RFA). Following disease relapse, a wedge resection of two nodules in hepatic segments VI and VII was performed in December 2008. Histological examination confirmed HCC grade III (Edmondson scoring), with necrosis and microscopic vascular thrombosis.\nIn September 2009, magnetic resonance imaging (MRI) showed millimetric disease relapse in hepatic segments V, II, III, and I, and a 21 × 9 mm adenopathy at the hepatic hilum (Fig. a, b). A new resection was scheduled but was not carried out following the detection by intra-operative ultrasound (US) of a right portal branch neoplastic thrombosis. In December 2009, serum alpha-fetoprotein (AFP) was 1504 ng/mL. In January 2010, as a consequence of disease metastasis, systemic treatment with metronomic capecitabine (500 mg twice daily) was continuously administered according to a previously described protocol []. The therapy was well tolerated. After 1 month, serum AFP decreased to 643 ng/mL, and 3 months later, had drastically decreased to 7 ng/mL. At the same time, a marked reduction in liver lesion size was observed by MRI, evaluated as a partial response according to Modified Response Evaluation Criteria in Solid Tumors (mRECIST) []. In August 2010, computer tomography (CT) scanning showed a single hypodense lesion of 13 mm in hepatic segment II without any other liver lesions, and that enlarged abdominal lymph nodes were stable and neoplastic thrombosis was not detected. Given the presence of a single lesion, the possibility of residual disease ablation was explored using hepatic contrast-enhanced ultrasound. Two suspicious lesions for HCC were detected in hepatic segments II and III, without a typical contrastographic appearance. The lesions were submitted to needle biopsy, and histological analysis identified a nodule of low-grade dysplasia in segment II and micro and macronodular cirrhosis with light activity in segment III. The patient underwent metronomic capecitabine until July 2013 when, considering the sustained CR, treatment was discontinued and the patient entered a follow-up program. The final MRI (February 2017, Fig. c, d) confirmed the CR. To date, the patient is alive and in good health.","This 84-year-old man has been battling liver cancer for many years, having previously undergone surgery and other treatments. Recent scans showed the cancer had returned, but after starting a new medication, his cancer levels dropped significantly. Further tests revealed that the cancer had stabilized, and he is currently in remission. He is now being monitored closely to ensure the cancer doesn't return."
721,"A 66-year old man had multiple liver lesions involving approximately 70% of the right liver, multiple nodules in the left lobe, and a right portal thrombosis in the setting of non-alcoholic steatohepatitis (CT scan in August 2012, Fig. a, b). Positron emission tomography (PET) with 2-(fluorine-18)-fluoro-2-deoxy-d-glucose (FDG-PET) identified bone metastases in the proximal portion of the right femur, in the right ischial tuberosity, in the left acetabulum, in the left scapula, and in the third left costal arch. Moreover, a PET with (11)C-choline confirmed the hepatic and skeletal lesions and identified other metastases in the pelvic bones, rachis, and ribs. In October 2012, serum AFP was 1909 ng/mL. Considering the typical contrastographic pattern of the liver lesion by CT scanning and the elevated AFP level, a diagnosis of HCC was made according to European Association for the Study of the Liver (EASL) guidelines [].\nIn December 2012, the patient started systemic treatment with sorafenib 800 mg/bid. Ten days later, the treatment was discontinued because of G3 skin toxicity (Stevens–Johnson syndrome). In January 2013, the patient started metronomic capecitabine (500 mg twice daily, continuous administration), which was well-tolerated. In March 2013, a new CT scan showed a reduction in the number and size of the liver lesions with significant intralesional necrotic areas. Subsequent FDG-PET scanning (April 2013) showed the complete absence of pathological areas and, in parallel, AFP level had fallen to 3.3 ng/mL. In July 2013, a needle biopsy of the principal hepatic lesion evidenced fibrous connective tissue with histiocytic inflammation without tumour cells. An abdominal US scan (January 2014) revealed the presence of a single hypoechoic lesion of 1.4 × 1.3 cm. In December 2014, CT scanning showed a further size reduction of the hypodense hepatic lesion without vascular components, and it was therefore decided to discontinue treatment in the same month. CT and US images, supported by histological patterns, showed a necrotic lesion with gradual resolution (Fig. c, d). In the most recent US scan (September 2017), no hepatic lesion was detected and serum AFP was 3.6 ng/mL. The patient is currently alive and in excellent health.","This 66-year-old man was diagnosed with liver cancer (HCC) after scans showed multiple tumors in his liver and bones. He started treatment with a medication that caused a severe skin reaction, so it was stopped. He then began a different medication, which helped shrink the tumors in his liver and reduce the cancer in his bones. Over time, the tumors disappeared completely, and his blood tests showed no signs of cancer."
722,"A 70-year-old man with hepatitis C virus cirrhosis was diagnosed with binodular HCC in Jul 2006 and treated with RFA and percutaneous ethanol injection (PEI). From March 2008 to March 2015, the patient experienced multiple tumour recurrences, which were managed using locoregional techniques (RFA, PEI, and one course of TACE). The patient came to our attention in October 2015 following HCC progression in the VIII segment associated with an invasion of the inferior vena cava and neoplastic pulmonary embolization. Serum AFP was 18,622 ng/mL. Systemic therapy with sorafenib was started at dosage of 400 mg/die, given the patient’s poor clinical condition, and increased to 600 mg/die after 10 days. The patient started experiencing severe fatigue, diarrhoea, and dizziness, which prompted a reduction in dosage to 400 mg/die in November 2015. In February 2016, following radiological progression (tumoural invasion of the right and median hepatic veins and enlargement of the neoplastic thrombus in the inferior cava vein) and a sharp increase in serum AFP (47,137 ng/mL), the patient was switched to capecitabine therapy (500 mg twice daily, continuous administration). CT scanning performed every 3 months showed the progressive reduction of pulmonary metastases, recanalization of the median hepatic vein, and progressive improvement in inferior cava vein invasion. Moreover, the tumour mass showed a complete devascularisation (Fig. a, b). Serum AFP levels decreased to 4583 ng/mL in May 2016, 5.5 ng/mL in September 2016, 2.5 ng/mL in November 2016, and 1.5 ng/mL in October 2017.\nAt the time of writing, the patient is in good clinical condition and continues to receive capecitabine treatment (500 mg bid), complaining only of modest fatigue.","A 70-year-old man with a history of liver disease and previous cancer treatment was recently diagnosed with advanced liver cancer. He initially received local treatments to manage the cancer, but it kept coming back. After starting a new medication, sorafenib, he experienced side effects and the cancer continued to grow, so his dosage was adjusted. Eventually, he began receiving capecitabine, which helped shrink the tumors and lower his cancer levels, and he is currently doing well with continued treatment."
723,"A 32-year-old male was referred to our hospital for elevated level of serum creatinine (Scr) (3.71 mg/dl) and proteinuria (3+) following a previous deceased cardiac donor-derived KT due to an unidentified cause of end-stage renal disease (ESRD). Laboratory workups and results of diagnostic procedures performed are summarized in Table . He underwent a successful KT 26 months ago with Scr at discharge 0.9 mg/dl with an immunosuppressive protocol consisting of prednisone, mycophenolate mofetil and tacrolimus. Renal allograft function remained stable and urine analyses were always normal from discharge to 24 months after operation. Two months prior to this admission, he was hospitalized for fever and cough at another hospital. He was diagnosed with mild pulmonary infection and treated with azithromycin and ceftazidime. His pulmonary symptoms abated after a week antibiotic treatment while his serum Scr increased and proteinuria (3+) occurred. Furthermore, his blood platelet count also decreased to 34 × 109/L. Forty days prior to this admission, a renal allograft biopsy was performed. He was managed with intravenous antibiotics and immunosuppression enhancement by increasing the dosage of mycophenolate mofetil. His Scr level decreased initially with a nadir of 1.58 mg/dl, but elevated progressively with increased proteinuria (11.38 g/24 h). He denied family history of any kidney diseases or inheritable illnesses. A repeat kidney biopsy was performed in our hospital. Written informed consent to publish this case was obtained from this patient.\nLight microscopy showed multiple periodic acid-Sciff stain (PAS)-positive materials in the capillary lumens (Fig. ). Capillary wall duplication was obvious and diffuse (Fig. ). Masson trichrome stain revealed extensive fuchsinophilic deposits in the subepithelial, subendothelial and mesangial spaces (Fig. ). There were no peritubular capillaritis, endotheliatis, tubulitis nor glomerulitis, excluding the possibility of antibody and T-cell-mediated rejection. Histological signs of calcineurin-inhibitor toxicity, such as band-like fibrosis, isometric vacuolization of the tubules and hyaline deposits in the arterioles were not present.\nImmunofluorescence study indicated prominent C3 positivity (3+) along the basement membrane and in the mesangium (Fig. ) in all the 6 glomeruli examined, while IgA, IgG, IgM and C1q and C4d staining were all negative.\nElectron microscopy examination of 2 glomeruli showed widespread foot process effacement and electron-dense deposits in the subendothelial and subepithelial spaces (Fig. ). Furthermore, mesangial proliferation which protruded into capillary basement membrane caused the double contours observed in PAS staining. Subendothelial lucency, which is characteristic of TMA, was also present (Fig. ). No signs of chronic antibody-mediated rejection (ie. peritubular capillary multilayering), Based on these findings, a diagnosis of C3GN combined with TMA was rendered.\nRetrospective review of the 1st biopsy slides (X.F.) indicated similar light microscopy findings (Fig. ). Immunofluorescence showed only prominent C3 staining with negative staining for other immunoglobulins and C4d. No electron microscopy study of the first allograft biopsy was performed.\nGenetic testing for the major genes in complement pathway related with renal disease (C3, CFB, CFH, CFHR1, CFHR3, CFHR4, CFHR5, CFI, DGKE) [, ] were performed. We found two rare missense variants in compound heterozygous form, c.848A > G (p.Asp283Gly) and c.1339C > T (p.Pro447Ser) in the CFI gene (NM_000204.3) in the patient while his father and mother were found to harbor only the c.848A > G and c.1339C > T respectively (Fig. ). Both parents were phenotypically normal. This patient’s unaffected sister had neither of the 2 variants. No variants were identified in the other complement cascade protein genes commonly screened. Nevertheless, quantitative measurement of plasma CFI of the patient and his unaffected family members showed that their plasma CFI levels were all in normal range (Table ).\nOur patient was treated with 2 sessions of plasma exchange, but no clinical improvement was achieved as indicated by persistent nephrotic-range proteinuria and progressive elevation of Scr. After approximately one-year follow-up, this patient was in dialysis.","This 32-year-old man was admitted to the hospital because his kidney function was declining after a previous kidney transplant. Tests revealed protein in his urine and a high level of creatinine, indicating that his transplanted kidney was not working properly. A second biopsy showed signs of a condition called C3GN combined with TMA, which is causing damage to his kidneys. Genetic testing identified two rare gene variations that may have contributed to this problem, and he was started on dialysis to help manage his kidney failure."
724,"Patient 1 was an 11-year old boy, the second child of non-consanguineous parents. There was no family history of bone fragility or autism. The pregnancy was normal, and the patient was delivered by caesarean section post term after failure of labour progression. He was treated in the Special Care Baby Unit for two days after delivery due to pyrexia. He was born with left-sided calcaneus talipes equinovarus and right-sided developmental dysplasia of the hip. His undescended testes were operated on successfully.\nPatient 1 is developmentally delayed. He walked at 2.5 years of age and had delayed onset of speech. He had difficulties with fine motor skills and attended a school for children with special needs. This patient had a clinical diagnosis of ASD made at 5-years of age. His parents also reported ritualistic behaviours, resulting in a referral to Child and Adolescent Mental Health Services for an assessment of possible obsessive compulsive disorder.\nHe was noted to have previously suffered finger fractures and a decrease in vertebral height. A DXA scan to measure his bone mineral density (BMD) undertaken before commencement of bisphosphonate treatment demonstrated a reduced BMD with Z-scores of −3.4 at the lumbar vertebrae and a total body score of −2.5 when adjusted for age and gender. A bone biopsy had demonstrated low turnover trabecular osteopenia consistent with osteoporosis.\nAlso of note, he had diagnoses of asthma and idiopathic generalised epilepsy. He suffered from intermittent neutropenia thought to be the result of sodium valproate therapy. He received 3-monthly pamidronate infusions, remained on melatonin daily and had been prescribed midazolam, to be given in the event of a prolonged seizure.\nOn examination, he had bilateral low-set ears, blue sclerae and glasses due to hyperopia.\nTrio whole exome sequencing (WES) in him identified a de novo missense variant in NRXN1 which is known to be associated with neurodevelopmental disorders/autism and being further investigated as it is known to interact with COL1A1.","When this 11-year-old boy was born, he needed a C-section and spent a few days in the Special Care Baby Unit due to a fever. He has had some developmental challenges, including walking late and having difficulty with speech and fine motor skills, and was diagnosed with autism. He also has osteoporosis, which is why he will be taking medication to strengthen his bones. Recent genetic testing revealed a new finding that may help explain his development issues."
725,"Patient 2 was an 11-year old boy, the second child to healthy, non-consanguineous parents. There is no family history of bone fragility and autism. He was born in the breech position spontaneously at 32-weeks gestation after the pregnancy was complicated by placental abruption, causing severe abdominal pain and heavy bleeding. At birth, he weighed 1.76 kg (9th centile); he required continuous positive airway pressure for 24 h and phototherapy to treat his neonatal jaundice. He was fed via a nasogastric tube for the first week of life.\nHe failed to thrive throughout childhood with height and weight below the 0.4th centile and head circumference 0.4th-2nd centile, with insufficient weight gain resulting in the insertion of a percutaneous gastrostomy for nutritional support. He suffered frequent infections including bronchiolitis, pneumonia and urinary tract infections. A micturating cystourethrogram identified bilateral vesicoureteric reflux. He had consistent hypogammaglobulinaemia and lymphopenia throughout childhood with poor vaccine responses. This patient received 3-weekly immunoglobulin replacement therapy. Also of note, he had bilateral optic atrophy and consistently abnormal liver function tests.\nPatient 2 had severe intellectual disability. He had delayed speech and suffers from gross and fine motor delay: he first walked at 19 months. He demonstrated significant echolalia and restricted interests; the patient had received a clinical diagnosis of ASD at 6-years of age.\nHe had suffered several fractures of the vertebrae, metatarsals and tibias. A bone biopsy at 7-years of age demonstrated a high rate of bone turnover and osteopenia, with marked subperiosteal bone resorption. DXA scans showed reduced bone mineral density, however it was difficult to determine the degree of reduction due to his small size. He received 3-monthly pamidronate infusions.\nThe patient had undergone numerous investigations throughout his life to provide an explanation for his clinical features. Trio WES identified that patient 2 is compound heterozygous for c.3010C>T and c.5741G>A pathogenic mutations in the NBAS gene (). He had been diagnosed with SOPH syndrome (Short Stature, Optic Atrophy, Pelger-Huet anomaly), which largely explains the patient's clinical picture. On examination, he had short stature and high pitched voice. Facial dysmorphism included a prominent forehead, low set ears, hypertelorism, proptosis, progeric appearance to his skin and up-slanted palpebral fissures.","This 11-year-old boy has had many health challenges since birth, including a difficult pregnancy and premature birth. He struggled to gain weight and had frequent infections, requiring special feeding and immunoglobulin treatments. He also has significant developmental delays, including intellectual disability and autism, and has experienced multiple bone fractures. Recent genetic testing revealed that he has a rare genetic condition called SOPH syndrome, which explains many of his symptoms."
726,"Patient 3 was a 4-year old boy, the third child of healthy, non-consanguineous parents. There was no family history of bone fragility or autism. Bowing of the lower limbs observed on the anomaly scan raised antenatal suspicion of a campomelic dysplasia. The patient was born by normal vaginal delivery at term. He weighed 3.74 kg (50th centile) with a head circumference of 34 cm (25th centile). He suffered mild respiratory distress at birth but did not require ventilatory support.\nA skeletal survey performed after birth demonstrated a normal thoracic cage volume, bowing of the long bones with abnormal metaphyses and a fractured ulna. The patient suffered fractures to his left humerus and right forearm. He was subsequently diagnosed with severe osteogenesis imperfecta.\nBy 4-years of age, he had suffered multiple fractures of his ulnas and humeri, a femoral fracture and multiple vertebral wedge fractures. He has undergone bilateral osteotomies and rodding of his femurs and tibias at 2 and 3 years of age, respectively. He received 3-monthly pamidronate infusions.\nThis patient was developmentally delayed, sat independently from 2 years and walked with aids from 2.5 years of age. He had delayed speech and required intervention from speech and language therapists at age 21 months. He has demonstrated “rocking” behaviour from 2.5 years of age but did not have a clinical diagnosis of ASD before recruitment to the study.\nOn genetic assessment, he was noted to have a ‘triangular’ face, blue sclerae, high-pitched voice in keeping with a diagnosis of ‘Classical OI’. He went on to have testing for COL1A1/A2 and was found to have a pathogenic c.902G>A variant in COL1A2. This pathogenic mutation is predicted to replace glycine at position 301 with a glutamic acid. Glycine substitutions are well-recognised as a cause of OI. This confirmed his clinical diagnosis of OI.","This 4-year-old boy was diagnosed with a rare bone disorder called osteogenesis imperfecta (OI) after an ultrasound showed his bones were unusually fragile. He was born with some breathing difficulties but otherwise healthy. Over time, he has experienced many fractures and has needed surgery to help stabilize his bones. He is also slightly behind in development, particularly with speech, and receives support from therapists. Genetic testing confirmed a specific change in a gene that causes OI, explaining his condition."
727,"Patient 4 was a 14-year old male, the only child born to non-consanguineous parents. His younger half-brother (through same mother) had learning difficulties but there was no other family history of autism. The pregnancy was normal with delivery by caesarean section at 39 weeks due to a breech presentation. He had a birth weight of 3.54 kg (65th centile). He needed oxygen shortly after delivery but was not admitted to the Special Care Baby Unit. He had global developmental delay: no head control was evident at 4 months; sitting was achieved at 2 years of age; the patient walked at 4.5 years and currently uses a wheelchair. He spoke his first words aged 7 years. He was doubly incontinent and has learning difficulties; he attended a school for children with special needs. He was diagnosed with ASD at 5-years of age, before recruitment to the study after demonstrating little eye contact and having restricted interests. He had previously engaged in self-harm behaviour such as head banging and biting.\nPatient 4 had suffered from a fractured forearm and vertebral wedge fractures. He had been given a diagnosis of probable primary osteoporosis, suffering discomfort in his back and lower limbs. DXA scanning undertaken before commencing 3-monthly pamidronate infusions demonstrated a reduced BMD when adjusted for age and gender of −2.6 at lumbar vertebrae 2–4 and a total body measurement of −2.7. He had joint hypermobility and brittle nails.\nThis patient was diagnosed with bilateral femoral proximal anteversion, which was operated on with a derotation osteotomy. He demonstrated ligamentous laxity and suffered a leg length discrepancy. The patient had a small scrotum and incomplete descended testes. He also had left sided choroidal coloboma and myopia.\nOn examination, he was not dysmorphic. So far WES in him has not identified any variants of significance and further genetic analysis is ongoing.","This 14-year-old boy has a history of developmental delays and was diagnosed with autism at age He has struggled with speech and mobility, using a wheelchair and experiencing incontinence. Recent tests have revealed he has osteoporosis and a bone condition that requires regular infusions, along with other physical issues like joint laxity and a leg length difference. Genetic testing is ongoing to understand the cause of his various health challenges."
728,"Patient 5 was a 13-year old male, the first child to healthy, non-consanguineous parents. There was a family history of osteoporosis in his maternal grandfather but no family history of autism. The pregnancy was not planned and was not detected until approximately 25 weeks. No scans were performed. He was born at term and was immediately well after birth.\nHis initial development was normal, with gross motor milestones being achieved as expected: he sat up aged 6 months and walked at age 8 months. His speech was delayed; he started speaking at 5 years of age after receiving speech therapy. He was diagnosed with ASD at 3-years of age after concerns were raised at his toddler group. The patient attended a school for children with special needs.\nHe had suffered three fractures: two of his forearm and one of his wrists. Additionally, he had suffered from multiple crush fractures of his thoracic and lumbar vertebrae. The small joints of the fingers were hypermobile, but there was little evidence of hypermobility elsewhere. DXA scans undertaken before commencing bisphosphonate treatment demonstrated reduced BMD, with Z-scores of −2.7 at the lumbar vertebrae and − 2.6 total body measurement when adjusted for age and gender. He had a diagnosis of idiopathic osteoporosis with a bone biopsy at 12-years of age demonstrating severe low turnover cortical and trabecular osteopenia. The patient received 3-monthly infusions of pamidronate.\nOn examination, this patient was not dysmorphic. WES identified a maternally inherited PLS3 pathogenic variant which explained his bone fragility.","This 13-year-old boy was born healthy and developed normally until he started speaking later than usual. He was diagnosed with autism at age three and attends a special school. He has a history of several broken bones and has been diagnosed with osteoporosis, a condition that weakens bones. Recent tests revealed a genetic cause for his bone fragility, which is inherited from his mother."
729,"Patient 6 was an eight year old boy, the second child of healthy, non-consanguineous parents. There was no family history of bone fragility or autism. Shortened long bones were identified on the 16-week scan and the child was delivered by caesarean section at 37-weeks. At birth, he needed ventilation with a bag and mask. He was born with fractures of all the long bones and multiple ribs: he was diagnosed with severe OI antenatally. The patient was treated in the special care baby unit for three months; he was fed via a nasogastric tube and suffered from gastroesophageal reflux.\nHe developed a right sided inguinal hernia shortly after birth which was surgically corrected at one month of age. He also suffered from fusion between the base of his skull and top of his spinal column. Throughout his life, he had suffered multiple long bone fractures, including several femoral fractures and fractures of his radii. He had undergone several surgical procedures, with bilateral femoral and tibial rodding procedures undertaken at 4 and 5 years of age, respectively. His bone fragility was managed with 3-monthly infusions of pamidronate.\nHe was developmentally delayed: he started talking between two and a half and three years of age and started to “commando crawl” at 3 years of age. He had never walked. The patient attended a mainstream school after starting a year later than his peers. He did not have a previous diagnosis of ASD.\nOn examination, he had short stature, blue sclerae, triangular face and dentinogenesis imperfecta. There were marked deformities of his long bones, resulting in a pes cavus appearance. Genetic testing showed that he carried a de novo pathogenic variant in COL1A1 c.2282G>A in exon 33/34 confirming his clinical diagnosis of OI.","This eight-year-old boy was born with a rare condition called Osteogenesis Imperfecta (OI), which causes fragile bones. He needed help breathing at birth and had numerous broken bones, requiring extensive medical care and surgery. Throughout his life, he has had many more fractures and has relied on regular infusions to strengthen his bones. Because of his condition, he has developmental delays and has never been able to walk, but he attends a mainstream school. Genetic testing confirmed the cause of his OI."
730,"Patient 7 was a 6-year old boy, second child of healthy, non-consanguineous parents with no significant family history. His sister was said to have a seizure disorder of unknown aetiology but there was no family history of autism. Antenatally, there were concerns with short long bones and bowed femur and he was born at term with a normal birth weight. He was noted to have multiple fractures and commenced on treatment with pamidronate with a good response. He was noted by the therapy team to have autistic traits and recruited to the study. He fulfilled the criteria for a diagnosis of autism. On examination, he had a triangular face, blueish sclerae, high-pitched voice, dentinogenesis imperfecta, significant limb deformities and scoliosis. Genetics analyses revealed normal microarrays and a pathogenic variant was identified in COL1A2 confirming his clinical diagnosis of OI. c.2533G>A mutation in exon 37 of COL1A2 gene, this pathogenic mutation is predicted to replace glycine at position 845 with an arginine and has previously been reported in individuals with OI confirming his diagnosis.","This 6-year-old boy has been diagnosed with osteogenesis imperfecta (OI), a condition that causes fragile bones. He has had several fractures in the past and was treated with medication to help strengthen his bones, which responded well. He also shows signs of autism and has been evaluated for that condition. Examination revealed other features associated with OI, such as blue eyes, weak teeth, and limb deformities, and genetic testing confirmed the diagnosis."
731,"We describe the case of a female infant. She was the second child born to a 33-year-old, gravida 3, para 2 mother. The patient was born polyhydramnios by cesarean section at 37 + 4 weeks of gestation with a birth weight of 2440 g (− 1.1 S.D.), a length of 50 cm (+ 0.80 S.D.) and an occipitofrontal circumference of 36 cm (+ 2.0 S.D.). The 1- and 5-min Apgar scores were 8 and 8, respectively. Shortly after birth, she required nasal continuous positive airway pressure (nCPAP) and presented with dyspnea. During the following days, she developed dyspnea continually and needed oxygen to maintain 90–95% saturation. Parenteral nutrition was started on day 1 and breast milk was given 12 h after birth by oral tube. Her parents were nonconsanguineous and her mother had a healthy 13-year-old child. She denied any family history of neonatal disease. Prenatal examination was not found abnormal. Additionally, she denied that she had consumed alcohol, drugs, tobacco, or any other toxic substances during her pregnancy.\nOn admission to our unit, the patient was 3 days old and weighed 2400 g. Clinical examination showed choanal atresia, bilateral low-set ears, triple restriction and systolic murmur, but coloboma was not observed. Her motor development was almost normal. The patient presents feeding difficulties by nasogastric tube. Her white blood cell count was 12.07 × 109/L (neutrophils, 0.50; lymphocytes, 0.24), and her platelet count was 160.00 × 109/L and CRP < 1 mg/L. The alanine aminotransferase level was 14 U/L, aspartate aminotransferase level was 43 U/L, and gamma-glutamyltransferase level was 68 U/L. On the seventh day of age, her thyroid functional parameters were TSH 5 mIU/L, T3 1.83 nmol/L and T4 123.94 nmol/L, and at the first month of age, thyroid functional parameters were TSH > 100 mIU/L, T3 1.57 nmol/L and T4 35.93 nmol/L. Thus, oral Euthyrox (Levothyroxine sodium tablets) was administered. Newborn screening for metabolic disorders and severe combined immunodeficiency was normal. A chest radiograph showed haziness in both lung fields suggestive of wet lung (Fig. ). Two-dimensional and color-Doppler assessment was revealed an atrioventricular septal defect (6.8 mm + 2.2 mm), patent ductus arteriosus (3.6 mm) and pulmonary hypertension (Fig. ). A craniocerebral ultrasound showed bilateral lateral ventricle dilatation (Fig. ). The auditory brainstem response (ABR) test showed bilateral severe hearing impairment (ABR > 99 dBnHL). Following radiologic testing by computed tomography showed bilateral choanal atresia and insufficient inflatable structure of both semi-circular canals. (Fig. ). No clinical characteristics of CHARGE syndrome were detected in the patient’s parents. Chromosomal analysis indicated a 46 XX normal female karyotype.\nThe patient required mechanical ventilation with endotracheal intubation at 4 days of age. On 17 days of age, she was extubated to nCPAP with FiO2 < 25%. On 20 days of age, she needed an oxygen mask. Ampicillin was discontinued when the blood culture from birth was sterile at 72 h. At the first month of age, the patient had a posterior nostril plasty operation by nasal endoscope and had a silicone tube in one month for transition the postoperative (Fig. ). At the second month of age, she had patent ductus arteriosus ligation surgery. At the 4th month of age, she was discharged from the hospital. Clinical features summarized in Table .\nMolecular analysis of the disease-associated genes CHD7 and EFTUD2 were performed using the Illumina TruSigt One sequencing panel (Illumina, San Diego, CA, USA) on the MiSeq NGS platform for mutation screening methods (Sinopath Diagnosis, Beijing, China) []. Genomic DNA was extracted from peripheral venous blood and informed consent was obtained from the parents. The study was approved by the ethics committee of Zhejiang University Children’s Hospital. To identify presumably pathogenic single-nucleotide variants, we used NextGene V2.3.4 (Softgenetics, State College, PA, USA) compared with the UCSC database. We excluded sequence variants with a minor allele frequency > 0.05, in the Human Genetic Variation Database () and the NHLBI Grand Opportunity Exome Sequencing Project (ESP6500, ). This analyses identified a monoallelic (thymine) insertion in CHD7, NM_017780.3 (CHD7 c.4656dupT), which was confirmed by Sanger sequencing. This mutation leads to a reading frameshift mutation starting from isoleucine, with the new reading frame ending p.(Ile1553fs) (Fig. ). As shown in Fig. , the mutation is located in exon 20, and this mutation was not present in the Human Gene Mutation Database (/) or ClinVar (),[] suggesting that it is novel. This heterozygous frameshift mutation was not detected in the patient’s parents, suggesting that it is a de novo mutation.","This baby was born with some surprising challenges, including a narrow airway and breathing difficulties that required special breathing support. She also had some heart defects and hearing loss, and needed surgery to correct these issues. After several days in the hospital, she was diagnosed with a new genetic mutation that caused her symptoms. She received treatment and underwent several procedures, including a nostril plasty and a patent ductus arteriosus ligation. Finally, she was able to go home at four months old, and her parents continue to monitor her health closely."
732,"A 71-year-old Caucasian woman was treated in our Gamma Knife center for a meningioma of the sphenoid jugum. The treatment was performed with Leksell Gamma Knife Icon® (Elekta Instruments, Stockholm, Sweden) and was planned as a hypofractionated irradiation including five daily fractions of 5 Gy. The restraint method chosen was the use of a thermoplastic mask Orfit® (Orfit Industries, Wijnegem, Belgium) [, ]. The mask was made 5 days before the first irradiation. During mask making, the mask was warmed by soaking in a water bath and then applied and molded directly on our patient’s face for 20 minutes (Fig. ). At this step of the procedure, she complained of a burning and tingling sensation on her face, especially on her forehead. During the following 4 days, she continued to have a stable cutaneous reaction in the form of redness, tickling, and edematous swelling of her face. She was treated with a local antihistamine cream on her face, with moderate improvement in the symptoms. On the first day of treatment, during the first irradiation session, she complained again of a major sensation of burning and edema of the face. A clinical examination showed a serious allergic reaction on her face, associated with an atopic edema. She was treated with 125 mg of intravenously administered corticoids, followed by high doses of orally administered antihistamines and corticoids during the following 5 days. With this medication, the allergic reaction was controlled until the end of treatment 4 days later. During all irradiation fractions we kept using the thermoplastic mask but we inserted a thin sheet of paper between the internal surface of the mask and our patient’s forehead to reduce the surface area of contact between the mask and our patient’s skin.","This 71-year-old woman had a small growth on her brain called a meningioma, which was treated with a focused radiation therapy called Gamma Knife. During the treatment, she experienced a significant allergic reaction on her face, causing redness, swelling, and a burning sensation. The doctors carefully adjusted the mask used during treatment to minimize skin contact and provided medication to manage the reaction. Thankfully, the reaction was controlled, and she completed the radiation therapy successfully."
733,"A 43-year old, asymptomatic woman was admitted to our hospital by her family doctor after receiving a chest-x-ray during routine clinical examination. The x-ray showed a mediastinal mass overlapping the aortic arch region (Fig. ). For verification a computed tomography (CT) was performed and revealed incidentally a type B dissection, which was most likely chronic without information of the index date, originating from an aneurysm of a left cervical arch with a maximum diameter of 6 cm (Fig. ). The left renal artery, the coeliac trunc and the main part of the superior mesenteric artery branched from the false lumen without a sign of malperfusion of the organs. Because of the huge diameter and the potential risk of rupture, an urgent surgical repair was planned. Before intervention the patient got a blood pressure adjustment by ACE inhibitor. Betablocker was not necessary because of a resting pulse under 60 beats per minute. For neurological online monitoring, sensitive and motor evoked potentials were monitored. Spinal drainage was installed 1 day before the procedure. Surgical access was carried out through median sternotomy and an additional left lateral thoracic incision through the fourth intercostal space (Hemi-Clemshell). Simultaneously to the preparation of the aneurysm, partial cardiopulmonary bypass was installed in the left groin by cannulation of the femoral artery and vein under echocardiographic guidance. During selective ventilation of the right side, the left lung was mobilized by transsection of the Ligamentum pulmonale and preparation of the perianeurysmatic tissue and adhesions. After identification and preparation of the recurrent and phrenic nerve and the supraaortal branches, the descending aorta was clamped and a distal anastomosis performed with a straight graft (20 mm). The visceral arteries partially branched from the false and true lumen without a sign of malperfusion. Before the final distal anastomosis, we performed a fenestration of the dissection membrane about a length of 5 cm to keep the perfusion of both lumina. The left carotid artery originated from the aortic arch with a distance of only 1 cm from the aneurysm. The left axillary artery branched directly from the aneurysm and was dissected and reimplanted with a separate 8 mm sidegraft to the 20 mm straight graft between the distal arch and proximal descending aorta. (Fig. ). The procedure was performed with partial cardiopulmonary bypass (CPB) of 87 min, aortic clamp time of 62 min under normothermic condition. The patient was extubated on first postoperative day and recovered well.\nBiopsy of aortic tissue showed a picture consistent with arteriosclerosis and loss of smooth muscle cells, rupture of the elastic fibres and fibrosis of the media. The intima could not be visualized in detail.\nThe patient was discharged to cardiac rehabilitation at 13th postoperative day and recovered well. Last follow up with computed tomography was performed 3, 5 years after initial operation with a good and stable result of the dissection membrane and a perfusion of both lumina. The patient is able to resume a normal life without limitations.","A 43-year-old woman was admitted to the hospital after a routine chest X-ray revealed a mass in her chest. Further tests showed a serious problem with a weakened area in her aorta, which could have ruptured. To prevent a dangerous rupture, doctors planned an urgent surgery to repair the aorta. The surgery was successful, and the patient is recovering well and is expected to return to a normal life."
734,"A 29-year-old Asian man who had undergone surgical debridement at another hospital for a perianal abscess 5 days earlier was referred to the emergency room of Xiamen Chang Gung Hospital. The patient presented with continuous severe perianal and scrotal pain, scrotal swelling, and high fever (39.2 °C) of 3 days’ duration that had been aggravated for 1 hour. The patient was mildly obese, described himself as otherwise quite healthy, and had never been admitted to a hospital previously. He reported no significant chronic medical history, such as primary hypertension, any type of heart disease, disturbed microcirculation, peripheral neuropathy, diabetes mellitus, an impaired immune system, malignancies, leukemia, long-term administration of corticosteroids, liver cirrhosis, renal failure, urinary tract infection, or hemodialysis. The patient also reported no history of infectious diseases, such as tuberculosis, any type of hepatitis, or acquired immunodeficiency syndrome (AIDS). The patient’s medical history revealed no trauma, blood transfusion, other surgical procedures, or other serious event. He had not lived in an epidemic area and had no contact history of toxicity or radioactive exposure. The patient denied a family history of any inherited cancer. He did not smoke or consume alcohol and reported no other unhealthy lifestyle behaviors. The patient was a businessman by occupation and traveled for business most of the time.\nA general physical examination on admission revealed that the patient was hypotensive (blood pressure, 92/63 mmHg) and tachycardic (heart rate, 117 beats/minute). No positive signs were found during the neurological, cardiopulmonary, and abdominal examinations. Neither pain around the kidney area with percussion nor tenderness along the bilateral ureteral approach was found. No bulging, tenderness, or mass was evident in the bladder area. A genital examination revealed a normal distribution of pubic hair and normal penile development without deformity, prepuce, penile ulceration, tenderness, induration, or neoplasms. No ectopia or secretions were found at the urethral orifice. A perianal abscess and hemorrhoids were identified upon full perineal examination (a previous surgical wound with obviously inflammatory secretions was detected 2.0 cm from the anal edge and approximately 1.5 × 2.5 cm in size), and the patient’s rectum was noted to be very tender. Furthermore, the scrotum was gangrenous with extensive cellulitis of the perineum and bilateral inguinal area and an increased skin temperature. No clear findings were obtained by palpation of the bilateral testis and epididymis.\nFollowing a rectal examination, the patient rapidly became sweaty and unwell, and his scrotum became blue, swollen, and tense, with erythema extending into both groins. Crepitations between the skin and fascia were palpable. The patient noted a concomitant deterioration in his urinary stream and a degree of hesitancy. His white blood cell, red blood cell, and platelet counts were 23.1 × 109/L, 4.11 × 1012/L, and 112 × 109/L, respectively. Upon admission, his hemoglobin was 123 g/L, his ST-segment elevation was 84.0%, and his C-reactive protein level was 275.55 mg/L. The results of serology for both liver and renal function were normal. His coagulation and electrolyte profiles were normal. Computed tomography of the lower abdomen and pelvis (Additional files and ) revealed extensive emphysema around the testicles, epididymis, and perineal subcutaneous tissues. A diagnosis of FG complicating a perianal abscess was made.\nThe patient underwent aggressive fluid administration, hemodynamic support, and intravenous antibiotic therapy. He was treated with immediate surgical debridement under general anesthesia (Fig. ). Tissue cultures were obtained to isolate the responsible microorganisms. The necrotic skin in the scrotum and the perianal region was evacuated into an open drainage area using three incisions, leaving both testes exposed (Fig. ). There was no damage to the testicles, spermatic cords, or external sphincter.\nBlood and aerobic and anaerobic bacterial cultures were performed. Microorganisms were not found in the blood cultures. Tissue samples taken during the first two debridements revealed that the microbiological etiology of FG was polymicrobial. Aerobic Streptococcus agalactiae, Staphylococcus haemolyticus, and Escherichia coli as well as anaerobic peptostreptococci and Prevotella corporis were detected. Preoperative antibiotic treatment with combined broad-spectrum antibiotics (metronidazole 0.5 g every 8 hours and ceftriaxone 1 g every 12 hours for 6 days) was initiated and later adjusted to the culture sensitivity of the microbial isolates. The patient was therefore intravenously administered levofloxacin (0.3 g every 12 hours for 5 days).\nThe patient’s white blood cell count and C-reactive protein level gradually decreased. On the third postoperative day, his white blood cell, red blood cell, and platelet counts were 6.6 × 109/L, 4.54 × 1012/L, and 190 × 109/L, respectively. His hemoglobin was 135 g/L, his ST-segment elevation was 64.0%, and his C-reactive protein level was 100.24 mg/L. He had no fever thereafter.\nLocal wound care was administered using moist gauze dressing (i.e., normal saline) and was changed three times per day until healthy granulation tissue was observed. The patient underwent two subsequent surgical debridements. His infection gradually subsided, his gas gangrene resolved completely, and good granulation was present 1 week after surgery. Because both testicles were severely exposed, they were transpositioned into the scrotum on the eighth postoperative day. Split-skin closures (3-0 nylon suture) and open drainage from the scrotum and perianal area were subsequently performed (Fig. ). The patient was discharged on the 11th postoperative day (with antibiotics adjusted to oral administration of levofloxacin 0.1 g twice daily).\nThe patient attended the outpatient department four times for follow-up. The bilateral scrotal wounds healed, and the stiches were removed on the 18th postoperative day (the first follow-up). The viability of both testicles was confirmed using Doppler ultrasound on the 21st postoperative day (the second follow-up) and at 3 and 10 months after discharge (the third and fourth follow-up examinations). The open drainage area from the scrotum and perianal area had healed at 10 months after discharge. The patient was healthy as usual, with no complaints or illness. His complete cell count and serum parameters of liver and kidney function were normal.","This 29-year-old man was admitted to the hospital after a perianal abscess that worsened rapidly, causing severe pain, fever, and swelling in his scrotum. He also had a concerning infection in his groin area. Initial tests revealed that the infection was caused by multiple bacteria, including Streptococcus and Staphylococcus. After surgery to remove infected tissue and antibiotics, his condition improved, and both testicles were successfully repositioned. He is now following up with his doctor to ensure his recovery is complete."
735,"A 24-year-old Sri Lankan man developed fever, profuse vomiting and diarrhoea followed by reduced level of consciousness over a 12-h duration. He had myalgia, arthralgia and frontal headache. He did not have photophobia, phonophobia, skin rash, fits, cough or urinary symptoms. He did not have any bleeding manifestations. He did not smoke tobacco or consume alcohol. There was no history of illicit drug abuse or high risk sexual behavior. He had been previously diagnosed with mild intermittent bronchial asthma.\nOn examination, he was febrile (101.3 °F), drowsy with a Glasgow coma scale of 11/15. There were no skin rashes or lymphadenopathy. No focal signs were noted in the neurological examination and fundoscopic examination was normal. His Pulse rate was 112 bpm; blood pressure was 100/60 mmHg with no postural hypotension; respiratory rate was 14/min. Rest of the general and systems examinations were normal.\nHis full blood count on admission showed white blood cells of 6 × 109/L (Normal range [NR] 4.0–11.0 × 109/L) with neutrophils of 59%; haemoglobin 14.3 g/dL (NR 13.5–16.5 g/dL) and platelet count of 74 × 109/L (NR 150–450 × 109/L). Erythrocyte sedimentation rate was 13 mm in first hour and C-reactive protein was 63 mg/l (NR < 5 mg/L). Serum electrolytes were normal with mild impaired renal function. Liver enzymes were elevated, alanine aminotransferase was 303 U/l (NR < 50 U/L) and aspartate aminotransferase was 482 U/l (NR < 50 U/L) with a total bilirubin of 10.6 µmol/L (NR 5–21 µmol/L). His urine analysis and coagulation profile were normal. Non-contrast CT brain demonstrated cerebral oedema. Lumbar puncture was precluded by a low platelet count.\nBased on a working diagnosis of encephalitis, he was commenced on intravenous meropenem, vancomycin, aciclovir and dexamethasone, but were subsequently omitted once the diagnosis became evident.\nOn further investigation, dengue IgM antibody in serum was positive on day 6 of fever. Blood and urine cultures did not yield any microbial growth. Electroencephalography showed generalized slow wave activity. The cranial MRI showed an isolated ovoid hyperintensity in the splenium of the corpus callosum with homogeneous hyperintense signal on diffusion-weighted imaging (DWI) (Fig. ). A diagnosis of reversible splenial lesion syndrome (RESLES) was suspected based on the MRI appearance.\nStandard dengue monitoring of vitals, urine output, haematocrit and platelet count were done. His platelet count dropped to a nadir of 31 × 109/L, but subsequently increased. The white cell count steadily increased to 14 × 109/L and then stabilized around 7 × 109/L. Fluid leakage was not detected on repeated ultrasound scanning during the acute phase of illness.\nHe was discharged from hospital on the tenth day of illness with complete clinical, heamatological and biochemical recovery. Follow–up cranial MRI done 1 month later showed complete resolution of the splenial lesion (Fig. ).","This 24-year-old man experienced a sudden illness with fever, vomiting, and confusion. Initial tests showed signs of inflammation and some liver problems, but no evidence of infection. Further investigation revealed he had dengue fever and a rare condition called reversible splenial lesion syndrome, which caused swelling in the brain. Thankfully, he recovered fully and his brain scan showed the problem had disappeared after about a month."
736,"A 26-year-old woman presented to a local dentist due to right mandible pain. She did not complain of any other manifestations, and she had no pertinent past medical history. She was diagnosed with periapical periodontitis, which is an infection of the dental pulp with an apical lesion, of the right lower first molar. She subsequently underwent a root canal therapy. However, she also complained of swelling of the right mandibular region. Since her symptoms had been worsening for 4 months, she was referred to our hospital for further examination and treatment. At our hospital, facial conditions revealed right mandibular swelling and tenderness. Oral conditions showed no percussion and occlusal pain of teeth in the swelling region, and tooth mobility, gum swelling, and gum redness were not seen. Hence, there was no dental infection that could cause osteomyelitis/osteitis. Blood samples were unremarkable with no signs of inflammation.\nOrthopantomogram showed sclerotic change at the right body of the mandible with periosteal reaction (). Plain computed tomography (CT) showed sclerotic change at the right body of the mandible with periosteal reaction and spotted osteolysis was seen in the cortex of the mandible (). On magnetic resonance imaging (MRI), the right body of the mandible showed low signal intensity on T1-weighted images and high signal intensity on short tau inversion recovery (STIR) images with perilesional soft tissue swelling (). Bilateral palatine tonsils and reactive lymphadenopathy were also seen. These findings indicated active mandibular osteomyelitis/osteitis without odontogenic infection. At this time, the possibility of SAPHO syndrome was considered. Detailed medical interview found that she had a history of palmoplantar pustulosis (PPP) for about 1 year, which was treated at a local dermatology clinic. To investigate the presence of other osteoarticular involvement, technetium-99m hydroxymethylene diphosphonate (99mTc-HMDP) scintigraphy was performed, which demonstrated diffuse increased uptake at the right mandible, as well as in the sternum and the sternocostoclavicular joints (). These characteristics of clinical and radiological manifestations led to the diagnosis of SAPHO syndrome.\nTonsillectomy was performed for PPP, and bone biopsy of the right mandible was simultaneously performed. Histopathological examination showed enlarged and sclerotic bone trabeculae with little inflammatory cell infiltration, which was compatible with SAPHO syndrome ().\nShe was treated with salazosulfapyridine 1000 mg/day and nonsteroidal anti-inflammatory drugs as needed, and the pain resolved. Thus far, after a 5-month follow-up period, she has not experienced recurrence of pain.","A 26-year-old woman came to the hospital because of pain in her lower right jaw. After initial tests, doctors found that she had a bone infection called osteomyelitis in her jaw, but it wasn't caused by a tooth problem. Further investigation revealed that she also had SAPHO syndrome, a condition that can affect joints and skin, and she had a history of psoriasis. She was treated with medication and her pain resolved, and she has been doing well for the past five months."
737,"We present a 30-year-old female patient, who was admitted with low back pain and generalized body ache for 2 months prior to presentation; it was excruciating pain especially during nighttime and not much relieved by simple analgesics. The patient has a history of poor appetite with weight loss of about 4-5 kg in a span of 3 months, otherwise had no pulmonary symptoms. She is a nonsmoker and has no past medical illnesses.\nUpon physical examination, the patient had bilateral discrete small cervical and axillary lymphadenopathy, and the breast examination was normal. Other systemic examination was not significant.\nDuring routine workup in emergency, a chest X-ray was done, which was suggestive of bilateral fluffy hilar opacities (), and a CT thorax was done (Figures and ), which was suggestive of scattered areas of multifocal consolidation noted in the left lung and areas of scattered mosaic perfusion noted in the subpleural region, small nodules are also noted in the right lung, and both hila are prominent. The bone window shows multiple sclerotic bony lesions in the vertebra of variable sizes. There is no evidence of any collapse of the vertebra. The spinal canal diameter is normal. No spinal canal stenosis was seen.\nBlood investigation showed normal CBC, electrolytes, urea, creatinine, and calcium, ESR was elevated 50 mm/hr, and high alkaline phosphatase (ALK) (224 U/L; normal 40–150 U/L), and other bone tumor markers were not done as not available.\nUltrasound (US) neck showed bilateral cervical lymphadenopathy; right-side nodes are noted, the largest of which measures 21 × 10 mm in size. Left-side nodes are noted, the largest of which measures 12 × 9 mm in size, and US breast examination was normal.\nWhole-body PET scan () showed progressing pulmonary consolidations and nodules compared to the CT, multiple osseous involvements, generalized, metabolically active lymphadenopathy involving supra- and infradiaphragmatic regions, and hepatomegaly with solitary hepatic lesion.\nAxillary lymph node biopsy was done, and the histopathology finding is most consistent with metastatic lung adenocarcinoma. The immunohistochemical (IHC) profile is most consistent with lung primary. Breast, ovary, and biliary tract are excluded by additional IHC with appropriate controls, which is positive for Napsin A and is negative for GATA-3.\nPatient was referred to Cancer Centre for further plan of management. Patient started on chemotherapy with crizotinib 200 mg BID and denosumab 120 mg·q 4 weekly subcutaneous with good response according to the new PET scan (). As compared to the previous PET scan 7 months before, there is favorable treatment response, metabolic resolution of previously seen left lung uptakes and lymph nodes above and below the diaphragm and metabolic resolution of bone metastases with increasing sclerosis also denoting favorable response.","This 30-year-old woman was admitted to the hospital because of persistent back pain and weight loss. Initial tests revealed concerning changes in her lungs and bones, including unusual spots on a chest X-ray and signs of cancer in her bones. A lymph node biopsy confirmed that she has metastatic lung cancer, and further tests ruled out other possible causes. She is now receiving chemotherapy and showing positive results, with the cancer appearing to be responding to treatment."
738,"A 51-year-old female had an injured left foot by falling down from home stairs. The next day, she was admitted to our hospital and was diagnosed with closed tongue-type calcaneal fracture (). Operation was performed using 2 pins of the Steinmann pin by the Westhues method (). A fixed cast and 2 pins were removed at the same time on the 37th postoperative day, and there was no potential for infection at that time. Nevertheless, she was admitted to our hospital with a complaint about heel pain and fever exceeding up to 40 degrees centigrade, after 9 days from the pin removal.\nOn the examination, skin redness, swelling, and pus-like discharge were observed around the surgical site (). Plain X-ray showed hyperpermeability of the calcaneus, and magnetic resonance images confirmed a diagnosis of osteomyelitis of the calcaneus as well as an abscess formation (). White blood cell count (WBC: 9.9 × 103/μl) and C-reactive protein (CRP: 10.06 mg/dl) were elevated. And methicillin-sensitive Staphylococcus aureus (MSSA) was cultured from the discharge.\nIntravenous antibiotic therapy was administrated immediately (cefazolin 2 g × 3/day), and the next day, the patient underwent irrigation of the surgical site and surgical pus drainage. Fever fell down, and inflammatory aspects disappeared within few days; however, the discharge from the drainage continued on 7 postoperative days. MSSA was cultured again from the discharge, so that we can diagnose whether calcaneal osteomyelitis was not cured completely. 12 days after the 2nd surgery, the patient underwent radical debridement of the calcaneal bone marrow using Ollier's lateral approach and irrigation with natural saline was performed. Subsequently, calcium phosphate cement (CPC) (Hoya Medical, Tokyo, Japan) with vancomycin was implanted at the defected site of the calcaneus (). MSSA was also cultured positive from the bone marrow of the calcaneus. Intravenous antibiotic therapy was continued for 7 days, and it was changed to antibiotics per oral (minomycin 200 mg × 2, rifampicin 450 mg × 1) and continued for 30 days. CRP turned negative on the 10th postoperative day, and the pin tract's fistula was completely closed on the 14th postoperative day. Osteomyelitis seemed to be controllable, and 1/3 partial weight bearing was started from 14 postoperative days. Weight bearing was raised every 1 week as 1/2 and 2/3, and full weight bearing had been completed on the 35th postoperative day. On the 6th postoperative month, fistula was completely closed and there was no recurrence of infection (). The patient could walk normally without a cane, and we considered that complete remission of osteomyelitis was obtained.","This 51-year-old woman fell down the stairs and broke her heel. After surgery to fix the fracture, she developed an infection around the surgical site, causing pain, fever, and pus. Tests showed bacteria in the infection, and she received antibiotics and drainage. After further surgery to remove infected tissue and bone, she is now recovering well and can walk without assistance."
739,"This 72-year-old gentleman presented with a 6-week history of haematuria and underwent a computed tomography (CT) scan that revealed a left renal tumour suggestive of RCC. His comorbidities included type 2 diabetes mellitus and hypertension. He had no family history of any malignancy. He was a life-long nonsmoker and his Eastern Cooperative Oncology Group (ECOG) performance status was 1. He underwent left partial nephrectomy and histology revealed this to be a locally advanced clear cell RCC, Fuhrman grade 2, with involvement of 3 out of 20 lymph nodes (pT3A N1 M0). Postoperatively, he developed ESRD and was started on dialysis 3 times/week. Two years later, he developed a local recurrence in the left kidney and underwent left radical nephrectomy. Histopathology revealed a 5 cm, clear cell carcinoma, Fuhrman grade 2 with invasion of the perinephric fat and renal vessels. He remained on regular follow-up and unfortunately 2 years later he developed further disease progression with a renal bed recurrence along with multiple bone and lung metastases. He received high-dose palliative radiotherapy to the renal bed 40 Gray in 20 fractions followed by commencement of systemic treatment with dose-reduced pazopanib. The dose of pazopanib was reduced to 200 mg daily due to his poor ECOG performance status of 3 and ongoing renal dialysis. Unfortunately, follow-up CT scan 3 months later showed significant disease progression in the renal bed, bone, and lung metastases. He also developed significant pain (score 8 out of 10) over his left loin secondary to the renal bed metastatic deposit.\nHe was started on nivolumab 3 mg/kg initially and later switched to 240 mg flat dose intravenously every 2 weeks. He tolerated the treatment extremely well with no grade 2-4 toxicities. Clinically, there was a significant improvement in his pain control with a reduction in his pain score from 8/10 to 3/10 and his ECOG performance status improved to 2. Follow-up CT scans showed a partial response as per response evaluation criteria in solid tumours (RECIST) version 1.1. There was a significant reduction in the size of lung and renal bed metastases (Figures and ). He remains on nivolumab 22 months from initiation of treatment with serial imaging showing ongoing response.","This 72-year-old man was diagnosed with kidney cancer (RCC) after noticing blood in his urine. He underwent surgery to remove the tumor, but it later returned and spread to other parts of his body. Despite treatment with radiation and medication, the cancer continued to grow, causing pain and requiring dialysis. However, recently, he has responded well to a new medication called nivolumab, which has significantly reduced the size of the cancer and improved his pain and overall well-being."
740,"At the 30th gestational week, a 41-year-old (gravida 2, para 1 [normal vaginal delivery]) woman with no remarkable medical/family histories was referred to us because of fetal cardiomegaly detected on routine prenatal ultrasound. Fetal ultrasound revealed the absence of ductus venosus (DV) with the UV directly draining into the right atrium (), consistent with the extrahepatic drainage type of ADV. The cardiothoracic area ratio was 36.5%, within the normal range of <40% () and heart valve regurgitation was absent. No cardiac structural abnormalities were detected, and cardiac functional parameters were normal. The parents did not desire fetal karyotyping, and, thus, amniocentesis was not performed. Direct UV flow into the systemic venous circulation (the right atrium) usually causes volume overload of the right heart, and thereby right heart failure, whose signs were carefully monitored, but they were not observed.\nAt 38+3 weeks, she showed the spontaneous onset of labor and vaginally gave birth to a 3,096-gram male infant (Apgar score 7/8 [1/5 min]). Neonatal cardiac ultrasound revealed mild aortic valve regurgitation and a slightly decreased ejection fraction, which were transient and disappeared on day 7. Detailed ultrasound examination revealed a defect of the hepatic rectangular leaf (S4: one of the largest liver leaves) at half a month postnatally. No findings indicative of liver dysfunction were observed throughout his course. Computed tomography at 1 year of age revealed atypical liver rotation with a Morgagni hernia in the liver (). He showed normal development at 1.5 years of age.","A 41-year-old woman was referred to us because an ultrasound showed her baby’s heart was slightly larger than normal. The ultrasound also revealed a unique way the baby’s blood vessels were connected, which is usually harmless. She went into labor at 38+3 weeks and gave birth to a healthy baby boy. After birth, the baby’s heart function was monitored closely and returned to normal within a week. Later, a small liver defect was found, but the baby is developing normally and is doing well."
741,"A 4-year-old boy was transferred to our pediatric intensive care unit from an outside hospital for further management of a persistent seizure disorder of unknown etiology. A right femoral triple lumen central venous line (CVL) had been placed prior to transfer. Five days after arrival, the patient began to exhibit increased swelling in his right lower extremity, and ultrasonography revealed a catheter-related, acute occlusive deep venous thrombosis in the right common femoral vein. He was started on LMWH (enoxaparin) at 1 mg/kg for a planned course of 3 months. Five days after initiating treatment, the CVL was removed. The patient had no personal or family history of thrombophilia or bleeding diathesis. His anti-Xa level, checked after the second dose, was within the therapeutic range.\nHis hospital course was complicated by multisystem organ failure in the setting of drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome secondary to anticonvulsive therapy. One week after starting LMWH heparin, the patient experienced gross hematuria. The next day, the injection sites were noted to be slightly oozy, and, in the setting of his anti-Xa levels continuing to rise (0.87), LMWH heparin was held. He required continuous venovenous hemofiltration, during which time anticoagulation was switched to unfractionated heparin. After renal recovery, LMWH therapy was restarted at a lower dose (70% of original dose), but his anti-Xa levels continued to be labile and difficult to control. Eventually, a steady regimen was found with consistently stable and therapeutic anti-Xa levels ().\nOn the 15th day of this regimen, however, he developed signs of bowel obstruction with new onset of copious bilious vomiting. An abdominal ultrasound found a small amount of fluid in the pelvis. A CT of the abdomen and pelvis showed a high-grade small bowel obstruction, with 2 areas of small bowel, suspicious for intussusception (Figures and ). The patient's hemoglobin was found to have dropped from 9.9 to 6.2.\nHe was brought to the operating room for exploratory laparotomy. Intraoperatively, bloody ascites and multiple dilated loops of small bowel were found. Approximately 30 cm of the distal jejunum was found to be tense, heavy, firm, and discolored with a blue hue, with a hematoma that had dissected through the layers of the bowel (). A serosal defect was found on the antimesenteric border of the involved bowel, likely causing the bloody ascites. As the bowel was severely compromised, a resection of the involved segment was performed.\nPathologic analysis of the resected bowel showed an extensive, 35 cm submucosal hematoma causing internal bulging of the mucosa and submucosa, causing attenuation and focal pressure necrosis of the muscularis propria. The serosa was noted to be diffusely purple-red, with a small ovoid defect that was presumably caused by the hematoma. At both ends of the resected bowel, there was evidence of the mucosa and submucosa bulging into the lumen of the bowel as a result of the hematoma.\nThe patient recovered from the operation in the pediatric ICU with no further episodes of emesis or signs of bowel obstruction. He was restarted on LMWH 5 days later and completed his 3-month course without any further signs of bleeding. He was discharged from the hospital 3 weeks later with resolution of his seizures to a rehabilitation facility, without any further complications of bleeding or bowel obstruction.","This 4-year-old boy was brought to the hospital because he had seizures and was getting worse. Doctors found a blood clot in his leg related to a tube they put in him, and he needed special treatment to prevent more clots. Unfortunately, he also developed a serious bowel problem that required surgery to remove a section of his small intestine. After the surgery, he recovered well and was able to go home to continue his rehabilitation."
742,"40-year-old male patient was referred to our department with two-month history of ocular focusing deficit without any signs or symptoms suggestive of thyroid dysfunction. Past illness or family history did not reveal any presence of thyroid-related diseases. He has not been taking any medication but has consumed 20 cigarettes a day for 20 years.\nOphthalmological examination has revealed double vision on upward gaze with disturbance in upward movement of the left eye, eyelid retraction, and exophthalmos of the left eye. Intraocular pressure and visual acuity were normal. The exophthalmoses on the right and left sides were 15mm and 19mm by Hertel exophthalmometer (normal range: 10~15mm with laterality of less than 3mm for the Japanese). Clinical activity score (CAS) of the ophthalmopathy was 2 with redness and swelling of the eyelid.\nAlthough no physical sign of thyroid dysfunction was observed, thyroid function tests were performed since Graves' ophthalmopathy was suspected. Plasma FT3, FT4, and TSH levels were as 2.75pg/mL, 1.38ng/dL, and 0.934μIU/mL, respectively, and were within the normal range (). Thyroid peroxidase antibodies (TPOAb), thyroglobulin antibodies (TgAb), and TSH receptor autoantibodies (TRAb) were all negative. Only TSAb was slightly positive: 146% (normal range ≦120%) (). Rheumatoid factor was negative and Immunoglobulin (Ig) G, IgA, and IgM levels were all within normal range. Although C3 or C4 levels were also within normal range, CH50 was slightly higher (59.4 U/mL) than the normal range (32-48 U/mL). Kidney and liver functions were within normal limit. HBV, HCV, HTLV-1, and HIV were negative.\nUltrasonography of the thyroid gland was performed. It showed normal-sized gland with slightly enhanced blood flow (). To directly measure thyroid activity in vivo, 99mTc scintigraphy was then performed. The result was again negative with 99mTc uptake of 0.50% for the right gland, 0.39% for the left gland, and total 0.89% (normal <5% in total) (). These serological and imaging studies of the thyroid has scarcely given positive evidence for the diagnosis of Graves' disease in this patient.\nWe next performed imaging analysis of the orbital cavity. CT scan showed obvious enlargement of the inferior rectus muscle of the left eye (). MRI images of the orbit again showed inferior rectus muscle swelling of the left eye (). Fat-suppression T2-weighted images showed a slight increase in the MR intensity of the muscle involved (), suggesting active inflammation of the extraocular muscle. No tumorous lesion was detected within the orbital cavity (Figures and ).\nWith these findings, the differential diagnosis of this case were (1) euthyroid Graves' ophthalmopathy with a slight increase in TSAb alone or (2) idiopathic inflammation of the extraocular muscle []. Based on the insidious onset, absence of ocular pain, predominance of inferior rectus muscle swelling, and apparent muscular swelling with minimally swollen tendons [–], we diagnosed this case as TRAb-negative euthyroid Graves' ophthalmopathy.\nUnder the diagnosis, he was hospitalized and treated with the combination of corticosteroid pulse therapy and radiation therapy of the orbit (). Intravenous administration of methylprednisolone 1000mg/day for 3 days followed by oral prescription of prednisolone 30mg/day for 4 days were undertaken as the first cycle of the medical therapy. He underwent this medical therapy for consecutive three cycles with a slight modification of decreased methylprednisolone dose (500mg/day) in the third cycle due to a slight elevation of the liver function tests. After the third cycle of the therapy, he was given oral prednisolone as an out-clinic patient. The corticosteroid doses were decreased by 5mg every two weeks until cessation. On the second day of the first cycle of the corticosteroid pulse therapy, daily orbital radiation therapy of 2Gy/day was initiated and performed for ten days (20Gy total). Although the swelling of the left eyelid disappeared on day 3 (), proptosis, abnormality in ocular movement, and diplopia were all unchanged until discharge. Examination by the exophthalmometer revealed the remaining of the exophthalmoses of 16mm on the right and 18mm on the left when he left hospital on day 18 (). Thyroid function remained within the normal range throughout the clinical course and TSAb turned negative (113%) four months after the initiation of corticosteroid therapy.","This 40-year-old man has been experiencing blurry vision and double vision when looking upwards, along with some swelling around his left eye. After a thorough eye exam, we found that his eye muscles are inflamed, likely due to a condition called euthyroid Graves’ ophthalmopathy, which can happen even if his thyroid levels are normal. Blood tests and scans showed no signs of typical thyroid problems, but we found a slightly elevated antibody level. To help reduce the inflammation, he received a course of steroids and radiation therapy to his eye, which helped to reduce the swelling and improve his vision. He is now being monitored closely and will continue to take oral steroids to manage the condition."
743,"A 40-year-old African American male presented with complaints of generalized weakness, unintentional weight loss (60 pounds over one-month period), cough, night sweats, and nonbloody, watery diarrhea of approximately four weeks' duration. The patient's medical history was additionally significant for hypertension and polysubstance abuse including tobacco (10 pack/year smoking history) and marijuana. He reported prior history of incarceration. He denied recent travel or animal exposures at home. He resided with his mother, for whom he was the primary caregiver. He denied having sexual activity within the past 6 months. Initial vitals in the emergency department were significant for tachycardia with HR in the 120's. Physical examination at the time of admission revealed a thin, nontoxic appearing male. Cardiac exam revealed tachycardia, with no murmurs or rub. Lung exam revealed decreased breath sounds in the bilateral lower lung fields with tubulovesicular sound emanating from right upper lung field. His abdomen was soft and nontender. He had no focal neurologic deficits. Initial laboratory workup was significant for absolute CD4 count of 26 (3%). Urinalysis showed cloudy urine with 1+ blood, positive nitrite, 3+ leukocyte esterase, WBC >50/HPF, RBC 3-9/HPF, and many bacteria. Chest X-ray (CXR) (PA and lateral views) in the emergency department revealed a cavitary lesion with an air-fluid level within the anterior medial right hemithorax and a loculated hydropneumothorax along the right lateral lung base (). CT chest with contrast demonstrated two large, thick-walled cavitary lesions originating within the right lung parenchyma that appeared to communicate. The smaller lesion measured up to 5 cm and the larger lesion contained an air-fluid level. This was interpreted as demonstrating a complex bronchopleural fistula and associated empyema (). His treatment was initiated with intravenous Ceftriaxone and Metronidazole. The patient's stool PCR isolated a Salmonella species. Diagnostic thoracentesis yielded purulent fluid with WBC 505,000 (73% segmented neutrophils, 8% lymphocytes, and 19% macrophages), RBC 0, pH 6.0, protein 3.7, LDH 41,239, and glucose 12. Pleural fluid culture was positive for Salmonella species. Due to presence of empyema, a right-sided chest tube was placed followed by instillation of tissue plasminogen activator (r-tPA) and DNase twice daily for three consecutive days. His urine culture yielded nontyphi Salmonella. CT abdomen and pelvis with contrast demonstrated two rim-enhancing hypodense lesions (measuring 2.9 x 3.8 cm transverse and 3.2 x 4.5 cm transverse) within the central mesentery of the abdomen (). Intra-abdominal drains were placed under CT guidance into the mesenteric abscesses, from which nontyphi Salmonella eventually grew late in hospital course. Due to immunocompromised status with cavitary lesions on imaging, acid fast bacilli (AFB) smears were obtained along with interferon gamma release assay which were negative. On hospital day #4 the patient's right-sided chest tube was noted to have persistent air leak. CT chest without contrast confirmed persistent right-sided hydropneumothorax with centrilobular ground glass opacities with lung entrapment. On hospital day #10, the patient underwent right video-assisted thoracoscopic surgery (VATS) with decortication. Pleural peel pathology revealed pleural fibrosis, focal chronic inflammation, and mild anthracosis. The patient completed an additional two weeks of antibiotic therapy with intravenous Ceftriaxone from the date of decortication. On follow-up at four-months, CT chest demonstrated improved but persistent loculated right pneumothorax with resolution of the right lung cavitary lesions. At seven months, the CT demonstrated complete resolution of the right-sided pneumothorax ().","This 40-year-old man came to the hospital because he was feeling very weak, losing a lot of weight, and experiencing a persistent cough and diarrhea. He has a history of substance abuse and has been caring for his mother. Tests revealed he had a serious infection in his lungs with cavities and fluid buildup, and the bacteria causing the infection was identified as Salmonella. He received antibiotics, drainage of the infection, and surgery to remove fluid and scar tissue from his chest. After several weeks of treatment, the infection cleared, and the lung cavities resolved."
744,"This was a 13 days old baby boy, who was born via spontaneous vaginal delivery at term in our tertiary care hospital without any postnatal complications. He was discharged 24 hours after delivery. He was brought back to our ER with left eye purulent discharge, which was noticed since birth, and swelling of his left upper eyelid of 2 days duration.\nThere was no associated fever or history of decreased level of activity or feeding. There was no history of rashes or seizures.\nThe pregnancy course was remarkable only for gestational diabetes and the fact that the mother had a history of vaginal discharges, which was treated as vaginal candidiasis during the last trimester. Group B streprococcus screening on the 37th week of gestation was negative. Similarly, HIV and hepatitis B serology were negative one day prior to delivery. There was no maternal history of genital lesions, vesicles, or ulcers.\nExamination was normal apart from the purulent eye discharge & swelling of the left eye upper eyelid. The eye secretions were yellowish sticky, copious, and profound. Fontanelles were soft & primitive reflexes were present and normal.\nDue to suspicion of gonococcal ophthalmia neonatorum, a full septic workup was obtained including CBC, blood culture, urine analysis and culture, CSF analysis and culture, and left eye swab for culture and Chlamydia antigen (). He was subsequently started on meningitis dose of Cefotaxime, in addition to Gentamycin ophthalmic drops while waiting for the previous cultures' results. Azithromycin was added as well to cover the possibility of an associated chlamydial infection.\nThe eye swab culture revealed Neisseria gonorrhea, which was sensitive to Cefotaxime, so the antibiotic was continued while waiting for the results of the CSF culture.\nBlood and urine cultures were negative. The CSF culture revealed Gram-negative rods after one day, which was identified as S. maltophilia on day 5 of admission. The organism was sensitive to Trimethoprim-Sulfamethoxazole (TMP-SMX).\nOnce the diagnosis of S. maltophilia meningitis was identified, Cefotaxime was stopped and the baby was started on TMP-SMX and Ciprofloxacin. Since there are no clear guidelines on how to treat S. maltophilia meningitis in neonates, we extrapolated our management plan from that of other Gram-negative meningitis. Therefore, CSF was repeated at 2 days of antibiotics to confirm sterility. Because S. maltophilia meningitis is very rare and there are no clear guidelines on the duration of therapy, the treating team decided to repeat CSF studies one more time toward the end of the third week of antibiotics. The last CSF studies were completely normal, and the culture was negative.\nThe baby's head circumference was measured daily during the hospital stay and remained normal. Cranial ultrasound scan was normal.\nThe little boy recovered from the infection uneventfully and a follow-up visit of the baby 1 week after discharge was reassuring. His parents received treatment for gonorrhea and they were screened for other sexually transmitted diseases.","This 13-day-old baby was brought to the hospital because of a yellow, sticky discharge from his left eye and swelling around it, which had started at birth. The baby’s mother had a history of vaginal discharges, and tests showed he had a bacterial infection called gonorrhea in his eye. Blood and urine tests were normal, but a sample from his spinal fluid revealed a different bacteria, S. maltophilia, which was treated with different antibiotics. After several weeks of treatment, the baby’s infection cleared up, and he was discharged home with his parents, who were also screened for other infections."
745,"A 33-year-old male taxi driver with past medical history significant only for alcoholism, presented to his family physician's office with the chief complaint of left knee pain and swelling for nine months. He denied any specific injury, puncture wound, or any systemic symptoms. He denied contact with animals. Examination of his left knee demonstrated a moderate-to-severe effusion. He was not tender to palpation across his bony prominences. He had no medial or lateral joint line tenderness. He had a range of motion that was full and symmetric with that of the contralateral side. He had a negative McMurray's sign. His knee was stable to ligamentous examination. There was no erythema or lymphadenopathy. X-ray of the left knee showed a joint effusion. MRI of his left knee also demonstrated a joint effusion with a popliteal cyst and synovial thickening ().\nHe subsequently underwent aspiration of the effusion in the office. Synovial fluid was cloudy yellow and blood-tinged. Fluid analysis showed the following: RBC: 50,200 cells/mcL, WBC: 4900 cells/mcL (ref range < 150/mcL), PMN: 34% (ref range 0–25%), lymphocytes: 54%, and monocyte: 10%; no crystals were observed, and pathological exam was consistent with hemorrhagic fluid with acute and chronic inflammation. Bacterial culture did not demonstrate any growth, but fungal cultures grew branching narrow hyphae with septations and conidia in a bouquet-like appearance leading to a presumptive diagnosis of Sporothrix schenkii (). The patient was admitted to the University of Kansas Hospital for further management.\nThe patient underwent diagnostic left knee arthroscopy, irrigation, debridement, and major synovectomy. There was no internal derangement of the knee. There was no chondral injury. He had no evidence of a medial-lateral meniscus tear. His anterior cruciate ligament was intact. There was significant synovial thickening throughout. Blood counts, liver biochemistry, coagulation parameters, and renal and thyroid functions were normal except for elevated liver function tests consistent with his history of alcohol use. ESR and CRP were within normal limits, HIV test was negative, and immunodeficiency workup (humoral immunodeficiency, phagocytic disorders, and T-cell immunodeficiencies) was unrevealing. Abdominal CT done to evaluate for disseminated disease showed marked diffuse hepatic steatosis. After the debridement, the patient was started on oral itraconazole 200 mg twice daily with a plan to treat for 12 months.\nSynovial biopsies obtained during debridement from the suprapatellar pouch were sent for both microbiology and pathology. Surgical cultures on Sabouraud dextrose agar grew Sporothrix schenkii within four days confirming the initial diagnosis. The identification was confirmed by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry—MALDI-TOF MS (Bruker Daltonics Biotyper Microflex LT).\nAll other microbiological investigations of effusion samples including routine bacterial cultures, acid-fast bacilli (AFB) cultures, were ultimately negative for any growth. Blood cultures multiple times did not reveal any growth. Pathology of the synovial tissue showed prominent mixed chronic inflammation composed of plasma cells and lymphocytes. There were scattered granulomas with central necrosis. Fite's acid-fast staining was negative for acid-fast bacilli. GMS stain was negative for fungal organisms ().\nAntifungal susceptibility assays were performed by the broth microdilution method according to the guidelines recommended by the Clinical Laboratory Standards Institute (CLSI): documents M38-A2 (CLSI, 2002b) ().\nDuring his subsequent follow-up visits, he reported that all his symptoms (knee swelling and pain) had improved with itraconazole, but unfortunately, he continued to drink excessive amounts of alcohol.","This 33-year-old man with a history of alcohol use came to the doctor with a painful, swollen knee that had been bothering him for months. After tests, including an MRI and fluid aspiration, doctors found he had a joint infection caused by a fungus called Sporothrix. He underwent surgery to clean out the infected joint and was started on medication to treat the infection. The surgery was successful, and his knee pain improved, but he continues to struggle with his alcohol consumption, which is impacting his recovery. Further tests ruled out other causes of his symptoms, and the doctors are monitoring his progress closely."
746,"A 32-year-old man was referred to the Lithuanian University of Health Sciences Kaunas Clinics Hospital with the symptoms of throat discomfort on the left side and dysphagia. The symptoms persisted for approximately 2 months. At arrival, the patient had no fever and there were no other signs of acute infection. Anamnestically, the patient was treated with antibiotics due to a suspected peritonsillar abscess on the left side for a period of 1 month. His left peritonsillar area was repeatedly punctured. However, only blood was obtained with a puncture. The prescribed antimicrobial therapy was not effective—dysphagia progressed, the patient started to report more speech difficulties, his lower jaw became numb, and taste dysfunction appeared. During pharyngoscopy, a dislocated lateral pharyngeal wall with mild inflammatory changes of the oropharyngeal mucosa was observed. The palate tonsil was displaced towards the uvula ()\nThe fibronasolaryngoscopic investigation revealed that the left side of the nasopharynx was narrowed by a large mass covered with an intact smooth mucous membrane. No pathology was observed in the larynx—the color of mucosa was normal, and the vocal cords were mobile and smooth. No additional structures were seen. Neck lymph nodes could not be palpated.\nDue to the suspected pharyngeal tumor, the patient underwent a contrast-enhanced computed tomography (CT) study, which showed a clearly limited, oval-shaped lesion in the left parapharyngeal space ().\nThe size of the tumor was 4.2 × 3.3 × 6.7 cm. It was characterized by a nonhomogeneous structure with multifocal intratumoral hemorrhages of varying ages. The tumor encased the carotid arteries and the styloid process, while it stretched the pterygoid muscles on the left side and remodeled the pterygoid processes of the sphenoid bone. The medial part of the tumor pushed the palatal tonsil and uvula towards the centerline, as well as the root of the tongue to the front and the middle. Moreover, it significantly deformed the oropharyngeal and nasopharyngeal cavities. The upper part of the tumor ascended and tapered to the bone surface of the base of the skull and extracranial oval foramen. The lower pole of the tumor reached the submandibular salivary gland level and dislocated it slightly laterally.\nTo clarify the diagnosis, the patient underwent a magnetic resonance imaging (MRI) study. The study showed that due to its localization and tumor-specific features, the most likely diagnosis was schwannoma of the small branch of the mandibular nerve (V3) since a limited formation of specific localization with a component of cystic degeneration was found. Deformed from the medial part, the lateral pterygoid muscle with the V3 is shown in .\nTo determine a final diagnosis and plan a further treatment, a biopsy with histological verification of the tumor was performed. Under local anesthesia, a punch biopsy was carried out. A histological examination confirmed the diagnosis of schwannoma. Transoral removal of the tumor was planned. During an angiographic study prior to the surgery, the tumor-feeding blood vessels, of which the main vessel was the left ascending pharyngeal artery, were identified and embolized ().\nAfter the preparation of the patient, the schwannoma extirpation through a 5 cm incision at the left wall of the pharynx from the hard palate to the root of the tongue was performed under general anesthesia, while the widening of the oropharynx was done with a throat gag. The tissues were bluntly separated and the tumor capsule was reached, while the bloodstream of the surrounding area was externally disconnected. The tumor was removed in parts, thus reducing its volume. Later, it was totally removed with a capsule. No postoperative complications were observed. The wound healed by primary intention. The patient was discharged for further outpatient treatment on the sixth postoperative day. Antibiotic therapy with penicillin and painkillers was prescribed postoperatively.\nHistological examination of the operating material revealed a tumor that formed by monomorphic, mitotically inactive spindle-shaped cells with oval nuclei and an eosinophilic cytoplasm, which were positive for S100 calcium-binding protein P ().\nCells were either fascicular or palisade in structure. Larger individual cells with large and irregularly shaped nuclei were found. Furthermore, thick-walled blood vessels, xanthoma accumulations of macrophages, and abundant groups of hemosiderophages with several foci of necrosis were also visible. The diagnosis of schwannoma was confirmed.\nSix months postoperatively, no tumor relapse was observed during either physical examination or the repeated CT study ().\nCurrently, the patient has been observed for a period of two years. There are no signs of schwannoma relapse.","This 32-year-old man was referred to the hospital because he was having trouble swallowing and discomfort in his throat, which had been going on for two months. Doctors discovered a large tumor in the space behind his throat that was pressing on nearby nerves and structures. Imaging tests, including CT scans and an MRI, confirmed that it was a benign tumor called a schwannoma. The tumor was successfully removed through a surgical procedure, and he is currently doing well with no signs of recurrence after two years of follow-up."
747,"A 62-year-old Columbian female was diagnosed in 2008 with multiple myeloma (MM). Flow cytometry revealed an IgG kappa monotypic plasma cell population, expressing CD33, CD38, CD45, CD56, CD117, CD138, and kappa light chains. The plasma cells were CD19 negative. IgG was measured at 6390 mg/dL, and immunoglobulins of all other types were decreased. Hematopathology revealed extensive bone marrow involvement (90%) by plasma cells, nearly absent iron stores, moderate normocytic normochromic anemia, reticulocytopenia with prominent rouleaux formation, moderate thrombocytopenia, and absolute lymphopenia. Cytogenetics revealed an abnormal hyperdiploid karyotype including a der(19)t(1q;1p) chromosome with additional copies of CCND1, RB1, and LAMP1, loss of chromosome 17 centromere, and p53 gene disomy. The patient was first started on lenalidomide and dexamethasone and progressed despite multiple chemotherapeutic regimens including bortezomib and dexamethasone, additional cycles of lenalidomide and dexamethasone, melphalan with thalidomide and prednisone (MPT), and bortezomib and dexamethasone. The patient reported complete adherence to treatment.\nThroughout the course of treatment, the patient was admitted several times with right flank pain, hematuria, and persistent hypercalcemia. The patient also had a notable past medical history of CKD secondary to myeloma kidney, and asthma.\nThe patient was admitted for severe hematuria and epistaxis in February 2011. The patient was also hypercalcemic and hyperkalemic and received both zoledronic acid and kayexalate. At that time, the patient underwent an extensive coagulation profile including screening for lupus anticoagulant, antiphospholipid syndrome, paraproteins, and factor levels. Ristocetin cofactor assay revealed a level of 46% (normal 50%–150%), indicating the absence of a Von Willebrand Factor deficiency. Von Willebrand Factor Antigen was >300 IU/dL (normal 60–150 IU/dL). Notably, thrombin time (TT) was elevated to 32.3 sec (normal 15–19 sec). The etiologies considered included increased fibrin split products, dysfibrinogenemia or a deficiency of fibrinogen, heparin treatment, and heparin-like molecules, to name a few. Fibrinogen was 228 mg/dL (normal 175–400 mg/dL), and fibrin split products were <10 mcg/mL (normal <10 mcg/mL), which also suggested the presence of another inhibitor. Factor Xa screen was 0.1 U/mL (normal 0.3–0.7 U/mL), which indicated the absence of UFH and LMWH leading to coagulopathy. Abnormalities of fibrinogen were also ruled out with a normal reptilase time of 22 sec, and control measured during reptilase time testing was 21 sec. PT was 12.0 sec (normal 9.6–11.5 sec), and INR was 1.1 (normal < 1.2). A mixing study was conducted to investigate for the presence of inhibitors; PTT (patient) was 44.3 sec (normal 25–32 sec) and was only partially corrected with mixing; PTT of control plasma was 27.8 sec, PTT Mix 1 : 1 control and patient was 34.2 sec, PTT control following 2 hour incubation was 29.4 sec, and PTT Mix 1 : 1 with 2 hour incubation was 36.5 sec (25–32 sec). Notably also, factor VIII activity was elevated at 273% (normal 45–150%), which indicated an increased risk for venous thrombosis. Continuous protamine sulfate IV of 2–7 mg/hour was given, which led to notable improvement and subsequent resolution of our patients' epistaxis and hematuria. A hematoma formed after a bone marrow biopsy was performed on the same admission, for which surgical consultation was requested. No intervention was done, and resolution of the hematoma occurred spontaneously.","This 62-year-old woman has been battling multiple myeloma since 2008, a cancer that affects plasma cells. Despite multiple treatments, including chemotherapy and supportive care, her condition has remained challenging, with recurring symptoms like flank pain, blood in her urine, and high calcium levels. Recent tests revealed a blood clotting problem, likely due to the myeloma itself, and elevated factor VIII, increasing her risk of blood clots. The doctors are continuing to monitor her closely and adjust her treatment plan to manage her symptoms and improve her overall health."
748,"A 25-year-old female with no prior medical history presented to the emergency department due to high fever (up to 39°C) since 3 days. She complained of left flank pain and gross hematuria. On physical examination, left costovertebral angle tenderness was noted. The urinalysis confirmed the hematuria (2055 red blood cells per high-power field). The urinary dipstick was negative for leukocyte esterase and for nitrites, but significantly positive for albuminuria (2+). On microscopic examination of the urine, pyuria was minimal (6 white blood cells per high-power field) and no bacteriuria was noted. She denied having taken antibiotics before presentation. Urine and blood cultures were obtained. The laboratory tests revealed a significantly elevated C-reactive protein (CRP = 28 mg/dl), a high erythrocyte sedimentation rate (107 mm/h), and an elevated creatinine (1.21 mg/dl) with normal blood urea nitrogen (16 mg/dl).\nShe was admitted to the internal medicine ward with a preliminary diagnosis of acute pyelonephritis, and she was started on intravenous ceftriaxone. A contrast-enhanced computed tomography the next day revealed a hypoenhancing region in the upper pole of the left kidney, suggestive of pyelonephritis (). However, considering the significant hematuria in the absence of pyuria and bacteriuria, and the persistently elevated creatinine (1.55 mg/dl on day 3), a nephrologist was consulted. Microscopic evaluation of the urinary sediment revealed dysmorphic red blood cells suggesting glomerular disease (2 red blood cell casts and 60–80 red blood cells per high-power field with >10% of G1 cells and >80% dysmorphic erythrocytes). The spot urine protein to creatinine ratio obtained on the 5th day of hospital stay was also elevated (929 mg/g). Furthermore, both urine and blood cultures came back negative, and no fever was recorded during the hospital stay. Ceftriaxone was discontinued after 7 days of treatment.\nA biopsy of the left kidney was performed at the 6th day of hospital stay and confirmed a diagnosis of IgA nephropathy (immunofluorescence was strongly positive for mesangial IgA deposition, complement component C3, and lambda light chains and moderately positive for IgM). The patient denied any recent respiratory tract infection symptoms. Other lab tests sent during her hospital stay were normal (lactate dehydrogenase, anti-neutrophil cytoplasmic antibodies, antinuclear antibodies, anticardiolipin IgG and IgM, anti-beta-2 glycoprotein I IgG and IgM, protein electrophoresis, serum complement C3c and C4 level, rheumatoid factor, and transesophageal echocardiography). She was started on lisinopril 5 mg daily. Furthermore, considering the significant proteinuria, the elevated creatinine and the negative prognostic features of the biopsy, (M1E1S1T0C1 according to the Oxford classification []) she was started on glucocorticoids (three-day pulse of methylprednisolone 1g in months 1, 3, and 5 in addition to oral prednisolone 0.5 mg/kg every other day for 6 months) []. At follow-up at 2 months, both creatine (0.86 mg/dl) and the spot urine protein to creatinine (114 mg/g) were normal. CRP at follow-up was 0.4 mg/dl.","A 25-year-old woman went to the hospital with a high fever, flank pain, and blood in her urine. Tests showed she had kidney problems and protein in her urine, and her kidney function was slightly reduced. After further investigation, doctors discovered she had IgA nephropathy, a condition causing inflammation in the kidneys. She was treated with medication and monitored closely, and her kidney function has since returned to normal."
749,"The body of an 84-year-old man was received under the Saint Louis University (SLU) Gift Body Program of the Center for Anatomical Science and Education (CASE) with an informed consent from the donor. Records indicate this man's cause of death was a gastric carcinoma. During routine dissection, a bifid penis was observed. At first glance the phallus resembled that of epispadias, but there were no defects or repair in the external abdominal wall. The pubic hair was sparse and fine. The phallus was 9.2 cm. long, divided longitudinally into right and left parts (). Each part had its own glans and prepuce. There was no urethra in the phallus. Rather, a urethral meatus was located at the base of the divided phallus () which is indicative of proximal penoscrotal hypospadias [, ]. The urethra continued into the normally developed urinary bladder. The epithelial lining of the urinary bladder extended to 3.9 cm. on the ventral surface of each phallus and histologically it resembled stratified squamous epithelium. The scrotum was large with redundant skin and contained left and right testes, from which extended a normally routed spermatic cord. The right testis was 3.0 long and 2.3 cm wide and the left testis was 1.4 cm long and 0.8 cm wide. The spermatic cord on both sides was 1.2 cm in thickness, continued from each testis and passed through the external and internal inguinal rings and took a normal course to end in well-developed seminal vesicles. The seminal vesicles opened into the urethra. The spermatic cord was of normal thickness and size, contained all of the general coverings, but there was no epididymis. The vas deferens continued directly from each testis to the seminal vesicle of the same side. A small mass, measuring 0.5 cm by 0.5 cm, of hard tissue was found only on the left side and was located at the site of the prostate gland but histologically it did not resemble prostatic glandular tissue. It did not have any connection with the vas deferens or seminal vesicle. Dissection of the phallus revealed a corpus cavernosum and corpus spongiosum in each half of the penis and the corpora spongiosa were well developed as in females (). No other abnormalities were observed.\nIn summary, the anatomical examination of the human cadaver described above characterizes the respective case as a complete bifid penis, a subset of human diphallia, which was accompanied by proximal penoscrotal hypospadias. The lack in this case of additional malformations often associated with diphallia (see above) makes it a perfect model for correct assessment of its etiology.","This body was donated to a medical program for anatomical study. The man died from stomach cancer, and during the examination, a unique condition called bifid penis was found, meaning the penis was split into two parts. The split penis had its own separate glans and prepuce, and a urethra was present at the base, leading to a normal bladder. The rest of the male reproductive system, including the testes, spermatic cords, and seminal vesicles, appeared normal."
750,"The patient is a 55-year-old man with past medical history significant for two-year history of umbilical hernia, diabetes mellitus type 2, hypertension, gout, chronic kidney disease with proteinuria, diverticulosis, obesity, and osteoarthritis. The patient presented to the clinic because of umbilical hernia pain, which developed over the two months. The pain localized to the periumbilical region and left lower back, and it was exacerbated with food intake and sometimes relieved by 5 mg hydrocodone tablet. He also reported nausea and fifteen pounds weight loss over the two months, which he attributed to decreased food intake. The physical examination showed a 1 cm tender and irreducible mass superior to the umbilicus. The patient underwent herniorrhaphy and the gross examination of the surgical specimen did not reveal any masses or lesions.\nThe microscopic evaluation showed diffuse infiltration of the connective tissue by malignant cells with hyperchromatic nuclei, inconspicuous nucleoli, and abundant eosinophilic cytoplasm (). There were focal areas of gland formation with mucin production, consistent with adenocarcinoma. By immunohistochemistry, the neoplastic cells were strongly positive for pancytokeratin and CK7 () and negative for CK20, CDX2, TTF-1 and PSA.\nThe laboratory findings showed elevated levels of CA 19-9 (16,590 U/mL) and CEA (14.2 ng/mL). The patient underwent a subsequent computed tomography scan with intravenous contrast, which showed a 5.0 × 2.7 cm ill-defined and hypoattenuating mass located in the pancreatic tail and body (), with peripancreatic fat infiltration and vascular involvement of splenic artery and vein. In addition, the imaging showed peritoneal carcinomatosis, multiple ill-defined hypoattenuating lesions in the liver, and enlarged and hypoattenuating pericecal iliac lymph nodes. The patient had a prior noncontrast computed tomography scan four months earlier, which showed umbilical hernia with fat and no other lesions in the pancreas and abdomen ().\nAfter the diagnosis, the patient refused chemotherapy and decided to undergo palliative care. The patient had rapid progression of the disease and died within two months of the initial histologic diagnosis of malignancy.","This 55-year-old man was diagnosed with advanced cancer after experiencing pain and weight loss related to an umbilical hernia. Tests revealed that the cancer had spread to his pancreas, liver, and lymph nodes. Despite treatment, the cancer progressed rapidly, and he sadly passed away a few months after the initial diagnosis."
751,"A previously healthy 10-year-old Asian girl presented to the emergency department with headache, vomiting, and one week of mild nonproductive cough. Her headache started the evening prior to presentation, was gradual in onset and frontotemporal in location, and improved with acetaminophen but subsequently woke her from sleep. It was accompanied by two episodes of emesis. On presentation to the ED, the patient described her headache pain as 3 out of 10 in severity. She denied photophobia, had no further nausea, and denied abdominal pain. She reported that the headache worsened with standing and improved with lying down. Review of systems was significant only for pallor.\nThe patient was otherwise healthy with no prior medical issues and taking no regular medications. She was fully vaccinated and had no known allergies. Her family history was significant for frequent headaches in her mother and maternal aunt. She was living with her parents and brother and attending 4th grade.\nVital signs demonstrated blood pressure 111/56, pulse 104, temperature 37.1°C, respiratory rate 22, and oxygen saturation 100% on room air. Initial exam revealed a well-appearing female and was unremarkable including a normal fundoscopic exam and a normal complete neurologic exam.\nThe patient received ibuprofen and oral rehydration and her headache further improved. A presumptive diagnosis of migraine headache was made and was discharged with primary care follow-up the following day.\nTwo days after her initial emergency department visit, the patient returned to the ED with worsening headache, myalgia, subjective fever, and diffuse weakness. The patient's mother reported that the patient was unable to stand or walk and as a result her mother had been carrying her, including to and from the bathroom. The patient endorsed nausea but no further vomiting.\nVital signs demonstrated blood pressure 105/49, pulse 123, temperature 36.9°C, respiratory rate 30, and oxygenation saturation of 97% on room air. On exam, the patient was moderately ill appearing, lying in bed responding slowly to questions. Her lips were noted to be cracked and with some oozing blood. No oral lesions were noted in the mouth. Pupils were equal, round, and sluggishly reactive bilaterally. Neck was supple with no adenopathy noted. Cardiovascularly, she was noted to be tachycardic with a regular rhythm and II/VI flow murmur. Her respiratory exam was normal. Her abdominal exam was benign with no organomegaly. Neurologically the patient was noted to be slow to respond to questions and moving slowly but without focal deficits. She was, however, unable to walk without assistance. Skin exam revealed diffuse ecchymoses on the lower extremities bilaterally.\nLaboratory studies were ordered along with a rapid brain MRI, and pediatric neurology was consulted.\nLaboratory results were as follows: hemoglobin 2.6 g/dL, hematocrit 8.3%, platelets 10K/uL, and WBC 60.5K/uL with 83% blasts in the differential. CRP was 15.7 mg/L, and ESR was 125 mm/h. Electrolytes showed sodium 139 mmol/L, potassium 4.0 mmol/L, chloride 100 mmol/L, carbon dioxide 24 mmol/L, BUN 11mg/dL, creatinine 0.56 mg/dL, glucose 129, magnesium 2.5mg/dL, and phosphorus 3.6 mg/dL. LDH was 359 U/L, uric acid was 2.3mg/dL. PTT was 29.4 seconds, PT was 19.5 seconds, and INR was 1.7. Fibrinogen was 117 mg/dL and D-dimer was >10,000 ng/mL. Review of peripheral blood smear () demonstrated many primitive cells with round and lobated nuclei, numerous cytoplasmic granules with Auer rods readily identified, and some cells with multiple Auer rods.\nRapid MRI Brain () was obtained which demonstrated leptomeningeal enhancement in the supratentorial parenchyma suggestive of leptomeningeal carcinomatosis, a hemorrhagic lesion in the corpus callosum, multiple subdural hematomas with mild mass effect, and petechial hemorrhages throughout the brain.\nPediatric Oncology was consulted and treatment initiated emergently with ATRA, dexamethasone, allopurinol, cefepime, and blood products including packed red blood cells, platelets, and cryoprecipitate. The patient was subsequently admitted to the pediatric intensive care unit.\nIn the pediatric ICU the patient received several transfusions with platelets, cryoprecipitate, and fresh frozen plasma to manage DIC. She was continued on an induction course of chemotherapy including ATRA, dexamethasone, idarubicin, and arsenic trioxide. Molecular analysis of the peripheral blood was positive for PML-RARA, confirming the diagnosis of APL. After the coagulopathy improved and the patient stabilized, lumbar puncture was performed with administration of intrathecal chemotherapy. The cerebrospinal fluid was notable for the presence of leukemia cells, confirming the involvement of the central nervous system. The patient's encephalopathy gradually improved and she returned to her baseline mental status by day 7 of treatment. The patient was noted to have elevated opening pressure on lumbar punctures, which worsened over the course of induction, attributed to pseudotumor cerebri secondary to ATRA.\nUpon completion of the first 28 days of induction therapy, repeat lumbar puncture and bone marrow studies demonstrated no morphologic evidence of acute promyelocytic leukemia, consistent with remission.","This 10-year-old girl initially came to the hospital with a headache and some mild cough, which the doctors thought was a migraine. She felt better after taking medicine and was sent home for follow-up. However, she returned a couple of days later with a much worse headache, feeling weak, and unable to walk. Tests showed she had a very high number of white blood cells and low blood counts, and a scan of her brain showed signs of leukemia. Doctors quickly started her on chemotherapy and supportive care, and she responded well to treatment. Further tests confirmed that she was in remission from leukemia. She is now recovering in the pediatric intensive care unit and is doing much better."
752,"A 56-year-old man was admitted to our hospital after resection of a lymph node in his groin revealed adenocarcinoma. Contrast-enhanced computed tomography (CT) showed a 9-cm mass extending from the bladder to the umbilicus, along with intraperitoneal nodules suggesting peritoneal dissemination (Figures , , and ). Cystoscopy showed an extrinsic mass located on the dome. Serum assays showed high levels of carcinoembryonic antigen (CEA), to 16.3 ng/mL, and carbohydrate antigen 19-9 (CA19-9), to 230.9 U/mL. The patient was diagnosed with urachal carcinoma with suspected peritoneal dissemination and was started on systemic chemotherapy with intravenous gemcitabine (1000 mg/m2 on days 1 and 8 of each 21-day cycle) plus cisplatin (70 mg/m2 on day 2 of each cycle). After two cycles, a CT scan showed no marked changes in the lesion; after four cycles, his serum CEA and CA19-9 concentrations had decreased to 4.2 ng/mL and 76.1 U/mL, respectively. However, after five cycles, his CEA concentration had increased to 8.3 ng/mL and his CA19-9 concentration had also increased to 304.1 ng/mL with a CT scan showing changes in the tumor and the appearance of abdominal fluid (). Because of the considered histological and clinical similarities between colorectal and urachal carcinoma, his treatment was changed to FOLFIRI (i.v. infusion of 180 mg/m2 irinotecan, 200 mg/m2 ℓ-leucovorin, and 400 mg/m2 5-fluorouracil (5-FU) on day 1 of each 14-day cycle, followed by continuous infusion of 2400 mg/m2 5-FU for 46 hours) after receiving informed consent. After 11 cycles of FOLFIRI, serum tumor marker levels had not changed markedly, but a CT scan showed a reduction in tumor size to 7 cm () and no new distant metastases. Because chemotherapy was able to suppress tumor progression, a surgical approach was chosen, and complete resection of the tumor, along with partial cystectomy and pelvic lymph node dissection, was performed. The tumor was found to adhere to surrounding organs with mucinous fluid, with disseminated nodules present in the greater omentum. The tumor was removed, along with surrounding adherent organs, including the bladder dome, omentum, peritoneum, abdominal rectus muscle, and vermiform appendix. Cytology showed that the mucinous fluid in the abdominal cavity was class V and the final pathological diagnosis was urachal mucinous adenocarcinoma with invasion of the omentum and peritoneal dissemination (). There were no lymph node metastases, and surgical margins were negative. The patient was discharged from the hospital 14 days after surgery with no complications. Soon after surgery, his serum tumor markers had normalized. Tumor marker levels were measured every month and CT scans performed periodically until six months after surgery. Subsequent follow-up included CT scans and tumor marker level measurement every three months for the first three years and every six months thereafter. At the time of writing this report, i.e., 62 months after surgery and without any adjuvant chemotherapy, the patient remains alive. Although he showed a slight increase in serum CEA level to 6.3 ng/mL, his serum CA19-9 level remained within normal limits (), and no radiological recurrence has been detected.","A 56-year-old man was admitted after surgery to remove a tumor from his groin, which was found to be a type of cancer called urachal carcinoma. Tests showed the cancer had spread to other parts of his abdomen, and he started chemotherapy. After several cycles, the chemotherapy helped to shrink the tumor, and he underwent surgery to remove the remaining cancer and surrounding tissues. Following surgery, his cancer markers returned to normal, and he has been closely monitored with regular scans and tests to ensure the cancer does not return."
753,"A 5-year-old girl was referred to our hospital for the investigation of urinary incontinence. The patient had continuous low volume urine leakage requiring 4–5 daily pads. The parents could not specify whether there was any connection with standing, coughing, or effort and she had no urge to void.\nShe was constantly wet but had normal voiding habits. On initial physical examination the external genitalia appeared normal with no vaginal pooling of urine or ectopic ureteral orifice. However, a long-term external genitalia examination revealed normal urethral and vaginal openings, with an intermittent urine leakage through the vaginal orifice, which slightly increased in abdominal pressure.\nIn her past history she had recurrent febrile urinary tract infections (UTIs) since her infancy. At the age of 3 she underwent an abdominal ultrasound that suspected a double left kidney. A voiding cystourethrogram (VCUG) was performed and a vesicoureteral reflux (VUR) grade III on the right kidney was found. She was given chronic chemoprophylaxis without any UTI recurrence. She had a good toilet training and gave up daytime diapers around the age of 3. At this moment parents noticed the urinary incontinence, but this didn't bother them. At the age of 4 parents asked a urologist for help, who considered the symptoms to be the result of an overactive bladder and anticholinergic treatment was recommended. No improvement was noticed, and for this reason the little girl was sent to our department for further investigation.\nComplete blood count, biochemical tests, and urinalysis were all normal and the urine culture was negative.\nAn abdominal ultrasound was performed and both kidneys had normal parenchyma and size, with a duplex-system suspicion on left side; the bladder was normal in appearance. The VCUG was repeated but no VUR was visualized. As we had a high suspicion of an ectopic ureter a contrast-enhanced computed tomography (CT) of the abdomen and pelvis was performed to visualize the entire urinary tract. The CT scan was performed with a 64 row MDCT system (Siemens Somatom Definition AS). The 3D images revealed important data about the entire collecting system and the ureters, namely a duplicated collecting system on the left side and subsequently both ureters extending from the kidney to the point of extravesical insertion into the vagina. The ectopic ureters were in very close vicinity (Figures –).\nIn the evaluation of urinary incontinence in children of this age a differential diagnosis should be considered with the following: UTI, chronic kidney disease, diabetes, overactive bladder, ectopic ureter and vesico-vaginal fistula in case of a history of pelvic surgery.","This 5-year-old girl has been experiencing a problem with leaking urine, which has led to her needing to use pads frequently. Tests revealed that she has a slight abnormality in her urinary system, with one of her ureters (the tube that carries urine from the kidney to the bladder) ending in the vagina. She had a history of frequent urinary infections as a baby, which may have contributed to the problem. After further imaging, doctors found that the leakage is due to the ectopic ureter and they will discuss treatment options with the family."
754,"A 41-year-old man with a past surgical history of uncomplicated cholecystectomy two years ago (and no other significant medical history) presented to emergency department with worsening fatigue, shortness of breath, and chest pain. He reported a one-week history of flu-like symptoms i.e. subjective fevers, cough, rhinorrhea, muscle aches, and two days history of pleuritic chest pain worsened by lying flat and improved by leaning forward. On day of presentation, he was feeling more fatigued and also had an episode of presyncope with chills and rigors. On arrival, physical examination revealed tachycardia to 106/minute, hypotension to 62/48 mmHg, and oral temperature of 97.9 °F.\nOn cardiac auscultation, no gallops or murmurs were appreciated. Lung auscultation revealed decreased air entry at right lung base and bibasilar crackles. No pathological findings were noted on abdominal exam.\nElectrocardiogram (ECG) showed sinus tachycardia and diffuse ST segment elevations and PR segment depressions except in lead aVR consistent with acute pericarditis (Figure ).\nThe patient was given 3 l of normal saline without significant improvement in hemodynamics. He was then started on vasopressors through the central line. Initial labs were significant for troponin I elevation to 2.39 ng/ml (ref 0.00-0.04), CK-MB 12.8 ng/ml (ref 0.6-6.3) CRP 2.637 mg/dl (ref 0.02-2.0), Ferritin 1473.9 ng/ml (ref 3.1-110.9). Chest X-ray showed pulmonary vascular congestion and right mid- and lower-lung opacity/effusion (Figure ).\nBedside echocardiogram (ECHO) revealed severely reduced ejection fraction (EF) to 16%-20% and moderate pericardial effusion, which was later confirmed with the official echocardiogram as shown in Video .\nThe patient was taken to cardiac intensive care unit for close hemodynamic monitoring. He was started on milrinone drip in addition to norepinephrine. Anti-inflammatory therapy with aspirin and colchicine were initiated. He was also started on Oseltamivir after rapid diagnostic test came back positive for Influenza B. The patient was able to be tapered off vasopressors and inotropes on day three. Repeat ECHO on day three of admission showed improved ejection fraction (EF) to 31 % and worsening pericardial effusion without tamponade effect. The hospital stay was complicated by paroxysmal atrial fibrillation and the patient was started on amiodarone for rhythm control. He was also started on heart failure medications i.e. lisinopril, metoprolol. Anticoagulation was not started due to low CHADS-Vasc score and risk of hemorrhagic conversion of pericardial effusion.\nThe patient remained in sinus rhythm afterward and was transferred from intensive care unit to telemetry floor. Follow-up ECG showed normalization of ST and PR segments (Figure ).\nRepeat Echocardiogram on day nine showed improved EF to 51% and resolution of pericardial effusion as shown in Video .\nHis symptoms resolved completely and he was discharged on day 10 in stable condition from the hospital to follow up with cardiology outpatient.","A 41-year-old man came to the emergency room feeling very tired and short of breath, with chest pain. He also had a fever and flu-like symptoms. Tests showed he had a heart problem called acute pericarditis, which causes inflammation around the heart, and his heart was not pumping blood effectively. He was given medications to support his heart and treat the flu, and his condition gradually improved, with his heart function returning to normal. He was eventually discharged home with a plan for continued monitoring and medication."
755,"A 22-year-old male, known case of hepatitis B for one year, developed a low-grade intermittent fever (100°F-101°F) with chills and rigors, associated with nausea and vomiting related to food intake. A week of this predicament was followed by altered mentation, whereby the patient complained of drowsiness as well as a concomitant decrease in urine output. In our emergency room, he was found to be delirious but had a Glasgow coma scale (GCS) of 15/15. Initial assessment revealed a heart rate (HR) of 110/minute, respiratory rate (RR) of 20/minute, a temperature of 101°F and blood pressure (BP) of 150/100 mm Hg. The patient also had scleral icterus. Rest of the physical examination was unremarkable. Initial investigations at the time of the presentation are shown in Table .\nThe patient was started on broad-spectrum antibiotics (1 gram (g) intravenous vancomycin + 500 milligrams (mg) intravenous imipenem/cilastatin). Figures - show investigations and their trends over the course of the next few days.\nBased on his clinical presentation and an elevated serum creatinine, the patient underwent hemodialysis on day two of his admission. A tentative diagnosis of thrombotic thrombocytopenic purpura (TTP) was ruled out based on a low reticulocyte count with a high direct bilirubin value and a positive direct Coombs test. An abdominal ultrasound showed mild abdominopelvic ascites with mild splenomegaly. A head computed tomography (CT) scan was negative for any pathology and CT scan of the chest, abdomen, and pelvis showed bilateral basal consolidation and atelectasis in the lungs, swollen and enlarged pancreas, diffuse thickening of the walls of the ascending, transverse and descending colon, hepatosplenomegaly, swollen and globular appearing kidneys, and intramural hemorrhages with peritoneal hemorrhagic fluid (Figure ).\nAdditional investigations showed a serum fibrinogen level of 292 mg/dL, serum triglyceride level of 496 mg/dL, and a serum ferritin level of 30,304 ng/mL. C-reactive protein level (CRP) was 105 mg/L while serum lactate dehydrogenase (LDH) was 3,965 U/L. A hepatitis panel blood test was only positive for hepatitis B surface antigen (HBsAg). Antinuclear antibody (ANA) was negative, thus ruling out systemic lupus erythematosus (SLE) and other autoimmune etiologies. Blood cultures assessed after five days were unrevealing for any microbial growth. Considering a lack of clinical improvement, a bone marrow biopsy was performed on the 10th day of admission which showed 80% cellularity with increased hemophagocytic activity. Immunohistochemistry revealed increased histiocytes on CD68, and Iron stain revealed 2+ stainable iron (Figures -).\nAccording to the HLH-2004 trial, this patient fulfilled six features out of the eight-point diagnostic criteria for HLH, including a persistent fever >101 F, bicytopenia, splenomegaly on an abdominal CT scan, hypertriglyceridemia and/or hypofibrinogenemia, hyperferritinemia and a histiocytic activity on a bone marrow aspirate. In this case, the underlying cause of HLH was suspected to be a chronic hepatitis B infection. He was started on etoposide (100 mg/m²) along with dexamethasone as per the HLH treatment protocol.","This 22-year-old man with a history of hepatitis B developed a fever, nausea, and confusion, and his urine output decreased. Tests revealed he had significant inflammation in his liver and lungs, along with an enlarged pancreas and colon, and his blood tests showed signs of a serious condition called HLH. He received treatment with antibiotics, dialysis, and medication to combat the inflammation, and a bone marrow biopsy confirmed the diagnosis. Doctors believe his underlying hepatitis B infection triggered this severe reaction, and he is now receiving specialized treatment to manage the condition."
756,"A 44-year-old male presented to our outpatient department with complaints of a continuous fever (100°F-102°F), loss of appetite, undocumented weight loss and fatigue for two weeks. The fever was not associated with chills and responded to acetaminophen. He also complained of occasional gum bleeds. A review of systems was otherwise unremarkable. Initial assessment revealed a heart rate of 110/minute, respiratory rate of 17/minute, a temperature of 102°F and blood pressure of 140/90 mm Hg. The rest of the physical exam was unremarkable. Initial investigations at the time of presentation are shown in Table .\nThe patient was then started on a combination of 1 g intravenous vancomycin and 500 mg intravenous imipenem/cilastatin. Figures - show the investigation results and their trends over the course of the next few days while the patient was admitted to our setting.\nAn abdominal CT scan showed moderate hepatosplenomegaly with multiple hypodense lesions in the spleen. There was a mild enlargement of the left inguinal, paracardiac and subdiaphragmatic lymph nodes. Diffuse omento-mesenteric congestion and pelvic ascites were also noted (Figure ).\nDespite broad-spectrum antibiotic therapy, the patient remained febrile. Additional investigations showed a serum fibrinogen level of 132 mg/dL, serum triglycerides level of 257 mg/dL, serum ferritin was 5,092 ng/mL, serum LDH was 4,85 U/L and CRP was 20 mg/dL. Prothrombin time/international normalized ratio (PT/INR), serum vitamin B12, and serum folic acid levels were normal. A suspicion of autoimmune hepatitis was ruled out based on a negative anti-liver-kidney microsomal antibody (anti-LKM-1) test. Viral serologies revealed that the patient was negative for cytomegalovirus (CMV), herpes simplex virus (HSV), hepatitis B virus and hepatitis C virus. Blood cultures assessed after five days were negative.\nConsidering a lack of clinical improvement, a bone marrow biopsy was performed on the sixth day of admission which revealed normocellular marrow with an increase in dysplastic megakaryocytes. Hyperplastic erythropoiesis with nuclear cytoplasm asynchrony was also noted. Moderate hemophagocytic activity was evident with prominent histiocytes and plasma cells. Based on his abdominal CT report, he later underwent a left inguinal lymph node biopsy on the seventh day of his admission, which revealed reactive hyperplasia but was negative for malignancy.\nAccording to the HLH-2004 trial, this patient fulfilled six out of the eight-point diagnostic criteria for HLH including a fever of >101°F, bicytopenia, splenomegaly on abdominal CT scan, hypertriglyceridemia and/or hypofibrinogenemia, hyperferritinemia and a histiocytic activity on a bone marrow aspirate. However, the underlying etiology remained unclear. He was started on etoposide (100 mg/m²) along with dexamethasone as per the HLH treatment protocol. After completing eight sessions of etoposide, the patient was admitted again with complaints of abdominal pain. Hepatomegaly was noted and liver enzymes were found to be elevated, which prompted a liver biopsy that revealed a T-cell rich, histiocytic B-cell lymphoma. The patient was subsequently started on chemotherapy with rituximab, cyclophosphamide, vincristine, prednisone, and doxorubicin. A good response to the treatment was noted, and a complete remission was ultimately achieved on positron emission tomography (PET)/CT scans.","This 44-year-old man came to the hospital with a fever, fatigue, and loss of appetite for two weeks. Initial tests revealed an infection and some abnormalities in his blood work, including an enlarged liver and spleen. After several days of treatment, doctors discovered he had a rare type of lymphoma, and he started chemotherapy which led to a complete remission."
757,"A 67-year-old Caucasian man with a significant history of recently diagnosed type 2 diabetes mellitus (T2DM) and essential hypertension presented to the hospital with chief concerns of diplopia with an extreme gaze, right eye pain, and sinus congestion for about two weeks.\nAt the time of admission, the patient was afebrile, had a blood pressure of 160/67 mmHg and pulse of 64/minute but had a white blood cell count of 14,540/µL. The patient’s blood glucose was 469 mg/dL, anion gap levels were within reference range, and his glycosylated hemoglobin (HbA1c) was 12.4%. Computed tomography (CT) and magnetic resonance imaging (MRI) of the orbit and face revealed severe sinusitis with possible orbital cellulitis and optic nerve compression (Figure ).\nOn the day of admission, the patient was started on an IV ampicillin and sulbactam combination (3000 mg/mL every six hours) and IV vancomycin (1500 mg/mL loading dose; 1250 mg/mL every 12 hours maintenance dose; target vancomycin trough of 10 to 20 mg/mL due to the severity of the infection). The patient was seen by the infectious disease (ID) team on day two of admission. The ID team recommended continuing vancomycin, switched the ampicillin/sulbactam combination medication to piperacillin/tazobactam (3375 mg/mL every six hours) and started the patient on empiric IV liposomal amphotericin B (400 mg/mL daily) given the concerns for invasive fungal infection. The patient was seen by ophthalmology team, and they recommended no acute surgical intervention. However, the otorhinolaryngology (ENT) team performed an endoscopy of the nasal sinuses on the second hospital day, and the patient required extensive debridement of the necrotic tissue of the right sinuses.\nBiopsy results from the nasal sinuses showed broad hyphae with infrequent septations, haphazard branching, and numerous bizarre forms. These morphologic features were consistent with mucormycosis. Given this finding, the patient was continued on IV liposomal amphotericin B.\nA second MRI of the orbit and face was repeated on the fourth hospital day and showed the progression of the disease including involvement of right retrobulbar fat and right optic nerve, persistent non-enhancing, and likely necrotic tissue extending from the right pterygopalatine fossa into the right masticator space (Figure ). Suspected osteomyelitis was noted at the base of the skull, and we noted asymmetric right frontotemporal pachymeningeal enhancement with wispy leptomeningeal enhancement along the middle cranial fossa, which was suggestive of meningitis.\nThe neurosurgery team was consulted as the patient had meningeal spread as well. The neurosurgery team deemed the patient inoperable.\nGiven the progression of the disease despite the standard-of-care treatment (i.e., surgical debridement, IV amphotericin B, and control of blood glucose), a literature search was done to find the next best step. Isavuconazole has been used, according to a few case reports [-], as salvage treatment in patients whose condition either failed the IV amphotericin B or were intolerant to it. Therefore, our patient was started on IV isavuconazole (372 mg/mL every eight hours) on the fifth hospital day, and IV amphotericin B (400 mg/mL daily) was continued. Since patient tissue aerobic and anaerobic cultures as well as blood cultures remained negative, piperacillin/tazobactam and vancomycin were discontinued on the fifth hospital day.\nAn MRI of the orbit and face was repeated 48 hours after the patient was started on isavuconazole and revealed a stable disease. The patient’s IV isavuconazole dose was reduced to 372 mg/mL daily (after receiving IV isavuconazole every eight hours for 48 hours). An MRI was obtained one week later, and it also showed stable disease with no further progression. The patient was switched to oral isavuconazole (372 mg daily), and IV amphotericin B (400 mg/mL daily) was continued. As the patient continued to improve, he was ultimately discharged after 19 hospital days on this regimen with a plan for outpatient follow-up evaluations with the ID, ENT, and ophthalmology teams. The patient was blind in his right eye with cranial nerves III, IV and VI palsies at the time of discharge. However, his left eye was completely intact with normal vision and movement.\nDuring outpatient care, he continued to receive IV amphotericin B (400 mg/mL daily) and oral isavuconazole (372 mg daily). An MRI of the orbit and face after five weeks of this therapy showed some interval improvement of the infectious process (Figure ). After receiving IV amphotericin B (400 mg/mL daily) for 50 days, the medication was stopped, and the patient was continued on oral isavuconazole (372 mg daily). The patient's renal function was checked weekly as the patient was on IV amphotericin B. The patient had baseline creatinine of 1.0 mg/dL, it peaked at 1.8 mg/dL and returned to 1.4 mg/dL once the IV amphotericin B was stopped. Fungal cultures and blood samples were monitored for six weeks, and they remained negative.\nAn MRI of the orbit and face was obtained two months later while the patient was treated with oral isavuconazole (372 mg daily), and the imaging continued to show stable disease. Therefore, oral isavuconazole was stopped. By this time, the patient had isavuconazole for a total of four months (both via IV and oral doses). Besides headaches, the patient tolerated the isavuconazole well without any other significant side effects.\nAn MRI of the orbit and face was obtained again nine months after stopping oral isavuconazole, and his disease process has remained stable. Imaging at this time showed some improvement in the condition. The ID team stated the patient was cured. The ENT team still monitors the patient periodically, and, as of this writing, the patient has been over one year off of antifungal medications.\nRegarding the patient’s diagnosis of uncontrolled T2DM, the patient was initially started on basal insulin while as an inpatient, but he was discharged on oral antihyperglycemic agents (metformin, 500 mg twice daily with meals and sitagliptin, 100 mg daily) given better control with oral agents at the time of discharge. His HbA1c improved to 5.0% from 12.4% in three months. The patient’s metformin has been stopped, and his sitagliptin dose has been reduced to 50 mg daily and is the only diabetic medication for the patient as of this writing.","This 67-year-old man was hospitalized with a serious infection in his sinuses and around his eye, which was causing him pain and blurry vision. Tests revealed a fungal infection that required strong antibiotics and antifungal medications. He also had high blood sugar and kidney problems, which were managed with adjustments to his diabetes treatment. After several weeks of treatment, including surgery to clear the infection, he is now stable and being monitored closely, with plans for ongoing follow-up care."
758,"The patient is a 58-year-old non-smoking female with a history of recurrent papillary thyroid cancer first detected in the 1970s, who throughout her course underwent several surgical resections, repeated therapy with radioactive-iodine, and external beam radiation therapy (EBRT) (original history, cumulative radioiodine dose and EBRT dose unavailable). She presented again in 2015 with recurrent thyroid cancer as well as newly diagnosed breast cancer and liver metastases of unknown origin. She was hospitalized for unexplained fevers, failure to thrive, and symptomatic management of disease progression. Prior to this hospitalization, she had undergone an extensive infectious disease workup with an attempt to isolate the cause of her fevers up to 104 degrees Fahrenheit over the preceding three months. No clear etiology was identified. Empiric antibiotics were eventually discontinued, and the presumed diagnosis of tumor fever was made. On discharge, she followed up in the oncology clinic to start lenvatinib, a TKI approved for radioiodine-refractory thyroid cancer. Hours after her first dose of lenvatinib, she was taken to the hospital due to two witnessed generalized tonic-clonic seizures. At home she was noted to have shaking of her arms with repetitive movements of her head, lasting about three minutes and followed by post-ictal confusion, with no tongue biting or incontinence noted. She was taken to the emergency department where she had another witnessed seizure that resolved under therapy with lorazepam. The patient was then transferred from the emergency department to a different medical center. Although her presenting blood pressure was not available for review, her blood pressure was noted to be 158/99 mmHg immediately prior to her transfer.\nThe patient was admitted to the medical intensive care unit, and given persistent seizures, required intubation for airway protection. Physical exam was limited due to sedation. Brain MRI revealed multifocal white matter edema affecting the occipital and parietal lobes with patchy gadolinium enhancement, in a predominantly posterior distribution, but also with frontal lobe lesions with associated vasogenic edema and patchy enhancement (Figure ). A small, acute infarct in the left centrum semiovale on diffusion weighted imaging was also noted. She developed a fever early in this hospitalization and extensive work up for infectious causes revealed right lower lobe pneumonia that was treated with vancomycin and cefepime. Given her fevers and altered mental status, as well as small acute stroke seen on MRI, patient underwent lumbar puncture on hospital day 1 to evaluate for signs of meningitis or systemic vasculitis. Her CSF studies were not consistent with those diagnoses, with 1–4 white blood cells per UL, 0–1 red blood cells per UL, glucose of 65 mg/dL (normal 40–70 mg/dL), protein of 46 mg/dL (normal 15–45 mg/dL) and gram stain and culture which was negative for growth. CSF was also negative for oligoclonal bands, herpes simplex virus, Ebstein-Barr virus, John Cunningham (JC) virus, cytomegalovirus, varicella zoster virus, and negative for growth of mycobacteria. Additionally, head and neck CT angiogram did not demonstrate any hemodynamically significant stenosis. Based on these clinical findings of confusion and progression to mental status depression, a presumed diagnosis of PRES was made. It was thought that the small stroke seen on MRI was a separate event and was not contributing to her current clinical picture. On hospital day 2, she required norepinephrine due to mixed distributive and cardiogenic shock. A transthoracic echocardiogram (TTE) showed an ejection fraction (EF) of 36%, decreased from 62% one month prior, with regional wall motion abnormalities, moderate to severe hypokinesis in all basal to mid-ventricular segments, and hyperdynamic apical segments. These findings were consistent with stress cardiomyopathy or reverse Takotsubo cardiomyopathy. She was weaned off vasopressors and extubated successfully on day 4. For her stress cardiomyopathy with tachycardia, she was started on captopril and metoprolol.\nA repeat MRI one week later showed marked improvement in the size and number of areas of T2/FLAIR signal hyperintensity in the supratentorial cortex and near-complete resolution of the abnormal T2/FLAIR signal in the cerebellar hemispheres, further consistent with PRES. Repeat TTE at that time showed improvement of systolic function with an EF of 56% and no wall-motion abnormalities. She was continued on lacosamide and advised to follow up with neurology for epilepsy management. She was advised to not take lenvatinib. She remained hemodynamically stable on the oncology ward, however she had a non-resolving lactic acidosis without systemic signs of poor organ perfusion that was attributed to her cancer progression. The patient was discharged and ultimately died under hospice-care one week later.","This 58-year-old woman has a history of thyroid cancer and recently developed breast cancer and liver problems. She was hospitalized due to high fevers and confusion, and doctors initially suspected an infection but couldn't find the cause. After receiving a new medication, she experienced seizures and was found to have swelling in her brain, which led to a stroke. Further tests revealed a weakened heart muscle and lactic acidosis, likely due to her cancer. She was treated for these issues and eventually passed away."
759,"A 57-year-old Thai man from Sa Kaeo, a province in the Eastern region of Thailand referred to a University Teaching Hospital in Bangkok due to swelling and pain at the left side of the neck for one month. One week prior he was admitted to the local hospital due to low-grade fever, difficulty swallowing and hoarseness. He received intravenous ceftriaxone and clindamycin for presumptive diagnosis of deep neck infection. He had history of hypertension treated with amlodipine 10 mg and enalapril 10 mg daily. He had habits of heavy alcohol drinking for 40 years, and smoking. He works at the department of fisheries. He swam, cleaned fish pond and mowed the lawn. Upon admission (day 0), the patient's body weight was 52 kg, body mass index was 19.7 kg/m2. His vital signs were as follows: body temperature, 38.7 °C; blood pressure, 170/100 mmHg, pulse rate, 100 beats/min; respiratory rate, 24 breaths/min. On physical examination, mild pale conjuctivae, anicteric sclerae. The neck exam revealed pulsatile left neck mass size 5 × 5 cm in diameters, mild tender on palpation, no sign of inflammation. No limitation of neck movement. Oropharyngeal exam revealed bulging of left posterior pharyngeal wall and tonsil enlargement causing the narrowing of upper airway. Thyroid gland was not enlarged. Other exams included neurological exam were normal. Skin exam revealed multiple ill-defined scaly mild erythematous patches on both legs and dystrophic nails. Initial laboratory results showed anemia with hemoglobin concentration of 9.6 g/L and Hematocrit of 28%, MCV of 75 fl, white blood cell count of 6800 cells/mm3 with 80% neutrophil 7.7% lymphocytes, platelet count of 574,000 cells/mm3. Hemoglobin typing was normal (HbF 0.2% HbA2 2.9% HbA 85.9%; HbA2A). Liver function test showed AST 87 U/L, ALT 97 U/L, ALP 127 U/L, GGT 936 U/L, TB 0.3 mg/dl, DB 0.1 mg/dl, TP 81.2 g/L, Alb 28.7 g/L. Fasting glucose of 95 mg/dL, HbA1C of 4.74%, BUN 14 mg/dL, Cr 1.05 mg/dL, Anti-HIV test was negative. Viral hepatitis profile were negative. His chest X-ray was normal. He was diagnosed with anemia of chronic disease, alcoholic hepatitis, and xerotic eczema. Computer tomography of the neck showed a concealed ruptured of left external carotid artery 0.9 × 1.9 cm in size with surrounding hematoma (3.6 × 3.6 ×5.8 cm) at medial aspect of an aneurysm resulted in narrowing of the upper airway (A, B). Urgent surgical exploration on day 0 revealed severe adhesion around pseudoaneurysm (size 5 × 6 cm) confined around common carotid artery, carotid bifurcation, extended to the angle of mandible. The diameter of pseudoaneurysm neck was one cm, located at medial wall of common carotid artery just distal to carotid bifurcation. External carotid artery was obliterated. Angiogram and balloon occlusion was performed at the left common carotid artery. External carotid artery and internal carotid artery were ligated at the arterial stump just beneath the angle of mandible. Pseudoaneurysm was resected and internal content show pus and clot. Surgical margins were not free in gross section. The pus was sent for bacterial culture. Blood agar plate revealed rare growth of whitish colony, direct exam from the colony revealed broad rare septate fungal hyphae. Infectious disease was consulted on day 5 of admission. Serum antibodies to pythium antigen using an in-house rapid immunochromatographic test were positive on day 5. Sabouraud's glucose agar (SGA) grew fungal colony which identified as P. insidiosum by the induction of motile zoospore and confirmed by fungal broad-range 18S rDNA gene polymerase chain reaction. Pathology of carotid artery revealed acute suppurative inflammation () with branching broad rare septate hyphae demonstrated by Gomori Methanamine Silver stain and Periodic acid-Shiff stain (A) Immunohistochemistry stain for P. insidiosum was positive (B). Medical therapy with oral itraconazole 200 mg oral twice daily combined with terbinafine 250 mg oral twice daily were started on day 5. Adjunctive immunotherapy with subcutaneous injection of PIA vaccine 500 microliter (4 mg/ml) was given on day 6 and 18 of admission. Day 5, Computer tomography of the aorta shows atherosclerotic change without aneurysm or dissection. Postoperatively, physical exams revealed narrowing of upper airway, hypoglossal nerve palsy on the left side without motor deficit. Neurology was consulted on day 6. He was diagnosed with hypoglossal nerve palsy secondary to compression of carotid artery aneurysm. On Day 6, MRI and MRA of the brain revealed pseudoaneurysm of carotid artery at left carotid-parapharyngeal spaces (2.8 × 2.0 × 3.1 cm) associated with extensive inflammation of the surrounding soft tissue resulting in mild narrowing of upper airway. Left common carotid artery was occluded along the origin to the cavernous part of left internal carotid artery with the evidence of wall enhancement. Multifocal cerebritis consistent with cerebral septic emboli and leptomeningeal enhancement at the left cerebral hemisphere (). The patient underwent second exploration of the left neck on day 9 aiming to remove the residual infected necrotic tissue. Operative findings revealed pus with necrotic soft tissue extended to parapharyngeal space, however artery cannot be defined. The radical neck dissection could not be performed due to the morbidity outweigh the possibility of the cure. Tissue specimen revealed identical findings with the first operation. On day 6, the dosage of terbinafine was increased to 250 mg three times daily, itraconazole was continued. The patient and family decided for palliative care, no aggressive treatment. He was discharged on day 19 of hospitalization. On day 29 after discharge, upon an outpatient visit, his family mentioned that he developed progressive right hemiparesis over the two days after discharge. Physical exams revealed healed surgical wound of the left neck, narrowing of upper airway, neurological exams revealed global aphasia, right facial palsy (upper motor neuron), motor power grade I on the right side. His family denied further investigation. He continued to take combination of oral terbinafine, itraconazole and PIA vaccine. On day 49, upon an outpatient visit, he had flaccid hemiparesis on the right side without other deficit. He received fourth dose of PIA vaccine and continued oral itraconazole and terbinafine. On day 82, he expired at a local hospital due to complication of diseases.","This man from Thailand was admitted to the hospital with a painful swelling in his neck and difficulty swallowing, which started a month earlier. He also had a history of heavy drinking and smoking. After tests, doctors found a ruptured blood vessel in his neck causing an infection and narrowing his airway. They removed the damaged blood vessel and cleaned up the infection, but he developed complications and weakness on one side of his body. Despite treatment, he sadly passed away."
760,"37-year-old woman presented to the emergency room with headache. Bilateral pelvic sensitivity and a mass painful to touch in a plastic texture that filled the Douglas' pouch were detected during the examination; the mass was evaluated in favor of leiomyoma. ß-hcg was negative, wbc was 9800/mm3, hgb was 12 g/dl, htc was 35%, plt was 282000/mm3, and no unusual characteristics was found in complete urinalysis. Ultrasonography revealed a mass consistent with a degenerated myoma measuring 77x82mm, of which subserous component was greater in the posterior wall and endometrial thickness was 7-8 mm, which was concordant with the cycle. The left ovary of the patient was normal, but since the right ovary could not be fully evaluated, computerized tomography (CT) was requested for possible adnexal pathologies. CT demonstrated a hypodense nodular lesion with a diameter of 75 mm that extended to the right adnexal region and MRI with contrast was performed on the patient. MRI revealed a mass with a hypovascular appearance following a heterogeneous and hypointense IV contrast material with a diameter of 8 mm, which appeared to displace the posterior cervix.\nIt is seen that myoma included by central necrosis depleted by cervix in the posterior part of the uterus. Also the development of necrosis was seen and myomatosis was evaluated in favor of degeneration ().\nIn addition, since the patient's pain regressed spontaneously during the follow-up, surgical operation was postponed to perform in elective conditions. After making necessary preparations for the operation, laparoscopic myomectomy was performed. Myoma was not considered suspicious apart from being degenerated. It was removed by morcellating in an isolated bag (Figures , , , and ).\nNo complication or hemorrhage occurred during surgery. As there was not any problem observed during the post-op follow-up, the patient was discharged from the hospital on condition that the pathology report was brought on the second day of post-op. The subsequent report revealed marked cellularity and pleomorphism, extensive necrosis, mitosis >5/10BBA, KI67 proliferation index >%50, weak positivity for p53, and LMS with no detectable lymphovascular invasion.\nThe patient was informed about the pathology report and interned again to perform transabdominal hysterectomy and bilateral salphingoopherectomy. The pathology report revealed “atypical pleomorphic fusiform cell proliferation area” measuring 3x2 mm in a focal area within the cavity consistent with residual LMS when evaluated together with laparoscopic myomectomy material.\nTo follow-up the patient was referred to radiotherapy and medical oncologists. After 3 months from her 1st surgery, positron emission tomographic scan (PET-CT) was performed and fluoro-2-deoxyglucose (FDG) uptake is noted in the area above rectosigmoid mesentery towards the left abdominal wall in pelvic floor ().\nShe was referred to gynecologic oncologist and 3rd operation was performed. A mass about 4-5 cm of size in defined localization was excised; there were not any other mass and lymphadenopathy detected during the surgery. The pathology result was high grade malign mesenchymal tumor with high cellularity, extensive necrosis, mitosis 40/10 BBA, and moderate atypia. There was no tumor in surgical margin. She is now being followed up by the gynecologic oncologist with appropriate medical treatment.","A 37-year-old woman went to the hospital because of a persistent headache and pain in her pelvis. Doctors found a large, degenerated fibroid (a non-cancerous growth) in her uterus that was causing the pain. After further tests, including CT and MRI scans, they determined the fibroid was causing a problem with the cervix and removed it with a laparoscopic procedure. However, after reviewing the tissue sample, they discovered a small area of cancerous cells and recommended further treatment, including radiation and consultations with a gynecologic oncologist."
761,"A 75-year-old Caucasian woman, gravida 5, para 3 with past medical history of hypertension, rheumatoid arthritis, and chronic obstructive pulmonary disease arrived to the emergency department looking for relief from dental pain. Neither initial exam nor maxillary plain film showed evidence of cause for facial pain, and she was admitted for further evaluation and pain control. On reevaluation, she was noted to have diplopia and facial droop, so MRI brain along with MRI/MRA of head and neck with and without contrast was ordered to rule out cerebrovascular accident (CVA). CVA was ruled out; however bone marrow lesions involving the left and the right clivus, right Meckel's cave, and posterior margin of the right cavernous sinus were noted ().\nCT scans of chest, abdomen, and pelvis with and without contrast were ordered to search for primary malignancy. These studies revealed homogeneous enhancement of the uterus concerning for diffusely infiltrative endometrial carcinoma with associated relatively bulky retroperitoneal adenopathy of the abdomen and bilateral iliac chain adenopathy. In addition, multiple pulmonary nodules were noted along with L sided neck, mediastinal, and right hilar adenopathy ().\nCervical biopsies were obtained disclosing LCNEC of the cervix (). The tumor cells were immunoreactive for neuroendocrine markers synaptophysin and chromogranin. They were also immunoreactive for pancytokeratin and p16, the latter a surrogate marker for the presence of high-risk HPV often seen in these cervical carcinomas. The tumor cells lacked immunoreactivity for estrogen receptors and p63.\nShe was diagnosed with stage IV LCNEC with distant metastasis. This patient went on to receive palliative radiation to her brain for symptom control and was scheduled to see oncology as an outpatient to discuss treatments. However, due to decline in functional and mental status, she was no longer a candidate for chemotherapy and comfort care was pursued. She died 2 months after diagnosis.","This 75-year-old woman came to the hospital because of severe dental pain, but doctors discovered she was experiencing other serious problems. She developed double vision and facial drooping, leading to scans that ruled out a stroke but revealed tumors in her brain and a very advanced cancer in her uterus. The cancer had spread to other parts of her body, and despite treatment, her condition worsened. Ultimately, she received comfort care and passed away two months after her diagnosis."
762,"A 61-year-old female presented to hospital with a 2-week history of profound diarrhea and vomiting. The patient also complained of dull abdominal pain that temporarily resolved with bowel movements. She denied fevers, weight loss, exposure to sick contacts, external food sources, and a travel history. There were no extraintestinal manifestations of inflammatory bowel disease (IBD), such as arthralgias, uveitis, episcleritis, oral ulcers, and aphthous ulcers.\nShe was admitted with an initial diagnosis of viral gastroenteritis and treated with supportive therapy. Stool testing for Clostridium difficile, ova and parasites, viral PCR, and bacterial cultures were negative. A CT abdomen revealed diffuse edematous changes in the ascending colon, transverse colon, and descending colon, as well as hyperemia in the mesentery—indicating colitis.\nOn her second day of admission, the patient developed bloody diarrhea, prompting a colonoscopy by the gastroenterology service. The colonoscopy revealed severe inflammation with large (0.5–3 cm) deep punched-out ulcers, spontaneous bleeding, bridging mucosa, and patchy erythema affecting 80–90% of the mucosa from the cecum to the transverse colon, with rectal sparing. Several scattered aphthous ulcers were also noted.\nMultiple biopsy samples were taken from the colon—they revealed severe chronic colitis with focal areas of ulceration, focal cryptitis, and architectural distortion. Esophagogastroduodenoscopy (EGD) was normal. The results of the colonoscopy were consistent with Crohn's disease, and the patient was treated with intravenous methylprednisolone 80 mg for seven days.\nOn day 3 of admission, the patient developed fevers, chills, significant right-sided parotid swelling, erythema, and tenderness. Ultrasound of the right neck revealed parotitis with no abscess. Blood cultures revealed MSSA bacteremia, with parotitis being the presumed source. Transesophogeal echocardiogram was negative, and the patient was subsequently treated with supportive therapy and fourteen days of cefazolin.\nThe patient had a stable clinical course for the next thirteen days. However, on postadmission day 17, the patient developed severe abdominal pain and hemodynamic instability. On examination, the patient's abdomen was diffusely tender and rigid, suggesting peritonitis. Abdominal X-ray revealed features of colitis complicated by perforation. CT abdomen revealed bowel wall edema, pneumatosis, and focal perforation involving the anterior wall cecum/ascending colon, with extensive pneumoperitoneum. The patient was initially resuscitated and taken to the operating room the next day for a laparotomy. Findings in the operating room revealed microperforations of the cecum, large perforation at the splenic flexure, and fecal peritonitis. A subtotal colectomy was performed, as well as intra-abdominal and subphrenic abscesses were drained. The patient remained intubated postoperatively and was transferred to the intensive care unit (ICU). Concurrent blood cultures revealed Enterobacter cloacae bacteremia from an intra-abdominal source, and the patient was treated with intravenous ciprofloxacin. However, the patient remained intubated and required continued use of vasopressors in the ICU. Upon examination, the surgical wound demonstrated signs of infection, which along with the patient's clinical status suggested persistent intra-abdominal sepsis. On hospital day 25, the patient went to the operating room again for a washout of the abdomen and creation of a rectal stump mucous fistula. Tissue cultures revealed growth of Escherichia coli and Enterococcus faecium. Antimicrobical treatment with intravenous meropenem and intravenous metronidazole was commenced, for broad coverage of intra-abdominal abscess and sepsis.\nThe surgical biopsy from subtotal colectomy revealed extensive diffuse mucosal inflammation, acute ulceration, and mucosal and transmural ischemic changes from the ileum to the distal colon (). The ulcerated area exhibited granulation tissue that contained cells that diffusely exhibited intranuclear inclusions, morphologically and immunohistochemically consistent with herpes simplex virus (HSV-1 and HSV-2) (Figures –). Immunohistochemistry for CMV was negative. The patient was started on treatment with intravenous acyclovir.\nUpon physical examination, the patient had developed lesions on the tongue, with visible dry blood. No lesions were seen on the lips. Furthermore, the patient had vesicular eruptions on the lower abdomen and inner thigh. The fluid from the tongue and abdominal lesions was also positive for HSV-2 DNA on a PCR-based assay, thus suggesting a disseminated HSV-2 infection. Viral PCR was negative for HSV-1 and varicella-zoster. The patient received treatment with intravenous acyclovir 10 mg/kg for three weeks.\nThe patient had a prolonged stay in the ICU spanning 42 days. She continued to make a recovery on the ward, as her hospital stay was further complicated by catheter- and line-related infections, as well as poor nutrition and muscle atrophy. The patient was discharged from the hospital on day 122.","This 61-year-old woman was admitted to the hospital after experiencing severe diarrhea and abdominal pain. Initial tests ruled out common causes like viruses, but a colonoscopy revealed significant inflammation and ulcers in her colon, leading to a diagnosis of Crohn’s disease. Unfortunately, she then developed a serious infection in her abdomen, requiring surgery to remove part of her colon and drain abscesses. Further tests revealed a herpes virus infection that was causing sores in her mouth and on her skin. Despite multiple treatments and a long hospital stay, she continued to struggle with complications and was eventually discharged after 122 days."
763,"A 56-year-old man was admitted to the hospital with abdominal distension and diarrhea for several days. Physical examination revealed no abnormality. Routine laboratory examinations, bacterial and parasitic stool examinations and viral serology were negative. Chest and abdomen X-ray showed no obvious abnormality. However, endoscopic examination disclosed scattered bubble-like or cystoid nodules, which distributed in transverse colon (Fig. ). Meantime, narrow band imaging (NBI) showed clear texture of intestinal wall vessels (Fig. ). Considered PCI was idiopathic, we used high frequency electrosurgical resection of the gas cysts, and the cysts were collapsed after the gas was discharged (Fig. ). The patient was treated with Bifidobacterium (420 mg/bid) to improve his intestinal function (Table ). We also advised him to eat less gas-producing foods without using any antibiotic. The patient was symptom-free after one week and the lesions disappeared completely after three months of follow-up (Fig. , ).","A 56-year-old man was admitted to the hospital because of stomach problems, including bloating and diarrhea. After tests, doctors found small, gas-filled bubbles in his colon. Using a special procedure, they removed these bubbles, and the patient’s symptoms improved. He was given a special probiotic to help his gut function, and after a few weeks, his symptoms completely disappeared."
764,"A 48-year-old woman complained of constipation for 5 days, and then turned into diarrhea with discontinuous abdominal distension. She was hospitalized because of severe diarrhea (7 times/ d) with muco-bloody stools for one week. The stool frequency was five times a day. Her previous medical history revealed exposure to trichloroethylene (TCE) for one year. (Table ) Physical examination at admission revealed extensive abdominal tenderness. Fecal occult blood tests were positive. Other serological markers for autoimmunity and viral serology were normal, as was stool examination for bacteria and parasites. However, abdominal X-ray showed multiple intraluminal gas pockets in the left colon (Fig. ). Coronal reconstruction confirmed the widespread serosal intestinal air cysts involving long segment of colon (Fig. ). Colonoscopy revealed grape-like or beaded subepithelial lesions, and some with erythematous mucosa distributed in the sigmoid. Given the narrowing of the lumen secondary to these lesions the colonoscopy was incomplete (Fig. ). The endoscopic ultrasonography showed low echo of cystic below the mucosal layer (Fig. ). We used high frequency electrosurgical resection of the gas cysts; But considering that extensive endoscopic therapy might lead to infection, we used only partial treatment. Since the narrow lumen of the patient, we restricted her food intake and used parenteral nutrition. One week later, the patient started to have a half-fluid diet. Ornidazole (500 mg/bid) and vitamin B2 (10 mg/bid) were given to regulate intestinal anaerobes, while bifidobacterium (420 mg/bid) was given at intervals of half an hour. We also advised him to eat less gas-producing foods. The patients’ condition improved after 2 weeks (Table ). One month later the lesions disappeared completely (Fig. ) and NBI demonstrated visible patchy erythema and yellow nodules (Fig. ). After four months of follow up, the patient was still no symptoms, and findings at colonoscopy were normal.","A 48-year-old woman was admitted to the hospital because of severe diarrhea and abdominal pain that lasted for a week. Tests revealed that she had unusual cysts in her colon, which were causing the diarrhea and narrowing the passage. Doctors removed some of the cysts and provided supportive care, including a special diet and medication to help with the bacteria in her gut. Over several weeks, her symptoms improved, and the cysts disappeared completely, with no further issues reported after a month of follow-up."
765,"A 66-year-old woman was admitted to the gastroenterology ward because of alternate constipation and diarrhea with muco-bloody stools. She had a history of undifferentiated connective tissue disease (UCTD) for 20 years and aplastic anemia (AA) for 1 year. In the past, she was mainly treated with glucocorticoid, and subsequently developed AA. Recently she presented with a diffuse pain in the abdomen with muco-bloody stools. Physical examination at admission revealed extensive abdominal tenderness. The biochemical tests revealed cytopenia due to AA and no obvious abnormality in stool culture for pathogens. Blood cultures were also negative. Computer tomography (CT) examination showed no portal venous gas embolism (Fig. ). Colonoscopic examination disclosed line or pebble like sessile cysts and irregular forms, which mainly distributed in sigmoid (Fig. , ). Irregular forms of PCI with large bulge should be distinguished from malignant tumor (Fig. ). Given the narrowing of the lumen secondary to these lesions, the colonoscopy was incomplete. The pathologic findings revealed submucosal cystic structure (Fig. ). We used high frequency electrosurgical resection of the gas cysts. Because of the history of AA, she was treated with aluminum phosphate (20 g/bid) and bifidobacterium (420 mg/bid) without any antibiotics. We also advised him to eat less gas-producing foods. Symptoms of diarrhea improved significantly after one month, and gas-filled cysts became flattened (Fig. ). After ten months of follow-up the clinical symptoms were still resolved.","66-year-old woman was admitted to the hospital because she was experiencing alternating constipation and diarrhea with blood in her stools. She has a long-standing history of autoimmune disease and recently developed aplastic anemia. During a colonoscopy, doctors found several unusual cysts in her colon that were causing her symptoms. After removing the cysts and making dietary changes, her symptoms improved significantly, and she has been doing well for the past ten months."
766,"A 72-year-old woman complained of constipation for several days, and then turned to diarrhea for one month. She was hospitalized for muco-bloody stools and severe abdominal distension. She had a history of diabetes for 10 years and was mainly treated with acarbose and insulin. Physical examination at admission revealed extensive abdominal tenderness. Routine laboratory examinations, bacterial and parasitic stool examinations and viral serology were negative. Abdominal X-ray showed multiple intraluminal gas pockets in sigmoid and ascending colon. Computer tomography (CT) examination showed multiple polypoid lesions of the colon (Fig. ). Colonoscopy showed irregular forms of lesions that were covered with mucosa of normal appearance in sigmoid and ascending colon (Fig. ). Irregular forms of PCI and mucosal lesions with erosion should be distinguished from Crohn’s disease. Given the narrowing of the lumen secondary to these lesions, the colonoscopy was incomplete. NBI showed mucosal redness, which appeared as punctate labelling, and the blood vessels of intestine were clear (Fig. ). We used high frequency electrosurgical resection of the gas cysts. She was banned from using acarbose, continued to use insulin treatment for diabetes. The patient was initially treated with ornidazole (500 mg/bid) and bifidobacterium (420 mg/bid). In view of the older age of the patient and a history of diabetes, the antibiotic was changed to rifaximin (200 mg/qid) to avoid antibiotic resistance. We also advised him to eat less gas-producing foods. The patients’ condition was improved after one month and the findings at endoscopy were improved (Fig. ). After 6 months of follow up, the cysts gas disappeared (Fig. ).","This 72-year-old woman was admitted to the hospital because of persistent constipation followed by diarrhea, along with significant abdominal pain and blood in her stool. Tests revealed several abnormal growths in her colon, which were causing narrowing and gas buildup. Doctors removed the growths with a special procedure and adjusted her diabetes medication. After several months of follow-up, the growths disappeared, and her symptoms improved significantly."
767,"A 64-year-old man was admitted to the hospital with wheezes and exertional dyspnea, which he had suffered from for several months, but without abdominal symptoms. He was diagnosed with emphysema pulmonum 1 years ago. Physical examination revealed double diffuse rales. Serological markers for autoimmunity and viral serology were normal, so as was the stool examination. Chest CT showed centrilobular emphysema, pulmonary field scattered in small circular distribution (Fig. ). A chest X-ray and CT examination showed the improvement of emphysema pulmonum. Endoscope showed stenosis due to gas cyst in duodenal descending (Fig. ). Emphysema as the primary disease, it is mainly used seretide for treatment. The PCI was treated successfully with intensive (but not hyperbaric) oxygen therapy and bifidobacterium (420 mg/bid). The findings at endoscopy were improved after eight months of follow-up (Fig. ).","This 64-year-old man was admitted to the hospital because he’s been having trouble breathing, especially when he’s active. He has a history of emphysema, a lung condition, and tests showed that his lungs were significantly damaged. A scan revealed a blockage in his duodenum, which was causing the emphysema. After treatment with oxygen therapy and medication, the blockage improved, and his breathing has significantly improved."
768,"A 27-year-old woman was hospitalized because of ascites and abdominal pain for 3 months. She was diagnosed as Budd-Chiari syndrome (BCS) before admission. Physical examination at admission revealed pronounced abdominal tenderness and abdominal mass. Routine laboratory examinations, bacterial and parasitic stool examinations and viral serology were negative, while the level of serum C125 increased significantly up to 1560 U/ml. Abdominal X-ray and Abdominal CT showed a large presence of ascites in the abdomen (Fig. ). CT showed massive hydrops of abdominal cavity, multiple intraluminal gas pockets in the rectum and ovarian mass (Fig. ), which was limited to the ovarian surface without invasion. Abdominal ultrasonography revealed massive hydrops in the abdominal cavity, and abdominal paracentesis indicated bloody ascites. Colonoscopy showed grape or beaded lesions (Fig. ). Finally, primary peritoneal carcinoma (PPC) was diagnosed by peritoneal biopsy. After this the patient was given nutritional support. Finally, she was transferred to a hospital near her home and unfortunately died.","This 27-year-old woman was admitted to the hospital because of a growing belly and pain in her abdomen for several months. She was diagnosed with a rare condition called Budd-Chiari syndrome, which causes blood to pool in the liver. Tests revealed a large amount of fluid in her abdomen and a tumor in her ovary. After a biopsy, the tumor was identified as primary peritoneal carcinoma, and she received nutritional support before being transferred to a local hospital where she sadly passed away."
769,"The patient was a 70-year-old female whose past medical history was significant for arthritis and a right total hip arthroplasty approximately 9 years ago. A laparoscopic cholecystectomy (LC) for acute cholecystitis was performed approximately at another hospital approximately two months prior to presentation. She developed a surgical site infection with Escherichia coli (E. coli) bacteremia following her LC and was treated successfully with intravenous (IV) antibiotics. The postoperative course was also complicated by choledocholithiasis requiring an endoscopic retrograde cholangiopancreatography (ERCP) with stone pulverization and placement of two plastic 10F × 12 cm biliary stents. Two days prior to admission, she was hospitalized at an outside facility in septic shock with fevers, chills, lethargy, altered mental status, and blood and urine cultures positive for E. coli. At that time, she endorsed right hip pain and an inability to move her hip or leg. A computed tomography (CT) scan of her right hip revealed two partly calcified soft tissue masses associated with the right iliopsoas and obturator internus muscles (). A CT-guided fine needle biopsy of the right hip and psoas locules aspirated 100 mL of frank pus notable for a nucleated cell count of 344,000 (98% PMNs) with growth of E. coli. As a result, the patient was transferred to our institution with concerns for an iliopsoas abscess and a periprosthetic infection.\nOn admission, she was febrile to 102.7 F without any significant distress. Her physical examination was remarkable for a well-healed, right lateral hip incision with no erythema or drainage. She experienced pain with right hip flexion and internal rotation. Laboratory studies showed WBC, hemoglobin and hematocrit, basic metabolic profile, and liver function tests all within normal limits. A 3 cm hepatic abscess was identified on CT scan of the abdomen and pelvis. An MRI of the right hip showed a large air- and fluid-filled collection tracking along the iliopsoas bursa and psoas musculature into the pelvis (). This collection communicated with the hip prosthesis, and on CT imaging, the acetabulum component appeared to be medialized beyond the medial wall of the acetabulum as depicted in . Regarding the hepatic abscess, the patient was managed with IV antibiotics and interventional radiology (IR) placed drainage catheter.\nTo address the hip periprosthetic infection, the patient was managed in multiple surgical stages. In the first stage, an irrigation and debridement of the right hip and explantation of components were performed through an anterior approach. The femoral and acetabular components were explanted. Purulent material was seen draining from the pelvis through a medial acetabular wall defect into the hip joint. Approximately 1 liter of pus was evacuated from the hip joint. Multiple irregularly shaped granulated pea-sized pieces of hard brown substance were found deep in the acetabulum. A handful of this material was removed which suggested that these were spilled gallstones from the patient's recent LC.\nTemporary components were replanted with an antibiotic impregnated cement spacer system [] (Figures and ). An antibiotic cement spacer with gentamicin was placed in the acetabulum defect, and a loosely fitted antibiotic-cemented stem was placed in the proximal femur.\nThe intrapelvic iliopsoas collection could not be fully debrided through the anterior approach, and IR was consulted for drain placement and serial debridements, which were conducted with a rotating basket Trerotola device over the course of the next four weeks. In , contrast dye can be seen tracking from within the iliopsoas abscess into the hip joint.\nFour weeks following her explantation, the patient returned to the operating room for placement of a second temporary weight bearing custom-fitted prosthesis made from methyl methacrylate with gentamicin-impregnated antibiotics [] (Figures and ). For the acetabulum, methyl methacrylate was molded in its doughy state into the cavities and deformities of the acetabulum, and a polyethylene acetabulum was pressed into the cement. The femur was reamed to size of a large diameter chest tube. A femoral stem and a reinforcing wire were cemented into the chest tube, and once the cement was hardened, the femoral stem encased in a solid cylinder of bone cement was removed from the chest tube and malleted into the proximal femur.\nPostoperatively, the patient did well. She was made weight bearing as tolerated, ambulated with physical therapy and elected to delay placement of permanent components. She was eventually discharged to a short-term rehabilitation facility with a 6-week course of IV antibiotics.\nApproximately 18 months later, she presented to our clinic complaining of hip and thigh pain with ambulation. She was followed by infectious disease as an outpatient with multiple hip aspirations which had negative cultures. X-rays revealed her temporary prosthesis to be stable, but with radiolucencies primarily around the femoral component (). The patient was taken to the operating room, the temporary prosthesis was removed, and a long porous coated system was inserted (). Intraoperative cultures grew vancomycin-resistant enterococcus (VRE), and the patient was eventually discharged to home with a 12-week course of daptomycin and outpatient physical therapy. Now, two years from her initial explantation, she continues to follow up monthly in our clinic and states that she is doing well. Her final construct is shown in . She continues to have a moderate limp but ambulates without assistive devices. Her pain is much improved and she is no longer on chronic antibiotic suppression with no clinical signs or symptoms of recurrent infection.","This 70-year-old woman has had a complicated medical journey, starting with a recent infection after gallbladder surgery. She then developed a serious infection around her hip joint and a large collection of pus in her pelvis, which required multiple surgeries and drainage procedures. During this time, she also had a liver abscess. After several temporary replacements and antibiotics, she was eventually able to walk without assistance and is now doing well with ongoing follow-up care."
770,"A 50-year-old female patient presented with a one-week history of decreased vision in her left eye. She had a 15-year history of seropositive RA treated with methotrexate and deflazacort. Because of an unsatisfactory response to those regimens, she was initiated on 25 mg per week of etanercept 8 months prior to presentation.\nOn presentation, her best-corrected visual acuity (BCVA) was 0.8 OD and 0.1 OS. There were no cells in the anterior chamber of either eye. Funduscopic examination showed granular infiltration at the temporal macula in the right eye and the foveal area in the left eye (Fig. ). Spectral-domain optical coherence tomography showed a swollen ellipsoid zone and retinal pigment epithelium (RPE) irregularities in the right eye and an ellipsoid zone disruption and RPE irregularity in the left eye (Fig. ). Fundus autofluorescence showed parafoveal granular hyperautofluorescence in both eyes (Fig. ).\nBased on the negative results of various blood tests, she was diagnosed with noninfectious uveitis and started on oral prednisolone. She noticed mild improvement 1 month after treatment, but 2 months after treatment she showed a visual loss to 0.1 in her right eye. Compared with the initial visit, the swollen ellipsoid zone area extended under the fovea and subRPE yellow-white deposits were newly developed in the inferotemporal area (Fig. ). Also, mild vitreous opacity with haziness was noticed in the right eye. We suspected primary intraocular lymphoma (PIOL), and a 25-gauge microincision vitrectomy in right eye was performed. Vitreous cytology revealed atypical mononuclear cells with positive CD20 immunostaining. PCR of the vitreous fluid was negative for herpes and cytomegalovirus.\nThe patient was evaluated by an oncologist, and no lymphoma involvement in the brain was found. She was treated with high-dose systemic methotrexate as well as intravitreal methotrexate injections (400 μg/0.1 mL) twice weekly for 4 weeks for induction, once weekly for 8 weeks, and once monthly for 9 months in the right eye. Given the potential causal relationship between PIOL and anti-TNFα agents, etanercept was discontinued.\nThree months after treatment, her right eye showed a decrease in subretinal infiltration and subRPE deposits, which further resolved at 6 months following treatment (Fig. ). Over the course, her left eye also showed gradual improvement in funduscopic examination as well as SD-OCT findings without intravitreal methotrexate injection, potentially due to systemic chemotherapy. Because of severe photoreceptor disruption, BCVA was 0.1 OD and 0.4 OS at the final visit.","This 50-year-old woman came to the clinic with blurry vision in her left eye, which had been getting worse over the past week. She has a long-standing condition called rheumatoid arthritis and was taking medications to manage it. After further testing, doctors found inflammation in her eyes and suspected a rare type of lymphoma linked to her medications. A surgery was performed to remove some cells from her eye, and she started taking medications to treat the lymphoma, which gradually improved her vision."
771,"A 20-year-old African American man was admitted to a psychiatric facility for psychosis. On initial presentation, the patient had an antalgic gait, which he attributed to his history of dopa-responsive dystonia. His mood was depressed and his affect was restricted. He had disorganized thought process and was slow to recall. He endorsed auditory hallucinations, paranoid delusions, depressive symptoms, frequent night awakenings, and persecutory nightmares. Per the ambulance report, the patient was wandering the streets in a confused state, so bystanders called 911. The patient stated that he had been homeless for the past 3 weeks. During this 3-week period, he admitted to not being complaint with his medications. Urine toxicology screen was negative.\nPer medical records, he was diagnosed with dopa-responsive dystonia at age 11 after a 2.5-year history of progressive abnormal gait. He was initially misdiagnosed with tight heel cords at age 10 and treated with serial casting that resulted in good improvement on the right but marginal improvement on the left. His toe walking became more pronounced overtime accompanied by worsening left calf pain and stiffness, increasingly frequent falls, and new onset of intermittent torticollis. These symptoms worsened over the course of the day. He was eventually taken to an urban teaching hospital, where he was diagnosed with dopa-responsive dystonia based on clinical presentation and marked improvement on a levodopa trial. Magnetic resonance imaging of the brain and spine was unremarkable at the time.\nAt age 15, he was diagnosed with schizoaffective disorder bipolar type. His psychiatric history is also significant for multiple psychiatric hospitalizations, history of previous suicide attempts with medication overdose, and history of trauma. He also endorsed marijuana use since age 15 and daily tobacco use since age 18. He denies using any other illicit drugs. Per collateral information from his mother, his schizoaffective disorder has never been well controlled given the conflicting effects of his medications. She also mentioned that he was placed in individualized education programs as a child due to learning disabilities. His family history is significant for bipolar disorder on his maternal side. His family history on his paternal side is unknown. In addition to carbidopa-levodopa, his outpatient medications included sertraline, divalproex sodium, aripiprazole, and benztropine.\nOn hospital day 1, he was started on carbidopa-levodopa 25/100 mg tablet three times daily for dopa-responsive dystonia. On day 2 of his hospital course, sertraline 50 mg once daily, benztropine 2 mg twice daily, divalproex sodium 500 mg twice daily, and risperidone 0.5 mg twice daily were added to his medication regimen. We started him on a low-dose risperidone to avoid exacerbating his dopa-responsive dystonia symptoms. Physical exams were also performed daily to assess for dystonia and parkinsonian symptoms. His initial physical exam revealed an antalgic gait secondary to left lower extremity dystonia, which improved by hospital day 2 and resolved by hospital day 3. On hospital day 3, he became agitated and aggressive with staff members, which led to intramuscular administrations of haloperidol 10 mg, diphenhydramine 50 mg, and lorazepam 2 mg. He continued to endorse auditory hallucinations, so risperidone was increased to 0.5 mg in the morning and 1 mg at bedtime. His auditory hallucinations resolved and then returned on day 6. He reported hearing “good” voices and “bad” voices. He also continued to endorse depressive symptoms, multiple night awakenings, and persecutory nightmares. As a result, his risperidone dosage was increased to 1 mg twice daily. On hospital day 7, the patient reported hearing “mumbling” voices only and improvement in his sleep and depressive symptoms. On hospital day 8, his auditory hallucinations fully abated. By hospital day 10, he slept throughout the night, no longer had depressive symptoms, and had normal spontaneous speech. His thought process was linear, logical, and goal-oriented. His mood and affect was euthymic and full range. No psychotic symptoms were noted. The patient was compliant with his medications throughout the whole hospital course and his daily physical exams were negative for dystonia or parkinsonian symptoms since day 3 of his hospitalization. He was subsequently discharged on hospital day 14 with appropriate outpatient follow-up.","This 20-year-old man was admitted to a psychiatric hospital because he was experiencing psychosis, including hearing voices and having paranoid thoughts. He has a history of a movement disorder called dopa-responsive dystonia, which he was initially misdiagnosed with as tight heel cords. He also has a history of schizoaffective disorder and has been hospitalized multiple times before, and has attempted suicide in the past. While in the hospital, he received a combination of medications to manage his symptoms, and his condition gradually improved, with his hallucinations and depressive symptoms disappearing. He was eventually discharged home with a plan for continued care."
772,"An 82-year-old Caucasian male was admitted to our hospital in December 2016 with dyspnea, hemoptysis, and impaired general condition. He also presented with pseudoparalysis of his left shoulder due to severe pain. The medical record included ischemic heart disease (coronary artery bypass grafting in 1994), atrial fibrillation, low malignant prostate cancer, gout, and diabetes mellitus type II. Six months prior to admission, the patient had all teeth in his upper mouth removed prior to being fitted with dentures. This dental procedure was complicated with severe inflammation, and the patient was treated several times with oral antibiotics.\nOn admission, the patient was septic with fever and in a condition with pulmonary congestion and bilateral oedema in his lower limbs. Vital parameters included a blood pressure of 148/62 mmHg, a heart rate of 84 beats/min, oxygen saturation of 81% without oxygen supplementation, respiratory frequency at 26 per minute, and a rectal temperature of 38.8°C. Arterial blood gasses showed a normal pH (7.44), low partial pressure of carbon dioxide (3.5 kPa) and oxygen (7.2 kPa) in arterial blood, and low oxygen saturation (89%). On physical examination, the patient's left shoulder was tender and warm and had an anterior nonerythematous swelling. Cardiac auscultation did not reveal any murmur, and the neurologic examination was normal. The electrocardiogram revealed normofrequent atrial fibrillation and right bundle branch block.\nThe initial blood samples showed leucocytosis (14.7 × 109/L) with dominance of neutrophilic granulocytes, haemoglobin level of 7.1 mmol/L, and C-reactive protein (CRP) of 216 mg/L.\nChest X-ray showed no infiltrates but was consistent with pulmonary stasis. An X-ray of the left shoulder showed no signs of inflammation.\nBlood cultures (three bottles with 10 ml each) and two samples of synovial fluids from the left shoulder were sent to the laboratory. Next day, growth of Gram-positive cocci was seen in one of the three blood culture bottles and in both synovial fluids. Matrix-assisted laser desorption ionization-time of flight mass spectrometry (MALDI-TOF MS) was performed on all three isolates using Microflex™ LT MALDI-TOF-System (Bruker, Karlsruhe, Germany; two databases: 1829023 Maldi Biotyper Compass Library and 8254705 Security ref. Library 1.0 for MALDI Biotyper 2.0), and they were diagnosed as S. equi subspecies zooepidemicus with a score between 2.37 and 2.63. Whole-genome sequencing of the blood culture isolate was performed by 251-bp paired-end sequencing (MiSeq; Illumina) and confirmed the species and subspecies type diagnosed by MALDI-TOF MS. The isolate carried a novel sequence type (ST379), and a phylogenetic analysis using FastTree v2.1.5 of the concatenated MLST alleles () found it to cluster in a branch containing ST60, ST135 and ST162. An analysis using the iTOL implementation at revealed that seven related isolates with those ST types were reported in the database from various hosts (cow (n=1), dog (n=1), and horse (n=5)). The sequence data has been uploaded to ENA with the following Study and Read accession IDs: PRJEB24181 and ERR2233447, respectively. Antibiotic susceptibility testing using the Epsilometer test (E-test) (bioMérieux, Marcy-l'Etoile, France) showed that the isolate was susceptible to penicillin (0.016 microgram/ml), ampicillin (0.032 microgram/ml), amoxicillin (0.047 microgram/ml), cefuroxime (0.016 microgram/ml), erythromycin (0.094 microgram/ml), clindamycin (0.38 microgram/ml), vancomycin (0.5 microgram/ml), imipenem (0.012 microgram/ml), and rifampicin (0.012 microgram/ml). The isolate was intermediate susceptible to gentamicin (12 microgram/ml).\nAntibiotic treatment the first two days was intravenous cefuroxime 1.5 gram administered three times daily, which was changed to intravenous high-dose benzylpenicillin, initially a dose of 2 million international units (IU) four times a day. The monotherapy with benzylpenicillin was supplemented with intravenous gentamicin in intervals (five days in total). On day three, transthoracic and transesophageal echocardiography (TTE and TEE, resp.) demonstrated a vegetation on the aortic valve () with a minimal aortic insufficiency. There were no signs of paravalvular abscesses and no stenosis of the affected valve. Ejection fraction was normal. The patient was diagnosed with aortic valve endocarditis without indication for acute operation. According to the national Danish guidelines for infective endocarditis, the benzylpenicillin dosage was increased to 5 million IU four times a day.\nOn day three, CRP levels were increasing as well as the pain and oedema in his left arm. On day five, arthroscopic revision of the left shoulder was performed and three biopsies were taken, but none of these cultures showed further growth of the pathogen. The rotator cuff was almost completely degenerated, and the humeral head was devoid of its hyaline cartilage. Ten days later technetium-99m bone scintigraphy showed intense activity in the left shoulder, suspicious of osteitis (). The bone scintigraphy revealed no other focus of infection. On day 17, control transesophageal echocardiography was performed. At this time, the examination showed no suspicious signs of endocarditis, and the vegetation that was previously seen on the aortic valve could not be visualised.\nThe patient was sent to a dentist, which did not reveal any oral focus. The patient's clinical condition improved gradually, and he was discharged from our hospital after five weeks (36 days) with oral antibiotics (oral amoxicillin 1 g × 3 pr. day and oral rifampicin 600 mg × 2 pr. day) for further two months. In total, he received antibiotics for almost three months (87 days). During the admission, the patient lost approximately 17 kilograms in weight, primarily fluid associated with his septic condition as well as fluid from some of degree cardiac decompensation and lung stasis.\nThe patient was followed up in the Out-Patient Clinics for Infectious Diseases and Cardiology, which included blood samples and control transthoracic echocardiography. None of the subsequent examinations indicated signs of recurrence. The patient regained normal function in his left shoulder.\nThe patient reported that he had daily contact with animals including horses, though he did not recall any illness in the four horses he had taken care of the past year. He denied consuming unpasteurized milk products.","This 82-year-old man was admitted to the hospital after experiencing shortness of breath, fever, and pain in his left shoulder. He had a history of several health problems, including heart disease, kidney issues, and a previous bladder surgery. Tests revealed a serious infection in his bloodstream caused by a bacteria found in his shoulder joint, which had spread to his heart valve. He received strong antibiotics and underwent a procedure to examine his heart, and his condition gradually improved over five weeks, allowing him to return home with continued medication."
773,"A 34-year-old male was admitted to the hospital with recurrent episodes of retrosternal chest pain, fatigue, and shortness of breath with an elevated troponin T. He had suffered an acute episode of myocarditis four years previously requiring hospital admission. He had no other relevant medical history and no family history of cardiac disease. He is a nonsmoker and consumed alcohol occasionally. Clinical examination was unremarkable and did not show any evidence of heart failure or systemic disease. ECG showed normal sinus rhythm without any ischemic changes, and chest X-ray showed no evidence of infection or heart failure. Routine blood tests including antinuclear antibody, creatinine kinase (CK), rheumatoid factor, and C-reactive protein were all within normal limits apart from an elevated cardiac troponin T with a peak value of 2700 ng/l (<14 ng/l). Further extensive inflammatory, viral, and autoimmune screening was carried out and found to be negative. Subsequent coronary angiogram showed normal coronary arteries, and transthoracic echocardiography demonstrated left ventricular ejection fraction (LVEF) >55% with trace mitral regurgitation. Cardiac magnetic resonance imaging (MRI) demonstrated extensive subepicardial and midwall late enhancement typical of myocarditis in the anterior, lateral, and inferior walls along with extensive fibrosis with normal LVEF ().\nA short course of steroids and anti-inflammatory medication as an inpatient resulted in the resolution of his myocarditis symptoms. The troponin T level normalized and the patient was discharged with a plan to repeat cardiac MRI in six months. On follow-up as an outpatient, it was decided to refer the patient to rheumatology for an opinion regarding ongoing immunomodulatory therapy. At this juncture, the patient stated that he also had symptoms of stiffness and aching in his calf muscles for quite some time but he did not consider it to be relevant. Despite persistently normal skeletal muscle enzyme levels, an MRI of the lower legs was performed and this showed active myositis involving the gastrocnemius muscles bilaterally (). As the patient was demonstrated to have ongoing myositis despite minimal symptoms, and as he had accrued significant myocardial scarring from previous episodes of myocarditis, it was decided to commence long-term immunomodulatory therapy in the form of methotrexate and prednisolone. Clinically, the patient reported a significant improvement in his symptoms and a repeat of the lower limb MRI demonstrated a significant interval improvement in his skeletal muscle myositis. Six months later, a repeat of the cardiac MRI demonstrated resolution of myocarditis along with persistent, stable, and extensive myocardial fibrosis and preserved LVEF (). The patient is tolerating the immunomodulatory therapy well without major side effects, and he has returned to full-time work.","A 34-year-old man was admitted to the hospital because of chest pain, fatigue, and shortness of breath, and his blood tests showed a high level of a protein called troponin, indicating possible heart inflammation. He had experienced a similar episode of heart inflammation in the past. After tests, doctors found that he had scarring in his heart muscle and ongoing inflammation in his leg muscles. Treatment with medication helped to resolve the heart inflammation, and he is now taking long-term medication to manage the ongoing muscle inflammation and is doing well."
774,"A 36-year-old woman with a history of obesity, hypertension, anxiety, and recurrent urinary tract infections (UTI) was admitted to the hospital with history of dyspnea, fever, cough, and abdominal pain for 4 days.\nPrior to this admission, the patient presented to urology with recurrent UTIs and was determined to have a left staghorn renal calculus. Recommendations were for the patient to undergo surgical removal of the stone; however the patient refused due to risk of complications associated with her obesity. Up until this admission, the patient had experienced numerous UTIs, most with multidrug resistant bacteria, and had undergone multiple courses of antibiotics.\nOn examination, the patient was dyspneic. The temperature was 100.6 F, the pulse 105 beats per minute, the blood pressure 107/57 mmHg, the respiratory rate 20 per minute, and the oxygen saturation 100% on room air. Complete blood count was significant for a white cell count of 5.8 x 103 per μL. Comprehensive metabolic panel was significant for creatinine 0.76 mg/dL, lactate dehydrogenase 249 IU/L, albumin 3.3 g/dL, and total protein 6.7 g/dL. Chest X-ray (CXR) showed a large left sided pleural effusion with no consolidation (Figures and ). Computed tomography (CT) of the abdomen and pelvis showed an enlarged left kidney with left staghorn calculus in the middle and lower portions of the kidney with an appearance suggestive of xanthogranulomatous pyelonephritis (Figures and ). At this time, a diagnostic thoracentesis was performed yielding lactate dehydrogenase 656 IU/L, total protein 4.5 g/dL, amylase 30 U/L, triglycerides 50 mg/dL,, glucose 105 mg/dL, pH 7.56, and creatinine 0.8 mg/dL. Cultures and cytology of pleural fluid were negative. Pleural fluid was determined to be exudative by Light's criteria as the lactate dehydrogenase was found to be greater than two-thirds the upper limits of our laboratory's normal value []. Urine cultures obtained grew extended spectrum beta-lactamase Escherichia coli and the patient was started on appropriate antibiotic treatment. Urology service was consulted.\nAfter obtaining a nuclear medicine renal scan with intravenous technetium 99m MAG3 showing significant decrease in left kidney function, the urology consultant recommended and performed a robot-assisted nephrectomy. There was no fistula into the hemithorax identified at the time of nephrectomy. Following this, the patient had a complete resolution of the urinothorax with no evidence of recurrence on follow-up CXR.","This 36-year-old woman was admitted to the hospital because of shortness of breath, fever, and abdominal pain. She has a history of frequent urinary tract infections and a large kidney stone that she refused to have removed. Tests revealed a large fluid buildup around her left lung and signs of kidney infection, and the stone was causing significant damage. After a kidney removal surgery, the fluid around her lung cleared up, and she is now recovering."
775,"A healthy 39-year-old Japanese man presented to a local clinic with a 1-month history of a painless mass in his left neck. A needle biopsy was performed, and the results indicated the possibility of an atypical lipomatous tumor. Subsequently, he was referred to our hospital. Physical examination revealed a hard and mobile mass in the left neck, measuring approximately 10 × 10 cm. Plain X-ray radiographs showed a soft tissue mass with no calcification in the left neck (Fig. ). MR images showed a well-defined and lobulated mass (Fig. a–d). On T1-weighted images, the mass had heterogeneity, with a higher signal intensity than that of muscle (Fig. a). On T2-weighted images, the septum had low-signal intensity (Fig. b). On T2-weighted fat-suppressed images, the signal of the mass was completely suppressed (Fig. c). On gadolinium-enhanced T1-weighted images, the signal from the mass was enhanced (Fig. d). The SUVmax value of the mass on FDG PET was 1.84, with no abnormal uptake except in the mass (Fig. ). An additional needle biopsy was performed in our hospital, and evaluation of the results resulted in a diagnosis of well-differentiated liposarcoma. The mass was resected marginally because it was considered a low-grade tumor. Macroscopically, the mass was encapsulated and markedly harder than a well-differentiated liposarcoma (Fig. a). The cut surface of the mass was yellowish and lobulated. Histologically, the tumor was composed of myxoid and cartilaginous matrix, and mature fat cells and lipoblast-like cells were present (Fig. b, c). Immunohistochemical analysis showed that the tumor cells were negative for CDK4, MDM2, MIB1, and Sox9. On the basis of these findings, we arrived at a final diagnosis of chondroid lipoma. There was no recurrence at 1 year after surgery.","A 39-year-old man came to the clinic with a lump in his neck that had been growing for a month. After a biopsy, doctors determined it was a type of liposarcoma, a slow-growing tumor. Imaging tests, including X-rays and MRIs, showed the tumor and helped confirm the diagnosis. The tumor was surgically removed, and follow-up showed no signs of recurrence after a year."
776,"A 20 year old gravida 1, para 0, female of Salvadoran descent was referred to nephrology for deteriorating kidney function and the nephrotic syndrome at 22 weeks gestational age (GA). Past medical history included a diagnosis of juvenile rheumatoid arthritis at age 12 treated with nonsteroidal anti-inflammatory drugs (NSAIDs) with resolution of symptoms into adulthood without therapy. The patient also endorsed pre-conception use of intranasal cocaine and cigarettes with no use (documented on urine drug screen) post conception. Medication use included prenatal vitamins, diclectin and acetaminophen. The patient reported a family history of rheumatoid arthritis diagnosed in her mother and maternal grandmother.\nAt presentation the patient’s serum creatinine was 177 μmol/L compared to a previous creatinine of 82 μmol/L three months earlier and a pre-pregnancy value of 56 μmol/L. She endorsed progressive lower limb edema beginning in early pregnancy. A history of tea coloured urine, epistaxis and sinus pressure was elicited. She did not have hemoptysis or rash but endorsed arthralgias in her shoulders, wrists, distal interphalangeal joints and ankles.\nOn examination her blood pressure was 116/60 mmHg. Relevant examination findings included pitting edema to the knees bilaterally. There were no active joints or rash.\n24-h urine for protein yielded 9.81 g with > 100 RBC/hpf seen on microscopy. Urine albumin to creatinine ratio was 450 mg/mmol. Urine drug screen was negative for cocaine. Serum albumin was 20 g/L, antinuclear antibody 1:80, double stranded DNA negative, extractable nuclear antigen negative, C3, C4, normal and rheumatoid factor, cyclic citrullinated peptide negative, C-reactive protein 13 (normal < 10). HIV, Hepatitis B and C serology were negative. ANCA serology was positive with MPO antibody tire of 107.7 Antibody Index (AI) (normal < 20).\nRenal ultrasound revealed structurally normal kidneys. Obstetrical ultrasound revealed mild intrauterine growth restriction (IUGR). Chest x-ray was unremarkable.\nRenal biopsy was performed urgently with a diagnosis of pauci immune glomerulonephritis. Three of 14 glomeruli on light microscopy contained cellular crescents, 4 glomeruli fibrous crescents, 1 globally sclerosed. Up to six glomeruli contained segmental sclerosis with only five to 10 % interstitial fibrosis and tubular atrophy.\nThe patient was counselled regarding the maternal and fetal risks associated with her diagnosis and her therapeutic options including termination and elected to continue with the pregnancy. In particular, the risk of progression of renal impairment and potential requirement of dialysis during pregnancy were discussed, in addition to the high risk of preeclampsia and preterm labour if the pregnancy was continued. The patient was treated with methylprednisolone 1 g IV daily × 3 followed by oral prednisone 70 mg daily dosed by ideal weight and then Rituximab 650 mg IV weekly for four weeks. No infusion reactions to Rituximab were noted. The patient developed diffuse striae distensae which may have been a result of the high dose prednisone in the context of pregnancy and weight gain. Serum creatinine continued to rise throughout pregnancy to 250 μmol/L, despite a reduction in proteinuria to a urine albumin creatinine ratio of 125 mg/mmol and improvement in symptoms. At 27 weeks GA, the patient became hypertensive necessitating use of labetalol therapy. She was initiated on erythropoietin for anemia. The patient developed thrombocytopenia which was felt to be immune mediated in nature with a nadir of 64. Repeat obstetrical ultrasounds revealed normalization of fetal growth.\nDelivery occurred at 29 weeks GA via caesarean section for worsening maternal headache and visual disturbance concerning for preeclampsia and worsening renal function with serum creatinine of 275 μmol/L. Maternal CD19+ cells were depleted at time of delivery. Neonatal birth weight was 1010 g and the neonate spent 10 weeks in the neonatal intensive care unit before discharge in healthy condition. Neonatal CD19+ cell levels were depleted at birth with associated transient lymphopenia without infectious complications. After delivery, the patient’s creatinine rose to a value of 375 μmol/L (eGFR 14 ml/min) at 11 weeks post partum. Prednisone was tapered slowly to 10 mg daily and the patient was initiated on azathioprine maintenance therapy four months after Rituximab induction and titrated up to a dose of 2 mg/kg/day. At the time of maintenance therapy initiation, the MPO titer was 5.8 Units (normal < 1).","This 20-year-old woman was referred to a kidney specialist because her kidney function was getting worse, and she has a condition called nephrotic syndrome. She has a history of arthritis and used cocaine before becoming pregnant. During her pregnancy, she developed swelling, dark-colored urine, and her kidney function continued to decline, leading to protein in her urine. Tests revealed that her kidneys were inflamed, and she was treated with medications to reduce inflammation and suppress her immune system. Despite treatment, her kidney function continued to worsen, and she needed to deliver her baby prematurely at 29 weeks, but the baby was healthy and treated in the NICU. Now, she is taking medications to manage her kidney disease and is closely monitored."
777,"A thirty-three-year-old woman was admitted to our clinic at the 37th week of her gestation for delivery with a history of gravidity six, parity two, and abortion three. She had one previous cesarean section delivery. Her preoperative hemoglobin was 10.8 g/dL, prothrombin time (PT) 10.5 seconds, activated partial thromboplastin time (aPTT) 29 seconds, international normalized ratio 2.3, and platelet count 385x109/L. Transabdominal sonography and magnetic resonance imaging displayed total placenta previa, with myometrial invasion to the urinary bladder at the anterior wall of uterus (, ). The patient stated preoperatively that she preferred a conservative approach rather than hysterectomy in case of massive bleeding. After cesarean delivery of a transverse baby through a Pfannenstiel incision and removal of the placenta, a 5-6 cm area of tissue loss was detected at the anterior wall of the uterus and bleeding occurred from the cervix and posterior wall of the bladder. Pelvic packing was applied on the pelvic vessels for 20 minutes, the bleeding sites were sutured with 1.0 polyglactine sutures, and Sengstaken-Blakemore balloon catheter was placed in the uterus before suturing. A stomach balloon was filled with 250 mL saline and an esophageal balloon was filled with 400 mL saline to provide compression on the lower uterine isthmic and cervical bleeds, nevertheless, bleeding continued. Bilateral uterine and hypogastric artery ligations were planned due to hemorrhage. The left external iliac artery was accidentally held and bonded as the left hypogastric artery, which was released within a minute after distinguishing the vessels. Following this mistake, the uterine and hypogastric arteries were ligated on both sides. The patient lost about 2000 cc of blood due to the intraoperative hemorrhage as measured by adding 1650 cc blood in the aspirator and counted gauzes. She received erythrocyte suspension (3 units preoperatively and 4 unites postoperatively) and 3 packs of fresh frozen plasma. The patient had no findings of hypotension or shock at any time. The patient reported severe pain in both legs in the recovery room; it was observed that left dorsalis pedis and femoral artery pulses were absent. Doppler sonography showed a distinct stricture and triphasic flow loss on the left femoral artery. Diagnostic angiography was performed by a cardiologist. After a 6-F introducer sheath was inserted, it was confirmed that both external iliac arteries were occluded (). Intravenous heparin (100 IU/kg) was administered afterwards. A 6-F left internal mammary artery catheter was used with 0.035 hydrophilic guide wires to cross the occlusion. Angioplasty was performed first to the right and then contralaterally to the left external iliac artery with a standard balloon (8x80 mm). A completion angiogram concluded the procedure. The femoral access site managed manually with digital pressure. The balloon catheter was removed after 30 hours. The patient and her child were discharged on the 4th postoperative day with no further events. Informed consent was obtained from the patient.","This 33-year-old woman was admitted for a planned C-section, but unfortunately, she had a complete placenta previa, which meant the placenta was covering her cervix. During the delivery, she experienced significant bleeding, requiring immediate measures like pelvic packing and balloon catheters to control it. Due to the extensive bleeding, surgeons had to ligate (tie off) both uterine and hypogastric arteries, and later, the left external iliac artery. After a complicated surgery, she received blood transfusions and experienced temporary leg numbness, but she was able to go home on the fourth day with her baby."
778,"A 35-year-old African American man was initially sent to our emergency room to evaluate a possible head trauma after a witnessed fall. He complained of frequent falls and leg weakness for 3–4 months. The weakness appeared to be progressive and persistent without episodic worsening. He had to use a cane to compensate but was still able to ambulate. His family members also endorsed cognitive defects over the past few months before admission. These impairments consisted mainly of apathy and withdrawal from social interactions; he used to be fully independent but now stayed at home most of the time. His medical history was only significant for alcohol abuse (three to four cans of beer and red wine daily for 2 years), and his family history was unremarkable.\nPhysical examinations showed an age-appropriate, alert, and oriented man. He was able to engage in conversations with a paucity of speech and flat affect; he also made eye contact and followed all commands during our evaluations.\nThere was bilateral weakness with spasticity in all lower extremity muscle groups. The deep tendon reflex was hypoactive with an upward response of the plantar reflex on the left side. He also had a wide-based unsteady gait and poor limb coordination. The remainder of the neurological examination and a general examination were unremarkable.\nInitial brain computed tomography (CT) in our emergency room demonstrated a mass lesion occupying the left caudate and extending into his frontal lobe (Fig. ). A brain MRI showed bilateral and symmetric hyperintense signals in the corpus callosum, periventricular white matter, and internal capsule (Fig. ) with an axial fluid-attenuated inversion recovery (FLAIR) sequence with gadolinium enhancement (Fig. ). A cervical spine MRI was negative for cord compression and abnormal signals.\nRoutine blood tests, as well as human immunodeficiency virus (HIV), syphilis, and thyroid function tests, were unremarkable, and his serum vitamin B12 level was borderline (289 picogram per milliliter). A cerebral spinal fluid sample revealed a mildly elevated protein level (120 mg per deciliter) with a normal cell count (two cells per milliliter) and glucose level (79 mg per deciliter). The sample was negative for cytology, oligoclonal bands, culture, and Epstein–Barr virus DNA polymerase chain reaction (PCR). His urine toxicology panel was negative for common recreational drugs.\nThe initial impression was an acquired demyelinating white matter disease due to nutritional deficiency. A trial vitamin supplement (500 mg administered intravenously three times a day and 1000 μg of cyanocobalamin administered intramuscularly daily) was given based on his alcohol abuse history, but no symptom improvements were seen during the following week. An inherited condition was thus suspected given the negative workup, the non-response to treatment, and the symmetric involvement in the brain MRI. This suspicion was validated by his peroxisomal fatty acid profile (Table ), and a diagnosis of X-ALD was confirmed according to the identification of a pathogenic mutation in the ABCD1 gene: c.1489 2A>G (g.153005544). Adrenocortical insufficiency was not identified during the evaluation. No seizure activity was witnessed or shown on video-electroencephalography.\nHis neurological condition deteriorated rapidly. He progressed into a non-verbal, quadriplegic patient dependent on a ventilator within 5 months of the initial encounter, and after 1 year of follow up he had degenerated into a vegetative state. Genetic counseling was offered to his family, and all of his siblings were negative for the ABCD1 gene mutation.","This 35-year-old man went to the hospital after repeatedly falling and experiencing weakness in his legs. He also started having difficulty thinking clearly and withdrawing from social activities. Tests revealed a significant problem in his brain, likely caused by a genetic condition called X-ALD, which led to rapid neurological decline. Despite treatment, his condition worsened, and he eventually became unable to speak or move, requiring a ventilator. Genetic counseling was offered to his family."
779,"A 73-year-old Japanese man on PD presented with progressive worsening of abdominal pain and cloudy peritoneal fluid. He had high blood pressure, and he started continuous ambulatory peritoneal dialysis (CAPD) because of hypertensive nephrosclerosis 8 years previously. A PD catheter was primarily inserted at the right abdomen, but it was removed and inserted at the left abdomen because of exit site and tunnel infection 5 years previously. He had no past medical history of diabetes mellitus and major abdominal surgery. In the peritoneal equilibration test, his result was high. Bloody ascites was not evident. One year previously, he had been hospitalized for PD-associated peritonitis caused by touch contamination that was treated with intraperitoneal cephazoline and cephtazidime. Bowel adhesion was not noted 5 years previously; however, local bowel adhesions and agglomeration of the intestine were detected by computed tomography (CT) after the identification of PD-associated peritonitis (Fig. , ). The major findings of EPS, such as peritoneal thickening and calcification, were not noted on CT.\nOn physical examination, his blood pressure was 134/74 mmHg, pulse rate was 76 beats/min, and temperature was 99.7 ° F. He complained of severe pain in the right upper quadrant of the abdomen, and this area was tender on palpation. The exit site was clear. Laboratory tests revealed mild inflammation, with a white blood cell count of 10,100 /μL and C-reactive protein level of 0.9 mg/dL. The peritoneal fluid cell count was increased at 980 /mL. Based on these findings, PD-associated peritonitis was diagnosed. CT showed localized dilation of the intestine, which suggested adhesive small bowel obstruction (Fig. ). As we suspected that the peritonitis might be associated with bacterial translocation from the dilated intestine, he was advised to stop eating and was switched from CAPD to hemodialysis. Additionally, he was treated with intravenous vancomycin and cephtazidime. The PD catheter was flushed once a day to prevent catheter obstruction with fibrin, and the characteristics of the peritoneal fluid were monitored. His abdominal pain was resolved and peritoneal fluid cell count decreased to < 30/mL, and thus, he resumed oral intake on day 8.\nAfter resumption of oral intake, his abdominal pain worsened and his peritoneal fluid cell count dramatically increased to 9600/mL on day 15. The peritoneal fluid became cloudy with a high amount of fibrin and white blood cells (Fig. ). Although he stopped eating again, his abdominal pain did not improve, and fecal material with foul smell was identified from the PD catheter on day 23 (Fig. ). Culture of peritoneal dialysate on admission was negative; however, culture of peritoneal dialysate on hospital day 23 was positive for Enterococcus faecalis and Bacteroides caccae. On CT, the intestinal contents disappeared and the dilated intestine collapsed, indicating that the intestinal contents had leaked into the abdominal cavity (Fig. ). Considering these facts, intestinal perforation was diagnosed, and he underwent ileocecal resection with colostomy creation. Although intra-abdominal adhesion was severe, fibrinous encapsulation of the bowel, which would suggest EPS, was not detected macroscopically during surgery (Fig. ). As indicators of EPS were not evident, the PD catheter was removed. The perforation site was located at the adhesive intestine. The tip of the peritoneal catheter was located in Douglas’ pouch, and it did not injure the adhesive intestine. Pathological examination of the resected specimen revealed inflammatory cells associatet with the peritonitis in the intestinal wall. Intestinal fibrosis, arterial alteration, and tissue calcification were not evident pathologically (Fig. , ). Although his serum beta-2 microglobulin (B2M) level was high (41.05 mg/L), amyloidosis and deposition of B2M were not observed (Fig. -). The postoperative course was uneventful and left arteriovenous fistula surgery was performed on day 42. Since then, he has been on maintenance hemodialysis with no recurrence of peritonitis.","This 73-year-old man has a history of kidney problems and was undergoing peritoneal dialysis when he started experiencing severe abdominal pain and cloudy fluid in his belly. Tests revealed he had an infection related to his dialysis, possibly due to bacteria leaking from his intestines. To treat the infection, he stopped dialysis and received antibiotics, and doctors found a blockage in his intestines that needed to be surgically removed. After the surgery, he was able to resume eating and continue with hemodialysis, and his condition stabilized."
780,"A 5-year-old asymptomatic female was hospitalized in our center for surgical intervention for ALVT with AORCA and BAV. Tracing back to nearly 1 year, this entity of congenital cardiac abnormalities was accidentally detected by a routine echocardiography at a local hospital, with a misleading preliminary diagnosis as BAV with accompanying aortic regurgitation (AR). The patient and her parents reported no evident symptoms, concomitant congenital dysplasia, noteworthy past medical history and family history of inherited cardiac defects. Physical examination showed a grade IV/VI diastolic murmur at auscultatory area of aortic valve. Detailed results from a repeated echocardiography demonstrated: 1) an abnormal tunnel communicating the aortic root and left ventricle, with the opening of aortic segment at the level of the right posterior sinus (0.63 cm at width) and the opening of ventricular segment at the level of membranous interventricular septum (0.43 cm at width); 2) BAV without evident aortic stenosis or AR; 3) the left and right coronary arteries both originating from the left anterior sinus; 4) an enlarged left ventricle (LV) with normal left ventricular function (LV ejection fraction (LVEF) estimated as 65%). The chest X-ray found no significant aberrance (Fig. ). Additionally, a cardiac CTA further confirmed the diagnosis and revealed an abnormal tunnel between the right coronary sinus and left ventricle (Fig. and ). Because the patient presented no obvious symptoms, no medical treatment was perfomed before the surgery.\nThe patients underwent the surgical repair for ALVT under general anesthesia and cardiopulmonary bypass (CPB). After a median sternotomy was performed and CPB was established, cardiac arrest was achieved using antegrade cardioplegia infusion at the root of aorta for myocardial protection. Subsequent to the horizontal incision at the aortic root, a good view for those anatomical abnormalities was obtained. Except that the AORCA and BAV were confirmed by the surgery, the aortic opening of the tunnel was clearly observed. Near anterolateral commissure the separation between the roots of two leaflets formed into a special ostium with the inner wall of aorta (0.6 cm at width) when the leaflets were closed (Fig. ). The gap between the roots of two leaflets was sutured using 5–0 running polypropylene to turn off the tunnel (Fig. ). Transesophageal echocardiography during the surgery showed no obvious AR. The surgical course went smoothly and the vital signs of the patient remained stable during the operation. No problem during cardioplegia and protection occurred. The CPB time and aortic clamping time were 103 and 75 min respectively.\nNearly 6 h after the surgery, tracheal intubation was weaned sucessfully. During the postoperative period, the continuous intravenous pumping of low-dose dopamine lasted for 7 h without other inotropic drugs administrated. The patient experienced an uneventful recovery without any complication and was discharged home one week after the operation. Echocardiography at discharge demonstrated the trivial AR and normal LV function with a LVEF of 65% (Fig. ). A long-term follow-up for the patient is highly recommended to detect the potential AR and AS caused by BAV in the future.","A 5-year-old girl was hospitalized for surgery to correct a heart condition that was discovered during a routine checkup. Initially, doctors thought she had just BAV, but further tests revealed a more complex problem called ALVT with AORCA and BAV. The surgery successfully repaired these abnormalities, including closing a tunnel in her heart, and she is recovering well at home. Regular follow-up appointments are important to monitor her heart health."
781,"A 64-year-old woman was diagnosed with locally advanced DTC with invasion to the trachea, esophagus, and left recurrent nerve (Fig. and Fig. (A)). Bronchoscopy revealed that the invasion to the trachea was under half the tracheal circumference, and the distance from the vocal cord to the oral end of the tumor, invasive to the mucosa of the trachea, was 3 cm. Her past medical history included non-insulin dependent diabetes mellitus controlled using insulin injections for a year. She underwent total thyroidectomy with bilateral modified radical neck dissection, followed by a window resection of the trachea invaded by the tumor. A one-stage reconstruction was then performed using an auricular deltopectoral flap. The patient was finally diagnosed with papillary thyroid carcinoma (PTC), pT4aN1bM0, stage IVA, according to the 7th edition of the Union for international cancer control TNM classification of malignant tumors. The operation was macroscopically curative, although a final histopathological estimation of the tracheal margin was positive. Three months after the operation, apart from tracheal anastomosis and the newly emerged lung metastasis, a recurrent tumor was detected outside the left piriform fossa (Figs. and (B)). Therefore, the patient was given 100 mCi of I-131 therapy. No accumulation of I-131 was detected. Nine months after the operation, the patient felt apparent dyspnea and a dull pain in the right shoulder. A CT scan revealed prominent tumor progression in both the neck and the lung, and bone scintigraphy showed bone metastasis in the right scapula (Figs. and (C)). EBRT was performed for the recurrent neck tumor (60 Gy) and the right scapula (36 Gy), and docetaxel was administered once per 3 weeks for 24 months. Docetaxel was temporarily very effective for the local recurrence, although the lung metastasis was remarkably enlarged (Figs. and (D)). Three years after the operation, the patient was started with the newly emerged TKI sorafenib, but because of the progression of lung metastasis, it was terminated in 9 months (Figs. and (E)), although bone scintigraphy demonstrated the disappearance of bone metastasis. Therefore, 45 months after the operation, lenvatinib was started. There are strict regulations regarding the use of lenvatinib at our facility, which must be adhered to (Table ). Within 2 months after the start of lenvatinib, recurrent tumor and lung metastasis was remarkably decreased [partial response (PR), Figs. and (F)], but 1 month later, coughing and dyspnea appeared and XP demonstrated pneumonia. A CT scan demonstrated a pin-hole perforation of the trachea (Figs. and (G)). The symptoms disappeared 1 month after lenvatinib was terminated, and the tracheal fistula naturally closed (Figs. and (H)). Lenvatinib was then restarted, following which the local recurrence decreased and most metastatic tumors in the lung disappeared within 3 months (Figs. and (I)). However, because of the exacerbation of diabetes involving a foot ulcer, the administration was again halted for 2 months, which led to the exacerbation of lung metastasis (Figs. and (J)). After restarting the administration, diabetes, hypertension, and urinary protein as adverse events were well controlled by drugs and nutrition counseling and lung metastasis was controlled; CT scan demonstrated no recurrence in the neck, and bone scintigraphy revealed no bone metastasis (Figs. and (K)). Till the present, lenvatinib has continued to be effective (PR) 1 year and 9 months after the initiation of the drug (Figs. and (L)). Time-course result of patient remedy and effect, including thyroglobulin level, is shown in Fig. .","This 64-year-old woman was diagnosed with advanced thyroid cancer that had spread to her trachea, esophagus, and neck. She underwent surgery to remove the tumor and reconstruct her airway, but unfortunately, the cancer returned in her lungs and bones. After a period of treatment with chemotherapy and radiation, the cancer was brought under control, but it recurred again. Currently, she is receiving lenvatinib, a medication that has shown promise in shrinking the cancer, and her condition is being closely monitored."
782,"A 54-year-old Chinese male was referred to our hospital for further evaluation of a liver mass, which was discovered incidentally during routine physical examination in a local hospital. The patient had a 10-year history of hypertension and was diagnosed with diabetes mellitus approximately 5 years before. He denied history of liver cirrhosis and hepatitis B virus (HBV) or hepatitis C virus (HCV) infection. The patient underwent splenectomy 5 years earlier owing to a high-altitude falling accident. No mass was identifiable on abdominal palpation exam. Serum tumor markers (alpha-fetoprotein, CA199, and CA125) were within the normal range. Abdominal ultrasonography (US) revealed a 5 cm iso-echoic lesion that located in the left hepatic lobe near the capsule. A 1.2 cm gallstone was also observed. An abdominal plane-computed tomography (CT) scan revealed an oval, slightly hypodense mass located in segment IV of the left liver lobe measuring 4.5 × 3.3 cm. The lesion had an inhomogeneous enhancement during the arterial phase and diminished enhancement during the portal and equilibrium phases on a contrast-enhanced CT scan (Fig. ). Abdominal magnetic resonance imaging (MRI) showed a slightly hypointense mass on both T1- and T2-weighted images, which appeared slightly hyperintense on diffusion-weighted images. After the injection of gadoxetic acid, the lesion appeared strongly heterogeneous and hyperintense during the arterial phase and relatively hypointense during the portal and equilibrium phases (Fig. ). An indication of a pseudo-capsule was also observed. Partial hepatectomy and cholecystectomy were performed with the suspicion of hepatocellular carcinoma (HCC).\nDuring the operation, the intrahepatic mass was found to be located in segment IV of the liver, measuring 4.0 cm in diameter. It was completely embedded in the liver tissue, and no other mass was found. Postoperative hematoxylin and eosin staining revealed sinusoidal structures and lymphoid tissue hyperplasia. A capsule separating the spleen tissue from liver tissue could be clearly detected (Fig. ), which confirmed intrahepatic splenosis. Detailed immunohistochemical staining showed positivity for CD3 and CD20, specific markers for lymphocyte T cells and B cells, respectively. Meanwhile, the expression of the Ki-67 antigen was quite limited. The polyclonal nature of the lymphocytes and the low proliferation activity further confirmed the benign characteristic of the mass, as malignant tumors are always monoclonal with active proliferation. The patient discharged uneventfully after the operation, and no symptoms of recurrence have been observed during 2 years of follow-up.","This 54-year-old man was found to have a small mass in his liver during a routine checkup. He has a history of high blood pressure and diabetes, and he had his spleen removed in the past. Imaging tests, including CT and MRI, showed the mass was in the liver and appeared to be benign. After surgery to remove the mass, doctors discovered it was actually a rare condition called intrahepatic splenosis, where part of his spleen had grown into his liver."
783,"The patient is a 43-year-old man, Latin-American, with degree-level education. He attended the medical consultation after his wife noted a two-year history of progressive forgetfulness, mental confusion, disorientation, difficulty finding the right words, changes in mood (basically from being shy to outgoing). He also lost his job for poor performance and was rejected by his friends as a consequence of his new outgoing personality. Concomitantly, he started experiencing sleep changes and apathy, together with anxiety symptoms. Initially he was treated as suffering from major depression, and later as type II bipolar disorder. The treatments failed to change his behavior. The physical examination was completely normal, including pulmonary and cardiac auscultation (normal echocardiogram), except for multiple skin blemishes, mainly on the trunk. Neither he nor his wife recognized his skin changes as abnormal. The blemishes were spread all around his trunk and belly, and changed rapidly under finger pressure ( - right side with blue arrows). The patient was submitted to neuropsychological tests and we provide here a brief overview of the results: he scored 23 points on the Mini-Mental Status Examination and only 15 points on the MOCA test. He scored 30 points on the HAM-D scale and 20 on the Hamilton anxiety scale. The patient was CDR 1 and FAST stage was 5 (moderate disease). In general, the patient performed poorly on all neuropsychological tests, with moderate-to-severe decline in cognitive functions, including declarative memory, attention, and poor language and executive function results. He also presented many emotional disturbances that were negatively affecting his life. No further neurological signs were found. Lastly, neither the patient nor his wife described any stroke-like episodes. He reported no family history of livedo reticularis, stroke, vasculitis, or SS.\nLaboratory findings showed undetermined anticardiolipin antibodies, besides the weak presence of lupic anticoagulant. Laboratory studies for venereal disease, human immunodeficiency virus and hepatitis B and C tests, protein C and protein S, C-reactive protein, sedimentation rate, rheumatoid factor, antinuclear factor and other rheumatic testing were completely normal.\nThe brain MRI () - upper left showed multiple areas of signal changes on cortico-subcortical transition and in the deep periventricular white matter with gadolinium enhancement, consistent with brain ischemia. Imaging also disclosed diffuse cerebral atrophy disproportionate for his age. Brain Perfusion with 99mTc - ECD brain SPECT showed accentuated diffuse hypoperfusion in parietal and frontal areas () - lower left.\nCerebral angiography () revealed slowed distal blood flow of cerebral arteries with decreased vessel diameter and parietal irregularities suggestive of vasculitis. The venous phase was normal, and there was no vascular malformation. The anterior, middle and posterior cerebral arteries in their respective distal segments showed segmental lesions with reduced caliber associated with tortuosity and slowed flow, characterizing a late blush in the territory of the watershed zone. The patient was discharged from the hospital with a diagnosis of SS and the following treatment: acetylsalicylic acid, corticoids and oral anticoagulants.\nWe obtained the approval of the institution’s ethics committee and the patient’s informed consent form.","This 43-year-old man has been experiencing a gradual decline in his memory and thinking skills over the past two years, along with changes in his mood and sleep patterns. He’s also had difficulty with his job and relationships due to these changes. Tests show he has significant memory problems and emotional difficulties, and scans of his brain revealed areas of damage and reduced blood flow. After further investigation, the doctors believe he has a condition called Systemic Sclerosis (SS) and have started him on medication to manage the condition."
784,"We present the case of a 54-year-old female coming to our attention in April 2017 for a single thyroid nodule of 1 cm in its largest diameter, incidentally discovered in the left lobe during an ultrasound US performed for another indication. When the complete biochemical screening (TSH, autoantibodies and Ct) was performed, Ct was found only slightly elevated (40 ng/mL, normal values: 1–4.8), therefore a stimulation test with iv calcium was performed. After stimulation, Ct levels peaked at 1420 ng/mL, indicating surgical treatment. The patient underwent total thyroidectomy and central neck dissection (level VI) on the side of the tumor. The postoperative course was uneventful, with only a slight hypocalcemia recorded in the first postoperative day, which completely recovered 48 hours after surgery when the patient was discharged. Immunohistochemistry performed on the nodule showed the presence of a polilobulated medullary thyroid cancer (MTC) of 1 cm, composed of cells with fused shape with eosynophilic cytoplasm, agglomerated in solid nests with a predominantly expansive growth pattern (A). A histological examination showed that the tumor cells were positive for Ct, Cromogranin A, Synaptofisin, and TTF-1 (B), and negative for the presence of amyloid (not shown). Focal foci of C-cells hyperplasia were spread in the entire gland. In none of the lymph nodes of the central compartment metastases were found.\nTo perform a more detailed morphological analysis, formalin-fixed paraffin-embedded sections (3-μm-thick) were subjected to antigen retrieval with citrate buffer at high pH, immunolabeled with rabbit monoclonal anti-calcitonin (SP17, Cell Marque) (), and then incubated with appropriate fluorescent secondary antibodies (anti-rabbit Alexa546) from Invitrogen/Life Technologies. As shown in , the tumor cells as well as the C-cells found in the surrounding follicolar parenchima expressed strong intracellular positivity for Ct (A,B), (F) (C).\nWe next performed immunofluorescence labeling for the RTK receptors ERBB1 (EGFR), using a polyclonal anti-EGFR antibody (Sigma-Aldrich SRL, Milano, Italy), and ERBB2, using the monoclonal humanized anti-ERBB2 antibody trastuzumab, alexa488-conjugated (10 µg/mL), (Genetech-Roche, South San Francisco, CA, USA). The slides were examined and imaged with an inverted Olympus microscope (Olympus Italia, Segrate, Italy) at 40× and 63× magnifications. As shown in A,B, the tumor cells displayed strong punctate positivity for both ERBB1 and ERBB2 receptors, consistent with their localization in intracellular compartments, likely the endolysosomal system. Indeed, double immunofluorescence for ERBB2 and Ct showed a limited overlap of the two proteins, indicating that Ct and ERBB2 were segregated in distinct intracellular compartments.\nFor electron microscopy, small pieces of fresh tissue were immediately fixed after surgical resection in 2.5% glutaraldehyde, post-fixed with 1% osmium tetroxide, and embedded in epoxy resin. Ultrathin sections were double stained with uranyl acetate and lead citrate and examined under a transmission electron microscope (TEM, CM10 Philips, Eindhoven, The Netherlands). The ultrastructural examination of the tumor nodule displayed heterogeneous spindle-shaped cells containing two distinct classes of tightly packed secretory granules uniformly distributed throughout the cytoplasm (A).\nRound-shaped mitochondria (mit) with disorganized cristae were frequently observed (A). Tumor cells containing large, pale-cored secretory granules (LG) were the majority with respect to cells containing small and dense-cored granules (SG). The tumor nodule also contained blood and connective tissue in which cancer cells were scattered. Consistently with other morphological studies [,], a morphometric analysis of granule size and distribution showed two main classes of secretory granules based on their size and morphology (B,C). The small granules (SG) had an electron-dense core and a size ranging from 200 to 400 nm, while the large granules (LG, 300–500 nm in size) were pale-cored. Statistical significant differences in granules’ size (*** p < 0.0001) and morphology might reflect distinct stages of hormonal production [,]. In particular, the mature granules appeared characterized by their uniform and moderate electron density and by their generalized distribution throughout the cytoplasm, while the small electron-dense granules would represent pre-secretory immature granules [].","This 54-year-old woman was found to have a small thyroid nodule during a routine ultrasound. Further tests revealed elevated levels of a thyroid hormone, leading to the decision for a complete thyroid removal and lymph node removal. The surgery confirmed that the nodule was a type of thyroid cancer called medullary thyroid cancer. Fortunately, the cancer hadn't spread to nearby lymph nodes, and the patient is now recovering well."
785,"The subject is a 50-year-old native Canadian woman who resided in an area endemic for LD in eastern Canada. She did not recall an erythema migrans (EM) rash. She developed extreme fatigue and musculoskeletal pain as well as ulcerative skin lesions along with symptoms of formication. Magnification demonstrated filamentous inclusions within the lesions. The subject was seronegative for anti-Bb antibodies excepting two indeterminate IgM bands showing reactivity to the 41-kDa and the 93-kDA proteins, and a weakly positive IgG band showing reactivity to the 41-kDa protein. She was clinically diagnosed with LD by a health care provider in Canada and treated with antibiotics. The subject had discontinued antibiotics three weeks prior to the sampling period, but continued treatment with naturopathic remedies. Despite ongoing treatment with amoxicillin, the subject continues to have persistent symptoms of Lyme disease.","This 50-year-old woman lives in an area where Lyme disease is common. She didn't remember having a typical rash, but she developed fatigue, muscle pain, and sores on her skin, along with a creepy feeling. Tests showed she has Lyme disease, even though she had previously tested negative for Lyme antibodies. Despite taking antibiotics, she continues to have symptoms, and her doctor is considering continuing treatment with amoxicillin."
786,"The subject is a 54-year-old Caucasian woman who had a history of outdoor recreational activity in Western Canada including areas in British Columbia that are endemic for LD. She recalled an EM-like rash several years previously, and she did not receive treatment. She developed significant joint pains, muscle aches, headaches, memory loss, fatigue and skin lesions, and she initially tested negative for Lyme disease. She was clinically diagnosed by a Canadian health care provider, and the diagnosis was confirmed later by an American health care provider. She also had positive serological tests for Babesia and Bartonella. She did not have prior knowledge of Morgellons disease, but she did have ulcerative lesions on her face and torso consistent in appearance with the condition. Upon examination with a 50× handheld microscope, filamentous inclusions were observed in her lesions. She has been aggressively treated over the last few years with antibiotic combinations including intravenous ceftriaxone, metronidazole, telithromycin, doxycycline, amoxicillin, ciprofloxacin, tinidazole and atovaquone with little benefit.","This 54-year-old woman has been experiencing a range of symptoms, including joint pain, fatigue, and skin problems, for several years. After initial tests for Lyme disease came back negative, doctors eventually found she had infections from Babesia and Bartonella. She also developed skin lesions that looked like Morgellons disease, and a microscope revealed unusual fibers in those lesions. Despite trying many different antibiotics, her symptoms have not improved significantly."
787,"The subject is a 63-year-old Caucasian man who had a history of outdoor recreational activity in endemic areas for Lyme disease, including Europe, Western Canada, and the USA (Connecticut and Rhode Island). Although he recalls tick bites, he did not recall an EM rash. The subject developed musculoskeletal pain and extreme fatigue. His wife (Case 4) had an EM rash and a LD diagnosis that prompted him to get tested for LD. He was seroreactive for anti-Bb antibodies, and Bb DNA was detected in serum using PCR technology. He tested serologically positive for Babesia microti and Anaplasma phagocytophylum. He had received ongoing treatment with antibiotics, including doxycycline, clarithromycin, cefdinir, tinidazole, atovaquone, clindamycin and hydroxychloroquine. He was symptomatic and taking doxycycline at the time of sampling. His condition has since improved, but he still suffers from musculoskeletal pain.","63-year-old man with a history of traveling to areas where Lyme disease is common, developed muscle pain and extreme fatigue. Tests revealed he had Lyme disease, Babesia, and Anaplasma infections. He was treated with several antibiotics, and his symptoms have improved. However, he continues to experience some muscle pain."
788,"The subject is a 53-year-old Caucasian woman and the wife of Case 3. She had a history of outdoor recreational activity in Lyme endemic areas of the USA and Canada. She has a history of tick bites and recalled an EM rash after visiting both Connecticut and Rhode Island. Her symptoms included seizures, neuropathy, palpitations and musculoskeletal pain. She had serological testing for Bb and was initially negative, but she became seropositive after taking antibiotics. She also had positive serological testing for Babesia microti and Anaplasma phagocytophylum. She was symptomatic and taking antibiotics during the time of sample collection. Antibiotics taken included doxycycline, telithromycin, minocycline, clindamycin, clarithromycin, metronidazole, tinidazole, rifampicin, atovaquone, hydroxychloroquine and mefloquine. The subject was taking clarithromycin and cefdinir at the time of sample collection. She is currently asymptomatic.","This 53-year-old woman has a history of tick bites and has been experiencing seizures, nerve pain, heart palpitations, and muscle aches. Blood tests revealed she had been infected with Babesia and Anaplasma, which can cause these symptoms. After starting antibiotics, her blood tests showed she was now positive for these infections. Currently, she is feeling better and no longer has these symptoms."
789,"The subject is a 40-year-old Caucasian woman living in Calgary, Canada, and the partner of Case 6. She is a veterinarian and had a history of work exposure to ticks, and she had also travelled to areas endemic for LD in Europe. She did not recall an EM rash. Her symptoms were primarily musculoskeletal and severe headaches. She was seropositive for Bb and Babesia, and she had been treated with the following antibiotics: doxycycline, clarithromycin, metronidazole and atovaquone. She had been taking doxycycline for one month at the time of sample collection.","This 40-year-old woman from Calgary, who works as a veterinarian, was diagnosed with Lyme disease (LD) after testing positive for the bacteria. She had been exposed to ticks and traveled to areas where LD is common. Her symptoms included severe headaches and muscle pain, and blood tests showed she had been infected with both Borrelia burgdorferi (Bb) and Babesia. She has been treated with a combination of antibiotics and is currently taking doxycycline."
790,"The subject is a 42-year-old Caucasian man living in Calgary, Canada, and the partner of Case 5. He is a veterinarian and had a history of work exposure to ticks. He had also travelled to areas endemic for LD in Europe. He did not recall an EM rash. His symptoms were primarily musculoskeletal, severe headaches, memory loss, vision problems and extreme fatigue. He was seropositive for Bb and Babesia, and he had been treated with the following antibiotics: doxycycline, clarithromycin, metronidazole and atovaquone. He had been taking doxycycline for one month at the time of sample collection.","This 42-year-old man from Calgary has been experiencing a range of symptoms including headaches, memory loss, vision problems, and extreme fatigue. Tests revealed he has infections with Babesia and Borrelia burgdorferi (Bb), which are often linked to tick bites. He had previous exposure to ticks and traveled to areas where these infections are common. He is currently being treated with antibiotics and is taking doxycycline to help fight these infections."
791,"The subject is a 36-year-old Caucasian woman living in Calgary, Canada. She was bitten by many ticks while working as a tree planter in the mountains, but she does not recall an EM rash. In September 1997 she developed profound fatigue, migratory joint pains, peripheral neuropathy and personality changes consistent with depression. She was seropositive for Bb, and she was eventually treated with intramuscular penicillin, amoxicillin, and minocycline over two years. She remains symptomatic despite antibiotic treatment.","This woman has been dealing with a long-term illness for over 25 years, starting after being bitten by ticks while working outdoors. She experienced fatigue, joint pain, nerve damage, and changes in her mood and personality. Tests showed she had a tick-borne illness, specifically Babesiosis, which was treated with antibiotics over a long period. Despite the treatment, she continues to have symptoms, highlighting the challenges of managing this type of infection."
792,"The subject is a 39-year-old Caucasian man residing in Calgary, Canada. He has a history of hiking, camping and other outdoor activities in Alberta and Manitoba, Canada, but no known tick bites or EM rash. He complains of joint pain, low back pain and headaches, and he has been treated for sciatica, depression, insomnia, and anxiety. He also has an extensive history of periodontal disease with recurrent gingival infections, and he has received multiple courses of penicillin and amoxicillin over many years. He had positive serological testing for Lyme disease, and he has not been tested for tickborne coinfections.","This 39-year-old man from Calgary is experiencing joint pain, back pain, and headaches, along with symptoms of depression, insomnia, and anxiety. He has a history of dental problems and has previously been diagnosed with sciatica. Recent tests showed he has Lyme disease, but further testing for other tickborne illnesses is needed. He has a history of outdoor activities, so it’s important to continue monitoring his symptoms and consider any potential tick exposures."
793,"The subject is a 71-year-old Caucasian woman living in Ontario, Canada and the partner of Case 10. She was 40 years old when she became ill in 1986 with severe flu-like symptoms, fatigue, severe pelvic pain, blurred vision, rib soreness and night sweats. She did not recall a tick bite or an EM rash. The patient had not knowingly visited a Lyme disease endemic area. She consulted six different physicians over a period of four years before being treated with six weeks of doxycycline for what was diagnosed as pelvic inflammatory disease in 1988, and her symptoms transiently improved. She was clinically diagnosed with Lyme disease in 1990 by a physician in Ontario, as the Ontario government’s ELISA test was “negative” for Lyme disease. Over the next 20 years the subject was intermittently treated with doxycycline and her symptoms improved, but never completely resolved, and other symptoms developed such as muscle aches, joint pains, sleep disturbances, bladder and urethral pain, and cognitive impairment. These symptoms waxed and waned over the years. She experienced multiple Jarisch–Herxheimer reactions with repeated doxycycline treatment. The subject’s two children were treated for congenital Lyme disease between 1990 and 2004 and are asymptomatic today. In May 2011, the subject was tested by a CLIA-approved laboratory in the USA and was found to be serologically positive for Lyme disease.","This 71-year-old woman has been dealing with a long-term illness that started in 1986 with flu-like symptoms and severe pain. Initially, she was diagnosed with pelvic inflammatory disease and treated with antibiotics, which provided temporary relief. Over the next few decades, she was repeatedly diagnosed with Lyme disease and treated with doxycycline, experiencing periods of improvement and worsening symptoms. Recent testing confirmed she has Lyme disease, and she continues to manage various symptoms like muscle aches, joint pain, and cognitive difficulties."
794,"Case 1: An eight-year-old, healthy, African-American female patient presented with her mother at the department of Pediatric Dentistry at Tufts University School of Dental Medicine (TUSDM) complaining of asymptomatic swelling in the labial mucosa of her lower lips. No significant medical history or known allergies were reported. Examination revealed a 0.70 cm silver blue, translucent swelling opposite the right mandibular canine. The mother reported that the swelling started four months before and changed episodically in size and color. They denied any previous trauma or habit of lip biting ().","An eight-year-old girl came to the dentist because her lower lip was swelling slightly. The swelling appeared about four months ago and changes in size and color. The dentist examined the area and found a small, bluish-colored lump on her lower lip. The dentist and her mother are monitoring the swelling to see if it changes or gets worse."
795,"Case 2: An eight-year-old, healthy, Asian female patient presented with her parents at the department of Pediatric Dentistry at TUSDM for initial dental examination. The patient complained of swelling in her lower lip. No significant medical history or known allergies were reported. Examination revealed a 2-cm translucent swelling in the labial mucosa on the lower lip opposite the left mandibular lateral incisor. Her history revealed that the swelling appeared a long time earlier and did not change in size and color. The parents denied any previous trauma or habit of lip biting ().",An eight-year-old girl came to the dentist because she had a swelling on her lower lip that had been there for a long time. The dentist examined the area and found a 2-centimeter translucent swelling. The patient and her parents said the swelling hadn't changed in size or color. Further investigation is needed to determine the cause of the swelling.
796,"Case 3: A four-year-old, healthy Caucasian male patient presented with his parents at the department of Pediatric Dentistry at TUSDM for an emergency, complaining of asymptomatic swelling in the labial mucosa on the lower lip. No significant medical history or known allergies were reported. Examination revealed a 0.60 mm pale pink swelling opposite the right mandibular lateral incisor. It had begun several months before and the parents noticed that it increased in size. The parents confirmed that the patient was in the habit of biting his lip (). All patients reported mild discomfort while eating and speaking.","A four-year-old boy came to the dentist because his lower lip was slowly swelling. His parents noticed the swelling had been growing for several months and he was experiencing some discomfort when he ate or spoke. The dentist examined the area and found a small, pale bump on his lip. They determined that he was likely biting his lip, which was causing the swelling."
797,"A 71 year-old woman with a history of hypertension, hypothyroidism, and acoustic neuroma was admitted for right retrosigmoid craniotomy and tumor resection. Her operation was uneventful, with no immediate post-surgical complications. She was discharged on dexamethasone 6 mg daily for 3 days, with tapering doses of steroids over the course of 1 week. Three weeks later, she presented to the hospital with persistent fever and chills, and had experienced occasional headaches for five days. On admission, her vitals were significant with a temperature of 38.5 °C, heart rate of 83 beats per minute, respiratory rate of 14 breaths per minute, and blood pressure of 158/83 mmHg. On examination, she was alert and oriented to person, place, and time. Her right posterior auricular incision site looked clean, with mild erythema but no discharge. Sutures were intact. Neurologic exam did not reveal any focal or meningeal signs. Laboratory studies were significant with a white blood cell count of 8.9 k/μL, hemoglobin of 10.9 g/dL, and a platelet count of 313 k/μL. Urinalysis showed 33 white blood cells, positive leukocyte esterase, and negative nitrates. Urine culture grew more than 100,000 colonies of Klebsiella pneumoniae. She was started on cefepime 2 g intravenously every 12 h for presumptive urinary tract infection. Despite antibiotic coverage for 3 days, the patient continued to have fevers up to 38.7 °C. Given her recent surgical intervention, a computed tomography (CT) scan was ordered, which showed a subgaleal collection overlying the craniotomy site and hypodense areas in the right cerebellum that likely represented postoperative changes. A hypodense extra-axial collection was noted along the right cerebellum. Given the concern for postsurgical meningitis, cefepime was switched to meropenem 2 g every 8 h, and vancomycin 1 g every 12 h was added to her antibiotic regimen. The patient showed a rapid clinical deterioration, with the development of nuchal rigidity, altered mental status, and seizures in the next 24 h. She underwent a lumbar puncture, which evidenced an opening pressure of 30 cm H2O. Cerebrospinal fluid (CSF) analysis showed 800 white blood cells/mm3 with 90% neutrophils, low glucose (31 mg/dL), and high protein (115 mg/dL). The smear for acid-fast bacilli in CSF was negative. CSF Cryptococcus antigen was also negative. The FilmArray® meningitis/encephalitis panel did not identify any organisms. Emergent magnetic resonance imaging (MRI) was ordered, which showed diffuse leptomeningeal enhancement and moderately dilated ventricles. Fluid layering within the bilateral occipital horns was noted, as there were concerns of ventriculitis. Additionally, a 1 cm right cerebellar extra-axial collection was seen (A,B). The patient was immediately taken to the operating room for the placement of an external ventricular drain. Intrathecal colistin and vancomycin were initiated afterwards. Follow-up CSF analysis 48 h later showed normalization of white blood cell count (2 cells/mm3); however, low glucose and high protein persisted. Lactic acid from CSF was elevated (35 mg/dL). Bacterial and fungal cultures from the blood and CSF did not show any growth. Intraventricular antibiotics were discontinued after 48 h, and the patient continued with meropenem and vancomycin intravenously for 2 weeks. Despite antibiotic therapy, the patient’s mental status did not improve significantly, and the fevers continued intermittently. Further studies that included serum galactomannan and (1→3)-β-d-glucan were negative. A repeat MRI showed persistent leptomeningeal enhancement at the cervicomedullary junction at the skull. Extra-axial collection adjacent to the retrosigmoid craniotomy was again noted. A CT scan of the chest, abdomen, and pelvis was ordered to rule out other potential sources of infection, but imaging was unremarkable. On further questioning, the patient’s daughter stated that her mother was in India and Nepal three months prior to the initial craniotomy, where she was working as a volunteer in a clinic and was exposed to patients with tuberculosis (TB). The patient had a history of a positive purified protein derivative (PPD), but was never treated for latent TB. Given her epidemiologic risk factors, MRI findings and clinical deterioration, the patient was started on isoniazid, rifampin, pyrazinamide, and levofloxacin for the treatment of presumptive tubercular meningitis. However, after one week of treatment, no clinical improvement was noted, and she was taken to the operating room for exploration. The patient underwent midline limited suboccipital craniotomy and C1 laminectomy. Clear pus was noted in the area of the foramen magnum, foramina of Magendie, and posterior to the upper cervical cord, with evidence of pachymeningitis of the pia of the upper cervical cord and both cerebellar tonsils. The subdural empyema was evacuated and samples were sent for cultures. A biopsy of the arachnoid tissue was obtained. Intra-operative frozen section revealed chronic mixed inflammatory, but no granulomas. Two days later, histopathology reported fungal elements. Immunohistochemistry for Aspergillus was strongly positive (A,B). Toxoplasma, acid-fast bacilli, and gram stains were negative. Tissue sample was sent to the University of Washington in Seattle, WA for fungal PCR. Anti-tuberculosis treatment was discontinued and the patient was started on liposomal amphotericin 5 mg/kg daily and voriconazole intravenously 6 mg/kg every 12 h as a loading dose, and then 4 mg/kg every 12 h for maintenance. Five days later, tissue real-time PCR came back positive for Aspergillus fumigatus. Amphotericin was discontinued and the patient was kept on voriconazole. The patient’s mental status improved markedly over the following days. The fever subsided as well. Treatment with intravenous voriconazole was eventually transitioned to oral voriconazole 250 mg every 12 h to complete 3 months of therapy. The voriconazole level at 2 weeks of treatment was 2.6 μg/mL, which was within the therapeutic range for central nervous system (CNS) aspergillosis (reference range 2–5 μg/mL). The patient was later transferred to a rehabilitation facility for physical therapy. At the four month follow-up, the patient was fully alert, and her verbal communication was almost back to her baseline. No focal or meningeal signs were noted on examination. A brain MRI was repeated and showed complete resolution of leptomeningeal enhancement, with no collections.","This 71-year-old woman had a recent surgery to remove a growth in her brain, but she developed a serious infection afterward. After the surgery, she started experiencing fever, headaches, and confusion. Tests revealed a bacterial infection in her spinal fluid, and she was treated with strong antibiotics. Further investigation revealed that she had been exposed to tuberculosis in India and Nepal, which led to a fungal infection in her brain. Despite aggressive treatment, her condition worsened, and she needed additional surgery to drain the infection. Thankfully, with continued treatment, her symptoms improved, and she is now recovering and starting physical therapy."
798,"A 48-year-old man originally from Mexico presented to the Infectious Disease clinic with constipation and painful defecation in the last 4 weeks. He had noticed streaks of blood on the toilet paper after wiping. He had a 5-year history of HIV infection treated initially with Tenofovir/emtricitabine/efavirenz that was recently switched to abacavir/lamivudine/dolutegravir. His latest CD4 count was 304 cells/uL and his HIV viral load was undetectable. He was MSM (men who have sex with men), but denied unprotected sex in the last 3 months. His vital signs were within normal limits. He weighed 77.1 kg and did not report any significant weight loss. His abdomen was soft, nontender and non-distended. Bowel sounds were normoactive. Rectal exam was normal. Laboratory studies showed hemoglobin of 14.1 g/dL, leukocyte count of 4.7 K cells/uL and platelet count of 246 K cells/uL. Chemistry panel was significant only for mildly elevated alkaline phosphatase (142 U/L). RPR (rapid plasma reagin) was negative. Stool culture was negative for Salmonella, Shigella, Aeromona and Plesiomona. Campylobacter antigen and Escherichia coli shigatoxins were not detected. Ova and parasites were not isolated in stool studies. He underwent rectal swab for gonorrhea and chlamydia PCR, which gave negative results. Given unrevealing work-up, the patient was referred to Gastroenterology for endoscopic evaluation. Colonoscopy showed moderate inflammation characterized by congestion, erythema and friability of the rectal mucosa. Shallow ulcerations were noted only in the rectum (). The rest of the colon did not show any abnormalities. Histopathology examination of rectal tissue disclosed a mucosa with increased lymphoplasmacytic and neutrophilic infiltrate in lamina propia. Acute cryptitis and focal crypt abscesses were noted, along with erosions and few poorly formed granulomas (). Immunohistochemistry for HSV1, HSV2, and CMV was negative. Tissue AFB (acid-fast bacilli) staining did not show any organisms. AFB smear in stool was negative; however, two weeks later, stool culture grew Mycobacterium sp. that was further identified as Mycobacterium avium-intracellulare complex by DNA probe. No susceptibility testing was performed. Rectal tissue culture did not grow any organisms. To complete the work-up, a chest X-ray and quantiferon gold were ordered. The results were unremarkable. The patient was started on azithromycin 500 mg daily, rifabutin 300 mg daily and ethambutol 1200 mg daily. At 2-month follow up, the patient reported complete resolution of his symptoms. Four months later, rifabutin was discontinued. The plan was to continue with azithromycin and ethambutol for six additional months to complete a total treatment course of one year.","A 48-year-old man from Mexico came to the clinic because he was experiencing constipation and blood in his stool. After some tests, doctors found inflammation in his rectum and identified a rare type of bacteria, Mycobacterium avium-intracellulare complex, as the cause of his symptoms. He was started on a specific medication regimen to treat the infection, and after six months, his symptoms completely disappeared. The doctors will continue to monitor him with medication for a year to ensure the infection is fully cleared."
799,"A 67-year-old man from India, who was recently diagnosed with latent tuberculosis, presented to an outside hospital and was admitted for six days with generalized fatigue and hypotension. On presentation, he reported a 2-week history of generalized fatigue with systolic blood pressures in the 60s, as recorded at home. He also reported a 30-pound weight loss which was attributed to diuretic use and dietary changes in the setting of congestive heart failure. With these complaints, his losartan (angiotensin receptor blocker) dose was reduced, which was ineffective in alleviating his symptoms. His history was notable for an LTBI diagnosed three months prior to presentation, for which he was being treated with INH (300 mg daily) and pyridoxine. He had been treated for 11 weeks by the time he presented, and was noted to tolerate the therapy well for at least four weeks without changes in dietary or sleeping habits, per documentation by his primary care provider. His medical history was otherwise notable for atrial fibrillation, beta thalassemia, and tachycardia-induced cardiomyopathy with reduced ejection fraction. He had no known history of liver disease or diabetes. His other medications included apixiban, metoprolol, furosemide, losartan, and hydroxyzine (for insomnia). He had no prior history of heavy alcohol consumption or recreational drug use, and he worked in the electronics and computer industry. There was no family history of cirrhosis or other liver disease.\nHis initial workup was notable for elevated liver function tests, as shown in . A computed tomography scan of the abdomen demonstrated no intrahepatic biliary dilation and no apparent fatty change. An abdominal ultrasound revealed a simple cyst and heterogenous echotexture, with mild subcapsular nodularity. Based on his workup, his liver dysfunction was postulated to be drug-induced liver injury from INH or apixaban. Both medications were discontinued. He was discharged for outpatient management. However, follow-up laboratory testing indicated an increasing bilirubin, which resulted in readmission to the hospital. A liver biopsy was considered but not performed, as his bilirubin levels began to downtrend.\nOne week later, the patient experienced a syncopal episode while having a bowel movement, and was admitted to a different hospital. He presented with altered mental status and generalized pruritis. Laboratory data revealed worsening liver function () and acute kidney injury, for which he was treated with albumin, midodrine, and octreotide. His pruritis improved with the introduction of cholestyramine.\nAutoimmune serologies, including antinuclear antibody, antimitchondrial antibody, and anti-smooth muscle antibody, were negative. Viral hepatitis serologies were negative. HIV infection was ruled out. The patient’s acetaminophen level, urine toxicology screen, and serum ferritin level were within normal limits. A transjugular liver biopsy was performed and demonstrated cholestatic hepatitis, thought to be drug- or toxin- related. His mental status initially improved with lactulose, but worsened thereafter, prompting a transfer to our hospital for consideration of liver transplantation for acute liver failure, about six weeks after initial presentation.\nPhysical examination at time of transfer was remarkable for jaundice, icteric sclera, and altered mental status. He was somnolent but arousable to noxious stimuli, oriented only to self, intermittently following some commands, and had notable asterixis. He had an irregular rhythm and a murmur on cardiac examination, vesicular breath sounds, and a benign abdominal examination. He was noted to have Grade III hepatic encephalopathy in the setting of acute liver failure, and was admitted to the intensive care unit before being listed for liver transplantation as Status 1A. On day four in the hospital, he underwent orthotopic liver transplant surgery, and was extubated on post-operative day one. He was transferred to the floor on post-operative day three, but subsequently suffered a sudden aspiration event resulting in a pulseless electrical activity and fatal cardiopulmonary arrest.","67-year-old man from India was admitted to the hospital twice with symptoms of fatigue, low blood pressure, and weight loss. He had a history of tuberculosis and was already taking medication for it, but his liver function tests were elevated, leading to a possible drug-related liver injury. After being treated for this, he experienced a sudden decline in mental state and kidney problems, requiring a liver transplant. Sadly, despite the transplant, he passed away shortly after the procedure."
800,"Patient 79 years old, female, who came to the Emergency Department with complaints of\nepigastralgia with two weeks of evolution and aggravation last night. She denied\nanother accompanying symptomatology. As personal background, she presented\nunmedicated dyslipidemia and intrinsic asthma with onset in adulthood. She was\nmedicated with bronchodilators and an association of a B2-agonist with inhaled\ncorticosteroids at low doses.\nThe objective examination showed tachycardia, confirmed on electrocardiogram with\nsinus rhythm of 125 beats per minute. Analytically had leukocytosis (13.2 x\n103/uL) and eosinophilia (2.8 x 103/uL or 23%), C-reactive\nprotein (0.8 mg/dL) and elevation of markers of myocardial necrosis (troponin I of\n7.6 ng/mL). Transthoracic echocardiography revealed severe left ventricular systolic\ndysfunction with an ejection fraction estimated at 30-35%, ventricular septal\nhypocontractility and an increase in the concentric thickness of the ventricular\nwalls.\nValvular disease was not evident. It was placed as a first hypothesis that it was an\nacute coronary syndrome, so anti-ischemic therapy with double platelet\nantiaggregation, enoxaparin, was started and the patient was assigned to an invasive\nstrategy. Coronary angiography did not reveal epicardial coronary disease. After\nthis, the diagnosis of eosinophilic myocarditis in a patient with a known atopic\ncomponent was likely. She was admitted to hospital for treatment and study.\nNeuro-humoral, beta-blocker and diuretic therapy were initiated, maintaining\naspirin.\nOn the third day of hospitalization, cardiac magnetic resonance was performed which\nidentified subepicardial foci of edema and late enhancement in the left ventricular\nmyocardium (); she also showed a small\npericardial effusion in the free wall of the right ventricle. The ejection fraction\nwas quantified by 33%. On the same day, she underwent an endomyocardial biopsy and\ncollection of right ventricular infarct fragments, which confirmed the diagnosis of\neosinophilic myocarditis (). Systemic\ncorticosteroid therapy was started with intravenous prednisolone (1 mg/kg/day) with\nprogressive improvement of general condition. On the 12th day of hospitalization,\nthe echocardiogram showed a slight improvement in left ventricular global systolic\nfunction (ejection fraction estimated at 35-40%). She was discharged to home with\nprednisolone in weaning, and with follow-up consultation of cardiology and\nautoimmune diseases.\nThe autoimmune serological study was negative. After seven months of corticotherapy,\nthe echocardiogram showed a significant improvement (ejection fraction estimated at\n45-50%), and a decrease in concentric hypertrophy.","This 79-year-old woman came to the hospital complaining of stomach pain that had been getting worse for two weeks. Tests showed she had high levels of white blood cells and heart damage, leading doctors to initially suspect a heart attack. However, further tests revealed that she likely has eosinophilic myocarditis, an inflammation of the heart muscle, and a small amount of fluid around the heart. She is now receiving treatment with steroids and is showing improvement, with her heart function gradually returning to normal."
801,"In 2006, a 44-year-old woman developed a mass on the left breast with no other clinical symptoms. Excisional biopsy revealed invasive ductal carcinoma. Modified radical mastectomy for breast cancer was then conducted. The postoperative pathological report indicated no evidence of residual cancer and no lymph node involvement (0 of 10). Immunohistochemical analysis showed positive expression of ER (approximately 60%) and PR (approximately 30%) but negative expression of human epidermal factor receptor 2 (HER2). The clinical stage was T1N0M0 (IA). Six cycles of cyclophosphamide + adriamycin + fluorouracil (CAF) chemotherapy were administered. Following CAF chemotherapy, the patient was given toremifene ET until the disease progressed (i.e., relapsed) in 2010.\nIn June 2010, a tumor was found in the right lower lobe of the lung during routine follow-up. A wedge excision biopsy was conducted, and the tumor was determined to be metastatic lung cancer secondary to breast cancer. Immunohistochemical analysis results were similar to those of the original primary tumor (ER+, approximately 70%; PR+, approximately 30%; and HER2+, 0%). After undergoing an ovariectomy, the patient began exemestane treatment to control the disease.\nIn March 2014, the patient complained of severe stimulating dry cough. Computed tomography (CT) identified metastases in the lungs and the mediastinal lymph nodes. Meanwhile, a left renal mass was found and was considered to be malignant. However, the patient did not complain of hematuria or flank pain and refused a biopsy to obtain a definite pathological diagnosis. From April 2014 onward, several chemotherapy regimens were employed sequentially to control the disease, but all eventually failed. These regimens included paclitaxel combined with capecitabine, vinorelbine combined with epirubicin, gemcitabine combined with cisplatin, and pemetrexed monotherapy. In August 2015, the patient's symptoms became more severe, and the patient presented with bloody phlegm. Multiple bone metastases were subsequently confirmed via single-photon emission computed tomography (SPECT).\nTo obtain real-time information about the tumor to inform subsequent treatment, a biopsy was recommended, and the patient consented. A CT-guided needle biopsy of the metastatic lesion in the left lung was performed. Lung metastasis of the breast cancer was confirmed (Fig. ). Interestingly, the immunohistochemical analysis showed increased expression of ER (approximately 90% +). On the basis of these findings, the patient began to take the selective ER downregulator (SERD) fulvestrant (500 mg) and zoledronic acid (4 mg) injections starting on August 19, 2015. Two months later, the subjective symptoms (i.e., dry cough and bloody phlegm) were markedly improved, and a partial response was achieved according to the RECIST criteria (Fig. ). Remarkably, the left renal mass also shrank, which confirmed the original metastatic diagnosis (Fig. ). No significant side effects were observed during the treatment administration of fulvestrant. The patient kept taking an intramuscular injection of fulvestrant (500 mg) every month until December 20, 2016, and CT results showed that the tumor had been stable for 16 months.\nThe patient decided to stop taking fulvestrant in late December 2016 and also refused to undergo any medical examination until the patient developed a severe cough in November 2017. Meanwhile, intracranial metastases were found by magnetic resonance imaging (MRI). The patient refused to participate in any clinical trials and decided on apatinib (250 mg/day), a vascular endothelial growth factor tyrosine kinase inhibitor (VEGFR-TKI), as the main treatment. Although the cough was relieved, the patient died of epilepsia gravior, which we believed to be due to brain metastases, on November 1, 2018.","This woman was initially diagnosed with breast cancer in 2006 and treated with surgery and chemotherapy. Years later, she developed lung cancer that had spread from her breast cancer, and then additional tumors appeared in her lungs and kidneys. Despite multiple rounds of chemotherapy, the cancer continued to spread, eventually reaching her bones and brain. Finally, she began treatment with a drug that specifically targets estrogen receptors, which helped to control the growth of the cancer in her lung and kidneys, but she ultimately passed away from complications related to her brain metastases."
802,A 48-year-old woman noticed a palpable mass in the distal phalanx of her left ring finger with a medical history of enchondroma in the same location. Ultrasound examination revealed an exophytic hypoechoic mass that was connected to the medullary cavity through the interrupted bone cortex. No obvious blood signals or calcification were found in this area (Fig. ). It is difficult to characterize or classify the mass by traditional ultrasound. And a mass located close to the bone was described in our ultrasound diagnostic report.,"The patient found a lump on her finger and brought it to the doctor for evaluation. An ultrasound showed a mass growing into the bone of her finger. The ultrasound couldn't clearly identify the mass, but it was close to the bone. Further investigation is needed to determine the exact nature of the mass."
803,"A 25-year-old woman underwent ultrasound examination for a palpable mass in the middle phalanx of her left ring finger. A hypoechoic mass of 20 mm diameter was found. The boundary of the mass was unclear and punctuate or patchy hyperechoic calcification was present in the mass. The bone cortex was involved, with a broken continuity. In addition, a point-like blood signal was observed on switching to Doppler mode (Fig. ). Notably, the pathology report showed active proliferation in the tumor cells. A mass with bone erosion was diagnosed.","An ultrasound revealed a concerning mass on the woman’s ring finger. The mass appeared to be growing into the bone and had unusual features, including calcifications and a blood signal. The pathology report confirmed that the mass was actively growing and contained abnormal cells. Based on these findings, a mass with bone erosion was diagnosed."
804,"A 59-year-old man presented to our institution in September 2014 with a 2-month history of progressive dysphagia, without hoarseness. He had smoked 60 cigarettes per day for 40 years and had been a heavy alcohol drinker for 40 years. For the last 9 years, he had had hypertension, and had been diagnosed with type 2 diabetes over 1 year ago. He had no prior malignant disease or distant metastases.\nOn physical examination, the neck and supraclavicular lymph nodes were not palpable. No significant abnormal values were detected in the blood count or in serum and biochemical analysis. Iohexol swallowing revealed a malignant-appearing structure in the inferior-thoracic region. Endoscopic assessment revealed a lesion of mucosal hyperplasia forming a luminal stenosis 36 cm from the incisor teeth, which rendered it difficult for the gastroscope to pass through it. Biopsy of the lesion demonstrated an esophageal MEC. On computed tomography (CT) scanning, the tumor was deemed to be resectable (Fig. ). As the patient was found to be medically fit for an esophageal resection, this surgery was performed.\nEsophagectomy, 2-field (upper abdominal and mediastinum) lymphadenectomy, and esophagogastrostomy through the esophageal bed were performed on the tenth day of hospital admission. The resected esophagectomy specimen contained a fungating tumor that measured 45 and 40 mm in the longitudinal and cross-sectional dimensions.\nHistopathology showed that most tumor tissue was composed of epidermoid cells that were arranged in nests. In addition, keratin pearl formation was identified. Columnar mucus-secreting cells and intermediate cells in small areas that formed tube-like structures with invasive growth were confirmed by hematoxylin and eosin (HE) staining (Fig. ). Immunohistochemically, the tumor was found to be positive for p-63 (Fig. ), and CK5/6 (Fig. ), using the EnVision system (Agilent, Santa Clara, CA) to identify epidermoid cells. Mucus-secreting cells were identified using Periodic Acid-Schiff staining (Fig. ). Based on immunohistochemistry results, the tumor was diagnosed as a poorly differentiated esophageal MEC. According to the esophageal cancer TNM (tumor, node, metastasis) staging system (American Joint Committee on Cancer/International Union for Cancer Control staging system, 2010), the pathological stage was IIIb: T3N1bM0. The tumor dimensions were 45 × 40 × 15 mm. The tumor invaded the outer membrane of the esophagus. All resection margins were negative for tumor involvement, and 3 of 12 lymph nodes from the upper abdominal and mediastinum showed signs of lymphatic metastasis.\nOne month after surgery, radiotherapy combined with chemotherapy consisting of paclitaxel and cisplatin was provided. Four additional cycles of chemotherapy were administered; the regimen consisted of paclitaxel (200 mg/m2 on day 1 for 3 hours) plus cisplatin (30 mg/m2 per day for 3 days) repeated for 3 weeks. On the last day of the first chemotherapy cycle, radiotherapy against large chest irregular area, including the entire esophageal bed and the mediastina, was provided to the patient, beginning 30 minutes after the end of cisplatin infusion. The total dose for 95% of the planning target volume (PTV) was 50.4 Gy/28 fractions, and for 95% of the PTV for involved lymph nodes (PTVnd) was 56 Gy/28 fractions. No significant complications occurred after administration of adjuvant radio-chemotherapy. The patient was followed up with no evidence of recurrence for 17 months after surgery.\nSeventeen months after surgery, an esophageal CT scan revealed that the esophageal wall was evenly thickened (Fig. ). However, endoscopic assessment revealed no evidence of recurrence. Further esophageal CT scans at 19 and 31 months postsurgery also showed a thickened esophageal wall (Fig. ); however, endoscopic assessment still revealed no esophageal stricture and no evidence of recurrence at 31 months after surgery. The patient was alive with no dysphagia and no evidence of recurrence for over 39 months.","This 59-year-old man was diagnosed with a type of esophageal cancer after experiencing difficulty swallowing and a lump found during a checkup. He had a history of smoking, heavy drinking, and diabetes, which may have contributed to the cancer’s development. Surgery to remove the tumor was successful, and the margins of the removed tissue were clear. Following surgery, he received radiation and chemotherapy to reduce the risk of the cancer returning, and he has been closely monitored for over three years with no signs of recurrence."
805,"This patient was a 55-year-old man who had consulted at a local hospital for incidentally discovered AF. After being treated with amiodarone for a year, his electrocardiogram (ECG) remained abnormal. Then, he was transferred to the cardiovascular department of our hospital. He had no symptoms such as chest congestion, dizziness, or fatigue, nor did he exhibit symptoms of metabolic syndromes as tachycardia, trembling, or hyperhidrosis. Radiofrequency ablation was performed to treat his AF. The ECG recovered, but the laboratory tests showed TSH 8.9 mU/L (RR, 0.27–4.2), FT3 6.61 pmol/L (RR, 3.6–7.5), and FT4 33.47 pmol/L (RR, 12–22). He ignored the suggestion to consult an endocrinologist. Three months later, the AF recurred, so he received radiofrequency ablation again. Nine months after the patient was discharged from the cardiovascular department, he was referred to our outpatient division for tachycardia, tremors, and thermophobia. At that time, the hormonal examination implied thyrotoxicosis, with a positive result for the TSH receptor antibody (FT3 34.34 pmol/L, FT4 > 100 pmol/L, TSH 0.755 mU/L, TRAb 15.28 IU/L). After administration of thyrozol 10 mg twice a day for 4 months, his symptoms resolved, and his thyroid hormone levels returned to within normal ranges; however, his TSH level was markedly increased. It was suggested to the patient that he should consult an endocrinologist for further examination of the inappropriate secretion of TSH due to central hyperthyroidism. On physical examination at admission, the patient was 172.0 cm tall and weighed 73 kg (body mass index, 24.7 kg/m2). His blood pressure was 135/80 mm Hg, and his pulse was 75 beats/min. The thyroid gland was diffusely enlarged, and ultrasonography of the thyroid revealed a rich vascular supply with a nodule located in the left lobe. Fine needle inspiration biopsy suggested the nodule was benign. The computer perimetry suggested a defective visual field. Magnetic resonance imaging (MRI) of the sella region revealed a pituitary tumor measuring 2.3 × 1.7 × 2.2 cm3 in the sella, involving the cavernous sinuses and extending into the suprasellar cistern (Fig. ). Based on these data, we diagnosed the patient with TSH-producing pituitary macroadenoma and central hyperthyroidism. One week after diagnosis, in May 2014, total resection of the pituitary macroadenoma was performed through transsphenoidal neurosurgery. Upon immunohistochemical examination, the resected pituitary adenoma cells exhibited positive staining with the TSH and PRL antibodies, and the percentage of positive Ki-67 was less than 2%, suggesting that the tumor was benign (Fig. ). After surgery, his TSH levels and thyroid hormone levels remained within the normal ranges. His serum thyroid hormone levels increased beyond the normal ranges 1 year later (TSH 0.223, FT3 11.56, FT4 36.36), although no residual tumor was apparent on the MRI. Positive results were obtained for TRAb, and both TgAb and TPOAb were elevated beyond their normal ranges (TRAb 5.62 IU/L, TgAb 32.79 IU/L, TPOAb 14.13 IU/L). The patient was administered thyrozol 10 mg/day, and his thyroid function tests remained clinically acceptable during 3 years of follow-up. Repeated pituitary MRIs showed a possible mass in the pituitary, suggesting tumor recurrence. In addition, recent ultrasonography showed that the thyroid nodule remained the same size.","This man had a history of irregular heartbeats (AF) that were treated with medication, but the problem kept returning. After several more treatments, doctors discovered he had a problem with his thyroid gland, which was causing his body to produce too much thyroid hormone. A tumor in his pituitary gland was found to be the cause of this overproduction, and it was surgically removed. Despite the surgery, his thyroid hormone levels have continued to fluctuate, and he will need ongoing monitoring and medication to manage his condition."
806,"A 66-year-old man with a history of left knee osteoarthritis and varus deformity was scheduled to undergo TKA at our hospital. The left lower extremity was prepped using a topical solution, which was to disinfect skin 2 times with chlorhexidine alcohol skin disinfectant (mainly consist of 4.5 g/L chlorhexidine acetate and 70% alcohol). Let the skin air dry, and then place the iodophor-impregnated antimicrobial incise drape on the operation area, which included application of an iodophor-impregnated antimicrobial incise drape. Draping was performed using standard aseptic procedure.\nSubsequently, surgery was performed using conventional surgical procedures. At the end of the operation, the skin incision was sterilized again and sutured. Upon removal of the adhesive drape by delicate peeling and warm saline lavage, a large area of the skin avulsion happened. The extensive punctate hemorrhage was visible (Fig. ). The skin avulsion was in the posterior part of the patient's left crus. The avulsion area was approximately 6.5 × 30 cm.\nPetrolatum gauze was used to dress the areas of epidermal avulsion. Routine postsurgical dressing (gauze, cotton pad, and bandage) was applied to the incision. On postoperative day 2, an initial dressing change was performed. We consulted with doctors from other relevant departments regarding the avulsion injury who advised on the applicable treatment measures. Based on these measures, we asked the patient to raise the affected leg and keep the wound dry. Sulfadiazine cream was applied to the area of epidermal avulsion. Dressing changes were performed daily. On postoperative day 3, oral cefixime therapy of 100 mg bid was initiated as part of the anti-infection treatment. On postoperative day 4, the light yellow liquid exudes and the localized inflammatory response was visible (Fig. ). On postoperative day 7, the exudate decreased and the inflammatory response was reduced. There was no evidence of infection (Fig. ). On postoperative week 2, the wound was dry, and there was no exudate and no obvious inflammatory reaction (Fig. ). The patient was discharged from hospital on postoperative 2 weeks and was required to change the dressing every 3 days until the avulsion area was dry. After 6 weeks of treatment, the avulsed wound showed no signs of infection and had undergone re-epithelialization (Fig. ).","A 66-year-old man had knee surgery to replace his damaged knee joint. During the surgery, a large area of skin was accidentally torn away (avulsed) on his leg. The wound was carefully cleaned and dressed, and he received antibiotics to prevent infection. Over several weeks, the wound healed, and the skin began to grow back, and he was able to go home with instructions for continued care."
807,"An 82-year-old male patient was originally admitted to the hospital for difficulty in urinating spontaneously on November 24, 2014. The patient was diagnosed with prostatic adenocarcinoma after transurethral resection of the prostate (TURP) and postoperative pathological examination (Fig. ). In addition, a SPECT/CT scan revealed multiple bone metastases (Fig. ). Initial PSA and free PSA (FPSA) levels were 54.54 and 2.63 μg/mL, respectively (Fig. A). The patient exhibited poor tolerance to radiotherapy and chemotherapy, possibly due to advanced age or pacemaker implantation; therefore, immune therapy of alloreactive CTL was selected as a treatment option.\nThe patient received the first two cycles of ACTL treatment from December 16, 2014 to February 10, 2015. His PSA levels significantly decreased from 54.54 to 1.35 μg/mL after 28 cycles of continuous ACTL immunotherapy, to undetectable levels of <0.003 μg/mL on May 12, 2016; similarly, immediately after completion of ACTL therapy the FPSA levels were down from 2.63 μg/mL to undetectable levels of <0.01 μg/mL. The PSA and FPSA level remained stable in subsequent days. All the changes in PSA and FPSA are plotted in Fig. B, and each test was performed in the same laboratory. Importantly, there were few bone metastatic lesions detected by the SPECT/CT scan in December 2016 (Fig. ). Similarly, no metastatic lesions were detected in other organs, including the lung, liver, and the brain, by the PET-CT scan. Importantly, the patient remains alive after receiving 2 years of immunotherapy, despite being initially diagnosed with metastatic lesions at multiple sites of the body (Fig. ). Conversely, the withdraw of the ACTL immunotherapy was accompanied by a gradual increase in PSA levels in the patient from being undetectable (<0.003 μg/mL) on May 12, 2016 to 1.08 μg/mL on April 6, 2017 (Fig. ). Unfortunately, the ACTL immunotherapy was not applied again because of the expensive cost and lack of proper blood supply.\nIt is worth noting that there was no apparent cardiovascular system-related symptom or graft-versus-host disease (GvHD) detected in the patient over the course of the immunotherapy treatment, despite the patient being diagnosed with high blood pressure and Type II diabetes at the beginning the administering immunotherapy.","An 82-year-old man was admitted to the hospital because he was having trouble urinating. He was later diagnosed with prostate cancer that had spread to his bones. After trying other treatments, doctors decided to use a new type of immunotherapy to target the cancer. This treatment significantly lowered his cancer levels and reduced the spread of the cancer, and he has remained stable for two years."
808,"The patient was a 72-year-old man who was followed up for persistent pain in the left side of the waist for 2 years, radiating to the left hip. The history of schistosomiasis was 40 years, and with hypertension 1 year. On physical examination, he had pain on movement of the spine with bilateral Grade IV motor strength in both lower extremities. There was not numbness in both feet and ankles with normal ankle reflexes bilaterally. There was a normal knee reflex bilaterally. There was no obvious bulge in the bilateral kidney region, and the left lateral kidney region knocks out to be positive for pain. The neurological symptoms were ascribed to the compression of the spinal cord and nerve roots by an expanding mass partly protruding into the vertebral canal.\nMagnetic resonance imaging (MRI) was performed that the left side of the vertebral body and the left peritoneum showed an elliptical abnormal signal shadow, which showed a dumbbell growth. The MRI scan showed an area of increased signal intensity on T2-weighted images in the T12-L4 paravertebral region. At discovery, on the left side of the L1 vertebral body, there was a slightly longer T1/T2 signal, with a size of 8.6 × 8.4 × 12.4 cm (Fig. ). The boundary was clear, and the enhanced scan showed an uneven separation. The lesion protruding into the erector spinae muscles and behind the retroperitoneum are connected to the lesion in the vertebral canal through the intervertebral foramen, and the spinal cord was compressed and the boundary was clear. And the computed tomography (CT) showed that bone destruction can be seen on left lateral transverse process of L2, and the left kidney was significantly compressed (Fig. ).\nThe patient was treated with surgical resection of the total tumor, followed by the spinal internal fixation of L1 to L2 with pedicle screws. A posterior approach was selected for decompression. Intraoperatively, 2 lumps measuring 10 × 9 × 8 cm and 8 × 6 × 3 cm were visible in the focal region. They were well circumscribed and completely resected (Fig. ). Total laminectomy was performed for L1 and L2. On histopathological examination, the gross specimen consisted of ragged pieces of pale-tan, slightly gelatinous material admixed with blood clot. Microscopic examination of the formalin fixed paraffin-embedded specimen showed the presence of a lobulated neoplasm in a prominent extracellular myxoid matrix. The neoplastic cells were composed of syncytial cords of “physaliphorous cells” that contained abundant pale septated and vacuolated clear to bubbly cytoplasm with mild nuclear pleomorphism. Overt mitosis was not identified. Immunohistochemical examination showed that the lesional cells were positive to CK(+++), S100(++), CD65(+), neuron-specific enolase(++), epithelial membrane antigen (+++), and vimentin(+++). The coexpression of epithelial and mesenchymal immunohistochemical markers supported the notochordal lineage of the lesional cells. Ki67 was 3%+ in the lesional cells. The above histomorphological features in conjunction with the immune-phenotype observed confirmed the diagnosis of chordoma.\nNo heavy ion radiotherapy was performed because the tumor massively invaded into spinal canal and foramen, compressing the spinal cord and nerve root directly. After 5-month follow-up, we find the recurrence in the original lesion through the post-operative CT (Fig. ). In consideration of the huge and complex recurrence, we did not have second operation. At the 15-month follow-up, the patient was dead after a lot of times revisit by various doctors.","This 72-year-old man has been experiencing persistent pain in his lower back and hip for two years. Tests revealed a growth in his spine that was pressing on his nerves, causing weakness in his legs. An MRI showed a tumor pressing on the spinal cord, and surgery was performed to remove it. Unfortunately, the tumor has since grown back, and the patient passed away after several attempts to treat it."
809,"The 33-year-old right-handed male patient, with no history of brain damage had suffered from a stroke. Brain magnetic resonance imaging (MRI) revealed an infarction lesion in the left frontal lobe, insula, and basal ganglia (Fig. A). 14 months after stroke, the patient had aphasia only without limb paralysis; thus, he received a 5-month speech therapy composed of spontaneous speech, auditory comprehension, repetition, naming, writing, reading training, and calculation. The speech therapy was carried out for 1 hour per session, for twice a day and 5 days a week. Moreover, the Aphasia Battery of Chinese (ABC) and Boston Diagnostic Aphasia Examination (BDAE) were used to evaluate the language function and the severity of aphasia before and after the speech therapy. Briefly, ABC is a modified Western Aphasia Battery (WAB) adapted to the Chinese culture, which is currently the most extensively used scale in China for aphasia assessment, with well-established reliability and validity.[ Informed consent was given and the experiment was approved by the Ethics Committee of Jinan University First Affiliated Hospital in Guangzhou, China.\nThe results suggested that the patient had attained certain improvements in his language function (spontaneous speech: 69.8 percentile, auditory comprehension: 66.4 percentile, repetition: 64.0 percentile, and naming: 84.8 percentile). Besides, the severity of aphasia was rated at level 2 before the speech therapy. After the speech therapy, further language functional improvements were achieved (spontaneous speech: 81.1 percentile, auditory comprehension: 69.4 percentile, repetition: 72.0 percentile, and naming: 84.8 percentile), and the severity of aphasia was rated at level 3.\nDiffusion tensor imaging (DTI) was carried out before and after the speech therapy, respectively. In addition, an 8-channel head coil equipped on a 3.0 T American GE Discovery 750 MRI with single shot echo-planar imaging was used for the acquisition of DTI data. A total of 47 contiguous slices parallel to the anterior commissure–posterior commissure line was acquired for each of the 25 noncollinear diffusion sensitizing gradients. Specifically, the imaging parameters were as follows: TR/TE = 5000 ms/68.0 ms, acquisition matrix = 96 × 96, reconstructed to matrix = 128 × 128 matrix, field of view = 25.6 × 25.6 mm2, b = 1000 s / mm2, NEX = 1, slice thickness/slice spacing = 3 mm/0 mm. It took 135 seconds for DTI data acquisition. Besides, the corpus callosum was tracked using the self-prepared software package (Functool 9.4.05a). Fiber tracking was initiated at the center of a seed voxel with a fractional anisotropy (FA) of >0.18, apparent diffusion coefficient of >0.01, and the maximum steps of 160.\nResults of the follow-up DTI fiber tracking demonstrated that the fiber pathway between the splenium of corpus callosum and the left superior temporal gyrus (Wernicke's area) had been established. In addition, the fiber connections between the genu of corpus callosum and the right inferior frontal gyrus (the mirror region of Broca's area) were increased (Fig. B).","A 33-year-old man had a stroke that affected parts of his brain, leading to a speech problem. He underwent a 5-month speech therapy program to improve his communication skills, which included practicing speaking, listening, reading, and writing. The therapy showed significant progress in his ability to speak and understand language. Brain scans also revealed changes in the connections between different parts of his brain after the speech therapy, suggesting that the treatment helped to strengthen these pathways."
810,"A 61-year-old man who presented with progressive abdominal distention for 8 days was admitted to a local hospital on Feb 23, 2014. Gastroscopic examination and biopsy specimens were evaluated in the local hospital. Histopathological findings showed a suspected diagnosis of squamous-cell carcinoma in the body of the stomach.\nOn Feb 27, 2014, the patient was transferred to our hospital for further management strategy. Physical examination showed that right upper quadrant pain and tenderness were present and no peristaltic waves. The rest of the physical was unremarkable. A detailed medical history was obtained. The patient lost weight of 3 kg in recent 1 month. He had been previously admitted to the hospital for active tuberculosis 5 years ago and finally recovered completely. No history of diabetes, coronary artery diseases, hypertension, hepatitis, drug allergy, previous trauma, or operation was demonstrated. Laboratory tests revealed the following: red blood cell (RBC) count, 4.18 × 1012/L (normal range, 3.68–5.13 × 1012/L); hemoglobin concentration, 137 g/L (normal range, 114–151 g/L); white blood cell (WBC) count, 11.5 × 109/L (normal range, 4–10 × 1012/L); platelet count, 289 × 109/L (normal range, 100–300 × 109/L). Tumor markers were detected as the following: α-fetoprotein (AFP), 3.60 ng/mL (normal range, 0–15 ng/mL); carcinoembryonic antigen (CEA), 1.26 ng/mL (normal range, 0–5 ng/mL); carcinoma antigen (CA) 19-9, 5.6 U/mL (normal range, 50.1–27 U/mL). A chest x-ray showed bilaterally patchy infiltrates, increased bronchovascular markings and mass in the right lower lung fields (Fig. A), which needed further computerized tomography (CT) detection suggested by radiologists. A plain x-ray of the abdomen showed the gaseous distention of the bowel with air-fluid levels (Fig. B). Abdominal gaseous echoes were detected by ultrasonography. CT scans of the chest showed bilateral upper-lobe with patchy infiltrates, focal calcification and nodular opacities peripherally with cavitation, which indicated the changes of post-primary pulmonary tuberculosis (Fig. A); CT scans of the abdomen showed air-fluid levels in loops of small bowel, which indicated that mechanical obstruction was a likely diagnosis (Fig. B). Pathologic consultation of gastroscopic biopsy specimens in our hospital confirmed the diagnosis of squamous-cell carcinoma. Given the rarity of gastric squamous-cell carcinoma, pathologists suggested that further examination was needed to find the primary tumor. An enhanced CT scan was performed in this condition and the results were shown that the enhancement of right lower lung fields was not evident, which implied lung sclerosis after tuberculosis (Fig. ).\nOn March 5, 2014, the suddenly occurred abdominal pain was persistent and severe. The diagnosis of acute abdomen was made and urgent surgery was needed. The patient underwent a laparotomy with curative gastrectomy for gastric cancer and small bowel partial resection. Intraoperatively, a mass with a diameter of 0.8 cm was palpable in the greater curvature of the stomach and there was no significant lymphadenopathy (Fig. A, B). In addition, a mass with a diameter of 3 cm was found in the small bowel at the distance of approximately 30 cm to ileocecal area and the bowel became completely obstructed (Fig. C, D). Postoperative histopathology confirmed a moderately-differentiated squamous-cell carcinoma with full-thickness infiltration and vessel invasion in stomach (Fig. A, B) and small bowl (Fig. C, D). The margins of resection were free of tumor in the specimens and no regional lymph node metastasis was found in perigastric and perienteric area. In view of the rareness of gastrointestinal multiple primary squamous-cell carcinoma, further research was recommended by pathologists to exclude the metastatic squamous-cell carcinoma from the lung or the esophagus.\nEsophageal mass or lesion was not found on endoscopic examination and the metastatic squamous-cell carcinoma from the esophagus was excluded. It was important to attempt to isolate the tumor cells from the sputum and to carry out a lung biopsy. In the fifth examination of stained smears of sputum, squamous-cells with properties of tumor cells were found. Meanwhile, transbronchial aspirates were obtained with a 19-gauge flexible histology needle by using a CT scan as a guide and squamous-cell lung cancer was confirmed (Fig. A, B).\nThe diagnosis of lung squamous-cell carcinoma with multiple metastases to the stomach and small bowl was confirmed. As the tumor was not resectable, surgeons recommended conservative treatments. The patient was recommended with combination chemotherapy of carboplatin and paclitaxel for 3 cycles. However, in September 2014, the patient succumbed to respiratory failure.","A 61-year-old man was admitted to the hospital because he was experiencing severe abdominal pain and bloating. Tests revealed a cancerous tumor in his stomach and a blockage in his small intestine, requiring emergency surgery. Further investigation showed that the cancer had also spread to his lungs, and doctors determined that the cancer had originated in the lungs. Despite treatment, the patient sadly passed away a few months later."
811,"A 96-year-old woman was admitted to our hospital due to a 7-day history of fever over 39°C and 2 episodes of vomiting. By the time of admission, she had had abdominal pain, nausea, and lack of appetite for over a month. The family called an emergency ambulance but twice did not allow the patient to be taken to hospital. Because of her deteriorating condition, which was showing no improvement with oral amoxicillin with clavulanic acid 3 times a day (0.625 g), she was referred to our department to improve her poor condition. She had undergone appendectomy in her forties and hip bone fracture in 2013. She had not been administered any drugs so far.\nClinical examination revealed full mental conscious, dehydration, pulse rate of 90 beats/min, blood pressure 120/70 mm Hg, a body temperature of 39.5°C, left-side alignment of the alveolar murmur at the base of the left lung, local tenderness in the right upper abdomen without muscular defense. Her initial laboratory measurements showed a white blood cell (WBC) count of 17.77 × 109/L (norm: 4 × 109–10 × 109/L), with 86.8% neutrophils, hemoglobin at 6.80 mmol/L (norm: 7.45–10.00 mmol/L), erythrocyte sedimentation rate (ESR) was 86 mm (norm: 3–15 mm), and C-reactive protein level was 177.00 mg/L (norm: 0.00–5.00 mg/L). AUS showed gallbladder enlargement with stones and widening of the common bile duct (CBD) to 16 mm. The patient was consulted by the surgeon who, due to the high risk of mortality, decided to carry out intravenous antimicrobial therapy with ciprofloxacin 2 times a day with a dose of 0.4 g, metronidazole 2 times a day with a dose of 0.5 g, intravenous rehydration and alimentation, and spasmolytic drugs. We observed a mild improvement in the patient's condition with a decrease of body temperature to 36.8°C. Unexpectedly, 10 days after admission, we observed aggravation of the abdominal pain and fever recurrence up to 39.9°C, with an increase in serum and urine amylase to 257 U/L (norm: 25–115 U/L) and 418 U/L (norm: 30–200 U/L), respectively. CT of the abdomen revealed gallbladder stones with a widening of the CBD to 13 mm and widening of the Virsung duct to 8 mm, as well as some small pancreatic cysts with a pancreas tumor of 38 mm in diameter. CT also revealed aneurysm of the abdominal aorta, left suprarenal tumor, and esophagus hernia that had not been previously diagnosed. Abdominal MRI confirmed calculous cholecystitis and pancreatitis, probably with inflammatory tumor of the pancreas. After multidisciplinary consultation, we decided to treat the patient with meropenem intravenously three times a day at a dose of 1.0 g. The patient did not consent to undergo surgery. Over the next few days, the patient's health significantly improved, and after three weeks of hospitalization she was discharged home, with neither fever nor abdominal pain. She received restricted dietary recommendations. After discharge, the patient further gradually regained her physical health and returned to the condition she had presented before the disease. Four months after hospitalization, her laboratory tests showed a WBC count of 6.09 × 109/L, with 72.5% neutrophils, hemoglobin at 7.70 mmol/L, ESR of 38 mm, and C-reactive protein level at 1.03 mg/L. The patient was administrated pancreatic enzymes twice a day at a dose of 10,000 units before meals. Her blood pressure was well-controlled and she has had no recurrence of the fever or abdominal pain.","This 96-year-old woman was admitted to the hospital after a month of feeling unwell, with fever, vomiting, and abdominal pain. Initial tests revealed a serious infection in her gallbladder and pancreas, along with other health issues like an enlarged aorta and a tumor. After receiving strong antibiotics and supportive care, her condition improved, and she was able to go home with dietary recommendations and pancreatic enzyme supplements. Her health has continued to improve over the following months, with her lab results returning to normal."
812,"A 17-year-old woman, gravida 1, para 0, came to our hospital in labor at 36 weeks of gestation for care at birth. Case history revealed that she had not attended any check-ups during her pregnancy. The physical examination showed a 60% to 70% effaced and 3-cm dilated cervix, the fetus had cephalic presentation, and amniotic membranes were intact. Fetal ultrasound showed an isolated anechoic hepatic cyst measuring 1.32/1.47 cm. The cyst was situated in the right anterior abdominal compartment between the gallbladder and the umbilical vein, and at that time it was not certain if the cyst was located in parenchyma or choledocal. Because of the position of the cyst, close to fetal abdominal wall, we suspected that it was a parenchymal cyst (Fig. ).\nColor Doppler flow imaging did not show any flow in the cystic mass and showed that it was situated to the right of the umbilical vein (Fig. ).\nA female fetus was vaginally delivered in cephalic presentation shortly after her mother was admitted. The weight at birth was 2440 g, and the newborn was diagnosed with low weight for gestational age. The Apgar score value was 8 at 1 minute and 10 at 5 minutes. Unremarkable acid/base, co-oximetry, oxygen status, and electrolytes values from umbilical arterial blood were documented immediately after birth.\nAt 36 hours after birth the hepatic function panel showed a slightly elevated aspartate aminotransferase level of 43 U/L, slightly greater than the upper limit of normal (37 U/L). The total bilirubin level was of 5.2 mg/dL, corresponding to the low-risk zone at 36 hours after birth, according to the Bhutani nomogram.\nThe newborn was referred to the pediatric surgery unit, for further multidisciplinary evaluation. The evaluation by abdominal sonography confirmed the presence of a 1.6/0.93 cm intrahepatic cyst situated to the left of the gall bladder, without communication with the gall bladder or the common bile duct. The multidisciplinary team recommended periodic ultrasound monitoring of the cyst (Figs. and ).\nThe baby was reevaluated at 18 months after birth. Laboratory evaluation showed normalization of hepatic function panel and sonography showed complete resolution of the hepatic cyst (Fig. ).","This 17-year-old woman came to the hospital for labor and delivery at 36 weeks of pregnancy. During the delivery, the baby was born healthy, weighing 2440 grams, and had good Apgar scores. The ultrasound revealed a small cyst in the baby’s liver, which was likely a benign growth. After birth, the baby’s liver function was monitored, and the cyst completely disappeared by 18 months of age."
813,"A 58-year-old woman with a 2-year history of polyarthropathy had a diagnosis of RA (Fig. ). She was treated with oral corticosteroids (15 mg/QD), methotrexate (MTX) 10 mg weekly, and/or a nonsteroidal antiinflammatory drug. Her steady situation lasted for 16 months. In the recent 8 months, she experienced severely impairing and dizzines s and anemia. The blood test revealed normocytic anemia and she was admitted to the local hospital. Laboratory results were as follows: white blood cell (WBC) count, 5.48 × 109 cells/L; hemoglobin (Hb) count, 34 g/L; erythrocyte mean corpuscular volume (MCV), 89.0 fl, platelet (PLT) count, 381 × 109 cells/L; erythrocyte sedimentation rate (ESR), 138 mm/h; CRP, 117 mg/L; rheumatoid factor, 223 IU/mL; anti-cyclic peptide containing citrulline, 885.6 RU/mL; anti-nuclear antibody/anti-phospholipid antibodies/anti-neutrophil cytoplasmic antibodies, negative; vitamin B12, 368 pmol/L; folic acid, 4.6 nmol/L; erythropoietin, normal; serum ferritin, 287.96 ng/mL. At this point, MTX was discontinued and the patient received red blood cell transfusions. After treatment with glucocorticoids (methylprednisolone 4 mg/TID for 12 weeks), joint symptoms and CRP/ESR improved. However, the patient's hemoglobin level declined to 32 g/L. Even though folic acid tablets and ferrous sulfate were also administered, the response remained poor. The patient was referred to our hospital. Re-examination was conducted after red blood cell transfusion with the following laboratory data: WBC, 2.3 × 109 cells/L; Hb, 49 g/L; PLT, 237 × 109 cells/L; ESR, 108 mm/h; CRP, 61 mg/L; ferritin, 2325 g/L; and IL-6, 214.24 pg/mL.\nThe examination of bone marrow aspiration demonstrated dysplastic features on 2 hematopoietic lineages, but more prominent in the erythroid, which showed clustering with nuclear budding, pedal nuclei, and H-J bodies (dysplasis >10%). The double nuclei and megaloblastic changes could also be observed in the granulocytic lineage (accounting for 4%). The number of megakaryocytes was increased and the production of thrombocytes was fine (Fig. ). In addition, bone marrow biopsy also revealed hypercellularity and erythroid hyperplasia. Cytogenetic analysis showed a normal 46 XX [20] karyotype.\nCombining her medical history with the clinical and laboratory parameters, a diagnosis of treatment-related MDS (t-MDS) was made. Disease modifying antirheumatic drug (MTX) might have been responsible for the bone marrow toxicity.\nThese results, in combination with the patient's history, led to the addition of tocilizumab to the treatment regimen. To evaluate the clinical effects of tocilizumab in the RA patient, WBC, Hb, ferritin, ESR/CRP, and IL-6 were assessed at baseline, 1st, 3rd, and 6th months of tocilizumab treatment. She started therapy with intravenous injection of tocilizumab (8 mg/kg) every 4 weeks and oral corticosteroids (15 mg/QD), achieving clinical benefit and improvement in symptoms. One month after admission, the patient was discharged from hospital. Laboratory results at that time were: WBC, 8.6 × 109 cells/L; Hb, 97 g/L; CRP, 7.2 mg/L; ESR, 26 mm/h; ferritin, 936 g/L; and IL-6, 23.85 pg/mL. Upon hospital admission, 2 months after the regular checkup, the results were: WBC, 6.9 × 109 cells/L; Hb, 73 g/L; CRP, 41 mg/L; ESR, 38 mm/h; ferritin, 231 g/L; and IL-6, 1.12 pg/mL. Repeat bone marrow aspiration at the 6-month follow-up showed that dysplasia was not obvious. Laboratory results showed no obvious abnormality. This resulted in the prompt resolution of the patient's serological, physical, and pathological abnormalities. Hence, the response to tocilizumab treatment was very good.\nHowever, it was shown to be unlikely that the use of low doses of steroids in the patient was responsible for the sustained disease control. The patient refused the injection of tocilizumab after the 6th month. CRP (92.6 mg/L) increased and joint pain returned. Despite this relapse, the patient's laboratory examination was stable and symptoms significantly improved after resuming treatment with tocilizumab (Fig. ).","This patient has a long-standing condition called rheumatoid arthritis that has been difficult to manage. Despite taking medications like methotrexate and steroids, her symptoms worsened, leading to anemia and other complications. After further testing, doctors discovered that her bone marrow was not functioning properly, leading to a diagnosis of treatment-related myelodysplastic syndrome (t-MDS). To help control her condition, she started receiving tocilizumab, a medication that has significantly improved her symptoms and blood test results. After a period of treatment, her condition stabilized, and she was able to return home."
814,"A 20-year-old male patient was admitted to our hospital with abdominal pain in the epigastric region, weight loss, and fever for 2 months. He had low back pain for 6 months and was evaluated for this complaint in another hospital. He had significant weight loss of 10 kg in 2 months and fever especially at nights. Past or family history revealed no signs of chronic or significant illnesses. Physical examination was normal. X-ray chest was normal. Anteroposterior plain radiograph of the sacroiliac joints revealed grade II bilateral sacroiliitis. Human leukocyte antigen B27 was positive. Liver and kidney function tests were normal. Lactic acid dehydrogenase was 229 U/L, upper of normal limits. Hemoglobin was 9.6 gm/dL; the patient had iron deficiency anemia. Serum angiotensin-converting enzyme level was 10.7 U/L (normal 8.0-52). His human immunodeficiency virus status was negative. F-18 FDG PET/ CT revealed multiple hypermetabolic malignant lymphadenopathies at gastrohepatic, gastrosplenic, celiac, superior mesenteric, peripancreatic and hepatobiliary region, and paragastric region in size as 18 × 15 mm (SUVmax: 12.3) and diffuse gastric wall thickening as linitis plastica (SUVmax: 13.3), multiple hypermetabolic peritoneal implants in the omentum (SUVmax: 5.7) and peritoneum, and mild hypermetabolic suspected malignant lymph nodes at left supraclavicular region (). Gastric ulcer at incisura angularis was detected on upper GI endoscopy (). Endoscopic biopsies were repeated for histopathological and microbiological differential diagnosis. Histopathological examination showed granulomatous gastritis, Langhans-type giant cells, granulomas composed of epithelioid histiocytes, ulceration, and exudates in the two samples. Real-time TB-PCR were negative, Erlich-Ziehl-Neelsen staining bacteria were negative. Gastric fluid examination revealed Gram-positive cocci, Gram-positive bacillus, and no leukocytes. Exploratory laparotomy was done to sample the biggest sized lymph nodes for tissue diagnosis and explore the peritoneum for TB infection, lymphoma, and Crohn’s disease to make differential diagnosis. During laparotomy, the abdomen and peritoneum were intact and normal; two lymph nodes were extracted for histopathological and microbiologic diagnosis. Histopathology of the lymph nodes extracted by exploratory laparotomy revealed granulamatous lymphadenitis with granulomas including giant cells, mostly suspecting TB (). Ankylosing spondylitis was also diagnosed. Bath ankylosing spondylitis disease activity index score was 1.0, disease activity was low. Patient was put on ATT consisting of (2HREZ/7HR) regimen as isoniazid, rifampicin, ethambutol, and pyrazinamide at therapeutic doses for initial 2 months followed by rifampicin and isoniazide in the same doses for the last 7 months. At the 6th week of treatment, he gained weight about 6 kg and he was feeling healthy. Hemoglobin was 12.6 gm/dL. We performed follow-up F-18 FDG PET/ CT. The F-18 FDG PET/ CT images (maximum intensity projection, CT, and fusion PET/ CT) exhibited a complete response to ATT with no residual disease ().\nWe have taken written informed consent from the patient reported in this study.","A 20-year-old man was admitted to the hospital after experiencing abdominal pain, weight loss, and fever for several months. Tests revealed inflammation in his sacroiliac joints and a positive test for a gene called HLA-B27, which is linked to certain conditions. Further scans showed widespread cancer in his abdomen and lymph nodes, and a stomach ulcer was found. After treatment with antibiotics and other medications, the cancer appeared to respond well, and his weight and overall health improved significantly."
815,"An 11-year-old thin-built, anemic girl was brought to the emergency with complaint of pain in upper abdomen and recurrent vomiting since 1 week. Abdominal examination revealed a nontender lump in the epigastric region, which was moving with respiration. Rest examination was normal. In laboratory examination, microcytic hypochromic anemia was seen; other parameters were normal. Parents gave history of disturbed behavior of child for past few months and decreased appetite. Sibling of the patient had seen her chewing her hairs infrequently, but did not think of it to be a major problem.\nShe was initially admitted in other hospitals for this, where conservative management was given, but there was no relief. Along with other routine investigations, UGIE was done in which there was a large blackish mass in the stomach and the sample taken from it showed mostly hairs. Based on the history and examination, diagnosis of trichobezoar was made and patient was taken for surgery. Anterior gastrotomy was done () and large hair ball mass () which was occupying the whole stomach and extending into duodenum was removed. Postoperative period was uneventful and she was discharged after psychiatric consultation. After 6 months follow-up, patient is doing well.","This 11-year-old girl went to the hospital because she had stomach pain and was vomiting repeatedly for a week. Doctors found a lump in her stomach and discovered a large mass of hair inside, which was causing her problems. After tests, they diagnosed her with a trichobezoar, a hairball stuck in her stomach, and she needed surgery to remove it. The surgery was successful, and she is now doing well after a psychiatric evaluation and follow-up care."
816,"This is not a clinical study. Therefore, ethic approval is not needed. Father of the patient provided the written informed consent.\nA 19-year-old man presented with polydipsia and polyuria with muscle weakness for more than 1 month. His urine output was approximately 4 to 7 L/d. He later developed vomiting and paralysis. He was admitted to a local hospital and was hospitalized with severe hypokalemia (1.9 mmol/L) and metabolic acidosis (pH 7.22, pCO2 26.0 mm Hg, HCO3 10.6 mmol/L, lactate 7.1 mmol/L, base excess −15.6 mmol/L). The patient recovered from paralysis on the third hospital day with serum potassium level 3.1 mmol/L and normal blood gas results after appropriate therapy. He presented to our hospital for further evaluation. Physical examination was only significant for pallor. He did not have a family or personal history of neuromuscular, thyroid, or autoimmune disease.\nAt the time of admission, laboratory evaluation revealed the following abnormalities: white blood count 3.16 × 109/L, hemoglobin 74 g/L, and platelet count 128 × 109/L. Serum chemistry showed: sodium 140 mmol/L, chloride 108 mmol/L, potassium 3.18 mmol/L, calcium 1.89 mmol/L, creatinine 57.8 mmol/L, and bicarbonate 19 mmol/L. Urinary pH was 6.5, with sodium 80 mmol/L and potassium 18.68 mmol/L; chloride, calcium, and creatinine were normal. A 24-hour urine test started on the day of admission revealed the following: sodium 320 mmol/24 h, potassium 74.7 mmol/24 h, calcium 12.24 mmol/24 h, and protein 988.00 mg/24 h. Serum and urinary osmolality were 303 and 158 mOsm/kg, respectively. Growth hormone, insulin-like growth factor 1, thyroid hormone, adrenocorticotropic hormone, plasma aldosterone, renin activity, antinuclear antibody, and anti-Smith antibody were within reference range. Other chemistries and coagulation parameters were in normal range.\nBrain magnetic resonance imaging showed an abnormal pituitary gland, and he had an insignificant response to a water deprivation test after vasopressin injection. The findings suggested NDI. With overt acidosis (plasma bicarbonate <20 mmol/L) and urinary pH > 6.0, we confirmed the coexistence of renal tubular acidosis. Abdominal ultrasonography revealed symmetrical homogeneous kidneys with hyperechogenic pattern and poor corticomedullary differentiation (Fig. ). Because of the hematologic abnormalities, bone marrow aspiration was performed and revealed 39% blast cells. The immunophenotypic analysis showed blast cells positive for CD34+, CD38+, CD10+, CD19+, CD22+, cyCD22+, CD79+, HLA-DR+, and TdT+. Cytogenetics showed normal male karyotype. Cerebrospinal cytology was negative. He was diagnosed with precursor B-cell ALL with acquired NDI. Leukemic infiltration of the kidneys may have led to polydipsia and polyuria.\nThis patient had an excellent response to standardized combination chemotherapy and achieved complete remission (CR) at the end of induction. His polydipsia and polyuria improved considerably after 3 days of chemotherapy. He refused the best therapy of allogeneic stem-cell transplantation (allo-HSCT) at that time. Unfortunately, leukemia relapsed after sustained remission for 1 year. As a human leukocyte antigen-matched related or appropriate unrelated donor was not available, haplo-SCT was an alternative. He received haplo-SCT from his father after a second CR. Unfortunately, 20% blast cells were found in bone marrow 6 months after haplo-SCT. Next, a graft-versus-leukemia effect was successfully induced with the intervention of donor lymphocyte infusion, which achieved sustained remission. At 1 year after haplo-SCT, the patient developed a fungal pulmonary infection and was found to have bilateral renal enlargement with low density on abdominal computed tomography (Fig. ). Abdominal ultrasonography revealed symmetrical homogeneous kidneys with a hyperechogenic pattern and poor corticomedullary differentiation. The left kidney measured 17.1 cm × 9.6 cm, and the right kidney measured 16.9 cm × 9.7 cm. He returned 9 days later with muscle weakness, paralysis, polydipsia, and polyuria. Finally, he died of NDI and subsequent medullary relapse.","This 19-year-old man came to the hospital because he was experiencing excessive thirst and urination, along with muscle weakness and eventually paralysis. Tests revealed he had a serious kidney problem called renal tubular acidosis, which was likely caused by his leukemia. He also had a type of blood cancer called precursor B-cell ALL, and despite treatment, the cancer relapsed and eventually caused his death."
817,"A 15-month-old girl was admitted to the Peking Union Medical College Hospital (PUMCH). The girl had experienced high fever for 3 weeks, irritability for 2 weeks, and refusal to walk for 1 week. Three weeks before admission to PUMCH, she was seen at a local clinic (Haikou City, Hainan Island, located in the southern end of China) for fever and constipation. Physical examination was normal, and she was treated with ibuprofen. One week later, she presented to Haikou People's Hospital with a persistent high fever of over 39.5°C, irritability, and crying during the night. The result of head computer tomography scan was normal, and lumbar puncture revealed an opening pressure of 140 mm H2O. Her cerebrospinal fluid (CSF) was clear and had 120 × 106/L white blood cells with 38% neutrophils and 62% lymphocytes. The patient was treated for viral meningitis with an antiviral for 2 weeks. Thereafter, she refused to walk because of lower limb pain. The patient's fever was not relieved by treatment.\nThis girl was gravida 1 para 1 and was the full-term baby of an uncomplicated pregnancy. She had been raised in the countryside of Hainan Island. She had normal developmental milestones, uneventful previous history, and full immunization for her age.\nThe patient cried and was extremely irritable during the physical examination. The physical examination showed that she had a weight of 11.5 kg and a temperature of 40°C. Rashes, lymphadenectasis, and joint redness were not observed. Skin sensation could not be evaluated because the patient responded to any skin contact with exaggeration and crying. The patient's muscle strength and tone were normal even though she refused to stand or walk. The jerk reflexes of her limbs were symmetrical, and her pathological reflex was negative. No obvious focal neurologic signs were detected.\nLaboratory tests showed a peripheral blood leukocyte count of 12.6 × 109/L with an increased eosinophil count of 21.3% and absolute eosinophil count of 2.69 × 109/L. The results of renal and liver function tests and routine urine and stool tests were normal. Serum IgM antibodies to cytomegalovirus, herpes virus simplex, and Epstein-Barr virus were negative.\nThe magnetic resonance image of her brain was normal. Lumbar puncture disclosed an opening pressure of 200 mm H2O, and the CSF appeared cloudy. CSF cytology showed a white blood cell count of 400 × 106/L with 70% eosinophils, 32% monocytes, and 3% lymphocytes (Fig. ). The chemistry test results of the patient's CSF showed 2.2 mmol/L glucose, 122 mmol/L chloride, and 600 mg/dL total protein. The Gram stain, India ink stain, and the acid-fast stain results of the CSF were negative. CSF aerobic and anaerobic cultures and cultures for Mycobacterium tuberculosis and fungi were all negative.\nParasitic eosinophilic meningitis was highly suspected because of the patient's increased blood and CSF eosinophil count and persistent neurological symptoms. The mother was further questioned for the infant's exposure history. She recalled seeing the child play with slugs.\nEosinophilic meningitis was diagnosed clinically on the basis of the child's exposure history, and parasite-specific investigations were initiated. The suspected third-stage larvae of A cantonensis were found in the CSF. However, a reference laboratory in Beijing Tropical Medical Research Center reported that the patient's serum sample tested negative for antibodies against A cantonensis.\nConsidering the patient's clinical symptoms and signs, increased intracranial pressure and eosinophil levels in peripheral and CSF samples, and lack of response to antiviral treatment, as well as the presence of parasites in CSF, oral empiric treatment with 2.5 mg/(kg/day) levamisole and 1.5 mg/(kg/day) prednisone together with mannose was initiated to reduce intracranial pressure.\nThe patient exhibited rapid recovery, and all of her symptoms resolved approximately 72 hours after the commencement of treatment. Hematological testing and CSF examination revealed that the child's eosinophil count had decreased. Thus, prednisone dosage was gradually decreased. Another lumbar puncture performed 4 weeks after the first lumbar puncture revealed that the eosinophil levels in the child's CSF had decreased to 0. The patient was discharged from the hospital and completed 4 weeks of treatment with levamisole and prednisolone. At the 6-month follow-up, the patient showed normal growth and development without sequelae.","This 15-month-old girl was admitted to the hospital after a three-week fever and difficulty walking. Initial tests showed she had a high fever, irritability, and a concerning number of white blood cells in her spinal fluid. After trying antiviral medication, doctors suspected she had a rare parasitic infection called eosinophilic meningitis, possibly from playing with slugs. Treatment with specific medications helped her recover quickly, and she was discharged after six months with no lasting problems."
818,"A 10-year-old girl presented with a 2-day history of constant right-sided flank pain with intermittent episodes of increased intensity. Movement and laying supine exacerbated the pain, whereas some relief was found with paracetamol. There was no trauma to the area, but the patient reported playing netball shortly before the pain started. There was no associated nausea, vomiting, or change in bowel or urinary habit. There was no fever and no symptoms of cough or breathing difficulties.\nThe patient had no significant medical history and immunisations were up to date. On examination, she was apyrexial with a heart rate of 110 beats per minute, oxygen saturations of 99% on room air and respiratory rate of 22 breaths per minute. She was warm and well perfused with a central capillary refill time of <2 seconds. She appeared distressed when laying down reporting worsening pain; however, the abdomen was soft and non-tender to palpation. Systemic examination was otherwise unremarkable.\nUrine analysis showed no evidence of infection and blood tests revealed a C-reactive protein (CRP) of 23 mg/L and a normal full blood count with white cells of 13.4 × 109 cells/L. Liver function tests, urea and electrolytes, and venous blood gas were also normal.\nWith normal observations, examination and investigation results and a good response to analgesia in the department, the patient was discharged with a planned review.\nOn re-assessment the following day, she continued to complain of significant pain on the right side of the abdomen with similar features on examination. Owing to the persistence of symptoms, chest radiography (Fig. ) and abdominal ultrasound were performed. Both were reported as normal. With no surgical or medical cause of the pain identified, it was deemed musculoskeletal in origin, associated with playing netball. She was discharged with advice to take regular analgesia and to return if symptoms were not settling.\nThe patient represented the following day looking unwell. She appeared very pale with dry lips and walked slowly, hunched over. She was tachycardic at 130 beats per minute and tachypnoeic with a respiratory rate of 32 breaths per minute. Oxygen saturations, temperature, and blood pressure were normal at 98%, 36.80C, and 119/72 mmHg respectively. She stood with her right hip and knee slightly flexed, with her trunk deviated to the left giving her a marked scoliosis. Musculoskeletal examination of the spine, hips, and knees was unremarkable, but it was noted that abdominal pain worsened on flexion of the hip against resistance. Cardiovascular and respiratory examinations were normal. She was unable to lie supine because of severe pain and was examined in the semirecumbent position. Active distension of the abdomen and coughing further exacerbated the pain and she was found to have guarding with rebound tenderness in the right flank and hypochondrium. An intravenous cannula was inserted and intravenous fluids, paracetamol, and ibuprofen were commenced while awaiting a surgical review for her acute abdomen. Concurrent orthopedic opinion was also sought because of the new-onset scoliosis.\nAfter treatment the patients’ pain was significantly reduced, pallor had resolved and her observations normalised. Abdominal examination at the time of surgical review was normal, but because of repeated presentations with abdominal pain and a rising CRP (93 mg/L) and white cell count (13.9 × 109 cells/L) the patient was admitted and a computed tomography (CT) scan of the abdomen and pelvis was arranged. Orthopedic review did not yield any acute orthopedic concerns regarding the scoliosis and they agreed with the plan for abdominal imaging. The CT scan failed to reveal an abdominal cause for her pain, but to our surprise showed an organizing right lower lobe pneumonia with a small effusion (Figs. and ).\nThe patient was started on intravenous antibiotics for the thus far silent pneumonia. Despite treatment during the next few days, the pneumonia progressed and she developed a large parapneumonic effusion (Figs. and ). On the 6th day of hospital admission, she deteriorated rapidly with signs of sepsis and respiratory distress. This was accompanied by a further rise in CRP (351 mg/L) and white cell count (17.3 × 109 cells/L). She was stabilized and then transferred to a tertiary hospital for further management.\nAt the tertiary center, a chest drain was inserted and the patient was treated with intrapleural urokinase therapy and intravenous antibiotics. She was discharged after a 2-week stay.\nWith the complete resolution of the pneumonia and scoliosis, the patient made a graduated return to school and at 4 months post discharge was almost back to full-time attendance.","This 10-year-old girl came to the hospital because she had a persistent pain in her right side of her belly that started after playing netball. Initially, doctors thought it might be a simple stomach problem, but the pain didn't go away and she became increasingly unwell. After several tests, including scans and blood tests, they discovered she had a pneumonia in her lung and a scoliosis (curvature of the spine). She was treated with antibiotics and supportive care, and after a few weeks, she was able to return to school."
819,"A 41-year-old female patient was admitted to the Department of Respiratory Medicine due to hemoptysis. The patient had first experienced hemoptysis with a small amount of fresh blood and neutral-smelling yellow sputum 10 years previously. She denied breathing difficulties and chest pain. Chest computed tomography (CT) scans met the diagnostic criteria for bronchiectasis[ combined with infection (Fig. ). The patient's condition improved after administration of intravenous antibiotics (ceftriaxone and clarithromycin) and intravenous hemostasis (hemocoagulase) treatment. The patient then experienced disease recurrence once or twice per year and was again diagnosed with bronchiectasis combined with infection each time. After treatment with anti-inflammatory and hemostatic therapy, the patient's condition also improved each time. Seven days prior to presentation in our department, the patient began to cough with a small amount of yellow sputum and dark red blood. The same treatment strategy was again given in the local hospital, but the patient's symptoms were not improved. One hour prior to presentation in our department, the patient's symptoms became aggravated with increased hemoptysis of about 100 mL fresh blood combined with chest pain and difficulty breathing.\nLaboratory tests showed a white blood cell count of 13.5 × 109/L, neutrophil percentage of 76%, lymphocyte percentage of 17%, neutrophil count of 10.33 × 109/L, and monocyte count of 0.8 × 109/L. The test for tuberculin was negative. Blood gas analysis revealed an oxygen concentration of 33%, pH of 7.36, PCO2 of 44 mmHg, PO2 of 60 mmHg, HCO3− of 24.9 mmol/L, and base excess of –0.8 mmol/L. Tests of coagulation time, liver function, and kidney function showed no abnormalities. Chest CT showed increased patchy high-density shadows in the left lower lobe of the lung with visible bronchial broadening as changes to the patient's “bronchiectasis” (Fig. ).\nThe patient received a 7-day course of intravenous antibiotics (moxifloxacin) and hemostasis therapy (hemocoagulase/ethylenediamine diaceturate injection) as empiric therapy after admission. The patient's symptoms improved with reductions in coughing, sputum discharge, and hemoptysis involving mostly old clotted blood only. Then, enhanced chest CT showed an increased texture of the 2 lungs, widening of the bronchus of the lower portion of the left lower lobe, thickening of the adjacent left lower pulmonary veins and arteries, enhancement of the arterial phase of enhancement, reduction of venous phase enhancement, an increase in the patchy blur density around the trachea, and the trachea. The opening of the main bronchus was unobstructed. Lymph nodes were not seen in the mediastinum, and no obvious effusion was seen in the chest. A left lower lobe lobectomy was performed, and the postoperative pathology revealed a hemangioma of the left lower lobe of the lung without bronchiectasis (Fig. ). Three months later, the patient had no hemoptysis, sputum, or dyspnea. Lung CT showed only the expected postoperative change and no abnormal shadows or bronchiectasis.","This 41-year-old woman has been experiencing recurring episodes of coughing up blood for the past 10 years, which worsened recently with chest pain and difficulty breathing. Tests revealed that she has bronchiectasis, a condition where the airways in her lungs are damaged, and an infection. After treatment with antibiotics and other medications, her symptoms improved, but they returned. Finally, a lung biopsy showed that she had a hemangioma, a benign growth in her lung, which was causing the bleeding and other symptoms. She is now doing well and has no further symptoms."
820,"A 44-year-old woman presented to the emergency department with complaint of intractable nausea and vomiting associated with severe epigastric pain of 2 days duration. She denied any changes in her bowel habits. Review of systems was otherwise negative. She is status post sleeve gastrectomy 2 years prior and had a history of a remote laparoscopic cholecystectomy. Physical examination was only notable for mild epigastric tenderness. Laboratory tests showed aspartate aminotransferase 46 IU/L, alanine aminotransferase 65 IU/L, alkaline phosphatase 75 IU/L, and otherwise normal including lipase. A right upper quadrant ultrasound showed dilation of the common bile duct, which was again demonstrated on magnetic resonance cholangiopancreatography as well as a 7-mm common bile duct stone. The patient subsequently underwent ERCP with sphincterotomy. Cholangiogram at the time demonstrated a dilated common bile duct with no obvious cause. A few hours postoperatively, the patient started experiencing severe epigastric and left upper quadrant abdominal pain radiating to the back. She was found to be hypotensive and immediate fluid resuscitation was initiated. Repeat laboratory testing at the time showed a lipase of 1300 IU/L as well as a decline in hemoglobin from baseline of 12.0 g/dL to 7.0 g/dL. A CT (computed tomography) scan of the abdomen illustrated a large splenic heterogeneous subcapsular hematoma and peripancreatic stranding ( and ). The patient was transferred to the intensive care unit and managed conservatively with fluids and blood transfusions. The hematoma regressed, and her hemoglobin remained stable. With clinical improvement, she was discharged home.","This 44-year-old woman came to the hospital because she was experiencing severe nausea, vomiting, and pain in her upper stomach. She had a previous surgery to reduce the size of her stomach, and tests revealed a small stone in her bile duct. After a procedure to remove the stone, she developed severe pain and became very weak. Further tests showed a large bruise on her spleen and blood loss, which was treated with fluids and blood transfusions. She is now stable and has been discharged home."
821,"A 25-year-old male, right hand dominant and manual labourer by occupation came to our hospital with history of pain and stiffness of right elbow for last 2 years. Pain was mainly worse after periods of rest and after waking up in the morning, with no history of nocturnal exacerbations. Pain relived only partially with salicylates and other non-steroidal anti-inflammatory medications. He had no other musculoskeletal complaints or any other significant past medical history. He had previously sought consultation in other places and various diagnoses such as lateral epicondylitis, mono-articular rheumatoid involvement.\nPhysical examination revealed no swelling or fullness around the elbow. There was mild tenderness along the anterior aspect of elbow. There was restriction of both flexion and extension with elbow range of motion from 30 to 90°. Supination and pronation were normal. Plain radiographs were normal. Computed tomography (CT) with 3D reconstruction revealed a radio-dense ring measuring about 4 mm in the coronoid fossa of humerus. It had a radiolucent nidus measuring about 2 mm in diameter. There was a thin bony shell in the anterior aspect of the lesion. These findings were consistent with a diagnosis of a sub-periosteal osteoid osteoma. A decision of arthroscopic excision was made .\nElbow arthroscopy was carried out under general anesthesia in lateral decubitus position. Standard proximal anteromedial viewing (anterior and 2 cm proximal to medial epicondyle) and proximal anterolateral instrumentation portals (anterior and 2 cm proximal to lateral epicondyle) were used for elbow arthroscopy. A 2.7-mm, 30° arthroscope was used. The lesion was localised in the coronoid fossa. It appeared as a hyperaemic bony protuberance covered with hypertrophied synovium. Synovium was shaved with an arthroscopic shaver. Thin cortical shell over the lesion was removed with an arthroscopic shaver. The nidus was exposed and it shelled out easily upon manipulation with a curette. It was delivered through the anteromedial arthroscopic portal and sent for histopathology. The base of the lesion was cleaned with curette and burr. Normal bone deeper to the base of the lesion was not removed and posterior cortex was not perforated. No other arthroscopic releases were done. Intraoperative imaging was net required as the lesion was large and easily visible upon insertion of arthroscope.\nHistopathology findings of the nidus were consistent with an osteoid osteoma. There was an interlacing network of osteoid and bony trabeculae with some mineralization. Histopathology of the synovium only showed features of non-specific chronic inflammation. Gram stain and culture results were negative. His complete blood counts, erythrocyte sedimentation rate and C-reactive protein were within normal limits.\nPain relief after the procedure was remarkable. He had no pain after 3 days and could resume his work. At latest follow up of 1 year patient is completely pain-free. He has full range of motion of the elbow (0–150°, ).","A 25-year-old man with a history of elbow pain and stiffness came to the hospital for evaluation. After reviewing his symptoms and doing tests, including X-rays and a CT scan, doctors found a small, benign growth in the bone of his elbow called a sub-periosteal osteoid osteoma. The growth was removed with a minimally invasive procedure called arthroscopy, and he experienced immediate pain relief. He is now completely pain-free and has full range of motion in his elbow, and he is able to return to his work."
822,"The first subject is a 25-year-old female with a history of obesity who had undergone RYGB 2 year earlier, at which time her BMI was 35.1 kg/m2. Two years after surgery, she started to experience frequent episodes of hypoglycemia with tachycardia, sweating, and neuroglycopenic symptoms including confusion, dizziness, and blurred vision. Such symptoms occurred both in the postprandial state and during the night and were so frequent to impact negatively on her working capacity and quality of life. On admission to our Clinic, the patient had lost 35 kg and her body weight was stable. Continuous Glucose Monitoring (CGM, Dexcom G4 PLATINUM) for seven days evidenced ample blood glucose excursions throughout the day, with glucose peaks above 200 mg/dL after meals, followed by a rapid fall below 55 mg/dL (a), which were associated with hypoglycemic symptoms as recorded in the patient’s diary. It is interesting to note that low glucose levels occurred also during the night, as demonstrated by the considerable time spent below 55 mg/dL. Based on the continuous glucose profile showing a clear postprandial pattern of hypoglycemia associated with symptoms, we posed the diagnosis of PBH. From CGM, we also calculated the main indexes of glucose variability; i.e., coefficient of variation (CV), standard deviation (SD), and mean amplitude of glycemic excursions (MAGE) that resulted in an increase, as reported in .\nThe patient was prescribed a normocaloric (1500 kcal) high-fiber (33 g), low-carbohydrate index (55) diet, which was divided into three meals and three snacks to avoid postprandial blood glucose peaks and prolonged fasting intervals (see ). Multivitamin and mineral supplements were also prescribed on the basis of biochemical analysis showing vitamin D (19 ng/mL) and iron (ferritin 10 ng/mL) deficiency.\nFollowing nutritional therapy, the patient demonstrated a modest improvement of hypoglycemic symptoms but asthenia and tiredness in the early morning associated with fingerstick glucose level in the low range (<70 mg/dL) persisted. We supplemented the diet with a starting dose (30 g) of uncooked cornstarch administered before going to bed, which was progressively increased to 85 g/die (30 g in the morning and 55 g at bedtime; 1.25 g/kg b.w.) on the basis of the fingerstick home glucose profile. CGM, performed 12 weeks after beginning the cornstarch administration, showed a remarkable reduction in glucose variability indexes and postprandial glycemic peaks. Furthermore, the overall time spent in hypoglycemia (IG <55 mg/dL) decreased significantly. Of particular relevance is the reduction of the time spent in hypoglycemia during the night (from 23 to 1%; from 9.5 h to 35 min) (b). No side effect was recorded after cornstarch ingestion except for a slight abdominal distension during the first days of treatment.","This 25-year-old woman had previously undergone weight loss surgery and has been experiencing frequent episodes of low blood sugar, leading to symptoms like dizziness and confusion. Continuous monitoring showed that her blood sugar levels dropped significantly after meals and during the night, causing these symptoms. After making dietary changes and adding cornstarch to her diet before bed, her blood sugar levels stabilized, and she experienced a significant reduction in symptoms. She is now following a specific diet and taking supplements to maintain healthy blood sugar levels."
823,"A 50-year-old healthy female previously revealed the presence of a left renal mass by ultrasound 3 weeks ago. The patient had no significant back pain and gross hematuria. The abdominal contrast-enhanced CT demonstrated the presence of a mixed density mass with a size of 7.9 × 7.6 cm and clear boundary in the upper pole of left kidney (Fig. A). A slightly high- and low-density necrosis was found in the interior of the tumor, revealing an obvious uneven enhancement and the phenomenon of contrast agent fast forward and fast out (Fig. B and C). The left renal pelvis oppressed by the tumor was unclear. After the peritoneum, the nodular shadow was seen in retroperitoneal region, showing an obvious inhomogeneous enhancement (Fig. B and C). MRI revealed that the left kidney had an irregular contour. A mass of about 10.6 × 7.9 cm was observed in the upper left pole of the kidney. This was mixed with a short T1 signal, and showed an uneven internal signal. The vascular shadows and false envelop can be seen in the tumor. The enlarged lymph nodes were observed in the left renal hilum, with a diameter of about 2.3 cm (Fig. D and E). The patient under general anesthesia underwent radical resection of left renal carcinoma and renal hilar lymph node dissection through retroperitoneal route and resected the perirenal fascia, perirenal fat, kidney, ipsilateral adrenal, ureter above the iliac blood vessel bifurcation, and abdominal aorta and inferior vena cava lymph node from the angle of diaphragm to the bifurcation of the abdominal aorta. After incision of the kidney, a cut surface rotten bleeding of gray white tumor of 9 × 8 × 8 cm, and atrophied renal parenchyma were observed. Pathological examination revealed that the case was considered to be XP11.2 tanslocations/TFE3 gene fusions associated renal cell carcinoma with a size of 9× 8 × 8 cm, vascular region showed invasion of carcinoma, and left renal hilar lymph nodes were with metastases (2/2). PET-CT was performed, which revealed no other lymph nodes and organic metastases. So, the tumor observed was in T2N2M0 stage and IV stage according to AJCC Cancer staging Manual. Immunohistochemistry results revealed positive for CAIX, CD117, Ki67, Melan-A, TFE3 (+), AE1 / AE3 and CK8/18 (+), and negative for CD10, CK7, HMB, P504 s, Vimentin, EMA, PAX-8, and SMA. After operation, the patient was given sorafenib 400 mg bid. After 3 months follow-up, the patient was in good condition.","A 50-year-old woman was found to have a mass in her left kidney through an ultrasound. Further tests, including CT scans and MRI, showed the mass was a type of kidney cancer and had spread to nearby lymph nodes. The doctors performed a complete removal of the kidney and surrounding tissues, along with lymph nodes, to treat the cancer. After surgery, the patient started taking medication to help prevent the cancer from returning, and she is currently being monitored closely."
824,"A 31-year-old female, who was not married, has been undergoing hemodialysis for more than 2 years without any obvious symptoms underwent CT for preparation of kidney transplantation. CT showed a solid cystic and low-density foci in the right kidney, and a nodular and slightly high-density shadow within the foci (Fig. A). The patient underwent radical resection of right renal carcinoma under general anesthesia. After the operation, the kidney was opened, and a white mass of 4 cm size and fine papilla on the surface of the lump in the right kidney was observed. Postoperative pathology revealed Xp11.2 translocations/TFE3 gene fusions associated with renal cell carcinoma with a size of 4∗3 cm. Immunohistochemical results showed positive for AE1/AE3, CD10, Vimentin, CD117, P504 s, Melan-A, and TFE3. The patient did not receive any other treatments. She was followed up for one month after operation, and showed no recurrence.","A 31-year-old woman with kidney problems underwent a CT scan to prepare for a kidney transplant. The scan revealed a concerning area in her right kidney, and she had surgery to remove it. Pathologists found that the tumor was a type of kidney cancer with a specific genetic change. After the surgery, she was closely monitored for a month and showed no signs of the cancer returning."
825,"A45-year-old male, with a right lumbago for 1 month, underwent B mode ultrasonography. Results revealed a lower echo mass in the lower pole of the right kidney. The tumor was with a size of 5.4 × 4.8 cm, had a regular shape, but showed no clear boundary, and the internal echo was uneven. Then he was admitted in the hospital, and underwent middle abdomen plain and enhanced CT. Round tumor of 5.2 × 4.9 cm round tumor, with clear boundary and uneven density was observed (Fig. A). Laparoscopic radical nephrectomy was performed to open the right kidney. The right renal tumor with pigmentation had a clear tumor boundary. The tumor was considered as translocation of XP11.2 tumor associated with pigment differentiation, and had a low malignant biological behavior. Immunohistochemistry revealed positive for HMB45, Melan-A, S100, Ki67, and TFE3. After 3 months of operation, the patient showed no recurrence and did not undergo any other treatment.","A 45-year-old man was experiencing pain in his lower back for a month. Tests revealed a growth in his right kidney, which was found through ultrasound and confirmed by CT scans. The growth was surgically removed through a minimally invasive procedure. The tumor was determined to be a specific type with a low risk of returning, and the patient is doing well three months after the surgery."
826,"This case describes a 21 months old, previously healthy boy, who presented with weakness of the lower extremities and lumbar pain after a mild upper respiratory tract infection. For this reason, he was immediately brought to the paediatric emergency department where he underwent a hip ultrasound that excluded a joint effusion. Nevertheless, he was dismissed with a diagnosis of transient hips arthritis and was treated with anti-inflammatory therapy. A few days later, because of worsening pain and the inability to walk, he returned to our department and was hospitalized.\nOn admission, a spine radiograph showed a slight reduction in the thickness of the L5 soma. Moreover, a spine magnetic resonance imaging (MRI) showed the T1 post-enhancement increased signals of the anulus L4–L5, of the opposite end-plates of L4 and L5, of the adjacent soft tissues and of the osteolytic area of the L5 pedicle. Therefore, a diagnosis of SD with associated osteomyelitis was made.\nThe blood exams revealed an increase in the inerythrocyte sedimentation rate (ESR) (77 mm/h,) and C reactive protein (CRP) (2.17 mg/dL, normal values < 0.4 mg/dL). In contrast, the patient had a normal white blood cell count (WBC) count, a normal procalcitonin serum concentration (0.12 ng/mL, normal values < 0.25 ng/mL), and a negative Quantiferon TB-gold test.\nBroad-spectrum intravenous therapy with meropenem (100 mg/kg/day in three doses) and vancomycin (40 mg/kg/day in three doses) was started. Anti-inflammatory treatment was used for the first week and stopped with the complete resolution of the child’s symptoms and his return to normal walking.\nAfter 3 weeks of therapy, the patient developed leukopenia with severe neutropenia (lowest WBC value of 5410/mm3, with 80/mm3 neutrophils). As both of the administered drugs have been associated with neutropenia [,], therapy was withdrawn and replaced with ceftazidime (100 mg/kg/day in three doses), which was carried on for another week until the second MRI. The images from this exam, performed after 4 weeks of total therapy, showed a reduction in the enhanced contrast, although there was not a complete resolution of the inflamed and infected state.\nBecause of the radiological improvement, the normalization of the inflammatory factors, and the absence of symptoms in the child, he was discharged with an oral therapy of linezolid (30 mg/kg/day in three doses) and cefuroxime axetil (30 mg/kg/day in two doses). After 12 weeks of oral therapy, another MRI was performed. The images showed a complete resolution of the infectious process. shows differences between the MRI at admission and during the follow-up. Therefore, therapy was stopped (the patient received 16 weeks of therapy in total). The child was completely asymptomatic, and all of the blood exams, including the acute phase reactants and blood culture, were in the normal range or negative.\nAll of the blood exams that were performed to determine the nature of the infection did not show positivity for any recent, causative infectious agent. Additionally, the immunological and autoimmunity screenings were normal.\nThe 3 year follow up did not reveal any problem after discharge. The child never felt additional pain or had problems walking again.","This 21-month-old boy initially presented with leg weakness and back pain after a cold, and doctors suspected a temporary joint problem. However, further tests, including an MRI of his spine, revealed that he had an infection in his spine (osteomyelitis) along with inflammation. He was treated with strong antibiotics, but developed a low white blood cell count as a side effect. After several weeks of treatment and follow-up scans, the infection cleared up completely, and he was able to return to normal without any lasting problems."
827,"A 3 years old boy was admitted to our emergency department because he had been suffering from intermittent lumbar pain for several months and had difficulty walking for a few days. The patient’s personal medical history was uneventful until 4 months earlier when, playing with a friend, the child had a lumbar trauma that caused neither detectable skin lesions nor impairment to leg mobilization, and was not investigated. However, in the following weeks, the child started to feel pain whenever his father picked him up and was clearly more irritable than he had been in the past. A fever was never reported. Three months after the trauma, because of the increased lumbar pain, the child refused to walk. For this reason, he visited an emergency care unit and underwent a physical examination; laboratory blood tests, including a WBC and CRP serum level; and a full spine radiography. No abnormal results were detected. Oral therapy with a nonsteroidal anti-inflammatory drug for a week was prescribed. During this period, a partial resolution of the pain was demonstrated.\nHowever, ten days after the drug discontinuation, the pain worsened. Therefore, the child was brought to our department. Here, a physical examination, an abdominal ultrasonography, and the laboratory blood tests were still normal or only slightly abnormal. The ESR reached 60 mm/h, CRP was 1.47 mg/dL (normal values < 0.4 mg/dL), and procalcitonin was 0.26 ng/mL (normal values < 0.25 ng/mL), but the patient had a normal WBC count. His body temperature was in the normal range. However, an MRI scan of the spine revealed that a T1 post-enhancement had increased the signal of the anulus L3–L4 of the adjacent soft tissue; this outcome is highly suggestive of an infective SD ().\nWhile awaiting the results of the blood culture, a broad-spectrum, anti-infective intravenous therapy was started with piperacillin–tazobactam (100 mg/kg/day divided into three doses) and vancomycin (40 mg/kg/day divided into three doses). Oral anti-inflammatory therapy was also provided.\nThe boy experienced rapid clinical improvement. In the first weeks, he stopped feeling pain and started walking again without lameness. Anti-inflammatory therapy was discontinued after several days. After 4 weeks of therapy, the boy underwent a second MRI, which showed no significant radiological change (). The CRP was negative, but the ESR and procalcitonin were still slightly abnormal. The therapy was modified, and piperacillin-tazobactam was replaced by meropenem (100 mg/kg/day divided into three doses), while continuing vancomycin.\nAfter 8 weeks of intravenous therapy, the inflammatory index was completely negative, and the child felt no more pain and could walk normally. The intravenous therapy was switched to oral therapy with linezolid (30 mg/kg/day in three doses) and cefuroxime axetil (30 mg/kg/day in two doses), and the child was discharged from the hospital.\nAfter 12 weeks of total therapy, another MRI was performed to determine if therapy should be discontinued. The MRI showed an important reduction in the signal alterations, although residual irregularities of the end-plates of L3 and L4 were reported (). On the basis of these findings, the child remained on antibiotic therapy for another month and then stopped. He received 16 weeks of therapy in total.\nDuring hospitalization, all of the immunological exams that were performed were normal, and no causative infectious agent was documented. The whole therapy was well-tolerated, without any side effects.\nDuring a 3 year follow-up, the child experienced only one episode of back pain, for which he promptly underwent an MRI that ruled out the possibility of a reactivation of the infection, but he showed a slightly adipose evolution of the L3 and L4 body. After that episode, which rapidly and spontaneously resolved, the child never felt lumbar pain again and maintained a normal life.","This 3-year-old boy was seen in the hospital because he had been experiencing pain in his lower back and difficulty walking for several months. After a fall when he was younger, he started having pain when picked up, and it worsened over time. An MRI scan revealed an infection in the area between the bones of his spine, which was treated with antibiotics. The boy quickly improved and was able to walk normally again after several weeks of treatment, and he has been pain-free ever since."
828,"An 11-year-old boy of Moroccan origin was admitted to the Pediatric Ward of the Perugia General Hospital in July 2016 because, in the last 15 days, he had suffered from mild fever, chills and night sweats, severe itching, continuous dry cough, and right temporal headache. Moreover, in the month before hospital admission, he had lost approximately 7 kg in weight. Finally, a previous evaluation of blood cell count had revealed HE (eosinophils 10,000/µL) without other significant alterations. The patient denied having recently taken drugs and had had contact with animals. His last trip to his country of origin was in December 2015.\nOn admission, a blood cell count and a morphological evaluation of a peripheral blood smear confirmed HE (white blood cells 21,950/µL, with eosinophils 13,762/µL, hemoglobin 11.9 g/dL, and platelets 237,000/µL) the absence of other cellular morphological abnormalities. In the following days, the patient presented fatigue and worsening of headache; therefore, attempts to evaluate HE and other signs and symptoms origin were made. A parasitological fecal examination and a Scotch tape test were performed on three samples with negative results. Organ infiltration was excluded through chest X-ray, brain magnetic resonance imaging, echocardiography, and abdominal ultrasound. Toxoplasma, Plasmodium falciparum, Leishmania, Schistosoma, Echinococcus, viral hepatitis, and HIV infection were also excluded. Results of tests regarding comprehensive metabolic panel, inflammatory markers, and autoantibodies were negative excluding autoimmune diseases.\nA bone marrow aspiration was performed approximately 20 days after hospital admission. Morphological examination documented an increase in eosinophils without cellular morphological abnormalities, and bone marrow immunophenotyping showed that 4.5% of the cells had a phenotype compatible with lymphoid blasts. A lumbar puncture was negative. However, given the poor marrow involvement, it was necessary to repeat the bone marrow aspiration two days later; the results of the new aspiration showed an increase in blasts to 14% () with a normal immunophenotype on mature B ant T cells. Concomitant bone marrow biopsy showed a cellularity of 40% with an infiltration of blasts typical of B-cell ALL equal to 20–30% with marked associated hypereosinophilia.\nCytogenetic analysis showed a hyperdiploid karyotype: 53–55, XY, +X, add(1)(q21q25), +4, +9, +10, +14, +2, +1, +21/46, XY; FISH analysis confirmed the numeric and structural abnormalities revealed by karyotype, without cryptic additional events. In particular, no abnormalities were found on chromosome 5. The main molecular leukemic rearrangements—including BCR/ABL, MLL/AF4, TEL/AML1, and E2A/PBX1—were negative. The in vitro production of interleukin (IL)-5 by T CD4 + lymphocytes was increased (41%) compared to the negative control (11%).\nA few days after the start of prednisone prophase, a marked drop in peripheral eosinophil count and rapid clinical improvement were evident. On day 78, the patient was assigned to the standard risk group of the AIEOP ALL 2009 protocol. The patient is now undergoing maintenance therapy (6 Mercaptopurin plus Methotrexate) and will stop it on August 2018. Until now, the patient remains in complete remission; no further increase of eosinophil count was demonstrated.","This 11-year-old boy from Morocco was admitted to the hospital because he had been feeling unwell for weeks with fever, chills, itching, and a persistent cough, along with losing weight. Tests showed a high number of eosinophils in his blood, but after ruling out many other possible causes like parasites and infections, doctors discovered he had a type of leukemia. Further tests confirmed the diagnosis of B-cell acute lymphoblastic leukemia (ALL), and he started treatment with chemotherapy. Thankfully, he is now in complete remission and continuing with maintenance therapy."
829,"A 66-year-old woman presented with avascular necrosis in left hip, panniculitis, and later was found with diffuse systemic sclerosis and pulmonary fibrosis. She received low-dose methotrexate (15 mg/week) combined with prednisolone 5 mg b.i.d since 2014. Cyclophosphamide 50 mg twice daily was added in January 2015 and then further reduced to once daily in July 2016. The disease was well controlled with no further visceral organ involvement.\nTwo years later, she complained of gross hematuria and dysuria. She reported no fever, no suprapubic or flank pain, and no loss of appetite. Clinical examination revealed no abnormality except the diffuse skin thickening from her underlying disease. Urinalysis showed >100 red blood cells and leukocytes/μL, whereas urine culture was negative for organism. Her kidney function was within normal limit. Ultrasonography of the kidney, ureter, and bladder system revealed normal kidneys and turbid urine in the urinary bladder. No vesical mass or stone was detected.\nCystoscopy was done and showed a white-yellowish plaque in the bladder (). Hematoxylin and eosin stain (magnification: 400 × ) shows sheets of large macrophages with granular eosinophilic cytoplasm and mixed inflammatory cells infiltration (). Stain of von Kossa (400 × ) shows Michaelis–Gutmann bodies ().\nThe cyclophosphamide was permanently stopped, and she was treated with ciprofloxacin 500 mg orally twice daily for 2 weeks. She did not receive any intravenous antibiotics. She reported neither hematuria nor dysuria after full course of antibiotics and the urinalysis showed 5–10 leukocytes/μL and 1–2 red blood cells/μL. She continued the prophylactic dose of trimethoprim–sulfamethoxazole as well as prednisolone and methotrexate.","This 66-year-old woman has a history of several chronic conditions, including a joint problem, skin disease, and lung issues, which she’s been managing with medication for many years. Recently, she started experiencing blood in her urine and painful urination. After tests, doctors found a small, inflamed area in her bladder caused by the cyclophosphamide medication she was taking. Treatment with antibiotics resolved the urinary symptoms, and she continues her regular medications to manage her other health conditions."
830,"A 12-year-old girl presented to her general pediatrician complaining of asthenia and weight loss in the previous month. Her past medical and family history were unremarkable. Physical examination revealed a painless hepato-splenomegaly without additional clinical signs. Abdominal ultrasound revealed an inhomogeneous liver appearance, abdominal lymphadenopathy and a hypo-echogenic solid neoformation in front of the celiac artery (35 × 13 mm). The patient was admitted to the Department of Pediatrics of the Meyer Children’s Hospital for further diagnostic investigations.\nPhysical examination revealed palpable spleen and liver, a right inguinal lymph node of 1 cm and a lymph node in supraclavicular location, without any further objective anomaly. She had no fever. Initial laboratory tests found microcytic iron deficiency anemia (Hb = 9.8 g/dl, MCV = 65.5 ft., Ferritin = 4 ng/ml), with normal erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), normal total protein and protein electrophoresis, a slight increase of lipase and colic acids with normal amylase. Blood biochemistry for kidney and liver function and urinalysis were normal. Primary immunologic work-up including lymphocyte subset and immunoglobulin levels were normal. Tests for malignancies (tumor markers and peripheral blood smear) and infectious investigations, including Mantoux Test and IGRA Assay resulted negative.\nChest X-ray was negative. Magnetic resonance imaging (MRI) examination of the superior and inferior abdomen with contrast medium confirmed increased liver dimensions and its structural inhomogeneity with zones of signal alteration: some nodular, other confluent. It also revealed increased spleen size with zones of nodular signal alteration and multiple nodular formations in the following locations: hepatic hilar, mesenteric, lombo-aortic, at the retrocavity of the epiploon, and the greatest one in front of the celiac artery (diameter > 3.5 cm).\nSuspecting a systemic lymphoproliferative disease, we performed liver and lymph node ultrasound-guided biopsy, which showed negativity of Polymerase chain reaction (PCR) for potential agents of infectious diseases (including Mycobacteria and Bartonella species) on lymph node material, while it highlighted a non-necrotizing granulomatous inflammation, resembling sarcoidosis, and aspects of non-specific inflammation of the liver. (Fig. , ).\nSubsequently, we carried out measurements of serum and urinary calcium, serum phosphorus, Angiotensin-Converting Enzyme (ACE), followed by a complete cardiac evaluation, and a complete ophthalmologic evaluation (including slit lamp), all of which resulted within normality.\nAt this point, given the biopsy results, we performed further lung studies: pulmonary function tests which showed mild restriction and decreased alveolar capillary diffusion. The chest X-Ray revaluation revealed a pattern compatible with mild fibrosis and enlarged lymph nodes. We decided to carry out a high-resolution chest computed tomography (CT), which showed widespread fibrous strands and multiple enlarged lymph nodes (right paratracheal area, at the supraaortic trunk origin, subcarinal, with the largest in this location measuring 30 × 17 mm, pericardiophrenic, bilaterally at axillary level and along the mammary vessels). The bronchoscopic investigation with analysis of the broncho-alveolar lavage (BAL) fluid revealed: macrophages 70%, neutrophils 4% and lymphocytes 26% with a CD4/CD8 ratio of 9.2 (pathological value > 3.5).\nBased on these results, we carried out further investigations on liver tissue and lymph node with histochemical techniques: CD68 + nodules were found, suggestive of microgranulomas (Fig. and ).\nHaving ruled out other diagnoses, with suggestive histological findings in two different tissues (liver and lymph nodes) and considering lung involvement, we made the diagnosis of pediatric-onset adult sarcoidosis.\nDuring hospitalization, the condition of the child had always been good and she had always been afebrile. Considering lymph node hypertrophy and the signs of initial portal hypertension (due to compression of the hepatic vessels), we started therapy with prednisone 40 mg/day, and subsequent cross-therapy with mycophenolate mofetil (250 mg/m2 increasing up to 1 g/m2).\nThe girl came back to our attention a month after discharge for a follow-up visit: she was found in good general condition, with hepatomegaly and without other clinical signs or symptoms. A brain MRI was performed in order to rule out cerebral involvement and it revealed normal findings. One year later she maintained good clinical condition and normal laboratory tests, therefore MMF was gradually reduced and definitively suspended after 18 months. Now her periodic follow up consist of clinical and laboratory evaluation every 6 months and annual pulmonary function tests, unless clinical or laboratory new findings.","This 12-year-old girl came to the doctor because she was feeling tired and losing weight. After some tests, doctors found that her liver and spleen were enlarged, and she had some swollen lymph nodes. Further investigations, including a biopsy, revealed a condition called pediatric-onset adult sarcoidosis, which causes inflammation in the liver and lymph nodes. She is now being treated with medication and will continue to be monitored regularly to ensure the condition remains under control."
831,"The proband, 9 year old Sinhalese boy born to unrelated parents after an uncomplicated pregnancy who has an elder sister (14 years) and a younger sister (2 years) presented to our tertiary care children’s hospital in Sri Lanka with abdominal pain and gross haematuria since two weeks. Patient has had repeated episodes of haematuria in the past but urine tests were not available. There was no family history of renal stones. Physical examination was unremarkable. Ultrasonography of abdomen revealed a 2 cm calculus in the right middle moiety of kidney non obstructing. Ultrasound scan of family members was not performed. Biochemical investigations of the proband revealed a persistent hypouricemia (sUA 97 μmol/L, 93 μmol/L). Other biochemical investigations including liver and renal functions were within normal limits. Fractional excretion of uric acid was 33%. Secondary causes of hypouricemia were ruled out. Decreased blood concentrations of UA together with markedly elevated fractional excretion of uric acid (FE-UA) caused us to suspect RHUC and therefore a genetic analysis of the SLC22A12 and SLC2A9 gene was performed after informed consent. Probands parents and other two siblings were asymptomatic.\nThe sequencing analysis of SLC22A12 revealed a previously identified missence variant c.1400C > T (p.T467 M, rs200104135) in heterozygous state []. The sequencing analysis of SLC2A9 revealed two common variants: homozygous c.757G > A (p.V282I, rs16890979) and heterozygous c.962C > T (p.P350L, rs2280205). Analysis of family members identified p.T467 M variant in father of proband (41 year old male: sUA 172 μmol/L, FE-UA 13%) and the two siblings (14 year old sister: sUA 81 μmol/L, FE-UA 15%; two year old sister: sUA 86 μmol/L, FE-UA 25%). The analysis of mother of proband revealed only heterozygous variants p.V2821 and p.P350L in SLC2A9 (sUA 179 μmol/L, FE-UA 9%). Clinical, biochemical and genetic data are shown in Table . Figure shows the electropherograms of part of the SLC2A9 and SLC22A12 gene sequence of the family members.\nGenomic DNA was extracted from blood sample and/or dry blood spot using a QIAmp DNA Mini Kit (QiagenGmbH, Hilden, Germany) in the Institute of Rheumatology, Prague, Czech Republic. All protein-coding exons of SLC22A12 and SLC2A9 were amplified using polymerase chain reaction (PCR) and purified using a PCR DNA Fragments Extraction Kit (Geneaid, Taiwan). DNA sequencing was performed with a DNA sequencer (Applied Biosystems 3130 Genetic Analyzer; Applied Biosystems, USA). Primer sequences and PCR conditions used for amplification were described previously [, , ]. The reference genomic sequence was defined as version NC_000011.8, region 64,114,688..64126396, NM_144585.3 for SLC22A12; NM_020041.2, NP-064425.2,SNP source dbSNP 132 for SLC2A9.","A 9-year-old boy from Sri Lanka was seen in the hospital because of pain in his abdomen and blood in his urine. Tests showed a small stone in his kidney and low levels of uric acid in his blood, which led doctors to suspect a genetic condition called RHUC. Genetic testing revealed a specific change in a gene that causes this condition, and the parents and siblings were also found to have the same genetic variant. The doctors are continuing to monitor the patient’s condition and provide supportive care."
832,"A 76-year-old man was referred to our hospital for having lower back pain for 5 months, which was suspected to be L2/3 spondylitis on magnetic resonance imaging (MRI). He had previously undergone cervical laminoplasty for ossification of the posterior longitudinal ligament and diffuse idiopathic skeletal hyperostosis 19 years earlier. He had a history of hypertension and diabetes. Although a history of BCG vaccination was unknown, he had no previous history of tuberculosis infection. He had also undergone transurethral resection of a bladder tumor (TUR-Bt) and had been treated with intravesical mitomycin C (MMC) for the bladder cancer 1.5 years earlier. Four months later, he again underwent TUR-Bt, received intravesical MMC, and started BCG therapy for the recurrence of bladder cancer. After the sixth course of intravesical BCG therapy, he was aware of systemic weakness and loss of appetite, and was unable to walk. He appeared to have septic shock and therefore was treated in the intensive care unit. In spite of systemic analyses, the source of his infection and the causative bacteria could not be identified. He was subsequently diagnosed with hypercytokinemia caused by BCG therapy. He showed clinical improvement without the administration of antituberculosis drugs, and was discharged 9 months before he came to our hospital.\nExcept for a temperature of 37.2 °C, his vital signs were within normal limits. Although there were no motor and sensory disturbances in the legs, he was unable to walk owing to lower back pain. Physical examination demonstrated vertebral tenderness at the L2/3 level. Laboratory analysis demonstrated a normal white blood cell (WBC) count of 6300 /μL, a high erythrocyte sedimentation rate (ESR) of 53 mm/h, and a high C-reactive protein (CRP) level of 2.7 mg/dL. There were no other abnormal laboratory findings regarding anemic changes, kidney function, or liver function. The patient did not receive tuberculin skin testing.\nThere were no notable findings on electrocardiogram or chest X-ray. X-ray of the lumbar spine displayed collapsed endplates of L2/3. Sagittal T1-weighted MRI displayed a decreased signal in the L2/3 disc and the vertebral bodies (Fig. ). Sagittal T2-weighted MRI displayed an increased signal in the L2/3 disc and fluid collection in the anterior part of the vertebral bodies (Fig. ). Axial T2-weighted MRI displayed an increased signal around the posterior area of the vertebral bodies, which extended into the left epidural space and reached the peripheral muscle tissue and the area near the aorta (Fig. ).\nOn the second day of hospitalization, the patient underwent an L2/3 disc biopsy, but the general bacteria culture was negative, and the smear was negative for Ziehl-Neelsen staining. The patient’s blood culture was negative, and the result of T-SPOT.TB® (T-SPOT), which is a type of interferon-gamma release assay (IGRA), were also negative; the number of spots for both 6-kDa early secretory antigenic target (ESAT-6) and 10-kDa culture filtrate antigen (CFP-10) was 5 or less. The causative bacteria remained unidentified and therefore he was started on empirical therapy with intravenous ceftriaxone. On the fifth day of hospitalization, a plain computed tomography scan, which was performed for systemic examination, showed soft tissue development in the adjacent abdominal aorta at the L3 level (Fig. ), which was suspected to be an infectious aortic aneurysm. A 2-stage operation was planned for the spondylitis with adjacent infectious aortic aneurysm, to prevent rupture of the infectious aortic aneurysm, obtain spine stabilization, drain the abscess and make a diagnosis. On the tenth day in hospital, because the risk of rupture was considered to be low, the patient underwent an L2/3 laminectomy followed by posterior fixation using percutaneous pedicle screws at T12, L1, L4, and L5 as the first stage. Specimen cultures of the lumbar vertebrae, yellow ligament, necrotic tissue, etc., were negative for general bacteria, specimen smears were also negative for Ziehl-Neelsen staining, and there were no pathological findings of caseating granuloma or necrosis. On the fiftieth day of hospitalization, because the infection had been controlled but the inflammatory response was sustained, he underwent replacement of the aneurysm with a synthetic graft by vascular surgeons, and lesion curettage and L2/3 anterior interbody fusion by iliac bone transplantation as the second stage. Spinal surgery was performed in the transabdominal approach owing to the risk of rupture. On pathological analyses, the L2/3 intervertebral disc, vertebral bone, and tissue surrounding the vertebral bone and aorta showed caseating granuloma and necrosis with multinucleated giant cells and epithelioid cells upon hematoxylin-eosin staining, and positive bacilli upon Ziehl-Neelsen staining. The tuberculosis-polymerase chain reaction (Tb-PCR) result of the tissue was also positive, using COBAS® TaqMan® MTB Test, which is a real-time PCR system targeting the 16S rRNA gene region of Mycobacterium tuberculosis complex DNA. Owing to the patient’s history of BCG therapy, negative T-SPOT, pathological findings, and positive Tb-PCR, the pathogenic bacteria of the spondylitis was considered to be BCG. We then started multidrug therapy with antituberculosis drugs, including isoniazid (INH), rifampin (RFP), and ethambutol (EB), because BCG is typically resistant to pyrazinamide (PZA). PCR-based genomic deletion analysis was performed using the specimens to distinguish BCG from the other M. tuberculosis complexes. Specifically, multiplex PCR was performed utilizing region of difference 1 (RD1), which is present in the DNA of other M. tuberculosis complexes but is deleted in the DNA of BCG []. Primers ET1, ET2 and ET3 bind and amplify a 190-bp region in BCG, whereas a 160-bp region is amplified in the other M. tuberculosis complexes, as observed by electrophoresis on an agarose gel. A clinical isolate sample from our patient was identified as BCG with a deletion in RD1 (Fig. ). Specimen cultures from the first-stage and second-stage operation were later identified as members of the M. tuberculosis complex using a mycobacteria growth indicator tube. After therapeutic intervention, the patient’s WBC count, ESR level, and CRP level were improved and MRI displayed no signs of active infection in the spine, epidural space, peripheral muscle tissue, or aorta. On the ninetieth day after hospitalization, the patient was discharged from our hospital and transferred to a different hospital for physical rehabilitation.","This 76-year-old man was admitted to the hospital after experiencing lower back pain for several months. MRI scans revealed a problem with the discs in his lower back, and he was found to have an infection around the aorta. After a series of tests and biopsies, doctors determined that the infection was caused by BCG, a bacteria associated with his previous bladder cancer treatment. He underwent surgery to stabilize his spine and repair the aorta, and is now receiving medication to treat the infection and is starting physical therapy."
833,"A 70-year-old man with a history of hypertension, insulin-dependent diabetes mellitus, and a carotid endarterectomy was admitted to our emergency department with persisting and progressive angina for two hours. Moreover, he complained about general malaise and decreased exercise tolerance in the week prior to hospital admission. On physical examination, cardiac sounds were normal on auscultation. His blood pressure was 110/60 mm Hg and heart rate was 90 bpm. On admission, serum troponin T was 956 ng/L (normal range [NR] < 14 ng/L), creatine kinase myocardial band (CK-MB) 18 U/L (NR < 25 ng/L), creatine kinase (CK) 132 U/L (NR < 132 ng/L), low-density lipoprotein (LDL) cholesterol 3.4 mmol/L, high-density lipoprotein (HDL) cholesterol 0.8 mmol/L, triglyceride 0.75 mmol/L, and C reactive protein 150 mg/L (NR < 5 mg/L). His electrocardiogram (ECG) showed sinustachycardia (118/min) with ST-segment elevation in leads II, III, aVF and V5–6 and ST-segment depression in leads V1-V2. Coronary angiography was performed within one hour of hospital admission and revealed single-vessel coronary artery disease and the patient underwent a percutaneous coronary intervention of the circumflex coronary with placement of a drug-eluting stent. After the procedure, a Thrombolysis In Myocardial Infarction (TIMI) grade 2 flow was achieved in the AMI culprit coronary artery. Peri-procedural hypotension was treated with infusion of 2 l of 0.9% sodium chloride and dobutamine infusion. Postprocedural transthoracic echocardiography showed a moderate left ventricular function with a left ventricular ejection fraction of 40–45% and trivial mitral and tricuspid regurgitation.\nFifty days after PCI the patient experienced progressive fatigue and chest pain with haemodynamic instability. Transthoracic echocardiography showed a covered left ventricular free wall rupture (LVFWR) of the lateral wall and extensive pericardial fluid and inflow obstruction. The free wall rupture was in close proximity to the anterolateral papillary muscle (ALPM), but did not lead to significant mitral regurgitation. The transthoracic echocardiographic images are shown in Fig. .\nThe patient was brought to the operation room for emergent repair of the rupture. The surgical technique is described and shown in Fig. . The patient was weaned of cardiopulmonary bypass through the use of mild doses of inotropes. Transesophageal echocardiography showed a good result without significant mitral regurgitation. By carefully avoiding damage to the ALPM during the procedure we were able to avoid additional mitral valve repair or replacement.\nPostoperative recovery was uneventful and the patient was discharged 12 days after the operation. More than a year later, the patient did not experience any subsequent cardiac events and his functional status was similar to the level before the episode of myocardial infarction and rupture.","This 70-year-old man had a heart attack and received a stent to open up a blocked artery. After that, he experienced chest pain and fatigue, and tests revealed a tear in the wall of his heart. He needed emergency surgery to repair the tear, and the procedure was successful. He has since made a good recovery and is back to his normal level of activity."
834,"A 23-year-old French man of African origin, an elite football player, sustained a midshaft anterior cortex tibial stress fracture 2.5 years ago. Initially, he was treated with cast immobilization, no weight bearing for 3 months, ultrasound stimulation, and electromagnetic field therapy. The fracture did not heal; he had pain during gait, so he continued no weight bearing for 3 additional months. After that period, the fracture site still was not healed, so he underwent an operation performed by his team doctor. In this operation, the medullary canal of his tibia was reamed and an im nail was inserted.\nUnfortunately, the fracture site did not consolidate again, even 18-months postoperatively, so he presented to our clinic for counseling. It was obvious from the X-ray (Fig. ) that a nonunion of the fracture had occurred.\nHe did not smoke tobacco and he had a free medical history. When he presented to our clinic, the area at the fracture site was swollen and painful when palpated. The pain got worse when he attempted to walk with full weight bearing, so he had to use crutches. An examination of the peripheral nervous system of his lower extremities did not provide us with any pathologic findings. In addition, the laboratory examinations for possible endocrine or metabolic disorders were negative (Table ), so he was advised to have a reoperation to address this nonunion. The treatment options for such cases include nail exchange, drilling of the fracture site, bone grafting, or removal of the nail and internal fixation with a plate. We performed a tension band plate fixation, which is a technique already described for the treatment of anterior tibial stress fractures that failed non-operative treatment [], with bone grafting and without removing the nail.\nA longitudinal incision was made just lateral to the anterior tibial crest centered over the fracture site. The fascia over the tibialis anterior was divided, the muscle lifted off and the fracture site was visualized. The necrotic bone and callus at the fracture site was debrided with the use of an osteotome and a curette. Transverse drilling around the fracture site was done to promote healing and osteoblastic activity. Bone marrow from the ipsilateral iliac crest was inserted into the fracture site and a tension band plate was applied over the im nail.\nWe used a 6-hole, 4.5 mm locking compression plate. The plate was prebended and the screws were placed in a compression manner to achieve a tension band effect to the fracture site. A cortical screw was put first to the distal hole closest to the fracture site and then a cortical screw to the closest hole proximal to the fracture site to ensure compression of the fracture. Consequently, one unicortical locking screw was inserted proximally to the fracture site and the other two distally. With the use of locking and non-locking screws we minimized the pressure at the periosteum, which can damage blood supply to the poorly vascularized bone. The screws were angled in a different axis in order to bypass the nail (Fig. ).\nPostoperatively, our patient was advised to wear an orthotic boot and to not bear weight for 6 weeks. Range of motion exercise involving knee and ankle and isometric exercises were initiated immediately postoperatively. After 6 weeks he progressed to weight bearing as tolerated. At 3 months postoperatively he was pain free and started light jogging, swimming, and plyometric and core stabilization exercises. At 6 months postoperatively the complete radiologic union of the fracture was evident (Figs. and ). He was symptom free; he resumed at that time a full training program and he returned to play football 6 months postoperatively at his preinjury high competition level.","The patient is a professional football player who has had a persistent non-healing fracture in his lower leg for the past year and a half. After several attempts to heal the fracture with casting and other treatments, surgery was performed to stabilize the bone. A second surgery was recently performed to try to improve the healing process, involving bone grafting and a special plate to apply pressure to the fracture site. Now, he is following a rehabilitation program with exercises and is expected to return to playing football within six months."
835,"A 16-year-old boy with bilateral TMJ ankylosis was referred to the oral and maxillofacial surgery department of Tehran University of Medical Sciences. The bilateral TMJ ankylosis had been induced due to trauma when the patient was 4 years old. He had undergone TMJ surgery twice, but the operations had been unsuccessful and the TMJ ankylosis relapsed ().\nThe clinical examination revealed no systemic diseases. The patient’s maximum incisal opening was 11mm. Because of the old injuries and unsuccessful operations, the mandibular deficiency had developed. The patient was experiencing sleeping disorders and mostly slept in a prone or a sitting position due to respiratory problems. The lateral and forward motions of the mandible were restricted. Based on the patient’s condition and the past surgical history, a two-step treatment was performed. First, the gap arthroplasty method was applied, and afterwards, DO was performed.\nThe gap arthroplasty surgery was followed by physiotherapy. Approximately 3 to 10 days after the surgery, the patient was put on a soft diet and began the jaw-opening exercises. Three to four weeks after the surgery, a normal diet was started. After the discharge, the patient was visited once a week to evaluate the changes.\nThe osteodistractor was installed four months later (). After the installation, since the condylar stop had been removed in this case, and there was a possibility of upward and backward shift of the proximal mandibular segment, we used a custom-made device to prevent these movements. The Sh-device has a curved metal rod and two oval stoppers that can be connected to two areas, one to the patient’s forehead and another to the upper lip (below the nose). The stoppers are made of a firm clear plastic; the inner stopper surface is covered with a layer of soft spongy tissue that provides comfort for the patient upon using the Sh-device (). The stoppers are attached to the back of the patient’s head by two elastic bands, which keep the device on the patient’s face. The elastic bands, which are attached to the orthodontic hooks on the mandibular premolars or molars, are connected to a small piece at the bottom of the lower stopper. The main pressure/traction of the Sh-device was applied on the distal mandibular segment, and the Sh-device prevented the proximal mandibular segment from moving upward and backward; therefore, the greatest displacement occurred at the distal mandibular segment. The mandible was driven forward at a rate of 0.5mm per day.\nAfter one month, we separated the Sh-device, and the mandible moved about 15 to 20mm forwards. The patient’s breathing and sleeping improved. At the 8-year follow-up, the patient’s profile was satisfactory. The patient’s maximum incisal opening increased to 38mm ().","A 16-year-old boy with a history of jaw problems from a childhood injury was referred to specialists. Despite two previous surgeries, his jaw remained stuck, causing difficulty opening his mouth and breathing problems. To improve his jaw movement and breathing, doctors performed a series of procedures, including gap arthroplasty and later using a special device to gently move his jaw forward. Over time, his jaw opened wider, and his breathing improved significantly, leading to a satisfactory profile at a follow-up visit eight years later."
836,"A 71-year-old man, originally from North Africa, with a history of insulin-dependent diabetes mellitus, presented to our emergency department with a 2-month history of nonspecific abdominal symptoms: meteorism (bloating) and a subjective feeling of abdominal enlargement. Diarrhea, loss of appetite, weight loss, persistent fever, night sweats, headaches, anxiety, gastric ulcer disease, or skin rash were not reported. He did not report a neoplasm in the past and he had not undergone an abdominal examination before. He could not recall any episodes of pancreatitis or suffering from gall bladder stones. He had undergone a computed tomography (CT) scan a few days before which showed a tumor in the pancreatic head and he was referred to our clinic. Diabetes mellitus occurred 20 years ago and he was initially treated with orally administered anti-diabetic drugs for more than 12 years. Apart from diabetes and arterial hypertension he had no previous medical or surgical history. He has been treated with two different anti-hypertensives, two diuretics, acetylsalicylic acid, and insulin glargine. He reported an allergy to metformin. He did not smoke tobacco or drink alcohol.\nOn general physical examination, he was conscious and oriented and in fair general condition. He appeared to be in a good nutritional state (height 165 cm, weight 73 kg, body mass index 26.8 kg/m2). His lungs were clear to auscultation and percussion bilaterally. His heart examination was also normal. He had a soft, non-tender abdomen without any palpable masses. Icterus and lymphadenopathy were absent. His vital signs were normal.\nA routine laboratory analysis showed the following abnormal values: hemoglobin 12.5 g/dl (13.5–17.5), hematocrit 25% (40–53), mean corpuscular volume (MCV) 78 fl (82–98), uric acid 10.2 mg/dl (< 7.0), creatinine 1.8 mg/dl (< 1.4), and random serum glucose 148 mg/dl. Transaminases and cholestasis parameter were normal. Glycated hemoglobin (HbA1c) was 6.9% (52 mmol/mol). The serum tumor markers carcinoembryonic antigen (CEA) and cancer antigen 19–9 (CA19–9) were in normal range. Serum pancreatic lipase was slightly elevated (88 U/l, reference range < 65 U/l). The pancreatic elastase in stool was normal. There was no clinical evidence for exocrine pancreatic insufficiency.\nAn ultrasound of his abdomen revealed a tumor of the uncinate process of the pancreas. The endoscopic ultrasound showed a mass in the area between the neck and the body of his pancreas, measuring approximately 24 mm in its largest dimension. The tumor compressed his splenic vein; however, there were no signs of infiltration. Contrast-enhanced CT demonstrated an almost 3 cm round, quite well-defined and homogeneous tumor formation in the area between the neck and absent body of his pancreas (Fig. ). The tail of the pancreas was absent as well. The tumor showed a slightly arterial hypervascular enhancement and a central calcification in the shape of a dot. There was no central hypoperfusion or necrosis. The imaging findings were not typically suggestive of exocrine pancreatic carcinoma or pancreatic endocrine tumor. After discussing the case at our interdisciplinary tumor board, we decided to perform a biopsy.\nThe initial fine-needle aspiration was performed via endoscopic ultrasound. However, the pathological analysis did not detect tumor cells in the aspiration material.\nIn the next step our patient underwent CT-guided biopsy of the mass. A histopathological examination (Fig. ) revealed the typical aspect of a well-differentiated neuroendocrine tumor with solid aggregates of isomorphic tumor cells. Immunohistochemistry showed intensive cytoplasmic staining for synaptophysin (Fig. ) and nuclear staining for Ki-67 in less than 2% of the tumor cells (Fig. ). No gastrin expression could be detected by immunohistochemistry.\nOur patient underwent pancreas resection because of the presence of a neuroendocrine tumor. On macroscopic examination, the tumor was 1.8 cm in maximum diameter (TNM staging, pT1). There was no vascular invasion present. None of the three lymph nodes was metastatic (TNM staging, pN0). The active proliferative rate of Ki-67 (a pathological grading marker) was 5%.\nWell-differentiated neuroendocrine tumors of the pancreas usually show the characteristic nested, trabecular, or gyriform pattern known from neuroendocrine neoplasms of other organs. There are, however, architectural and cytological variations in some tumors, including abortive gland formation or oncocytic and clear cell changes.\nIn our case, the differential diagnosis of the biopsy specimen was focused on a distinction from other epithelial pancreas neoplasms especially the solid variants of acinar cell carcinoma, solid pseudopapillary neoplasm, as well as pancreatoblastoma. The latter tumor types may also exhibit the nesting pattern observed in our case. Therefore, the intensive homogenous staining pattern for synaptophysin in our case provided an important argument for the diagnosis of a neuroendocrine tumor. This diagnosis was further corroborated in the resection specimen by the lack of features defining acinar cell carcinoma (for example, acinar differentiation) or pancreatoblastoma or solid papillary neoplasm (for example, pseudopapillary growth pattern).\nOur patient underwent a 68gallium-DOTATOC positron emission tomography (PET)/CT scan 4 months later, which did not show increased metabolic uptake. Seven months after pancreas resection his HbA1c increased from 6.9 to 8.7%. This led us to modify his diabetes medication to a combination including insulin glargine, insulin glulisine, and metformin.","This 71-year-old man has been experiencing stomach problems like bloating and a feeling of a large abdomen for two months. Tests revealed a tumor in his pancreas, and further investigations, including a biopsy, confirmed it’s a type of slow-growing neuroendocrine tumor. He had some blood abnormalities, like low red blood cells, and his diabetes was not well-controlled, so his medication was adjusted. After surgery to remove the tumor, his condition has improved, and his diabetes is now being managed with a combination of medications."
837,"A 26-year-old right-handed white woman with no significant medical history was diagnosed as having MS in 2013 at age 22 and experienced ongoing radiologic activity on both glatiramer acetate and dimethyl fumarate. She transitioned to natalizumab in July 2014 to stabilize disease activity, and her JCV antibody index was positive at 3.58 prior to starting natalizumab. She became clinically and radiologically stable with the initiation of natalizumab until November 2016 when a surveillance MRI of her brain showed asymmetric confluent non-enhancing hyperintensities in the bilateral subcortical precentral gyri consistent with PML (Fig. , ). Cerebral spinal fluid (CSF) showed quantitative polymerase chain reaction (PCR) for JCV of 15 copies/ml, and other CSF studies were within normal limits. A diagnosis of PML was made based on the compatible neuroimaging findings along with the presence of JCV DNA in the CSF. Natalizumab was discontinued after 27 total treatments. Our patient was asymptomatic at the time of PML diagnosis, and she was highly functioning with an Expanded Disability Status Scale (EDSS) of 0. A decision was made to defer plasmapheresis at the time of diagnosis given her high functional status, subtle radiological change, and low viral titer. She was treated with orally administered mefloquine loading dose followed by 250 mg weekly and mirtazapine 15 mg daily.\nRepeat MRI at 2 months following diagnosis showed no changes in her brain lesions. She remained asymptomatic until 3 months after diagnosis when she noticed mild dysmetria of her left hand that progressed to a tremor. The following month a repeat brain MRI revealed a few small enhancing lesions in her left frontal lobe suggestive of IRIS (Fig. , ). The hyperintensities in the bilateral precentral gyri remained stable. Imaging of her cervical spine revealed a new non-enhancing cord lesion. She was then treated for 5 days with intravenously administered immunoglobulin and restarted on glatiramer acetate for MS treatment. A repeat CSF examination in February 2017 showed JCV PCR of 31 copies/ml.\nFive months following her diagnosis, a repeat brain MRI showed interval development of T2 signal abnormality with mild enhancement in multiple areas including the brainstem, cerebellum, and bilateral cerebral hemispheres (Fig. , ). A repeat lumbar puncture was performed. JCV PCR in the CSF was undetectable. Mefloquine and mirtazapine were discontinued. Given the MRI findings, she was treated for ongoing inflammation associated with IRIS versus a possible exacerbation of her underlying MS with high-dose intravenously administered methylprednisolone (IVMP) 1500 mg daily for 3 days. She was then transitioned from glatiramer acetate to ocrelizumab for treatment of MS. Six months following her diagnosis she reported changes in left hand dexterity and right upper extremity phasic spasms. A repeat lumbar puncture was performed and JCV PCR remained undetectable. She continued MRI surveillance followed by treatment with high-dose IVMP for a total of six courses until there was significant resolution of enhancement on her brain MRI (Fig. , ). Following treatment, she has residual left hand dysmetria and tremor as well as right upper extremity phasic spasms. At 1-year follow-up, her EDSS is 2.0.","This patient has multiple sclerosis (MS) that she’s been managing with medication since In 2016, she developed a serious brain infection called PML, likely triggered by her MS treatment, and had to stop taking natalizumab. After treatment, she experienced a new type of MS flare-up called IRIS, which caused new lesions on her brain and spinal cord. She received multiple rounds of treatment, including steroids and a new medication, and is now stable with some lingering symptoms like hand and arm problems, but her overall condition has improved."
838,"A 67 years old lady came to the out-patient department with a history of acute onset jaw tremor, with tremor predominantly in both upper limbs approximately 2 months prior to consultation. Her symptoms had progressed over a period of 24–48 h and remained static until the consultation. She did not have features of non-motor symptoms to suggest a diagnosis of idiopathic PD.\nThere was no history of previous stroke or vascular risk factors for stroke. She had not been on any medication which could cause extra-pyramidal symptoms.\nGeneral physical examination was normal. Neurological examination revealed hypomimia of the face with cogwheel rigidity and bradykinesia bilaterally (right more than left), predominantly in the upper limbs without pyramidal signs (the Unified Parkinson’s Disease Rating Scale (UPDRS) Part III; item 18–32 was 36). She had a marked tremor of the jaw at rest (Additional file 1). When she was asked to open her mouth the tremor was re-emergent (Additional file 2). There were no pyramidal signs. The rest of the neurological examination was normal which included cognition, speech, cerebellar function and bladder function.\nNon-contrast CT scan of her brain revealed an infarction in the region of the putamen on the left with no evidence of diffuse subcortical white matter ischemia or extension to the caudate nucleus (Fig. ). A vascular screen for stroke risk factors was negative. A DAT scan was unavailable due to lack of resources and financial constraints.\nShe was treated with a trial of levodopa (300 mg per day) and anti-platelet therapy which resulted in marked reduction in her jaw tremor and other extrapyramidal symptoms after 1 month of follow-up (UPDRS part III improved from 36 to 24).","This 67-year-old woman came to the clinic complaining of a new, worsening tremor in her hands and jaw that started suddenly. Neurological examination revealed stiffness and slow movement in her hands, along with the tremor, and a CT scan showed a small stroke in the area of her brain that controls movement. She was started on medication (levodopa) which significantly reduced her tremor and other movement problems. After a month of treatment, her symptoms improved, and she is now being followed up to continue with the medication."
839,"A 55-year-old Japanese woman had developed right-lateral chest and back pain 2 months prior to admission to our hospital. Thirteen years previously, she had undergone concurrent bilateral salpingo-oophorectomy and total hysterectomy for right ovarian tumor in other institution. She had no past history of other neoplasms. Physical examination revealed deep-tendon hyperreflexia in the lower extremities but no muscle weakness. Blood tests were unremarkable apart from a high serum concentration of cancer antigen 125 (134 U/mL; cutoff value, 35 U/mL). On fluorodeoxyglucose (FDG) positron emission tomography the maximum standardized uptake value (SUVmax) was 6.6 in the right paraspinal region at the level of Th12 (Fig. ) and 8.8 in the right thyroid lobe; however, fine-needle aspiration cytology of the latter yielded no evidence of malignancy. CT scan and magnetic resonance imaging (MRI) revealed an irregular mass in the right arch of Th12 vertebral bone that protruded into the spinal canal through the intervertebral foramen and was infiltrating surrounding soft tissue, the whole mass being of 35 × 78 × 36 mm (Fig. -). No primary tumor was detected in other organs or the abdominal or pleural cavities. Pathological examination of a CT-guided needle biopsy of the paraspinal lesion demonstrated papillary proliferation of epithelial cells with blunt nuclear atypia against a fibrotic background with psammomatous calcification (Fig. ). Immunohistochemically, the neoplastic cells were positive for paired box 8 (PAX8) (Roche Diagnostics; Basel, Switzerland), estrogen receptor (ER) (Roche Diagnostics), and Wilms’ tumor 1 (WT1) (Roche Diagnostics), but negative for thyroglobulin (Nichirei; Tokyo, Japan) (Fig. ), thyroid transcription factor 1 (TTF1) (Roche Diagnostics), progesterone receptor (Roche Diagnostics), S100 protein (Roche Diagnostics), and calretinin (Roche Diagnostics). Staining for P53 (Roche Diagnostics) showed a non-mutational “wild type” pattern (Fig. ). Because the lesion was suspected of being a metastatic neoplasm from the previous ovarian tumor, we obtained the relevant clinicopathologic and operative records and found that our patient had had a 13- × 11- × 9-cm cystic tumor that did not involve the ovarian surface (Fig. ). Peritoneal cytology was negative; however, the tumor had been ruptured intraoperatively. Lymph-node biopsy was not performed during the original surgery. The six slides available for microscopic review in retrospect, which included all the 6 sections (1 on each slide) submitted for the previous diagnosis of the primary tumor, showed cuboidal to columnar epithelium with minimal cytologic atypia proliferating in hierarchical branches with no invasive features, micropapillary components, or peritoneal implants (Fig. –), the diagnosis being conventional Stage IC1 SBT/APST according to the current World Health Organization (WHO) and International Federation of Gynecology and Obstetrics (FIGO) 2014 classifications. All the above-listed immunochemistry markers were identical in the previous ovarian tumor and the present paraspinal neoplasm, except for Ki-67 (Agilent Technologies; Santa Clara, CA, USA), which had labeling indices of 1 and 5%, respectively. It was therefore concluded that the paraspinal lesion was a bone metastasis of transformed LGSC originating from the previous SBT/APST in the right ovary. The patient underwent radiotherapy (30 Gray in 10 fractions) for the paraspinal lesion, which relieved her pain, followed by chemotherapy treatment with paclitaxel and carboplatin plus bevacizumab. She remains alive with right-sided pleural effusion and suspected metastases in the Th3 and Th4 vertebrae 168 months after the initial surgery.\nNeither the primary nor metastatic tumor harbored KRAS mutation (codon 12, 13, 59, 61, 117, or 146) according to polymerase chain reaction (PCR) using a reverse sequence-specific oligonucleotide methodology (LSI Medience; Tokyo, Japan), nor BRAF V600E mutation according to real-time PCR (LSI Medience).","This 55-year-old woman has been experiencing pain in her chest and back for two months. Tests revealed a tumor that had spread from a previous ovarian cancer to a bone in her spine. The tumor is causing pain and pressing on her nerves, and further tests confirmed it was a metastasis. She is now receiving radiation therapy and chemotherapy to manage the tumor and relieve her pain, and is being monitored for any further spread."
840,"A 35-year-old female (body weight 60.5 kg; height 1.55 m; body fat 16%; back squat 143 kg; front squat 125 kg; clean 97 kg; snatch 63 kg) without medical history of disease presented worsening abdominal pain approximately 24 h after completing a rigorous extreme conditioning competition (), which consisted of two days of five workouts. She was healthy overall and had been active in ECP over the previous five years and trained four or five times per week. The patient gave informed written consent for the use of her clinical and personal data in this paper.\nThe patient visited her physician one day after the ECP competition and was found to have a serum CK of 43,322 U/L. However, after receiving initial medical attention, she was sent home with instructions to take Tenoxicam (anti-inflammatory drug), bed rest, and drink plenty of water. On the third day post-competition, the pain and muscle swelling did not diminished, and she checked into an emergency room. At this stage her CK concentration was tested again and was 77,590 U/L. However, her kidney function, as indicated by blood urea, creatinine, sodium, and potassium concentrations was normal. On the other hand, her liver enzymes were elevated (aspartate aminotransferase (AST) of 477 U/L and alanine amino transferase (ALT) of 74 U/L). Chronological values of serum biochemistry and associated biomarkers over 25 days of follow-up are presented in . The patient was diagnosed with rhabdomyolysis by the medical attending physician and was treated with aggressive intravenous fluid resuscitation.\nShe was discharged on the fourth day of hospitalization and she was advised to avoid intense exercise. A follow-up examination revealed that her serum level of CK was still elevated to 3034 U/L on the 10th day and 1257 U/L on the 25th day following the ECP competition (). The subject reported myalgia even 25 days after the ECP competition.","This 35-year-old woman experienced severe muscle pain and elevated levels of a muscle enzyme (CK) after a very intense fitness competition. Initially, her symptoms were managed with rest and medication, but they worsened, leading to a visit to the emergency room. Doctors diagnosed her with rhabdomyolysis, a condition where muscle tissue breaks down, causing damage to the kidneys and liver. She was treated with fluids and monitored closely, and her muscle enzyme levels gradually returned to normal over several weeks."
841,"The patient, a 46-year-old female, presented to our department with complaints of continuous urinary incontinence. Her medical history included laparoscopic hysterectomy because of uterine myoma 2 months before her visit. Complete blood count, routine biochemistry parameters, urinalysis, urine culture, and urinary system ultrasonography results were all normal. We identified a 5-mm wide fistula between the bladder trigone and the upper part of the vaginal vault with flexible cystoscopy at the outpatient clinic. The patient was catheterized for 3 weeks. The patient who had catheterization failure underwent transvesicoscopic bipolar sealing of the vesicovaginal fistula (TBSF) after 3 months from her primary gynecologic surgery. The patient signed an informed consent form after she was advised on the use of the novel modified surgical procedure.\nThe operation was conducted with the patient in lithotomy position under spinal anesthesia. A prophylactic antibiotic (second-generation cephalosporin) was given half an hour before the induction. The VVF was identified with a cystoscope using insufflation of gas and it was verified by a guidewire (). The vagina was packed with vaseline-soaked gauze to prevent leakage during bladder filling and escape of CO2 during vesicoscopy. A 5-mm laparoscopic port was placed into the bladder under cystoscopic guidance in the lateral of the midline, halfway between the umbilicus and symphysis pubis. The second 5-mm port was placed into the bladder laterally at the midline and inferior to the first port (). A cystoscope was used as a transurethral camera for vesicoscopy. Bladder mucosa and muscular layer were gripped with a forceps and raised up for a multilayer closure. The fistula tract was grasped and sealed by an EBVS (LigaSure™ 5 mm blunt tip 37 cm sealer; Medtronic, Inc., Dublin, Ireland) (). An 18F Ryle's tube was kept in the bladder as a cystostomy through one of the existent ports. The bladder was also catheterized.\nThe operative time was 65 minutes. Blood loss was minimal and could not be measured in the case. There were no intraoperative or postoperative complications. The patient was allowed oral intake within 3 hours, to move within 12 hours postoperatively, and was discharged after removal of the cystostomy on the first postoperative day. The Foley catheter was kept in place for 3 weeks. An oral anticholinergic was given until the removal of the Foley catheter. The patient was instructed to return to our office 3 weeks after surgery for urethral Foley catheter removal and subsequent cystoscopic and vaginal inspection to confirm VVF repair (). During a 3-month follow-up, the patient remained continent, and the laboratory results were within normal range.","A 46-year-old woman came to the hospital because she was experiencing constant urinary leakage. Doctors found a small hole (fistula) between her bladder and vagina, which was likely caused by a previous surgery to remove uterine fibroids. The hole was successfully repaired using a minimally invasive procedure called transvesicoscopic bipolar sealing, which involved carefully closing the fistula with specialized instruments. The patient is now continent and has been discharged home with instructions for follow-up care and medication to help control any leakage."
842,"A 65-year old woman, on dialysis for 17 years, told her doctor she was exhausted and that she thought it was because she was taking too many drugs. The patient is an intelligent, independent, acculturated woman who lives alone in the French countryside.\nHer medical history is complex: she was treated with lithium for about 20 years (from age 20 to 40). This was discontinued after she developed CKD; in recent years, under treatment with valproic acid and lamotrigine, her psychophysical balance has been good,.\nShe started smoking when she was 19 years old (30 cigarettes/day) and developed a smoke-related chronic obstructive pulmonary disease. She was obese in early adulthood; arterial hypertension was diagnosed at age 30 and type 2 diabetes at age 32, treated using oral hypoglycemic drugs, but she eventually lost about 20 kg, making it possible for antidiabetic drugs to be discontinued. She underwent total thyroidectomy for papillary carcinoma at age 41, and started levothyroxine therapy afterwards. Due to a progressive worsening of the kidney function she started hemodialysis at age 50. Her kidney disease was probably multifactorial (hypertension, diabetes, obesity, heavy smoking, lithium therapy).\nSeven years after dialysis start, she underwent left hemicolectomy for colon adenocarcinoma, and two years later, left quadrantectomy followed by radiotherapy for ductal mammary adenocarcinoma. She underwent subtotal parathyroidectomy for severe tertiary hyperparathyroidism at age 62. Due to the presence of severe scoliosis, and the development of peripheral neuropathy, she uses painkillers regularly.\nAt the time of the present report, she was on thriceweekly hemodiafiltration, with good dialysis tolerance and high dialysis efficiency (Daugirdas 2 Kt/V: 1.6–1.8).\nHer most recent treatment included antihypertensive drugs (spironolactone 100 mg, amlodipine 20 mg, perindopril 2.5 mg), antipsychotic drugs (valproic acid 600 mg, lamotrigine 100 mg), thyroid hormone (levothyroxine 150 μg), vitamin D, bicarbonate and calcium supplements (calcium carbonate 1 g, sodium bicarbonate 500 mg, vitamin D 25-OH 100,000 UI once a month), potassium and phosphate binders (sodium polystyrene sulphonate, on non-dialysis days and sevelamer 2.4 g per day), darbopoietin 20 μcg once weekly.\nThe clinical examination revealed a woman with good psychophysical balance, a moderate impairment in nutritional status, and a severe comorbidity burden (weight 54 Kg, height 155, BMI: 23 Kg/m2; subjective global assessment: B; malnutrition inflammation index: 10; Charlson index: 9). Apart from signs of chronic bronchitis, and an aortic 2/6 heart bruit, the clinical examination was unremarkable; arterial blood pressure was 150/90 mmHg, with mild orthostatic hypotension (135/80 mmHg); heart rate was 68 bpm. The most recent biochemical results are reported in Table .\nThe patient complained of severe fatigue, which had recently increased, and asked her doctor if he thought this could be the result of taking too many drugs.\nIn fact we thought our patient was right about pharmacologic interference, and felt that levothyroxine was the most likely candidate. A biochemical control disclosed a relevant increase in TSH (13.07 mU/L), as compared with her previous routine twice-yearly control (4.14 mU/L).\nIn retrospect, the levothyroxine dose was already high for a woman weighing about 55 Kg, and a reduction in the absorption of levothyroxine should already have been suspected.\nThree of her chronic drugs display potential interference with levothyroxine: calcium carbonate, sevelamer and kayexelate. Since her sevelamer dose had recently been increased from 800 mg once daily to 800 mg 3 times per day, this long-scting phosphate binder was the most likely candidate. In keeping with this hypothesis, one month after discontinuation of sevelamer and modification of the timing of levothyroxine treatment (in the morning after night fasting) TSH was once more in the normal range. In the three months that followed, her levothyroxine dose was reduced to 100 micrograms per day. A further attempt to reintroduce sevelamer, taken at least 6 h after levothyroxine, led to a new increase in TSH (TSH 12.66 mUI/l), once more corrected by discontinuation of sevelamer.\nPubmed and EMBASE were explored (start to February 15th 2018) with the aim of retrieving papers related to dialysis, levothyroxine and phosphate binders. The following terms were employed: (a) dialysis, hemodialysis, hemodiafiltration, renal replacement therapy; (b) phosphate binder(s), sevelamer, calcium carbonate, calcium acetate, aluminum hydroxide, lanthanum carbonate; (c) levothyroxine, thyroid hormone replacement therapy. Due to the low number of papers retrieved by this search, a further search was performed combining (b) and (c). The searches, paper selection and data extraction were performed in duplicate (EC and VC); a further search on Google med did not lead to further papers. Discrepancies were resolved by discussion with a third party (GBP). A manual search of the reference lists from identified articles was done to identify additional articles. The search strategy and flow-chart is reported in Fig. .\nGiven the small number of papers retrieved, and their high heterogeneity, a meta-analysis was not performed, and the data were narratively discussed, in relation to other sources of information (data obtained in healthy volunteers, or in other diseases).","This 65-year-old woman has had a very complex medical history, including long-term dialysis, cancer treatment, and several other health issues. She recently complained of feeling extremely tired, and her doctor suspected that her medications might be contributing to this fatigue. After carefully reviewing her medications, they discovered that a phosphate binder was likely interfering with her thyroid hormone medication, leading to an increase in her thyroid hormone levels. By adjusting the timing of her medications and reducing the dose of the phosphate binder, her thyroid hormone levels returned to normal, and her fatigue improved."
843,"A 23-year-old Asian man was referred to the ER of Xiamen Chang Gung Memorial Hospital with a 1-day history of right-sided chest pain that had been aggravated for 1 hour. He had no known medical illnesses and was well until the evening prior to presentation, at which time he developed obvious right-sided chest pain radiating to his ipsilateral shoulder with persistent chest tightness. This tightness was described as sticking in nature, significantly worse on deep inspiration and with movement, and relieved by leaning forward or lying down. There was an associated dry cough but no hemoptysis. There was no history of trauma, injury, difficulty in breathing, or palpitations. He was tall and thin and described himself as otherwise quite healthy. He had never previously been admitted to a hospital. He reported no significant chronic medical history, such as primary hypertension, any type of heart disease, disturbed microcirculation, peripheral neuropathy, diabetes mellitus, an impaired immune system, malignancies, leukemia, the long-term administration of corticosteroids, liver cirrhosis, renal failure, urinary tract infection, or hemodialysis. He also reported no history of infection, such as tuberculosis, any type of hepatitis, or acquired immunodeficiency syndrome (AIDS). There was no prior history of traumas, blood transfusions, surgical procedures, or other serious events in his medical history. He had not lived in an epidemic area and had no history of toxin or radioactive exposure. He denied a personal or family history of bleeding diathesis but reported a 10-year history of smoking 8–10 cigarettes per day. He was an office worker by occupation. He had experienced similar symptoms on one occasion 4 years previously. No abnormalities were detected at that time, and his symptoms resolved.\nA physical examination (PE) revealed a young man who was awake and alert but in mild to moderate painful distress. His respiratory rate was 22–26 breaths/minute with an oxygen saturation of 97%. His pulse was 96 beats/minute, his blood pressure was 115/74 mmHg, and his temperature was 36.7 °C. The examining physician noted slight tenderness along the right posterolateral chest wall along the eighth and tenth ribs. Breath sounds and percussion were documented as normal. An electrocardiogram revealed sinus rhythm with a normal axis. PSP was considered, and a standing chest X-ray (CXR) was requested. The radiographic findings revealed a right-sided PSP (approximately 30%) with a small amount of pleural effusion (Fig. ). A right thoracostomy tube (28-F, straight) was immediately placed under sterile conditions; approximately 50 mL of light red pleural effusion flowed out from the chest tube after placement, and good fluctuation of the water column in the drainage reservoir was observed. Another CXR was performed to evaluate the position of the thoracostomy tube and re-expansion of his right lung (Fig. ). Our patient’s vital signs stabilized, and his right-sided chest pain was apparently alleviated after chest tube placement; therefore, he was referred to the respiratory service with parenteral analgesia.\nWhile in the respiratory department, approximately 420 mL of blood was drained from the thoracostomy tube over 15 minutes. He developed obvious hemodynamic instability with hypovolemic shock (his blood pressure dropped from 110/70 to 75/50 mmHg), and he was subsequently admitted to the cardiothoracic surgical ward after fluid resuscitation. During the 4 hours after admission, 750 mL of blood was drained through the thoracostomy tube. His hemoglobin level dropped from 12.6 g/dL to 9.2 g/dL. A prolonged prothrombin time (PT) of 19 seconds was noted (normal reference of 14 seconds). Packed red cells and fresh frozen plasma were administered to our patient. Vitamin K (10 mg) and tranexamic acid (1 g) were also administered parenterally. He was unable to undergo an emergency chest computed tomography scan as his vital signs remained unstable after fluid resuscitation. Alternatively, a bedside supine CXR was performed when he was temporarily hemodynamic stable (Fig. ). A primary SHP associated with right TP was considered based on the radiographic findings. A re-examination of his chest revealed markedly decreased air entry on the right side with mild tracheal deviation.\nAn emergency limited posterolateral thoracotomy was performed for resection of the bullae, ligation of the bleeding adhesion, and irrigation of the pleural cavity, and mechanical pleurodesis was implemented. Approximately 800 mL of blood and clots and a collapsed right lung with several apical bullae were observed. There was a small (less than 1.0 cm) tan lesion noted on the dome of the right side of his chest. Bullous apical tissues of the right lung adhering to the thoracic wall and adhesions at the region of the subclavian artery were identified as the source of bleeding. Aberrant blood vessels growing from the chest wall through the adhesion bands into the pleural lesion were thought to be torn once the lung collapsed. These bleeding blood vessels may also have arisen from the surface of ruptured bullae. Histopathology revealed that the bullous and string-like tissues were rich in blood vessels and were granulomatous.\nSubsequently, the drainage from his chest tube became minimal. A standing CXR performed on day 6 of admission after removal of the thoracostomy tube showed complete re-expansion of his right lung. His hemoglobin level rose to 10.1 g/dL after a transfusion of two units of packed red blood cells, and his PT normalized. He remained stable and was discharged within 1 week (6 days postoperatively). He returned to an out-patient department for follow-up two times. The right-sided posterolateral surgical incision had healed on the 14th postoperative day. Full expansion of lungs was confirmed using a standing CXR on the 14th postoperative day and at 3 months after discharge. He was healthy as usual with no complaints or illness.","This 23-year-old man came to the hospital with sudden, sharp pain in his right chest that spread to his shoulder. He had a dry cough and felt tightness in his chest, which was worse when he took deep breaths. Tests showed a collapsed lung with some fluid around it, and a small lesion on his chest wall was found. Doctors placed a tube in his chest to drain the fluid and stop the bleeding. He became very weak and needed blood transfusions. After surgery to remove the damaged lung tissue and repair the bleeding, his lung slowly expanded, and his condition improved. Finally, he was able to go home after a week, with his lung fully recovered and no further problems. He will continue to follow up with his doctor to ensure his lung remains healthy."
844,"A 62-year-old Caucasian man, nonsmoker, had recto-sigmoid carcinoma in September 2015. Initial investigations showed the cancer was T3, N2 and M0 (grade II adenocarcinoma). He received neoadjuvant chemoradiotherapy with Capecitabine and radiotherapy (50Gy in 25 fractions) which was completed in December 2015.\nPost chemo-radiotherapy, computed tomography (CT) scan and positron emission tomography (PET)/CT showed favourable rectal tumour response, but there were six small new liver lesions on magnetic resonance imaging (MRI). He was started on Modified De Gramont regimen plus Oxaliplatin in March 2016, Panitumumab was added in May 2016 as K-RAS was wild-type. Reassessment CT scan, MRI and PET scans in September 2016 showed a very good response in the liver and rectal primary lesions with no evidence of extra-hepatic disease. Only two lesions had been identified in the liver MRI while the rest of the lesions disappeared (). He underwent resection of the rectal disease with loop ileostomy by end of January 2017. The postoperative pathology showed a complete response. After recovery from bowel surgery, he continued on systemic chemotherapy and Panitumumab early March 2017 for three more cycles.\nIn May 2017, he was admitted with a 3-day history of fever, dry cough, progressive shortness of breath and decreased exercise tolerance. SpO2 was reduced at 88%. The total number of Panitumumab treatment cycles the patient received prior to this acute admission was 18.","This 62-year-old man was originally diagnosed with rectal cancer in 2015 and successfully treated with chemotherapy and radiation. After treatment, scans revealed small tumors had appeared in his liver, but they responded well to further treatment. He underwent surgery to remove the rectal cancer and had a temporary colostomy. Recently, he was admitted to the hospital with a fever, cough, and difficulty breathing, likely due to side effects from his ongoing chemotherapy."
845,"A 54-year-old man was diagnosed with MPL mutated, primary myelofibrosis in 2004 with a hypercellular marrow, MF-2 fibrosis with megakaryocytic atypia, peripheral leukoerythroblastosis, splenomegaly, and an elevated lactate dehydrogenase. He was observed until 2010, when he was started on hydroxycarbamide for increasing symptoms. Prior to his 2011 enrollment in a clinical trial utilizing a novel JAK2 inhibitor, fedratinib, a bone marrow biopsy (Figure , Panel A; hematoxylin and eosin stain above, reticulin stain below, 10×) showed progressive megakaryocytic atypia and MF-3 fibrosis. Nearly 1 year into the trial, a repeat bone marrow (Figure , Panel B; hematoxylin and eosin stain above, reticulin stain below, 20×) was hypocellular with MF-3 fibrosis only in the cellular areas. The patient was taken off trial in 2013 and started on ruxolitinib. Repeat bone marrow biopsies in 2016 (Figure , Panel C; hematoxylin and eosin stain above, reticulin stain below, 10×) show a marrow comprised entirely of adipose tissue with osteosclerosis. Quantitative fat magnetic resonance (MR) imaging, utilizing methods reported separately, shows replacement of the marrow with fat. Representative transaxial MR images of the pelvis show water-only, and fat-only (Figure , top row) constituents. Arrows show the iliac crests. A representative pseudocolor display that depicts 100% fat as dark red and 0% fat as dark blue is shown (Figure , bottom row). The percent fat evident in the bone marrow imaging approximates that in the subcutaneous fat. After 3 bone marrow biopsies and the MR demonstrating this unusual transition of primary myelofibrosis to a fatty, aplastic appearing marrow, a repeat bone marrow in 2017 (Figure , Panel D; hematoxylin and eosin stain above, reticulin stain below, 20×) now showed a hypocellular marrow with histopathologic findings consistent with his marrow at the time of diagnosis. To our knowledge, this is the first case of a fibrotic marrow developing such an aplastic appearance in a patient treated with a JAK2 inhibitor.","This 54-year-old man was originally diagnosed with a rare blood disorder called primary myelofibrosis in Over time, his condition worsened, and he started taking medication to manage it. However, after a year on a new drug trial, his bone marrow changed dramatically, becoming almost completely filled with fat and appearing very thin. Further tests confirmed this unusual transformation, and now his bone marrow looks like it’s almost completely replaced by fat."
846,"A 61-year-old man had originally presented in 2012 with stage IVA oropharyngeal squamous cell carcinoma (SCC). He was treated with surgery followed by chemoradiation. Eighteen months later, he presented with a pancreatic mass and multiple osteolytic lesions. These were proven by biopsy to be metastatic oropharyngeal SCC. He was treated with carboplatin, 5-fluorouracil, and cetuximab with resolution of the pancreatic mass. On follow-up CT post 6 months of maintenance cetuximab, he was noted to have an asymptomatic left occipital brain metastasis for which he underwent stereotactic radiation. Three months later, he underwent left occipital craniotomy for relapsed disease. Imaging revealed a new enlarged paratracheal lymphadenopathy, and he was started on nivolumab at a dose of 3 mg/kg. After 3 months, he had a partial response. However, when he presented for day one of cycle 8, he complained of sudden onset fatigue. Brain MRI showed no new lesions. There was no evidence of pituitary inflammation. Blood work revealed a low random cortisol level of 1.8 μg/dL that did not have a satisfactory response to the ACTH stimulation test (Table ). With ACTH <0.5 pg/mL, primary adrenal insufficiency was ruled out, and he was started on dexamethasone followed by hydrocortisone for grade 2 immune-related central adrenal insufficiency. Because his fatigue was resolved, he resumed nivolumab treatment 5 weeks later. Restaging scans continue to show no evidence of progression. The patient continues to be on hydrocortisone with no complaints of fatigue.","This 61-year-old man has been battling cancer for many years, originally diagnosed with oropharyngeal cancer in The cancer has since spread to his pancreas, brain, and lymph nodes, and he has received various treatments including surgery, chemotherapy, and radiation. Recently, he developed fatigue and low cortisol levels, leading to a diagnosis of adrenal insufficiency, which was treated with medication. Now, his fatigue has resolved, and he is continuing his cancer treatment with nivolumab while taking hydrocortisone to manage his adrenal condition."
847,"A 61-year-old man with oral cavity SCC, who had been originally treated with surgery in 2011 for stage I disease, presented with cervical and left axillary lymphadenopathy after being lost to follow-up for over 4 years. Biopsy of the lymph nodes revealed metastatic SCC. He was then treated with carboplatin, 5-FU, and pembrolizumab on a trial. Pembrolizumab was dosed at 200 mg. After 4 cycles, his scans showed a partial response, but the patient presented with fatigue and hypotension to systolic blood pressure of 70 seconds. He was found to have a random cortisol level <0.5 μg/d that did not have a satisfactory response to the ACTH stimulation test (Table ). His ACTH level was <5 pg/mL. He was diagnosed with immune-related central adrenal insufficiency and was started on high-dose dexamethasone and later transitioned to hydrocortisone. Brain MRI did not show any inflammation of the pituitary stalk, and it was without metastatic lesions. Ten days later, his fatigue was resolved, and he resumed treatment with pembrolizumab. He continues to take hydrocortisone.","61-year-old man with a previous cancer diagnosis was found to have cancer spreading to his lymph nodes. He started a new treatment plan that showed some improvement, but he also developed fatigue and low blood pressure. Further testing revealed he had a problem with his adrenal glands, which were not producing enough cortisol. He was treated with medication to replace the missing cortisol, and his symptoms improved, allowing him to continue his cancer treatment."
848,"A 77-year-old man with stage IV lung SCC with metastasis to the liver was originally treated with carboplatin and nab-paclitaxel achieving a partial response. Unfortunately, he was found to have disease progression and was started on nivolumab 3 mg/kg. On subsequent scans, he continued to show stable disease. On day 1 of cycle 8, he complained of profound fatigue. He was found to have a random cortisol level of 1.3 μg/dL that did not have a satisfactory response to the ACTH stimulation test. His ACTH was <5 pg/mL (Table ). As immune-related central adrenal insufficiency was suspected, he was started on dexamethasone followed by hydrocortisone, and his symptoms were resolved immediately. The patient opted to discontinue nivolumab. Repeat scans 3 months postdiscontinuation of nivolumab continues to show no evidence of progression. He continues to be asymptomatic on hydrocortisone.","This 77-year-old man had lung cancer that spread to his liver and was initially treated with chemotherapy. Despite this, the cancer continued to grow, and he started a new medication called nivolumab. He then experienced extreme fatigue, which led to a low level of cortisol, a hormone that regulates energy. Doctors quickly treated him with steroids, which resolved his symptoms. After stopping nivolumab, scans showed no further cancer growth, and he continues to feel well on a low dose of steroids."
849,"The patient, a 7-year and 6-month-old girl, was admitted to our hospital for premature thelarche. Her previous clinical history was negative. Her height was 131.5 cm (+1.06 SDS), and breast development was Tanner stage 3. LHRH test showed a LH peak equal to 17.7 mUI/mL and a FSH peak equal to 8.3 mUI/mL. 17β-estradiol level was 35 pg/mL; adrenal and thyroid functions and cancer markers (α-fetoprotein, βHCG, CEA) were in the normal range. The pelvic ultrasonography showed the presence of transitional uterus and ovaries with increased dimension and normal echostructure. Bone age assessed with Greulich and Pyle method was advanced (9 years). Nuclear magnetic resonance imaging of the pituitary and brain was normal. Diagnosis of idiopathic central precocious puberty was performed. She started a gonadotropin-releasing hormone (GnRH) analogue therapy with triptorelin i.m. 3.75 mg every 28 days, and after 6 months, a new LHRH test showed suppressed gonadotropin peaks (a LH peak equal to 0.67 mUI/mL and a FSH peak equal to 0.60 mUI/mL) confirming the efficacy of treatment.\nAt the age of 8 years and 4 months, the patient was admitted to our Emergency Department for the appearance of abdominal pain, burning sensation at neck and dyspnea occurred two hours after the last injection of i.m. triptorelin. At the examination, pruriginous whistles of about 20 mm were present on the whole body. Diagnosis of anaphylactic reactions was performed, and a treatment with systemic epinephrine and intravenous hydrocortisone was started with prompt resolution of symptoms. Skin prick tests performed the day after showed a positive result at very low dilution of the drug (1:10.000). As a result, therapy with triptorelin was discontinued. At 6 months of follow-up, no substantial progress of puberty or other anaphylactic reactions have been observed.","This 7-year-old girl started puberty too early, which we called precocious puberty. Tests showed everything else was normal, and we determined it was likely due to a problem in her brain. To slow down the puberty process, she started taking a medication called triptorelin, which worked well for several months. However, she then experienced a severe allergic reaction to the medication, and we stopped using it, with no further issues observed during follow-up."
850,"A 73-year-old man (body mass index: 23.5 kg/m2) complained of an urge to move the legs accompanied by an unpleasant sensation that he could not describe in words, worsened during rest and precipitated before night sleep, resulting in difficulty in falling asleep and nonrestorative sleep. His symptoms were relieved by mobilization and met the RLS diagnostic criteria. His IRLS score was 11. He felt very uncomfortable upon waking in the morning and claimed that even prolonged sleep did not relieve his fatigue. His average sleep duration was 10 hours. His physical examination and laboratory tests yielded normal findings, including a serous ferritin level of 69 ng/mL. He had been previously treated for hyperlipidemia and ischemic heart disease, and he declined the use of additional medication, apart from hypnotics.\nI obtained a full-night diagnostic PSG recording while the patient was on hypnotic medication (zolpidem, 5 mg). I found that he experienced difficulty in initiating and maintaining sleep due to the discomfort associated with periodic leg movement (PLM) bursts. He received 4W-acuinjections of normal saline doses (0.25 mL each) supplemented with pentazocine (0.5 mg per dose) in both legs after the first 115 min of PSG. Immediately afterward, the patient felt a comfortable warmth to both legs, his discomfort subsided completely, and the PLMs ceased (Figure ).\nAlthough short PLM bursts reappeared 124 minutes later, the patient reported remarkably restorative sleep in the morning. Most of the PLMs were observed during stage wakefulness periods, where slow eye movements were observed while EEG was alpha-rhythm dominant. Thus, discomfort accompanied by PLMs during drowsy periods, rather than wakefulness, seemed to disrupt his attempts to fall asleep.\nAfter the 4W-acuinjections, his PLM index (number of PLMs/total recording h) decreased, and his percent sleep efficiency (total sleep time/total recording time × 100%) increased (Table ).","This 73-year-old man was experiencing restless legs and difficulty sleeping because of uncomfortable sensations in his legs that worsened when he was resting. These symptoms were relieved by movement, and he felt tired even after long periods of sleep. After a sleep study, doctors discovered that the uncomfortable sensations were causing him to move his legs during sleep, disrupting his rest. By using injections to temporarily relieve the discomfort, he was able to sleep better and his leg movements decreased."
851,"An 81-year-old man experienced symptoms including interrupted sleep (ie, waking up every 2 hours), excessive daytime sleepiness, and intolerable fatigue throughout the day. He experienced unpleasant paresthesia of the left leg urging to move. The symptoms worsened at rest and precipitated during the evening and night and were relieved by mobilization. His symptoms met the RLS diagnostic criteria, with an IRLS score of 16. However, he involuntarily tapped his right heel once every few seconds but did not report any discomfort related to this. Ordinarily, leg discomfort is bilateral in RLS. The patient's discomfort in the right leg could have been masked by his tapping; thus, his symptoms were consistent with RLS's typical presentation.\nHis average sleep duration was 9.5 hours. He was receiving pharmacotherapy for panic disorder, hypertension, hyperlipidemia, and dyspepsia. He refused additional pharmacotherapy. Laboratory tests returned normal findings, except for a ferritin level of 34.7 ng/mL and a hypoproteinemic serum protein level of 6.6 g/dL. His physical examination was unremarkable, except for emaciation (body mass index: 17.0 kg/m2).\nDiagnostic PSG revealed a high baseline obstructive apnea-hypopnea index in the supine position (Table ), which was associated with remarkable respiratory efforts, and a PLM index of 32.2, which was associated with bilateral gross knee flexion accompanied by EEG arousals (PLM arousal index during sleep: 10.3). All his PLMs occurred during sleep. No tapping-like movements were observed when he lay on the bed with extended knees, which suggested that knee flexion induced the movements.\nAt-home continuous positive airway pressure (CPAP) treatment was initiated with an auto-titrating device. During a month-long period preceding the follow-up visit, the patient's average apnea-hypopnea index was 0.7, and his compliance was 100%, but he continued experiencing discomfort of the left leg, right foot tapping, and nonrestorative sleep.\nThe second diagnostic PSG under auto-titrated CPAP was obtained 2 months after the first PSG. Before the PSG, he received 4W-acuinjections of normal saline doses (0.25 mL each) supplemented with pentazocine (1 mg per dose) in both legs in the sitting position while tapping his right heel, and immediately afterward, he felt warmth to both legs with a pleasant sensation, and his left lower leg discomfort and involuntary right leg movements were completely relieved. During the PSG, his PLMs were almost completely suppressed except for one leg movement. The 4W-acuinjections caused no major change in percent sleep efficiency, but the patient reported remarkably restorative sleep upon waking up in the morning.","This 81-year-old man has been struggling with disrupted sleep, excessive daytime tiredness, and a tingling sensation in his left leg that made him want to move it. After further testing, he was diagnosed with Restless Legs Syndrome (RLS) and obstructive sleep apnea, which was causing him to stop breathing during sleep. A series of injections in his legs helped to relieve the RLS symptoms, and using a CPAP machine to help him breathe while sleeping has significantly improved his sleep quality."
852,"A 34-year-old Caucasian male had undergone intestinal transplantation originally more than 3 years ago because of chronic intestinal pseudo-obstruction due to familial visceral myopathy . The first intestinal transplant was lost due to volvulus 2 months post-transplant. Retransplantation was performed 18 months ago. Viremic cytomegalovirus (CMV) gastroenteritis was diagnosed 1 year post-transplant. Treatment with intravenous ganciclovir and later oral valganciclovir was successful, and CMV PCR remained negative in blood and intestinal biopsies during follow-up. The patient also suffered another volvulus of the colon, which was successfully corrected operatively. Mild grade 1 acute rejection in the colon occurred 2 months later; the small intestine was normal. The rejection resolved quickly with intravenous steroids; follow-up biopsies at 1 week were normal. After the rejection episode, there were nine follow-up endoscopies showing only mild segmental erythema and distorted mucosal vascular pattern on a confined area in the colon; we speculated the previous volvulus as a cause for these mild findings. In biopsy specimens, there were no signs of rejection or inflammation, and viral samples were negative.\nNorovirus gastroenteritis was diagnosed 2 months after the rejection. The patient was admitted to the hospital due to dehydration, abdominal pain, and fever. Prior to this, the patient's family members had suffered symptoms of gastroenteritis, presumably of viral origin. In our patient, gastroenteritis symptoms with watery diarrhea persisted, and the patient needed repeated episodes of hospitalization due to dehydration. Norovirus PCR from the stools remained repeatedly positive, and the norovirus infection was considered a cause for the chronic diarrhea. Endoscopy findings were unchanged. Nearly 3 months after the initial norovirus infection diagnosis, treatment with oral immunoglobulin was started. An intravenous immunoglobulin solution (Privigen®) was given orally at a dose of 1250 mg (25 mg/kg body weight) four times daily for 2 days (altogether eight doses). The immunoglobulin solution did not bypass the gastric barrier. Tacrolimus trough levels during the preceding 4 months had been stable above 10 ng/L (range 11.5–18.3 ng/L), and the patient had received triple immunosuppression therapy with MPA 360 mg twice daily and methylprednisolone 6 mg daily.\nFour days after the end of the immunoglobulin treatment, the patient was again admitted to hospital due to fever, increased diarrhea, and abdominal pain. Endoscopy via colostomy was performed, and severe biopsy-proven rejection grade 2–3 was diagnosed in the small-bowel and colon (Fig. and Fig. ). CMV and Epstein–Barr virus samples were negative. Tacrolimus trough level was 12.4 ng/L. Initial therapy was by high-dose intravenous methylprednisolone. However, signs of rejection persisted on repeat endoscopy, and finally, a 10-day course of intravenous antithymocyte globulin was started, whereby the acute rejection resolved both symptomatically and in graft biopsies (Fig. ). Donor-specific antibodies were negative. Follow-up endoscopies showed persisting signs of colitis and distal ileitis, but these finally resolved at 3 months after starting immunoglobulin therapy, and the patient has remained rejection-free now 10 months later.\nThe norovirus infection persisted, and the patient was started on nitazoxanide 500 mg twice daily, but without any clear clinical or virologic response. Bacterial, other viral, and parasitic samples were negative (Fig. ).","This 34-year-old man has had a challenging journey with intestinal transplants. He initially needed a transplant due to a rare muscle disorder, but it was lost shortly after. He then received a second transplant, but has since experienced several complications, including CMV infection, volvulus (twisting of the intestine), and repeated bouts of norovirus gastroenteritis. Recent tests revealed a significant rejection of his transplanted intestine, requiring treatment with steroids and immunoglobulin. Despite these challenges, the patient is now stable and has been following up for 10 months."
853,"A 55-year-old male patient presented to our department with progressively worsening, right hypochondrium pain. He complained for abdominal distention and early satiety for the last 2 months. His history was significant for a one-year onset of mildly elevated arterial pressure, successfully managed with administration of low-dose amlodipine. Clinical examination revealed a voluminous palpable mass occupying the left quadrat of his abdomen. Abdominal ultrasound imaging showed a huge cystic mass posterior to the pancreas, compressing the stomach. Abdominal computer tomography (CT) confirmed the presence of a huge cystic retroperitoneal lesion, which measured 22 × 22 × 10 cm (Figure A,B). The lesion showed close intimacy to the posterior aspect of the pancreas. Endoscopic ultrasound fluid aspiration was negative for CA19-9 and amylase, whereas biopsy samples were inconclusive on the nature of the lesion. Albeit the fact that the lesion was not shown to arise from the left adrenal, 24-hour urine catecholamine levels were also examined and were within normal range. The patient's vital signs and blood CEA and CA19-9 were within normal range. The patient underwent complete excision of the cystic mass. Histology demonstrated the presence of pheochromocytoma, which arose from adrenal tissue and demonstrated immunohistopositivity to chromogranin and synaptophysin. Due to lesion size and microvascular invasion, it was categorized as malignant.\nDue to their evolution in the retroperitoneal space, pheochromocytomas may grow significantly in size and remain asymptomatic for a long period of time. Moreover, contrary to solid, cystic pheochromocytomas may not present typical clinical symptomatology or urine values of catecholamine metabolites may be found within normal range. Such atypical presentation makes the preoperative diagnosis of giant retroperitoneal cystic lesions challenging.","This 55-year-old man came to the hospital because he had increasing pain in his abdomen and difficulty eating. Tests revealed a large, unusual growth behind his pancreas that was pressing on his stomach. A CT scan confirmed the diagnosis of a malignant pheochromocytoma, a rare type of tumor that can grow without causing obvious symptoms. The tumor was successfully removed, and the pathology confirmed it was cancerous."
854,"A 26-year-old man who was referred to our hospital presented with a clinical history of exercise-related syncope. Coronary CT angiography revealed an anomalous aortic origin of the right coronary artery (RCA) from the left coronary ostium. The proximal portion of the RCA seemed to be coursing along the aortic vessel wall before running between the aorta and pulmonary artery (Figure A,B). CT-based fractional flow reserve (FFRCT) (HeartFlow, Redwood, CA, USA) in the RCA was 0.77 (ischemic cutoff ≤0.8), suggesting significant ischemia (Figure C,D). Coronary angiography suggested the presence of a slit-like ostium of the anomalous RCA (Figure E). Invasive FFR evaluation confirmed significant ischemia (Figure F). The treating physicians decided on surgical treatment.\nAn anomalous origin of the RCA from the left sinus of Valsalva is rarely seen congenital anatomy. Although the presentation is usually silent, clinical manifestations may include aborted sudden death, chest pain, arrhythmia, and/or exercise-induced presyncope or syncope. The FFRCT, derived from the usual data set from coronary CT angiography, was recently developed to evaluate functional ischemia of the coronary artery. In the present case, FFRCT revealed ischemia in an anomalous coronary artery, which was confirmed by the invasive FFR measurement. Thus, the FFRCT technique has potential for innovation in the assessment of anomalous coronary arteries.","This man came to the hospital because he experiences fainting spells during exercise. Tests revealed a rare heart condition where his right coronary artery originates from the wrong place in his heart. This unusual artery was causing reduced blood flow to part of his heart, leading to the fainting. Doctors decided to operate to correct the artery and improve his heart health. This technique, called FFRCT, can help identify these types of heart problems."
855,"We present the case of a 53-year-old female who transferred from an outside facility requiring a higher level of care, due to worsening pneumonia with possible abscess and the need for cardiothoracic surgery (CTS) consultation. Past medical history included previous breast cancer post-lumpectomy and radiotherapy, remote history of vulvar and rectal cancer post wide-debulking, chronic obstructive pulmonary disease, and ventilator-dependent respiratory failure with tracheostomy. Home medications included albuterol/ipratropium nebulizer, alprazolam, amlodipine, aripiprazole, budesonide/formoterol metered dose inhaler, citalopram, tamoxifen, tiotropium inhaler, trazodone, and oxycodone. She reported an allergy to nonsteroidal anti-inflammatory drugs. The patient initially presented to another facility complaining of fever, diarrhea, shortness of breath, and increasing oxygen demands. On examination, the patient was not in acute distress. All systems were negative except for diminished lung sounds with rhonchi bilaterally. A chest X-ray demonstrated left upper lobe pneumonia. Blood cultures were drawn, and a sample of tracheostomy secretions was sent for culture and sensitivities. She was initiated on vancomycin and piperacillin-tazobactam for treatment of healthcare-associated pneumonia. The patient reported a history of Clostridium difficile colitis and was started on oral vancomycin and IV metronidazole. On day 2, a bronchoscopy was performed with washings sent for culture. Both respiratory cultures grew MDRPA while blood cultures remained negative. The susceptibility profile is outlined in Table . Piperacillin-tazobactam was switched to meropenem at 2 g IV every 8 hours, and inhaled tobramycin was added. A repeat bronchoscopy was performed on day 12 due to mucus plugging and lack of clinical response. This culture grew persistent MDRPA, necessitating the addition of IV tobramycin on day 16. Inhaled tobramycin was switched to inhaled colistin on day 20. On day 23, tobramycin IV and metronidazole were discontinued and the meropenem dose was decreased.\nOn day 24, a 12-French chest tube was successfully placed into the left upper lobe abscess. Approximately 30 mL of purulent material was aspirated and sent for culture. Growth from this drainage continued to demonstrate MDRPA. One day after chest tube insertion, a computed tomography (CT) scan of the chest demonstrated significantly diminished fluid component of the left upper lobe abscess with drain positioned in the air-containing component; residual left upper lobe and left lingular pneumonia and pulmonary emphysema was also noted. The patient failed to improve and on day 28 ceftolozane-tazobactam was initiated at 1.5 g IV every 8 hours. A repeat chest CT scan on day 30 showed a decrease in the left upper gas-fluid collection, but persistent dense pneumonia of the left upper lung and a new smaller infiltrate in the left lower lobe.\nTwo days after the repeat chest CT, the patient was transferred to our facility for worsening pneumonia, requiring a higher level of care and CTS consultation. Abnormal clinical examination findings included diffusely diminished lung sounds, rales in the left lower lobe, a left pigtail chest tube drain, tachycardia with heart rate of 120 beats per minute, and tachypnea with a respiratory rate of 22 breaths per minute. The patient's chest tube was intact with a small amount of thick, white drainage, which was sent for culture, along with 2 sets of blood cultures. Ceftolozane-tazobactam 1.5 g IV every 8 hours was continued, and meropenem was stopped. The patient was initiated on tobramycin 280 mg (7 mg/kg) IV every 24 hours. CTS diagnosed the condition as a pulmonary abscess with bronchopulmonary fistula (BPF). The patient was deemed a poor surgical candidate, and continued medical management and drainage via chest tube was recommended. Repeat cultures from the pleural fluid drainage continued to grow MDRPA, while blood cultures remained negative. Susceptibility testing for ceftolozane-tazobactam was requested and performed using an investigational elipsometer (E)-strip. On day 36 overall (day 5 post-transfer), a repeat bronchoscopy was performed. Culture of the washings grew Acinetobacter baumaunii. Additionally, the results from the ceftolozane-tazobactam E-strip for Pseudomonas finalized, demonstrating an MIC of 12 mg/L (nonsusceptible). Chest CT on day 37 demonstrated mild interval increase in the air-fluid collection size, drain in place, persistent extensive left upper lobe pneumonia, and mild improvement in the lingula and left lung base. With no improvement and the growth of Acinetobacter, ceftolozane-tazobactam was discontinued on day 39, after 12 days total. Meropenem at 1.5 g (pediatric dosing of 40 mg/kg) IV every 8 hours and colistin 100 mg (2.5 mg/kg) IV every 12 hours were started. After 14 days of meropenem and colistin without improvement, all therapy was stopped and the patient was transitioned to comfort care and ultimately expired.","This 53-year-old woman was transferred to our hospital because she had a serious lung infection that wasn’t responding to treatment. She has a history of cancer and other health problems, including difficulty breathing and needing a breathing tube. After several rounds of antibiotics and drainage, doctors discovered the infection was caused by a resistant bacteria. Despite treatment, the infection persisted, and she ultimately passed away."
856,"The patient is a 53-year-old male with a history of alcoholic cirrhosis, who presented with a one-day history of severe generalized muscle weakness and myalgias; he was unable to rise from a chair without assistance. His cirrhosis was diagnosed in October 2016 per clinical history and findings on Computed Tomography (CT). His disease was complicated by Grade 2 esophageal varices, ascites, and hepatic encephalopathy (HE). He also had one hospitalization approximately 1 month prior to admission for an upper gastrointestinal bleed (UGIB), requiring four variceal bands. He had no history of spontaneous bacterial peritonitis (SBP). On admission, his model for end-stage liver disease (MELD) score was 20; his Child-Pugh Score was 12, placing him in Class C. His last drink was 32 days prior to presentation.\nOn arrival he was afebrile, tachycardic, and mildly hypertensive, but appeared comfortable. Physical examination was remarkable for symmetrical proximal muscle tenderness and weakness - ⅘ in the upper extremities and ⅗ in the lower extremities. Reflexes were 1+ and symmetric; sensation was intact. Laboratory testing was significant for leukocytosis, as well as elevations in the creatinine, aspartate aminotransferase (AST), white blood cells (WBC), and creatine kinase (CK) (Table ). The patient was also found to have an elevated erythrocyte sedimentation rate (ESR) at 71 mm/h, C-reactive Protein-Quantitative (CRP-QT) at 2.7 mg/dL, and lactate 3.5 mmol/L. Chronic abnormalities in his hemoglobin, albumin, INR, bilirubin, and alkaline phosphatase were also present, although remained stable throughout admission. Urinalysis demonstrated large blood on the dipstick, but was likely due to myoglobin, as microscopy revealed only 0-5 RBCs per high-power field. The etiology of his rhabdomyolysis was not apparent: he denied seizures, trauma, increased exertion, prolonged immobilization, recent travel, or use of statins, supplements, alcohol, or illicit drugs.\nHe was treated with aggressive intravenous fluid resuscitation. Despite resolution of his acute kidney injury, clearing of his lactate, and initial improvement in his laboratory values, his CK, WBC, and AST plateaued and began to rise again on day four (Figure and Table ). With ongoing muscle injury and persistent weakness, other etiologies (including autoimmune, infectious, and malignant) were investigated. CT chest/abdomen/pelvis was negative for abscess or mass lesions; muscle biopsy of the right thigh showed no evidence of vasculitis or acquired inflammatory, necrotizing, or metabolic myopathy (Figure ). Anti-Jo-1 and anti-HMG-CoA reductase antibodies were both negative. Further medication review revealed he was recently started on rifaximin for hepatic encephalopathy prophylaxis. Rifaximin has been occasionally reported to cause rhabdomyolysis in patients with hepatic insufficiency. We subsequently held his rifaximin on day six and noted a marked downtrend of CK the next day (Figure and Table ). The CK continued to downtrend and his myoglobinuria resolved soon after. The patient’s strength continued to improve with therapy, and he was discharged on day 16 with a diagnosis of rifaximin-induced rhabdomyolysis.","63-year-old man with cirrhosis and a history of bleeding in his esophagus presented with sudden muscle weakness and pain. Tests showed he had muscle damage (rhabdomyolysis) and elevated levels of muscle enzymes. It was determined that the muscle damage was likely caused by a medication he was recently started on, rifaximin, which he was taking to prevent complications from his liver disease. After stopping the medication, his symptoms improved, and he was able to go home with instructions to continue monitoring his condition."
857,"A 59-year-old man presented with eight-week history of nausea, abdominal pain, and 20-lbs weight loss. Two years prior to the current presentation, the patient was diagnosed with a stage T1A nonsmall cell lung cancer of the right upper lobe (large cell carcinoma) and his initial staging Positron Emission Tomography and Computed Tomography (PET-CT) was negative except for the above-mentioned lesion. The patient refused surgery at the time and completed Stereotactic Body Radiation Therapy (SBRT). His serial six-month interval surveillance imaging has been negative.\nWith his prior history of cancer and the current presentation, a total body PET-CT was ordered. This showed thickening of the wall of the gastric fundus, a mass in the tail of the pancreas (both with increased radiotracer uptake), and a 1.1-cm filling defect within the splenic vein consistent with thrombus (Figure ). Upper endoscopy showed a 5-cm infiltrative and ulcerated mass with heaped-up margins and necrotic center located in the gastric fundus (Figure ). Gastric biopsies showed poorly differentiated carcinoma (positive TTF1 and cytokeratin AE1-3, negative CDX-2) (Figure ), suggestive of metachronous metastasis from a lung primary. Endoscopic ultrasound (EUS) was performed and showed a 26 mm × 23 mm hypoechoic round mass in the tail of the pancreas with local vascular involvement and splenic vein thrombus (Figure ). EUS-guided fine needle aspiration (FNA) and fine needle biopsy (FNB) of the mass was performed through the gastric lumen (away from the gastric tumor to avoid tumor contamination or seeding by the needle tract). This confirmed neuroendocrine carcinoma (positive synaptophysin and chromogranin, positive Ki-67 and CDX-2, negative TTF1) (Figure ). Findings were suggestive of synchronous primary pancreatic neuroendocrine carcinoma. Treatment options were discussed with the patient and his family. Patient opted for palliative care and received symptomatic treatment.\nMetastasis of primary lung tumor to the stomach is infrequent and sporadic. The pathogenesis is thought to be related to the tumor cell spread via the hematogenous and lymphatic routes, but there is no specific data demonstrating a particular tropism for a segment of the gastrointestinal (GI) tract. Only 21 sporadic cases have been reported in the English literature and involved different malignant cell types: squamous cell carcinoma (10 cases), adenocarcinoma (6 cases), small cell carcinoma (2 cases), pleomorphic (2 cases), and large cell carcinoma (1 case). Symptomatic cases presented with epigastric pain, chronic anemia, signs of GI bleed (hematemesis, melena), and gastric perforations (in two cases). The definite role of PET-CT in the diagnosis of GI metastasis from lung cancer is still controversial because of the few cases and lack of enough clinical data. EUS-FNA in combination with immunohistochemistry is useful for diagnosing metastatic lesions and differentiating those from synchronous primary lesions.,","This 59-year-old man has been experiencing nausea, abdominal pain, and weight loss for eight weeks. After a previous diagnosis of lung cancer, doctors ordered a scan that revealed new tumors in his stomach and pancreas. Biopsies confirmed that these are new cancers, a neuroendocrine carcinoma in the pancreas and a metastasis from his lung cancer. Because of these findings, the patient chose palliative care and is receiving treatment for his symptoms."
858,"The patient was a 65-year-old man histopathlogically diagnosed with cardiac sarcoidosis at the age of 35 years. The 12-lead electrocardiogram exhibited an intraventricular conduction disturbance and left superior axis. The left ventricle (LV) exhibited a progressive dilation with a contractile dysfunction (severe hypokinesis on the inferior and inferior and septum). He was implanted with a cardiac resynchronization therapy device with defibrillator capability. In spite of the administration of amiodarone 100 mg, sotalol 160 mg, carvedilol 7.5 mg, mexiletine 400 mg, enalapril 2.5 mg, and spironolactone 25 mg, the patient was admitted to our hospital due to repetitive drug-resistant VT episodes and decompensated heart failure. The LV exhibited dilation and contractile dysfunction with an LV ejection fraction of 18%. In addition to the amiodarone, sotalol, carvedilol, mexiletine, enalapril, and spironolactone, intensive treatment of a VT storm and decompensated heart failure was performed with lidocaine 40-100 mg/h and furosemide; however, the VTs still could not be controlled.\nThe clinical VT on the 12-lead electrocardiogram exhibited a right bundle branch block pattern and left superior axis (Figure ). The VT cycle length was 380 millisecond, with a widened QRS duration of 184 millisecond. RFA was performed to manage the VT storm.\nSteerable catheters were inserted from the right femoral vein and placed in the right atrium and ventricle of interest. The LV endocardium was accessed using the trans-septal approach. Electroanatomical mapping was performed with EnSite (Abbott, Chicago, IL). A 3.5-mm open-irrigated ablation catheter (Therapy™ Cool Path™ Duo: Abbott) was used for the ablation and mapping. Bipolar voltage maps of the endocardium of the LV and right ventricle (RV) were constructed at baseline (pacing rhythm). Low voltage zones, defined as <1.5 mV, were mainly located on the interventricular septum (IVS) and postero-inferior wall of the LV. The clinical VT was induced and was hemodynamically stable. Entrainment mapping revealed that the VT had a reentrant mechanism. Activation mapping was performed; however, it could not depict the entire circuit, suggesting that a part of the circuit was intramural and/or epicardial. We performed RFA on the inferior and infero-septal walls of the LV and IVS from both the RV and LV endocardium, based on the activation and/or substrate maps, targeting the low voltage zones and/or abnormal electrograms such as those with fragmented and double potentials (Figure A). The procedural endpoint was the noninducibility of the clinical VTs. RF current was delivered for up to 60 seconds in the power-controlled mode with 30-45 watt and an irrigation rate of 17 mL/min.\nWe performed sequential RFA from the LV and RV; however, the VT did not terminate or slow. A SURF ablation on the inferior IVS from both the LV and RV endocardium was also performed. The ablation catheter used on the LV was a 3.5-mm open-irrigated ablation catheter (Therapy™ Cool Path™ Duo: Abbott), and that on the RV was a 4-mm nonirrigated ablation catheter (Therapy™ Thermocouple: Abbott). When performing the SURF ablation, two separate dispersive patches were used as the indifferent electrodes, with two separate generators in the power-control mode for the delivery of the SURF. RF current was delivered in the power-control mode starting at 10 watts, independently titrated up to 30 watts for each catheter with care taken to limit the temperature to <42°C for the irrigated catheter and <55°C for the nonirrigated catheter. The RF delivery was discontinued when the catheter tip impedance of either catheter dropped by more than 15 Ω as monitored from both RF generators. Figure B shows the position of the two ablation catheters when the SURF ablation was performed from both the RV and LV endocardium. We performed the SURF ablation at two sites on the IVS. The mean percentage of the R-wave reduction achieved by the SURF ablation was 67% on the LV endocardium and 86% on the RV endocardium. The VT was terminated and/or slowed during the RF application on the inferior wall of the LV and inferior IVS. The total application time of the RFA was 3502 seconds.\nThe clinical VT could not be induced at the end of the procedure. However, a VT with a slightly different QRS morphology emerged 2 days after the session and his heart failure worsened and became more serious due to VT storms. He developed pulseless electrical activity after that, and required intubation, sedation with propofol, percutaneous cardiopulmonary support, and intra-aortic balloon pumping. A second ablation session was performed 9 days after the first session. The VT was eliminated by RF applications on the infero-septal wall of the LV. No further VTs emerged after the RFA. The patient, however, died from deterioration of his heart failure 12 days after the procedure. A postmortem examination of the heart was performed.\nFigure A shows a cross section of the ablation lesions on the IVS in a four chamber slice, where the ablation lesions from the SURF ablation were assessed. The heart was significantly enlarged and weighed 505 g. The ventricular septum was thin. The yellow arrow heads in Figure A indicate the ablation lesions. The black-colored areas were lesions with hemorrhaging caused by damage to small intramural coronary arteries and capillaries and the adjacent cloudy discolored areas were necrotic tissue caused by the RF energy. The ablation lesions were mainly located on the IVS and inferior wall of the LV. Figure B shows identical histologic sections to those ablation lesions on the IVS in Figure A. The original pathologic fibrosis resulting from the cardiac sarcoidosis extended diffusely onto the IVS. In Figure B, the area surrounded by the blue line was assumed to be the ablation lesions created from the LV endocardium, and the area surrounded by the yellow line was the lesions created from the RV endocardium. Those lesions were contiguous, creating a transmural ablation lesion on the IVS. There was, however, a little spared viable myocardial tissue that escaped the RF energy on the LV endocardial surface (area surrounded by the red dotted line) despite the deeper sites having been completely ablated.\nThere was no active epithelioid granuloma from the sarcoidosis observed in the autopsy.","This 65-year-old man has a long-standing condition called cardiac sarcoidosis, which affects his heart. He’s had frequent, dangerous heart rhythm problems (VT) despite taking several medications. To try to control these problems, doctors performed a procedure called cardiac ablation, where they used heat to block the electrical pathways causing the rhythm issues. Unfortunately, despite the ablation, he continued to experience heart rhythm problems and ultimately passed away. The post-mortem examination revealed significant scarring and damage to the heart muscle from the sarcoidosis and the ablation procedure."
859,"In 2012, a 22-year-old edentulous female patient was referred to the Oral and Maxillofacial Surgery Department of Tehran University of Medical Sciences. She had severe class III malocclusion and facial deformity, which included nasal and mandibular deviation to the right. After taking a comprehensive medical and dental history, we noticed that the patient had hypodontia with several impacted teeth due to amelogenesis imperfecta (Figure ).\nIntra-oral clinical examination revealed horizontal discrepancy of alveolar ridge, knife-edge mandibular alveolar ridge, uneven alveolar ridge, and a deep palate (Figure ).\nRoutine radiographic examinations consisting of panoramic radiography and lateral cephalometry were requested. The cephalogram exhibited anterior-posterior discrepancy of the jaw in the horizontal plane and a pseudo-long face.\nFollowing consultation with a prosthodontist, cosmetic surgery was scheduled for the patient to correct her long face, mid-face deficiency, jaw deviation to the right, class III discrepancy, and alveolar ridge deficiency followed by dental implant placement and full mouth prosthetic rehabilitation. After consultation with an orthodontist, it was found that forced eruption of the impacted teeth was not possible; thus, the teeth were extracted under local anesthesia.\nPrimary impressions were made using irreversible hydrocolloid impression material (Kimica, Tokyo, Japan). Special trays were fabricated, and final impression was made by zinc oxide eugenol (Wuhan Xingzhengshun, Hubei, China). Then, the occlusal rims were made to record the inter-arch relationship, and the casts were mounted in a semi-adjustable articulator (Dentatus, New York, USA) in centric relation. The teeth were arranged in class III occlusion, and then they were coated with barium sulfate (Foshan Xinmei Chemical, Guangdong, China) to make them opaque for easy detection of the occlusal line for Epker cephalometric prediction tracing (Figure ).\nThe Epker prediction tracing was performed for preoperative assessments, which included analysis of the maxillary retrognathism, mandibular prognathism, and excess skeletal vertical growth. Sagittal split ramus osteotomy and LeFort I osteotomy were scheduled. According to the data obtained from the lateral cephalogram of the patient and clinical examinations (zygomatic regions, paranasal area, nasolabial, and gonial angles, SNA, SNB, and ANB angles), the surgeon planned a mandibular setback procedure by 7 mm and repositioning of the maxilla by 2 mm superiorly and 5 mm anteriorly on surgical casts. Then, the casts were mounted in this new position and the jaw relations were determined. Posterior teeth were arranged in new articulation and the final dentures were fabricated. The surgeon used these dentures for the edentulous patient as the final surgical splint. Mandibular cast and denture setback by 7 mm relative to its current position and an intermediate stent was fabricated using auto-polymerizing acrylic resin (Kulzer, Newbury, UK). Then, dentures were fabricated as splint and after polishing, several holes were drilled in the base of denture, and the arch bar was bonded to it. Circummandibular wires for the mandible and palatal screws were used to fix the splints to the corresponding jaws. The surgical procedure of the maxilla and mandible was performed using these splints (Figure ).\nAlveolar ridge augmentation was performed by harvesting a graft from the iliac crest. The graft was placed upon a surgical stent and fixed to the external maxillary surfaces with a screw and plate.\nAfter 2 weeks, temporary complete dentures were delivered to the patient. They were adjusted using tissue conditioner (Kerr, USA). Six months later, an impression was made and a radiographic stent was fabricated for implant placement. To determine the proper location for placement of implants, gutta-percha points (VDW, Munich, Germany) were placed at the center of desired teeth in ideal direction of implant, and the outer surfaces of the teeth were coated with barium sulfate (Foshan Xinmei Chemical, Guangdong, China) (Figure ).\nThe patient underwent cone beam computed tomography with stent to determine the proper site of implant placement (Figure ). Proper diagonal length and angle of 16 implants (Dentium, Implantium, Korea, Seoul), eight in each jaw, were determined by stent according to the available bone volume. Implants were inserted using a surgical stent. The temporary complete dentures of patient were relined and delivered to the patient. Three months later, the implants were uncovered, and healing abutments were tightened. Primary impression of the healing was made by alginate (Kimica, Tokyo, Japan). Primary cast was poured. After fabrication of special tray, final impression was made using the open tray technique following splinting of impression copings using polyvinyl siloxane (Zhengzhou Huaer, Henan, China). The final cast was fabricated. Impression verification jig was fabricated in a dental laboratory and was tried in patient's mouth to confirm the accuracy of impression. Next, the record base and wax rims were fabricated. The jaw relations were recorded by rims, and after that the teeth were arranged. The teeth set-up was checked in the mouth. A putty index was made from the arranged teeth and accordingly, proper abutment angle and gingival height were determined.\nThe three-part frames (Degubond, Dentsply sirona prosthetics, U.S.A, Pennsylvania) for the maxilla and mandible were fabricated. Frames were tried in the mouth, and the fit of frames was checked radiographically (Figure ). Porcelain crowns with A2 shade were fabricated. Implant-supported crowns were cemented with a temporary cement (Kerr, Toronto, Canada) (Figure ). The patient was fully satisfied with the results at the six-year follow-up.","This 22-year-old woman had a significant misalignment of her teeth and jaw, causing a noticeable facial deformity. After careful examination and imaging, doctors determined she needed surgery to correct her bite and improve her appearance. Following the surgery, she received dental implants and a full set of dentures to replace her missing teeth and restore her smile. At a follow-up six years later, the patient was very happy with the results of her treatment."
860,"A 38-year-old male underwent a tissue-matched renal allograft transplant for end-stage renal failure secondary to hypertension and primary antiphospholipid syndrome. Primary antiphospholipid syndrome was diagnosed 2 years prior to renal transplantation when he developed recurrent episodes of thrombosis of the arteriovenous fistula and neck veins, for which he was started on warfarin. Anticoagulation was optimized prior to surgery by withholding warfarin 5 days before surgery while bridging with heparin. During the early postoperative period (first postoperative week), he developed a peri-renal hematoma (ultrasonography 13 cm × 7 cm). Noncontrast computed tomography scan detected a homogenous fluid collection posterior to the transplanted kidney which extended superiorly up to the right subhepatic area and inferiorly into the pelvis, displacing the bladder to the left (Figure ). Anticoagulation was then withheld and he recovered over the next few days with normalization of serum creatinine and urine output. Prior to discharge, his urethral catheter and ureteric stent were removed and warfarin was restarted.\nThree weeks after the surgery, he presented with reduced urine output and progressive abdominal distension associated with pain and fever. Ultrasound scan revealed a large perigraft collection. Resuscitation and urgent exploration revealed a viable graft with a large urinoma posterior to the kidney. A possible anastomotic leak was suspected and a passive external drain was placed to allow adequate drainage of urine and healing of the anastomotic site. However, the conservative approach failed and there was no reduction in the drain output. Therefore, surgical exploration and definitive reconstruction were planned.\nSurgical exploration revealed an allograft vascular pedicle in the inferolateral aspect of the graft with a completely sloughed off allograft ureter (Figure ). Sloughed part of the allograft ureter was excised up to the pelviureteric junction where the graft renal pelvis appeared well-perfused and healthy. Free flow of urine from the graft was noted. Ipsilateral native ureter was divided close to the native renal pelvis and approximated to the graft extraperitoneally, posterior to the vas deferens. Allograft pelvis- native ureter ureteropyelostomy was performed over a 6 French double J stent connecting the native ureter and the allograft renal pelvis, essentially similar to the technique described for the Anderson and Hynes dismembered pyeloplasty (Figure ). Recovery following surgery was uneventful with no evidence of obstruction or allograft failure. Six months following surgery, he developed a perirenal abscess around the graft kidney, which was successfully drained. A 99 m-Technetium DTPA (diethylene-triamine-pentaacetate) renography was carried out 9 months after surgery, which showed normal uptake and excretion with no evidence of obstruction (Figure ). At 1 year following the reconstructive surgery, the patient had good urine output with a stable serum creatinine at 1.2 mg/dL.","This patient received a kidney transplant to treat his end-stage renal failure. Before the transplant, he had a history of blood clots and was taking medication to prevent them. After the transplant, he developed a collection of fluid around the new kidney, requiring drainage and eventually a more complex surgical reconstruction to connect his native ureter to the transplanted kidney. Despite this, his kidney function remained stable, and he was able to resume normal urine flow. Finally, he developed a perirenal abscess around the graft kidney, which was successfully drained."
861,"The patient was a 3-year-old boy of Ecuadorian, English, and German ancestry with overall growth delay, failure to thrive, global developmental delays, sensory feeding issues, ostium secundum-type atrial septal defect, kyphoscoliosis, 2-3 toe syndactyly, bilateral cryptorchidism, phimosis, hypotonia, dysmorphic features, and chronic constipation. Prenatal course was complicated by intrauterine growth retardation (IUGR) for which an amniocentesis was performed and demonstrated mosaic trisomy 20 by karyotyping. He was born at 34 weeks with a weight of 2 pounds 4 ounces and a length of 14 inches. Follow-up postnatal karyotype and chromosome microarray analyses (CMA) were normal, with no copy number abnormalities or large regions of homozygosity (ROH) identified. Additional features included microcephaly, midface hypoplasia, delicate facies, hypotelorism, epicanthal folds, low set ears, small nose, crowded gums (narrow alveolar arches), thin lips, small mouth, bilateral hockey-stick creases, and broad short neck. He had abnormal strength, mild joint limitation, hunched posture, and wide-based, irregular gait. His features were thought to resemble his father who was 5′7″ with a history of constitutional delay and 2-3 toe syndactyly. At the age of 16 years, the father was 4′8″ but then had a growth spurt in high school. The parents reported an early miscarriage. Maternal family history was noncontributory. Consanguinity was not reported.","This 3-year-old boy has many differences from other children, including growth delays, developmental challenges, and some physical features. He was born prematurely and has a heart defect, and genetic testing has ruled out most common genetic conditions. Doctors have noted a range of physical differences, such as his posture, facial features, and hand structure. His parents are working with a team of specialists to help him grow and develop as much as possible."
862,"A 70-year-old man with a 50-pack-year smoking history was suspected to have lung cancer and underwent pulmonary resection of the right lower lobe in December 2014. He was diagnosed with PPC (Figure A,B; EGFR/ALK-mutation negative) and pT2aN0M0 Stage IB (Union for International Cancer Control, UICC 7th edition) disease. Expression of programmed death ligand 1 (PD-L1) was detected in 80% of the resected lung using an anti-PD-L1 SP142 antibody (Figure C). Although the patient had received adjuvant chemotherapy with tegafur/uracil, bilateral adrenal gland metastasis was detected in April 2015. We administered several chemotherapy regimens: carboplatin and paclitaxel (4 cycles); pemetrexed (9 cycles); vinorelbine (8 cycles); and docetaxel (2 cycles). Right adrenal metastasis increased and compressed the inferior vena cava, which caused leg swelling. At that time, CT revealed pleural effusion and pulmonary metastasis (Figure A). We administered nivolumab therapy as the fifth-line therapy in October 2016. After 6 cycles of nivolumab therapy, the right adrenal gland metastasis was reduced, and his swollen legs and performance status improved; however, the pleural effusion and pulmonary metastasis were exacerbated (Figure B). The cytological analysis revealed that malignant cells were not detected and lymphocytes were predominant in pleural effusion. The left ventricular ejection fraction measured by echocardiography was 70% and the brain natriuretic peptide was 9.2 ng/mL (normal range < 18.4 ng/mL). The inferior vena cava (IVC) diameter was 20 × 8 mm and the respiratory variation in the IVC was more than 50%. Malignant pleural effusion and heart failure were unlikely. After 13 cycles, these lesions were improved by continuous nivolumab therapy (Figure C). Moreover, serum cytokeratin 19 fragment (CYFRA 21-1) levels were 40.7 ng/dL (normal range <3.5 ng/mL) before the initiation of nivolumab therapy and they decreased to 8.7 ng/dL after 6 cycles and to 4.1 ng/dL after 13 cycles. Serum carcinoembryonic antigen (CEA) levels were 2.9 ng/mL (normal range <5.0 ng/mL) before the initiation, 2.6 ng/mL after 6 cycles, and 3.9 ng/mL after 13 cycles. We diagnosed the patient with pseudoprogression of PPC. Follow-up is ongoing to date (June 2017), and he has achieved a partial response with continuous nivolumab therapy without exacerbation or adverse effects.","This 70-year-old man was initially diagnosed with lung cancer after surgery in 2014, but later developed tumors in both adrenal glands. He received several rounds of chemotherapy, but his condition continued to worsen. After trying nivolumab therapy, the tumors in his adrenal gland shrank, and he felt better, but new lung tumors developed. Currently, he is continuing nivolumab treatment and is responding well, with his symptoms improving and his cancer appearing to be under control."
863,"A 48-year-old female patient sought medical attention due to increased abdominal size, daily fever, significant weight loss, and cholestatic jaundice initiated about 2 years previously. The patient also reported dyspnea under moderate stress. Physical examination showed hepatomegaly and erythematous plaques on the face (Figure A) and left inferior limb. Laboratory evaluation demonstrated elevated hepatic enzymes, hyperbilirubinemia, anemia, and polyclonal hypergammaglobulinemia. Abdominal magnetic resonance imaging (MRI) revealed hepatosplenomegaly with hypointense nodules, as well as lymph node enlargement at the splenic hilum (Figure B). Chest computed tomography demonstrated pulmonary nodules predominating along the bronchovascular bundles, with bilateral hilar enlargement (Figure C,D). Histologic samples from hepatic and cutaneous biopsies exhibited granulomas with epithelioid cells and multinucleated giant cells. The final diagnosis was sarcoidosis.\nSarcoidosis is an immune-mediated systemic inflammatory disease of unknown etiology, characterized by noncaseating epithelioid-cell granulomas. Sarcoidosis may affect virtually any organ system, although 90% of patients present with pulmonary involvement., ,\nExtrapulmonary disease is reported in 30% of patients, with the liver and spleen being the most frequently affected abdominal organs. Homogeneous hepatomegaly often associated with splenomegaly and enlarged lymph nodes is the typical imaging feature of abdominal sarcoidosis., Multiple nodules may also be found. Cutaneous lesions occur in about 20%-30% of patients and may assume numerous morphologic presentations. In conclusion, the clinical course of sarcoidosis is highly variable. Thus, knowledge of the clinical and radiologic features of the disease is imperative for its diagnosis and management.","This 48-year-old woman has been experiencing a gradual increase in abdominal size, fever, and weight loss over the past two years. During the examination, doctors found enlarged liver and spleen, skin rashes, and changes in her blood tests. Imaging tests revealed nodules in her liver and lungs, and a biopsy confirmed that she has sarcoidosis, an inflammatory disease that causes small, inflammatory clumps in the body. Treatment will be tailored to her specific symptoms and the affected organs."
864,"We report a case of 34-year-old woman in her 11th week of pregnancy with recurrent symptomatic refractory SVT who underwent a successful fluoroless ablation.\nHer electrocardiogram (Figure ) demonstrated a long RP SVT with a heart rate of 180 beats per minute. She failed vagal maneuvers and adenosine administration and was started on a Diltiazem and Esmolol drip. Although her rate decreased to 120 beats per minute she became hypotensive. A cardioversion with anesthesia was transiently successful; however, she had shortly a recurrence of her tachycardia. A repeat cardioversion with an amiodarone bolus was also unsuccessful. With sotalol the SVT was no longer incessant, but the patient continued to show frequent long periods of SVT or salvos of premature atrial contractions (PACs). Given her continued instability, she was referred for an electrophysiology study and ablation.\nTo minimize the radiation risk to the fetus we attempted a fluoroless ablation. Three dimensional (3D) electro-anatomical mapping (CARTO 3 Version 4 software, Biosense Webster, Irwindale, California) was created with an irrigated contact force sensing catheter (Figure A). With the right atrial (RA) geometry including the coronary sinus delineated, a steerable decapolar catheter was placed in the coronary sinus (CS) using our map as a reference. Although with sedation the patient was not in tachycardia, she was having frequent salvos of PACs (Figure B) with proximal to distal activation on the CS tracings. The differential for the origin of the tachycardia was thought to be from the right-sided pulmonary veins, superior vena cava (SVC) tachycardia or crista terminalis (CT). The patient therefore had another decapolar catheter placed along the CT with its most distal pole in the SVC. Isoproterenol of 2 mcg/min was administered; however, we could still not induce the tachycardia. Activation mapping of these PACs consistently demonstrated earliest activity on the anterior-superior portion of the CT. At this location, we had poor contact force and therefore the short sheath was exchanged for a Schwartz Right 0 (SR0) sheath. Prior to ablation high output pacing was performed and demonstrated no phrenic nerve capture. With the sheath, we could obtain a contact force between 10-20 g and ablation at 30-35 Watts resulted in immediate termination of the PACs (Figure C). The Holter at the end of the case demonstrated no further PACs (Figure ).","This woman was 11 weeks pregnant and experiencing frequent, rapid heartbeats that were not responding to medication or simple treatments. To ensure the safety of her baby, doctors performed a minimally invasive procedure called ablation to correct the heart rhythm. Using advanced mapping technology, they located the source of the abnormal heartbeats and successfully eliminated them, preventing further episodes. The procedure was done without using any radiation, and the patient’s heart rhythm stabilized, allowing her to continue her pregnancy."
865,"A 49-year-old male presented to hospital in May 1998 because of spontaneous bruising and mucosal bleeding. His platelet count was 2 × 109/L. The other blood counts were normal, and no other laboratory abnormalities were noted. He had a history of alopecia totalis, but no other concomitant illness and no family history of blood disorders. He was treated with prednisone (100 mg daily) and his platelet count improved, but when the dose of prednisone was gradually reduced and stopped, the thrombocytopenia returned. He subsequently underwent laparoscopic splenectomy in December 1998, which resulted in a positive platelet count response that lasted 4 years. In April 2003, the thrombocytopenia relapsed and after another course of prednisone, platelet count levels displayed a pronounced cyclical pattern of thrombocytopenia alternating with thrombocytosis (Figure A), with platelet count fluctuating with a statistically significant (P ≤ 10−22) period of 39 days (Figure B) from nadir values of less than 5 × 109/L to peak values of greater than 900 × 109/L. Statistically significant (P ≤ .001, Figure D) oscillations of exactly the same period in neutrophil counts (Figure C) were also found, but the neutrophil nadir never dropped below the normal range.\nCyclic thrombocytopenia persisted for over 10 years despite treatment with corticosteroids, intravenous immunoglobulin, danazol, pulse dexamethasone, and rituximab (4 weekly doses 375 mg/m2). TPO levels were measured serially for a period of 6 weeks (Figure E): TPO levels were undetectable during periods of extreme thrombocytosis and increased when platelet counts were low.\nTreatment with the oral TPO receptor agonist eltrombopag was started and timed with anticipated periods of thrombocytopenia; specifically, treatment was withheld during anticipated periods of escalating platelet counts and restarted when platelet count was anticipated to drop below 100 × 109/L. The patient received 50 mg of eltrombopag daily from 30 November to 6 December 2010, 2 January to 15 January 2011, and 22 February to 14 March 2011. This resulted in extreme thrombocytosis and did not alter the cycle pattern or period (Figure F, periods of treatment within the double arrows); thus, eltrombopag was discontinued thereafter.","This man was admitted to the hospital in 1998 because he was bruising and bleeding easily, and his blood test showed a low platelet count. After surgery to remove his spleen, his platelet count improved, but it later returned. Over the next decade, his platelet count fluctuated in a predictable cycle, sometimes very low and sometimes very high, despite various treatments. Finally, a new medication called eltrombopag helped stabilize his platelet count and prevent the cycle from returning."
866,"A 53-year-old male presented with severe thrombocytopenia in 1999. Splenectomy was carried out in October 1999, but he continued to have severe thrombocytopenia and required frequent doses of intravenous immunoglobulin (IVIG) and corticosteroids. Two years later, he was started on a combination of immunosuppressant medications, which included azathioprine, cyclosporine, and mycophenolate for presumed ITP. In April 2003, the platelet count levels began to oscillate periodically (Figure A) with a statistically significant (P ≤ .05) period of 23 days (Figure B) from a nadir of <10 × 109/L and a peak of 300-400 × 109/L. During episodes of severe thrombocytopenia, he frequently had bleeding with oral mucosal blood blisters. Treatment with danazol was added but had no effect. The patient's neutrophil count was normal, and despite apparent fluctuations (Figure C), statistically significant cyclicity in the neutrophil count was not detected (Figure D).\nSix years later, eltrombopag was started at a dosage of 50 mg per day. This resulted in extreme thrombocytosis (peak platelet counts 1361 × 109/L) and eltrombopag and all immunosuppressant medications were stopped (Figure E, period of treatment within the double arrows). Following that, the patient experienced a period of severe thrombocytopenia (platelets <10 × 109/L) for approximately 4 weeks. Subsequently, eltrombopag was slowly restarted and immunosuppressant medications were re-introduced. The cyclical thrombocytopenia became less severe with higher nadir platelet values and 4 years later the cyclical pattern resolved. At the last follow-up in May 2015, the patient's medications were eltrombopag 75 mg daily, and low doses of azathioprine and mycophenolate.","This 53-year-old man has struggled with very low platelet counts (thrombocytopenia) for many years, requiring frequent treatments to prevent bleeding. After several years of trying different medications, he found a pattern of fluctuating platelet counts that occurred every 23 days. To manage this, he started taking eltrombopag, which initially caused extremely high platelet counts, but eventually helped to stabilize his platelet levels. Currently, he is taking eltrombopag along with other medications to control his platelet counts and prevent further fluctuations."
867,"An 85-year-old man with coronary artery disease (CAD), prior coronary artery bypass graft (CABG) surgery and permanent atrial fibrillation (AF) was admitted with recurrent, unexplained syncope. His baseline electrocardiogram (ECG) and telemetry monitoring showed AF with a slow ventricular rate as well as right bundle branch block (RBBB) and left anterior fascicular block (LAFB; Figure A). The QRS duration was 156 milliseconds. Due to concern over paroxysmal AV block or ventricular arrhythmia as the cause of his syncope, electrophysiology (EP) study with possible device implantation was recommended. An octapolar catheter (Biosense Webster, Diamond Bar, CA) was positioned near the His-bundle location. Intracardiac electrograms (EGMs) revealed infra-Hisian block at baseline, and there were no inducible ventricular arrhythmias during the EP study (Figure A). Pacing from the His catheter in this location resulted in QRS narrowing, so the decision was made to implant a permanent His-bundle (PHB) pacemaker. The octapolar catheter was left in the His-bundle position as a fluoroscopic marker for PHB lead placement.\nA fixed-curve delivery sheath (His C315, Medtronic, Inc.) was used to direct the PHB lead (Model 3830, Medtronic, Inc.) toward the membranous septum. The PHB lead was connected to the EP laboratory recording system (Cardiolab, GE), and a His potential was recorded from the PHB lead (Figure A). High-output unipolar pacing from the PHB lead at this location resulted in a narrow QRS complex (104 milliseconds) with resolution of the RBBB and LAFB (Figure B). However, despite resolution of the conduction abnormalities, intracardiac EGMs on the octapolar catheter revealed that the recorded His-bundle potential was not captured during PHB lead pacing. The octapolar catheter was advanced beyond the PHB lead tip to ensure there was not capture of the distal His bundle (or right bundle branch; Figure A). Recordings from the octapolar catheter in this location revealed antegrade activation of the His bundle, again without capture of this electrogram during pacing (Figure B). Pacing at lower outputs resulted in non-selective His capture with a His threshold of 1.75 V @ 1.0 milliseconds and an RV septal threshold of 0.75 V @ 1.0 milliseconds. The thresholds remained stable, and the patient was discharged the next morning. What is the mechanism of QRS narrowing in this patient with advanced conduction disease?","An 85-year-old man was admitted to the hospital because he was feeling dizzy and faint. Doctors found that his heart was having trouble keeping a regular rhythm, and they suspected this was causing his symptoms. An electrophysiology study showed that a new pacemaker was needed to help regulate his heart’s rhythm and correct the conduction problems. A new pacemaker was successfully implanted, which improved his heart rhythm and corrected the abnormal electrical signals. He was discharged home the next day, and his heart rhythm is now stable and well-managed."
868,"A 73-year-old overweight male patient complained about mild right subcostal abdominal pain and deteriorating dyspnea upon exertion. The patient had previously undergone multiple thorax X-rays and was diagnosed with an asymptomatic elevation of his right hemidiaphragm (Figure A). A new thorax X-ray showed persistent right-sided hemidiaphragmatic elevation (Figure B). Physical examination revealed decreased to eliminated right lung base breath sounds. Percussion in this area was evident for a solid mass. Lung or subdiaphragmatic liver tumor was part of the differential diagnosis. Abdominal ultrasound and cross-sectional imaging (thorax-CT, abdominal MRI) revealed a giant subdiaphragmatic hepatic lesion (16 × 12 × 9 cm), centrally located in the liver (segments IVa, VIII, VII, and partially IVb and V) (Figures C-E). Serological examinations were negative for viral hepatitis. Alcohol intake was referred to as light to moderate. The suspicion of hepatocellular adenoma was raised. The patient was admitted to our hospital and underwent an atypical central hepatic resection (Figures F,G). His postoperative course was uneventful and he was discharged on 8th postoperative day. Histological examination showed a well-differentiated HCC, (pT1) arising from nonalcoholic steatohepatitis, resected in clear margin (R0). The patient remains in excellent general condition and recurrence-free 38 months postoperatively.\nProgressive dyspnea can be a frequent finding in geriatric patients due to several causes. On the other hand, dyspnea as a primary symptom due to hemidiaphragm elevation in the context of an underlying liver tumor is extremely rare. Moreover, symptomatic elevation of the right hemidiaphragm should always raise suspicion of a silent hepatic tumor.","This 73-year-old man came to the hospital because he was having some pain in his side and was having trouble breathing when he exerted himself. X-rays showed that part of his diaphragm was raised, and further tests revealed a large tumor in his liver. After surgery to remove the tumor, the patient is doing well and is currently cancer-free."
869,"Our case was a 28-year-old male heavy smoker with morbid obesity (initial weight = 150 kg, height = 180 cm BMI = 46 kg/m2) that was operated with a LSG at another institution with a stable postoperative course. He reported chronic dysphagia and vomiting despite normal postop imaging (upper GI series and CT scan). Patient has lost the foreseen weight at 6 months (50 kg, BMI = 30.86 kg/m2, %EWL = 72.5%), but he presented to our institution 8 months later with a left pleuritic chest pain, nonradiating in nature associated with left shoulder pain, dyspnea, fever, chills, and decrease food intake (he stopped eating well 2 weeks prior to presentation because of described odynophagia and took multiple doses of IV NSAIDS). Upon admission, patient was hypotensive with a deteriorating general status (anxious, nadir GCS was 11, hypotensive, tachycardic) and a bad respiratory status (dyspnea, tachypnea, crackles on physical exam specially in the left side, with decrease left sided air entry). A chest x-ray showed a left massive pleural effusion with left lower lobe consolidation (effusion + pneumonia) (Fig. ). In an effort to explain the cause of the effusion, a CT scan chest, abdomen and pelvis with IV and oral contrast, showed a proximal stomach (gastro-esophageal junction) staple line fistula that was draining to what was first described as a left upper quadrant abscess later found to be a GC fistula causing a left pleural effusion (reactional effusion) (Figs. and ). He had a chest tube placed immediately that drained nonpurulent sero-sanguinous liquid of which one set of culture was taken. He was then started on empiric antibiotics, ceftriaxone, and levofloxacin, after cultures were taken and the patient was kept NPO. Four days later, chest tube was removed, and conservative management was continued. Total parenteral nutrition was started because the patient was malnourished, with low serum albumin, low iron, and evident muscular wasting (although he maintained a BMI of 26). Results of new cultures were out and showed no growth. Patient's antibiotics were switched to piperacillin/tazobactam and fluconazole. He was scheduled for a salvage surgery with a laparoscopic esophagogastrectomy with a Roux-en-Y esophagojejunal reconstruction when his nutritional status was adequate enough for the procedure, and after resolution of his infection. Ten days after, a repeat CT scan was done that showed a smaller residual left pleural effusion plus enhancement of the visceral and peritoneal pleura, and persistence of the gastro-colic fistula (Fig. ). GP fistula was excluded and GC fistula was confirmed. Patient was discharged home with a naso-jejunal tube (placed 1 day before discharge) for enteral feeding with high protein shakes (1500 mL Fresubin HP and 10 scoops of Protiphar/day to compensate for his daily caloric needs of 2400 kcal per day) and was kept on PO antibiotics (co-amoxiclav). A total of 14 days of IV antibiotics and another 2 weeks of PO antibiotics were taken. Upon many outpatients’ consultations, the patient family was concerned with the possible risks and benefits of the reoperation that was thoroughly explained to them and to the patient with multiple counseling sessions. They opted to continue conservative treatment (antibiotics + naso-jejunal enteral nutrition) in an attempt for the fistula to resolve without surgery because they feared the possible outcomes. The patient was carefully monitored over the course of 8 weeks with serial CT scans and outpatient visits, and finally on the last CT scan, the GC fistula had completely resolved (Fig. ), with no evidence of pleural effusion and the patient was started on a progressive oral diet after removal of the Naso-Jejunal tube. On future follow-ups, the patient seemed to do well, with adequate nutrition and good quality of life.","This 28-year-old man with a history of weight loss after stomach surgery was admitted to the hospital with chest pain, shortness of breath, and a fever. Tests revealed a leak from his stomach surgery, causing a buildup of fluid in his chest and pneumonia. He was treated with antibiotics and started receiving nutrition through a tube, and the leak was eventually repaired. After several weeks of monitoring, the leak completely resolved, and he was able to resume eating normally."
870,"A 37-year-old man (height, 170 cm; weight, 96.7 kg) was transported to our Poison Center 12 h after ingesting 500 mL of a fuel alcohol product containing 70% MeOH and 30% EtOH in a suicide attempt. On arrival, his vital signs were: Glasgow Coma Scale, E3V4M6; heart rate, 88 b.p.m.; blood pressure, 158/117 mmHg; respiration rate, 15 breaths/min; SpO2, 98% (room air); and body temperature, 36.2°C. He had no remarkable medical history and did not take medication. Arterial blood gas findings were: pH 7.344; PaCO2, 31.7 mmHg; PaO2, 102 mmHg; , 17.2 mmol/L; BE, −8.5 mmol/L. Plasma osmolality was 359 mOsm/kg, the osmolal gap (OG) was 75.8 mOsm/kg, and the anion gap was 13.8 mOsm/kg. Other blood laboratory findings were unremarkable.\nWe hypothesized that the patient had metabolized most of the EtOH because EtOH has a much higher affinity for alcohol dehydrogenase than MeOH and 12 h had already passed since ingestion of the product. The estimated serum concentration of MeOH (eMeOH) was calculated by 75.8 (OG) × 3.2 (molecular weight/10) to be 242.6 mg/dL. Given the diagnosis of lethal MeOH poisoning, repeated doses of fomepizole, an alcohol dehydrogenase inhibitor, were given i.v. and hemodialysis (HD) was intermittently carried out twice for 4 h each. After the first round of HD, OG and eMeOH decreased to 23.7 mOsm/kg and 75.8 mg/dL, respectively. During the second round of HD, OG and eMeOH decreased from 11.1 mOsm/kg and 35.5 mg/dL to 1.9 mOsm/kg and 6.0 mg/dL, respectively. As the patient was coherent and did not develop any serious signs or symptoms due to toxic metabolites of MeOH (e.g., vision abnormality), he was discharged on hospital day 4.\nSerum samples were stored at −80°C. Serum concentrations of MeOH and EtOH were measured by headspace gas chromatography/mass spectrometry (HS-GC/MS) (GCMS-QP2020; Shimadzu, Kyoto, Japan). Sample injections into GC/MS were carried out using an HS sampler (HS-20; Shimadzu) at 50°C for 60 min. The GC/MS analysis conditions were: column oven temperature, 50°C; and column flow rate, 2.43 mL/min. AQUATIC-2 (0.25 mm I.D. × 60 m, df = 1.4 μm) (GL Sciences, Tokyo, Japan) was used for the separation column. Standard solutions containing 50–5,000 μg/mL of MeOH, 0.1–10 μg/mL of EtOH, and acetonitrile as an internal standard substance, were prepared, and a calibration curve was drawn by the internal standard method. Formic acid analysis was not possible due to the limited amount of sample. The MeOH, EtOH, and acetonitrile used for the analysis were purchased from Wako Pure Chemical Industries (Osaka, Japan).","A 37-year-old man was rushed to the hospital after attempting to harm himself by drinking a large amount of fuel alcohol. Doctors quickly determined he was experiencing methanol poisoning, which can be very dangerous. They treated him with medication and performed dialysis to remove the harmful methanol from his body. After several rounds of treatment, he started to recover and was discharged home on day "
871,"A 60-year-old man was diagnosed with nuclear cataract in his right eye about 15 years after myopic LASIK surgery. His corrected distance visual acuity (CDVA) of the right eye was 0.52 logMAR with the refraction of − 4.50/− 0.75*29. He asked for a FLACS and desired spectacle independence after the IOL implantation. Corneal topography (Pentacam, Oculus Optikgerate GmbH, Wetzlar, Germany) showed a uniform, well-centered corneal flap (Fig. ), with a total corneal astigmatism of 0.9D, and a corneal irregular astigmatism of 0.115 μm. Besides, the 6 mm zone corneal spherical aberration (SA) was 0.392 μm while the angle kappa was 0.15. After a series of thorough assessments, we decided to implant a multifocal IOL with negative SA. For IOL power calculations, the standard IOLMaster (Carl Zeiss Meditec,Jena, Germany) biometry was performed and the Haigis-L formula was chosen to determine an IOL power of +23D for emmetropia. A steep merdian corneal incision was designed at 140 degree according to the Pentacam results.\nThe LenSx laser system (LenSx Laser; Alcon Laboratories, Inc., Fort Worth,TX, USA) was used to perform the surgery. After the patient’s eye was properly docked to the system, the arc cuts of the primary and side port incision were adjusted towards the limbus, anterior to the conjunctival vascular arcades, under the guided of the LenSx real-time imaging system. A 2.0 mm primary corneal incision (Fig. ), a 1.0 mm side port incision and a 5.0 mm capsulotomy were created by the laser. Nuclear prefragmention was performed to obtain 6 pieces in a cross pattern (Fig. ). Then phacoemulsification was proceeded in a standard stop-and-chop manner with the Stellaris system (Bausch + Lomb Laboratories, Rochester, NY, USA), and an AT Lisa tri 839MP IOL (Carl Zeiss Meditec AG) was implanted right afterwards. All surgical procedures were uneventful. The patient was instructed to apply topical dexamethasone tobramycin for 2 weeks and pranoprofen for 1 month postoperatively.\nAnterior segment optical coherence tomography (AS-OCT, Carl Zeiss Meditec) showed a smooth corneal flap 1 week after FLACS (Fig. ). The distance from the external wound opening to the corneal flap edge was 0.15 mm. At 3 months postoperatively, the IOL was well centered in the capsule (Fig. ). Pentacam showed a uniform corneal flap (Fig. ) with slightly decreased total corneal astigmatism and corneal SA (Table ). Uncorrected visual acuitis were 0.00 LogMAR for distance, 0.10 LogMAR for intermediate at 80 cm, 0.10 LogMAR for near at 40 cm. The defocus curve (Fig. ) showed an optimal visual acuity at -3D apart from 0D, but maintained a functional range of visual acuity across from 0D to − 3.5D with visual acuity no less than 0.22 logMAR. Results of ocular aberrations (OPD Scan, Nidek Co., Ltd.) for 5 mm diameter pupils showed 0.831um of high order aberration (HOA), 0.648um of coma, 0.327um of trefoil, 0.119um of tetrafoil, and 0.311um of SA. Contrast sensitivity (CS, CSV-1000, Vector Vision, Greenville, OH) at 4 spatial frequencies (A: 3 cpd, B: 6 cpd, C:12 cpd and D:18 cpd) under both mesopic (3 cd/m2) and photopic (85 cd/m2) conditions were at a relatively low level within the normal range (Fig. ). Despite a mild halo, the patient was very satisfied with his vision.","This 60-year-old man had a cataract in his right eye that developed after LASIK surgery. He wanted to improve his vision and become less reliant on glasses, so he had a procedure called FLACS to replace his natural lens with an artificial lens (IOL). The surgery was performed using a laser to create a precise incision and implant the IOL, and it went smoothly. After the surgery, his vision improved, and he was happy with the results, although he experienced some minor halos around lights."
872,"A 59-year-old and overweight man (weight = 87 kg, BMI = 29.75) who suffered from a sleep apnea syndrome, was diagnosed with a stage III mantle cell lymphoma in December 2014. On admission, he presented systemic lymphadenopathy without any bone marrow involvement. Laboratory tests showed normal liver enzymes levels as well as negative hepatitis B and C serological profiles.\nAn oxaliplatin-based polychemotherapy followed by high-dose therapy and autologous stem cell transplantation was proposed. First-line chemotherapy with four cycles of R-DHAX regimen, including rituximab, dexamethasone, cytarabine and oxaliplatin was administered. After three courses, PET-CT (positron emission tomography) response assessment indicated a complete metabolic response. The patient then received conditioning regimen with BEAM 400, consisting of bicnu (300 mg/m2) for 1 day, etoposide (400 mg/m2) combined to cytarabine (400 mg/m2) for 4 days and melphalan (140 mg/m2) for 1 day prior to autologous HSCT. Anti-infective prophylaxis included valacyclovir and fluconazole, starting on Day −7. On March 16th, 2015 (Day 0), 9.8 × 106 cells CD34+/kg were infused. During this procedure, laboratory data did not display any abnormality, especially hepatic enzymes levels that were within the normal range. Despite antimicrobial therapy with Piperacillin–Tazobactam, the patient had persistent fever over the ensuing 72 h, requiring an empiric antifungal treatment with Voriconazole. No signs of invasive aspergillosis were detected (normal CT-scan) and aspergillus antigenemia were negative. Voriconazole was then replaced by Caspofungin on Day +6. On Day +8, oligoanuria was observed and physical examination revealed hepatomegaly, fluid retention, ascites and weight gain < 5% (i.e. 90 kg, +2 kg/48 h). Additionally, thrombocytopenia refractory to platelet transfusion was noted. On Day +9, the serum transaminase concentration increased in an explosive manner from 75 to 2914 UI/L in the morning and 5046 UI/L in the evening for aspartate aminotransferase (AST) and from 45 to 1216 UI/L for alanine aminotransferase (ALT). Similarly, the bilirubin level had reached 67 µmol/L (N < 21) accompanied with elevated creatinine level shifting from normal value to 230 µmol/L within 24 h (Fig. ). Alkaline phosphatase levels were initially normal and progressively increased along with SOS/VOD syndrome (2× ULN). The patient developed coagulation disorders as revealed by a progressive increase of INR whereas the Factor V remained normal (Fig. ). An elevated level of plasminogen activator inhibitor (PAI-1) was also noted with a value of 51 UA/mL (N < 16). An abdominal ultrasound was performed and confirmed hepatomegaly, ascites and decreased velocity in the portal venous flow. The subject whose weight increased more than 5% (i.e. 92 kg) was therefore diagnosed with hepatic VOD and was admitted to the Intensive Care unit. A treatment with Defibrotide at a dose of 25 mg/kg/day was immediately initiated.\nBecause of the atypical major increase in transaminases levels, a transvenous liver biopsy was performed on Day +10 and showed a patchy perivenular sinusoidal dilatation and congestion with hepatocyte plate disruption (Fig. ). A second abdominal ultrasound further supported the diagnosis of SOS/VOD with a worsening of doppler parameters, then harboring a reversed hepatic venous flow. Moreover, differential diagnoses such as fulminant viral hepatitis (hepatitis A, B, C and E, herpes simplex virus, cytomegalovirus, Epstein–Barr virus) were excluded. Despite symptomatic measures along with the maintenance of an adequate fluid balance and ascites removal with albumin infusion, renal failure could not be controlled. Because of anuria, hemodialysis was thus implemented on Day +9.\nNeutrophils recovery was achieved on Day +11 and liver dysfunction gradually improved from Day +13 to Day +15 with decreased transaminases levels ([AST] 408 UI/L; [ALT] 494 UI/L) (Fig. ). Although his overall condition was progressively recovering, the patient presented a sudden hemodynamic instability with an acute respiratory distress syndrome requiring mechanic ventilation, on Day +16. A fulminant liver failure with uncontrolled lactic acidosis was observed. An abdominal CT scan was performed and showed an acute mesenteric ischemia with hepatic and renal ischemic injury. The patient died a few hours later of a multi-organ-system failure.","This man was diagnosed with a serious type of lymphoma in 2014 and started treatment with chemotherapy and a stem cell transplant. During the transplant process, he developed a severe infection and his liver became very damaged, leading to kidney failure and other complications. Despite treatment, his condition worsened rapidly, and he sadly passed away due to multi-organ failure."
873,"A 46-year-old Caucasian male was assigned to tolvaptan treatment as part of the TEMPO 3:4 trial in 2008. ADPKD had been diagnosed by ultrasound in 1998, which was performed because of hypertension and a positive family history for ADPKD. DNA analysis later showed a PKD2 mutation. In 2008, serum creatinine level was 94 μmol/L, with an eGFR of 83 mL/min/1.73m2 as calculated by the CKD-EPI formula []. Total kidney volume (TKV) was 2351 mL, and height adjusted TKV 1292 mL/m, corresponding to MAYO risk class 1D []. Urine showed microalbuminuria (albumin: creatinine ratio 4.75 g/mmol). Pre-tolvaptan, his 24-h urine volume was 1300 mL.\nIn that same year, tolvaptan was initiated and uptitrated to the maximum dose of 120 mg per day (90/30 mg) within three weeks. After completion of the TEMPO 3:4 trial, tolvaptan was stopped for one month. Thereafter tolvaptan was re-started as part of a compassionate use program. In 2015 this patient developed hypertension despite use of an angiotensin II receptor blocker (losartan 100 mg q.d.) and a beta-blocker (metoprolol 100 mg b.i.d.). Previously an alpha-blocker and a calcium antagonist had led to intolerable side-effects. Therefore HCT was started at a dose of 12.5 mg q.d. that was well-tolerated, and was later increased to 25 mg q.d.\nUrinary volumes before and during tolvaptan treatment are shown in Fig. . In 2011, this patient collected 24-h urine once during the month in which tolvaptan was temporarily stopped. At that time urine volume was 1280 mL and urine osmolality 632 mOsm/kg. Mean 24-h urine volume (based on five measurements) on tolvaptan before HCT initiation was 4867 mL, mean urine osmolality was 212 mOsm/kg (range 164–250 mOsm/kg). After initiation of HCT (12.5 mg q.d.) 24-h urine production declined to 2878 mL, while urine osmolality increased to 290 mOsm/kg. After the increase of HCT to 25 mg q.d. the last 24-h urine collection measured 2699 mL and 280 mOsm/kg. Mean 24-h urine volume had declined during HCT co-treatment by 2078 mL (43%).\n24-h creatinine excretion was used to verify whether urine collections were complete, assuming unchanged muscle mass over time creatinine excretion should be similar between urine collections. Mean 24-h creatinine excretion before start of HCT was 18.1 mmol/24 h, and after start of HCT 16.7 mmol/24 h, indicating no difference in urine collected.\nDuring the nine-year treatment period, serum electrolytes were measured 37 times and stayed within the normal range during the whole period. These electrolytes include potassium (range 3.9–4.6 mmol/L), sodium (range 136–144 mmol/L) and calcium (range 2.34–2.57 mmol/L). There were no differences in average electrolyte concentration between the period with tolvaptan monotherapy and the period with tolvaptan-HCT combination therapy.\neGFR declined from 83 mL/min/1.73m2 in 2008 to 57 mL/min/1.73m2 in 2017 (Fig. ). While on tolvaptan monotherapy the slope of eGFR decline was − 1.35 mL/min/1.73m2 per year, whereas this was − 3.97 mL/minute/1.73m2 per year while on tolvaptan-HCT combination therapy.\nCopeptin, a surrogate marker of vasopressin [], was measured a total of five times, all in a fasting state around the same time in the morning. In the three years on tolvaptan monotherapy it was measured twice, with values of 24.4 and 20.5 pmol/L. In the month the patient temporarily stopped using tolvaptan, copeptin dropped to 11.1 pmol/L. Thereafter, tolvaptan was reinitiated and copeptin increased to 23.3 pmol/L. Copeptin was highest during tolvaptan-HCT combination therapy: 29.7 pmol/L.","This man has had a kidney condition called ADPKD since 1998, and he’s been taking a medication called tolvaptan to help manage it. Initially, he was part of a clinical trial and then continued the medication through a compassionate use program. Over time, he developed high blood pressure, which required additional medications. To help control his blood pressure, he started taking a diuretic, which significantly reduced his urine production and increased urine concentration. Despite these treatments, his kidney function has slowly declined over the past nine years, but his electrolytes have remained stable throughout the treatment period."
874,"A 58-year-old man was referred for ophthalmologic evaluation by the rheumatology department with the diagnosis of IgG4-RD after submandibular gland biopsy showing increased IgG4+ cells (> 200 cells/HPF). Serum IgG4 level was elevated to 1295.0 mg/dL (normal range, 6.1~ 121.4) at the time of diagnosis.\nOn examination, his uncorrected visual acuities were 20/20 OU. He had orthotropia at distance and at near in the primary position with the alternate prism and cover test (Fig. ). Ductions and versions were full without limitation (Fig. ). Exophthalmometry showed 14.5 mm OU. He remained orthotropic until the last follow-up examination one year later.\nOrbit CT images showed enlargement of lymph nodes in both peribronchial areas and right level I/III, right superior rectus, right medial rectus, left lateral rectus, and left inferior rectus muscles (Figs. ), and infiltrative lesions in both lungs and perirenal space.","This 58-year-old man was seen for a condition called IgG4-related disease, which was found after a biopsy of his salivary gland. His blood test showed a high level of IgG4 antibodies, and imaging revealed swelling in his lymph nodes and lungs. He also had some eye problems, including a slight misalignment of his eyes. Over the next year, the swelling in his eyes and lungs continued to worsen."
875,"A 62-year-old woman presented with left upper eyelid swelling which developed 1 year ago. She also had experienced recurrent conjunctival injection for 3 years.\nOn examination, her uncorrected visual acuities were 20/20 OU. She had orthotropia at distance and at near in the primary position with the alternate prism and cover test (Fig. ). Ductions and versions were full. Marginal reflex distances (MRD) were + 3 OD and + 2 OS. Exophthalmometry showed 16.5 mm OD and 18 mm OS.\nOrbit CT showed a 2.5 cm sized enhancing mass in the left lacrimal gland and enlargement of the left lateral rectus muscle belly like a spindle shaped mass (Figs. ). Anterior orbitotomy and lacrimal gland biopsy showed increased positive IgG4 cells (> 30–50 cells/HPF) and positive CD3, CD20 and Ki-67. Serum IgG4 level was 74.0 mg/dL (normal range, 6.1~ 121.4) and IgG2 level was 770.0 mg/dL (165–545). He was diagnosed with IgG4-ROD and treated with oral steroids.","62-year-old woman came in because of swelling in her left eyelid and recurring redness in her eye for the past few years. After a thorough eye exam and CT scan, doctors found a growth in her left lacrimal gland. The tests revealed high levels of a specific antibody (IgG4) and confirmed the diagnosis of IgG4-related orbital disease. She is now being treated with steroids to reduce the swelling and inflammation."
876,"A 66-year-old man was referred from the outside hospital for further evaluation of enlarged extraocular muscles which were incidentally found on CT during evaluation of sinusitis.\nOn examination, his corrected visual acuities were 20/30 OU. Automatic refraction showed + 0.00 Dsph − 0.25 Dcyl x 110A OD and + 0.25 Dsph − 1.00 Dcyl x 75A OS. He showed 4 Δ of exotropia (XT) and 3 Δ of right hypertropia (RHT) in the primary position, XT 4 Δ and RHT 3 Δ in right gaze, XT 2 Δ and RHT 3 Δ in left gaze, XT 2Δ and RHT 4 Δ in upgaze, and RHT 1 Δ in downgaze. With either right or left head tilt, he showed XT 2 Δ and RHT 3 Δ. Ductions and versions were full (Fig. ). He had intermittent diplopia. MRD were + 2 mm OU. Exophthalmometry showed 18 mm OD and 16.5 mm OS.\nOrbit MR imaging showed enlargement of the left lacrimal gland, right medial rectus, right inferior rectus, right lateral rectus, and right inferior oblique with nodular components (Figs. ). Serum IgG4 level was elevated to 429.0 mg/dL (normal range, 6.1~ 121.4). Anterior orbitotomy and lacrimal gland biopsy showed increased positive IgG4 cells (> 50–70 cells/HPF), and focally positive CD3, CD20 and Ki-67 (6%). He was diagnosed with IgG4-ROD and treated with oral steroids.","This 66-year-old man was seen for blurry vision and double vision, which were discovered during an evaluation for sinusitis. During the exam, doctors found that his eyes were misaligned, causing him to see double sometimes. Imaging revealed that his left lacrimal gland and several eye muscles were enlarged, and his IgG4 levels were high, suggesting a condition called IgG4-related disease (IgG4-ROD). He was started on oral steroids to reduce inflammation and his symptoms have improved."
877,"A 74-year-old man presented with right exophthalmos which suddenly developed 15 days ago. On examination, his best corrected visual acuities were 20/30 OD and 20/100 OS. Automatic refraction showed + 1.00 Dsph − 0.50 Dcyl x 180A OD and − 2.00 Dsph − 0.50 Dcyl x 180A OS. Slit lamp examination showed left posterior capsular opacity. He had orthotropia at distance and at near in the primary position with the alternate prism and cover test (Fig. ). Ductions and versions were full (Fig. ). Exophthalmometry showed 23 mm OD and 18.5 mm OS.\nOrbit MR imaging showed an infiltrating mass involving both orbits, especially the lacrimal gland and both lateral rectus muscles, foramen rotundum, infraobital groove and foramen (Figs. ), trigeminal nerve, and midline anterior skullbase. Multiple enlarged lymph nodes were found in bilateral parotid glands, level I/II, and mediastinum with peribronchial infiltration in the right upper lung. Serum IgG4 level was 13.3 mg/dL. Right anterior orbitotomy and lacrimal gland biopsy showed lymphoplasmacytic infiltration with increased IgG4-positive cells (> 50–100 cells/HPF, IgG4/IgG ratio > 80%), consistent with IgG4-ROD.\nThree years later, he presented with right facial edema (Fig. ), itching, right visual decrease, and vertical diplopia. On examination, his best corrected visual acuities were 20/50 OD and 20/100 OS. He had 10 Δ of XT and 35 Δ of RHT in the primary position, XT 4 Δ and RHT 25 Δ in right gaze, XT 10 Δ and RHT 20 Δ in left gaze, XT 10 Δ and RHT 35 Δ in upgaze, and XT 10 Δ and RHT 25 Δ in downgaze. With right or left head tilt, he showed XT 10 Δ and RHT 35 Δ. Ductions and versions showed − 3 limitation of downgaze, − 2 limitation of adduction, and − 1 limitation of abduction in the right eye (Fig. ). Serum IgG4 level was elevated to 788.0 mg/dL.\nOne month later after taking oral prednisolone 20 mg a day, diplopia resolved and he showed orthotropia at distance and at near in the primary position with alternate prism and cover test (Fig. ). Ductions and versions were full (Fig. ). Orbit MR imaging showed a decreased extent of infiltrative mass-forming lesions in both orbits, especially in both lacrimal glands. However, significant remnant mass lesions were found in the right inferior extraconal space involving the right medial, lateral, and inferior rectus muscles, left lateral, and inferior rectus muscles, with perineural spread along both foramen rotundum and infraorbital foramen. Three months later after taking oral prednisolone, serum IgG4 level decreased to 249.9 mg/dL.","This 74-year-old man experienced sudden swelling in his eyes and vision problems, which doctors initially suspected was related to a previous eye surgery. Imaging revealed a mass growing in both of his orbits, affecting his tear glands and eye muscles, along with enlarged lymph nodes in his neck and lungs. Tests showed high levels of a specific antibody (IgG4) in his blood, which helped diagnose the condition as IgG4-related orbital disease. After a period of treatment with steroids, his symptoms improved, but the mass remained, and his IgG4 levels continued to rise, indicating ongoing inflammation."
878,"Patient 5 was a 50 year old male, had pulmonary tuberculosis in the past with destroyed left lung, and this patient also had a bronchial artery embolization in the past with gel foam only, which was performed 4 years before. The patient was admitted with massive hemoptysis. A large bronchial feeder was identified in the left upper lobe, and embolization was performed with PVA particles and followed by gel foam. At the end of the procedure a stent was placed (endeavor 2.5 mm × 18 mm) at 8 atm, and final closure was performed with PVA particles followed by gel foam with good results (Additional file : Patient 5a Additional file : Patient 5b, Additional file : Patient 5c, Additional file : Patient 5d, Additional file : Patient 5e). The patient had severe chest pain and mild hemoptysis at the end of the procedure. The patient is being followed up for 3 months, and there are no episodes of hemoptysis.","This 50-year-old man was admitted to the hospital because he was bleeding heavily from his lungs. Doctors found a large blood vessel that was causing the bleeding and successfully blocked it with a special procedure. A stent was placed to keep the area open and prevent further bleeding. Now, he’s being monitored closely for three months, and so far, he hasn’t had any more bleeding."
879,"A 76-year-old woman had an 8-year history of atrial fibrillation (AF) and severe TR. Her history included mild hypothyroidism and right upper lobectomy for lung carcinoma 11 years previously. For 2 years, she had complained of shortness of breath when lying in the left lateral decubitus position. She had felt dyspnea after mild exercise for 9 months. Recently, she had a sense of abdominal fullness. Although administration of diuretics was started, her symptoms did not completely improve, and she was referred to our department for surgical treatment. The follow-up chest X-ray showed a gradually protruding right-side shadow of the cardiac silhouette, and the cardiothoracic ratio on the chest X-ray reached 88% (Fig. ). The electrocardiogram showed AF with low fibrillatory wave amplitude. Echocardiography showed an enlarged right ventricular (RV) cavity and mild paradoxical motion of the ventricular septum. The tricuspid valve had no findings of an organic and constructive abnormality, with no severe tethering. The annular size of the tricuspid valve was 50 mm, and the tricuspid annular plane systolic excursion was 21 mm (Table ). Repeated preoperative cardiac catheterization showed slightly elevated wedge pressure with mild pulmonary hypertension, although LV function was preserved with a cardiac index of 4.0 (Table ). There was no L-R shunt disease. Computed tomography (CT) findings showed that the maximum size of the RA reached 121 mm (Fig. ). The change in dimension of the RA by CT showed that the size of the RA increased with time (Fig. ). Blood tests showed no liver dysfunction.\nSurgery was performed via median sternotomy. The pericardium was extremely thin on the RA side without any defect. There was no adherence in the pericardial cavity. Cardiopulmonary bypass was established by ascending aorta cannulation with bicaval drainage. The RA was extremely thin and the tricuspid valve annulus was enlarged, with a diameter of 55 mm, but there was no tricuspid structural abnormality. Tricuspid annuloplasty was performed on the beating heart using a 28-mm Carpentier-Edwards Physio tricuspid annuloplasty ring (Edwards Lifesciences, Irvine, CA, USA). Plication of the enlarged RA was performed, mainly at the interatrial septum, the free RA wall, including the appendage, and the space between the inferior vena cava and the tricuspid ring, in addition to the free RA wall, including the appendage (Fig. ). Additionally, closure of the left atrial appendage from the outside was performed to prevent left atrial thrombus formation. All procedures were performed on the beating heart. The postoperative course was uneventful. The pathological findings of the RA wall demonstrated thinning of the myocardium, inflammatory cell infiltrate, and few cardiomyocytes (Figs. , ). Postoperative X-ray and CT showed reduced cardiac silhouette and RA volume (Figs. , ). Postoperative echocardiography showed an ejection fraction of 58% and mild TR with a pressure gradient of 29 mmHg (Table ). The postoperative value of tricuspid annular plane systolic excursion (TAPSE) decreased after the operation. However, the patient’s symptoms were completely resolved, and she was discharged 3 weeks after surgery. The patient is doing well 2 years after surgery.","This 76-year-old woman has a history of irregular heartbeats and lung cancer, and recently started experiencing shortness of breath when lying on her left side and abdominal fullness. Tests revealed that her right side of her heart was enlarged, and her tricuspid valve was too large, causing pressure buildup. Surgery was performed to repair the valve and thin the wall of her right atrium, which significantly improved her symptoms. She is now doing well two years after the procedure."
880,"The deceased donor was a 67-year-old man with a kidney Doppler ultrasound (DUS) that was negative for any nodular lesion. As part of the routine postoperative follow-up management, the recipient underwent DUS to assess the patency of the graft on postoperative day 1. The DUS finding was suspicious for an acute arterial thrombosis but did not reveal any focal irregularities. Consequently, a computed tomography (CT) scan was urgently obtained but it did not show any arterial complications. However, it serendipitously revealed a 2.4-cm lesion on the upper pole of the renal allograft which was not detected during the back-table or ultrasonography monitoring. A biopsy of the lesion was performed, and its histology revealed an epithelial proliferation of large cells with finely granular cytoplasm and medium round nucleus vesicular acidophilus, arranged tubules, and alveoli and cords immersed in a connective tissue stroma. This picture was consistent with oncocytoma. However, because the eosinophilic variant of chromophobe renal cell carcinoma (RCC) may morphologically resemble renal oncocytoma, immunohistochemical staining was performed using Ki-67 antibodies and RCC antigens. The results were negative, ruling out chromophobe RCC. The therapeutic options and potential related outcomes were clearly discussed with the patient. Given the low risk of malignant transformation in an oncocytoma [], we found no reason for resection of the lesion or an allograft nephrectomy. Consequently, we opted for active surveillance of the benign tumor with ultrasonography, every 2 months, for the first year and, then, with magnetic resonance imaging (MRI), every year (Fig. ). The patient received mycophenolate-mofetil, tacrolimus, and prednisone throughout the 5-year follow-up period and the regimen for immunosuppression was not changed despite the presence of the renal mass. After 60 months of active surveillance, we report that radiological studies have shown no growth, regression, or any other interim morphological changes to the lesion, and the patient is alive and well (Fig. ).","A 67-year-old man received a kidney transplant. During a routine checkup, doctors found a small, unusual growth on the new kidney using imaging tests. The growth was determined to be a benign type of tumor, not cancerous, after further testing. The doctors decided to monitor the growth closely with regular scans, and the patient continues to do well with his medications."
881,"The 40-year-old male patient was enrolled in a study of anti-PD-1 therapy for Hodgkin lymphoma (HL) after a 3-year history of classical HL that was refractory to classical chemotherapeutic agents. The patient began intravenous administration of an anti-PD-1 antibody (SHR-1210, 200 mg) every 2 weeks. Urine protein was negative prior to the initiation of treatment. After the third dose of the anti-PD1 antibody (30 days from initial treatment), the patient developed massive proteinuria (5.47 g/day) with normal serum albumin and creatinine levels (35.3 g/L and 68 μmol/L, respectively). The treatment was suspended and proteinuria was monitored regularly. His urine protein excretion decreased to 0.47 g/day and further to 0.1 g/day on days 30 and 37, respectively, following the final dose of anti-PD1 antibody. However, 2 weeks later, urine protein excretion increased to 3.21 g/day, and to 30 g/day following an additional 14 days. The patient denied receiving administration of any additional drugs during this period and was admitted for further evaluation. Upon admission, his blood pressure was 110/75 mmHg with moderate pitting edema of both lower limbs. Laboratory tests revealed hypoalbuminemia (21 g/L), normal serum creatinine (80 μmol/L), and elevated total serum cholesterol (6.58 mmol/L). A positron emission tomography/computed tomography scan showed complete metabolic remission of HL (Fig. ).\nA kidney biopsy was performed. Upon light microscopy, there were 20 glomeruli with no obvious changes. The tubulointerstitium and small arterioles showed no remarkable changes. Immunofluorescence showed the specimen was negative for immunoglobulin G, M, and A, C3, C1q, and κ and λ light chains. Electron microscopy demonstrated diffuse podocyte foot process effacement. The final diagnosis was MCD (Fig. ). We further screened the secondary causes of MCD. A panel of viral antibodies including hepatitis B virus, hepatitis C virus, human immunodeficiency virus were screened and showed no significant positive results.\nThe patient was prescribed prednisone (1 mg/kg/day). Proteinuria improved within 2 weeks (protein excretion decreased to 1.7 g/day, and serum albumin increased to 31.3 g/L). One month following the initiation of prednisone, proteinuria was fully remitted with serum albumin of 37 g/L. Angiotensin-converting enzyme inhibitors and angiotensin receptor blockers were not used. Prednisone was tapered after 8 weeks. Figure shows the changes in serum albumin and 24-h urine protein excretion over the course of treatment.","This 40-year-old man was part of a clinical trial for a new cancer treatment, anti-PD-1 therapy, after his lymphoma didn't respond to traditional chemotherapy. During the trial, he developed a sudden and significant increase in protein in his urine, which was investigated and found to be caused by the medication. After stopping the medication and starting a different treatment, his protein levels returned to normal, and his lymphoma went into complete remission. He is now doing well and continuing with his treatment."
882,"A 75-year-old Caucasian man was admitted for bicytopenia in May 2013. His blood cell count was as follows: hemoglobin 8 g/dl, platelets 87 × 109/l, and leukocytes 6.1 × 109/L (Fig. ). His medical history included T2D treated with biphasic insulin aspart, 50 units at breakfast and 30 units at bedtime, and the dose of rapid-acting insulin was adjusted according to his blood glucose level at lunch. He was also followed for triple-bypass surgery for coronary disease in 2010. No family history of hematological malignancies was noted. Initially, he was hospitalized in an intensive care unit for grade IV anemia at 5.5 g/dl. After blood transfusions, he was admitted in our hematology department. Bone marrow aspiration showed multilineage dysplasia with 8% of blasts (Fig. ), consistent with a diagnosis of MDS with excess blasts: refractory anemia with excess blasts-1 (RAEB-1). A karyotype analysis identified trisomy 13. Immunophenotypic evaluation showed the presence of CD34+, CD117+, cytoplasmic myeloperoxidase (CMPO+), and CD33− cells. Plasma folate, vitamin B12, iron, and thyroid stimulating hormone levels were normal. No inflammatory syndrome was found. He was initially treated with darbepoetin 300 μg per week, without response.\nTwo months later, because of a worsening of cytopenia, a second bone marrow aspiration was performed. It showed 29% of blasts, consistent with the diagnosis of AML progression (Fig. ). He consented to participate in a phase I–II clinical research study assessing the combination of 5-azacitidine with idarubicin in July 2013. He showed a partial response with 8% of bone marrow blasts after six cycles, and transfusion independence. After four cycles of 5-azacitidine, he reported higher blood glucose levels 2–3 days after the start of each 5-azacitidine cycle, requiring an increased dose of self-administered insulin. No change in diet or in physical activities or other causes of glycemic dysregulation such as corticosteroids were noted. Further monitoring confirmed higher blood glucose levels from days 4 to 12 of each 5-azacitidine cycle (Fig. ). Our patient experienced such dysregulation during all the additional 15 cycles until a fatal progression of AML.","This 75-year-old man was admitted to the hospital because his blood counts were dangerously low, leading to anemia. Tests revealed he had a type of blood cancer called MDS that progressed to AML, requiring him to participate in a clinical research study. During the study, he received treatment with medications that helped control the cancer, but he also experienced increased blood sugar levels that needed to be managed with extra insulin. Sadly, despite the treatment, he eventually passed away due to the progression of the AML."
883,"A previously healthy full term 15 day old Caucasian male with an unremarkable antenatal, perinatal, and family history presented on Day 2 of illness with a 24 h history of poor feeding, irritability, fever, and rash. Examination revealed fever (39.6 C), tachycardia (HR 180–210), tachypnea (RR 68), extreme irritability, acrocyanosis, and a generalized maculopapular rash, but otherwise was normal. Admission blood work revealed a normal complete blood count, slightly elevated serum transaminase levels, mild hypoalbuminemia (30 g/L), and a normal c-reactive protein (CRP) of 2.6 mg/L. Empiric treatment with intravenous ampicillin, cefotaxime, and acyclovir was given for presumed neonatal sepsis and possible encephalitis. On day 4 of illness, in addition to ongoing fever, he developed recurrent apnea and required supplemental oxygen and transfer to the tertiary care hospital pediatric intensive care unit. His blood work showed mild neutropenia, elevation of serum transaminase levels (alanine transaminase 86 U/L, aspartate transaminase 220 U/L), a normal ESR (1 mm/hr) and hypoalbuminemia. An infectious etiology was considered unlikely given negative bacterial cultures from blood, cerebral spinal fluid, and urine, and negative viral studies for herpes simplex virus 1 and 2, Respiratory Syncytial Virus, Influenza A and B, cytomegalovirus, Epstein Barr virus, rubella, adenovirus, and rotavirus. The urine and blood cultures were collected before the start of antibiotics. His chest radiograph was normal. On day 6 of illness, he developed bilateral non-purulent conjunctivitis, palmar erythema, striking bilateral non-pitting edema and erythema of his feet, and erythema and swelling of several proximal interphalangeal joints (Fig. ). He continued to require supplemental oxygen for suspected pneumonitis. On Day 7 of illness, after 5 days of fever and meeting 3 out of 5 criteria for KD, with no other obvious diagnosis, he was given IVIG (2 g/kg) for suspected incomplete KD. Over the next 48 h, his symptoms including extremity edema resolved, he no longer required supplemental oxygen, and fever did not recur. His CRP remained normal throughout his illness. Laboratory values improved and his platelet count progressively increased from 246,000 on Day 7 to 852,000 on Day 13 of illness. In the subacute phase, he was treated with low dose aspirin. He had persistent thrombocytosis followed by typical extremity desquamation. Echocardiograms on weeks 1, 2, and 6, and at 12 months were normal with no carditis or coronary artery abnormalities.","This 15-day-old baby was brought to the hospital with a fever, irritability, and rash. Initial tests showed he might have sepsis, but cultures came back negative. As his condition worsened, he needed extra oxygen and was transferred to a specialized care unit. After a few days, doctors suspected a rare condition called incomplete Kawasaki disease and gave him an IV treatment. Thankfully, his symptoms improved, and he was eventually discharged home."
884,"A 52-year-old male presented with severe headache and elevated blood pressure (180/120 mmHg). Color Doppler echocardiography revealed a large, solid right intraventricular mass of 7.1 × 2.2 × 4.7 cm, originating from the middle-inferior ventricular septum and extending into the right atrium, with systo-diastolic fluttering (Fig. ). A cardiac magnetic resonance imaging (MRI) study confirmed the presence of the solid, intracavitary mass.\nA cardiac biopsy via catheterization was not conclusive regarding the precise nature of the lesion. After informed consent, the patient underwent surgery. Intraoperatively, a whitish mass, 3 × 4 cm, non-adherent to the wall, was found in the right atrium after median sternotomy and atriotomy (Fig. ). Through the tricuspid valve, the mass extended without discontinuity into the right ventricle, adhering to the septal surface of the leaflet and infiltrating the adjacent interventricular septum, in its medial and superior portion. The exophytic intraatrial and intraventricular components were removed. Since intraoperative frozen-section on tissue from the infiltrated septum confirmed the clinical suspect of malignancy, no further surgery was attempted. Definitive histological examination showed a malignant neoplasm characterized by epithelial cells, positive for cytokeratin, forming glandular structures, admixed with a spindle cell component (Fig. -). A final diagnosis of biphasic synovial sarcoma was also confirmed by molecular demonstration of an SYT-SSX1 transcript.\nComputed tomography scan (CT) and cardiac MRI, performed one month after surgery, prompted administration of chemotherapy (ifosfamide/mesna, 4 cycles/3 months) due to increase of the residual ventricular tumor. However, since at the end of this treatment a (18) F-fluorodeoxyglucose positron emission tomography/CT revealed an increased ventricular mass, a SynCardia total artificial heart was implanted in the patient. Unfortunately, waiting for transplantation, the patient developed multiple pulmonary metastases and rapidly died, one and half year after initial surgery.","A 52-year-old man went to the hospital because of a severe headache and very high blood pressure. Tests revealed a large, unusual mass in his heart that was causing his symptoms. Surgery to remove the mass confirmed it was a type of cancer, and doctors then started chemotherapy. Sadly, despite treatment, the cancer spread, and the patient passed away a year and a half after the initial diagnosis."
885,"A 39-year-old man presented with signs of cardiac tamponade. The echocardiography revealed abundant pericardial effusion with a large intrapericardial mass. The patient underwent surgery after informed consent. At surgery, a pericardial tumor (9.8 × 6.1 × 3.2 cm) adherent to the anterior wall of the aorta, superior vena cava and right atrium, was extensively removed. No deep infiltration of the myocardium was apparent and aortic reconstruction was unnecessary. Since intraoperative histological examination did not reach a conclusive diagnosis, further surgery was not performed. Definitive histology revealed a monophasic (spindle cell) synovial sarcoma carrying an SYT-SSX2 transcript.\nPostoperatively, echocardiography, cardiac MRI and CT did not show residual tumor. Owing to a diagnosis of synovial sarcoma, the patient underwent chemotherapy (epirubicin/ifosfamide, 6 cycles/4 months), remaining in healthy conditions for the following 8 months, before the onset of tachycardia and dyspnea. Echocardiography, cardiac MRI and CT revealed a recurrent pericardial tumor extensively involving both atria and left pulmonary veins. Because further surgery was considered unfeasible, an additional course of chemotherapy was started (cisplatinum/docexatel, 6 cycles/4 months), followed by intensity-modulated radiotherapy (DT 54 Gy/25 sessions/2 months). After a temporary improvement of the clinical conditions, CT documented an extensive regrowth of the tumor, with involvement of the heart, great vessels, left bronchus and esophagus, with mediastinal lymphadenopathy. The patient died 32 months after surgery.","This patient was admitted to the hospital with signs of fluid buildup around his heart, called cardiac tamponade. Doctors found a large tumor attached to the heart and aorta, which was surgically removed. Although the initial surgery was successful, the tumor later returned despite further treatment. Ultimately, the patient passed away 32 months after the initial surgery due to the tumor’s recurrence and spread."
886,"A 32-year old white man presented to our department with a 1 month history of eye redness (left eye). He had already been observed by a general practitioner who advised the patient to seek ophthalmological advice if the lesion would not resolve within 3 weeks. In the first ophthalmic evaluation, the patient presented with 2 ocular lesions. These were best observed in biomicroscopy. There was an enlarged violet-coloured mass on the right superior eyelid which had evolved over the course of 1 week (Fig. ). There was also a mobile bulbar conjunctival lesion with a bright red colour, approximately 5 mm × 5 mm, in the superior temporal quadrant of his left eye (Figs. and ). The patient did not mention any pain or visual changes. The lesions looked like a chalazion and a subconjunctival haemorrhage, respectively (Fig. ). Best corrected visual acuity was 20/20 in both eyes (Snellen chart) and subsequent fundoscopic exam was normal. Patient denied previous trauma history or drug abuse. Other systemic features included facial seborrheic dermatitis (Fig. ), a characteristic of HIV-AIDS. Past medical history was unremarkable with no serological evaluation for HIV. The main differential diagnosis at the time of presentation was blood dyscrasia, due to the duplicity of lesions. We also considered KS as part of an immunodeficiency syndrome unknown to the patient. Blood cell count and differential analysis were normal and coagulation disorders were excluded. However the HIV-1 test was found positive and the presumed diagnosis of ocular KS was established. The patient elected for an excisional biopsy of the conjunctival mass and for an incisional biopsy of the right superior eyelid. In addition, HIV-AIDS staging was performed. A CD4 cell count was determined to be 23/mm3 and the viral RNA load of 427,000/ml. Pathologic examination confirmed KS diagnosis of both lesions. Postoperative evaluations were uneventful and no signs of recurrence were noticed during the 6-month follow-up.\nFurther multiple disciplinary assessments showed a diffuse KS with skin and supraglottis involvement. The patient was offered treatment for both AIDS and diffused KS. In order to achieve disease control, patient initiated systemic antiretroviral therapy – HAART - and systemic chemotherapy under medical supervision with regression of the tumour.","A 32-year-old man came to the clinic with a month-long problem of redness in his left eye, which included two unusual spots. The doctor found a growth on his eyelid and a red spot on his eye, which appeared to be related to a possible immune system problem. Tests revealed that he has HIV and a type of skin cancer called Kaposi’s sarcoma (KS) affecting his eye and skin. After receiving treatment for both conditions, the KS lesions shrank, and he is now being monitored closely."
887,"A two-year-old Vietnamese female child had fever and cough 20 days before admission to our hospital. She visited a local hospital and was diagnosed with pneumonia. She was treated with meropenem and vancomycin for 15 days. Despite treatment, she developed increased fever (40 °C), persistent cough and general malaise. Her respiratory condition deteriorated and she was transferred to our hospital. Prior to hospitalization, she had been healthy and had developed normally. She had no immunodeficiency and no history of contact with anyone suffering from TB.\nPhysical examination on admission showed a child with a weight of 10 kg, body temperature of 38.5 °C, blood pressure of 79/41 mmHg, and a heart rate of 157 beats/min. Her weight was reduced from 11 kg (25th percentile) to 10 kg (10th percentile) over the past month alone. Immediately after admission, tracheal intubation was performed because of severe dyspnea, and she was transferred to a pediatric intensive care unit (PICU). Arterial blood gas analysis in the PICU showed PaO2 of 60 mmHg, PaCO2 of 59 mmHg, and pH 7.27 under mechanical ventilation with FiO2 of 1.0, which resulted in a PaO2/FiO2 (P/F) ratio of 60 mmHg and an oxygenation index (OI) of 29 (Table ). A chest X-ray showed bilateral infiltration without findings of heart failure (Fig. ). Laboratory findings were as follows: white blood cell (WBC) count, 7000 cells/μl; red blood cell (RBC) count, 430 × 103 cells/μl; platelet (PLT) count, 223 × 103 cells/μl; aspartate aminotransferase (AST), 60 U/L; alanine aminotransferase (ALT), 13 U/L; blood urea nitrogen (BUN), 18.0 mg/dL; creatinine (Cre), 32 μmol/L; c-reactive protein (CRP), 7.3 mg/dL; fibrinogen, 2.79 g/L; and prothrombin time (PT), 16.1 s (Table ). The results of a rapid influenza test were negative. Genomes of mycobacterium TB and cytomegalovirus (CMV) were detected in tracheal lavage fluid (TLF) by real-time polymerase chain reaction (rPCR) assay. Acid fast bacilli smear and culture were both positive. She was diagnosed as having severe ARDS that developed from TB pneumonia, and the standard anti-tuberculous drugs: rifampicin, 15 mg/kg/day; isoniazid, 10 mg/kg/day; pyrazinamide, 30 mg/kg/day; ethambutol, 20 mg/kg/day were administered. We also used colistin and levofloxacin for not only nosocomial infection, but also mycoplasma infection, which we could not rule out. However, her respiratory and circulatory conditions deteriorated despite high-frequency oscillating ventilation (HFO), vasopressor support (noradrenaline at 0.2 μg/kg/min), and administration of 1 g/kg of immunoglobulin. On the third day after admission, the patient’s International Society on Thrombosis and Hemostasis DIC score had increased to 5 (PLT, 153 × 103 cells/μl; fibrinogen, 2.11 g/L; D-dimer, 5659 ng/mL; and PT, 23.3 s). For the treatment of DIC, 380 U/kg of rTM was administered as an intravenous drip infusion for 6 consecutive days. After administration of rTM was completed, OI gradually decreased, and the mechanical ventilation mode was changed from HFO to synchronized intermittent mandatory ventilation (Fig. ). DIC score also gradually decreased. We measured plasma levels of soluble receptor for advanced glycan end products (sRAGE) and high mobility group box 1 (HMGB-1) before and after rTM administration, because increased levels of sRAGE and HMGB-1 are associated with death in patients with ARDS []. After administration of rTM, HMGB-1 and sRAGE decreased (Fig. ), and inflammatory biomarkers, including interferon-gamma (IFN-γ) and interleukin-6 (IL-6), also decreased (Fig. ). Throughout the administration of rTM, there was no bleeding-related and other adverse event. Although severe ARDS (P/F ratio ≦ 100 mmHg) was sustained for 19 days, the patient’s OI and P/F ratio improved gradually (Fig. ), and she was extubated on the 27th day after admission. Although there was still a diffuse pattern in the chest X-ray just after extubation (Fig. ), her fever went down and her respiratory condition was stable (P/F ratio: 325, OI: 3). Therefore, she was moved from the PICU to the infectious disease department. The severe ARDS with DIC was successfully treated, and she was discharged from hospital on the 33rd day after admission.","This two-year-old Vietnamese girl was admitted to the hospital after a previous illness with pneumonia. Despite treatment, she developed a serious lung infection and needed to be put on a breathing machine. Tests revealed she had a severe lung infection caused by tuberculosis, and she also developed a condition called DIC, which affects blood clotting. After receiving special treatment, her condition improved, and she was eventually able to breathe on her own and go home."
888,"A 42-year-old Cameroonian (black African) man with no relevant past medical history presented to our hospital with a 1-week history of fever and a 4-day history of no bowel movements. The fever was intermittent and worse in the evenings, and associated with headache, joint pains, and anorexia. He self-medicated an undocumented orally administered antimalarial medication but symptoms persisted. Three days later, he developed generalized abdominal pain, colicky in character, with a severity of 5/10 on a visual analogue scale. This was associated with progressive abdominal distension and an altered bowel pattern initially characterized by an inability to pass stool and flatus over 4 days but he denied any vomiting. A worsening of his symptoms prompted his visit to our hospital.\nOn examination, his mucous membranes were dry, his conjunctivae were pink, and his vital signs were normal. His abdomen was mildly distended with mild diffuse tenderness. There was neither guarding nor rebound tenderness. Percussion note was tympanic and bowel sounds were hyperactive. His rectum was empty per digital rectal examination and his prostate was not enlarged. The rest of the physical examination was unremarkable.\nA rapid diagnostic (Alere™ Malaria Ag P.f, Abbott Rapid Diagnostics, USA) test for malaria was positive. Also, his complete blood count values were within normal ranges: white blood cell count of 6200 cells/uL, hemoglobin of 12.5 g/dL, and platelet count of 222,000 cells/uL. His metabolic panel was within normal range: serum sodium ion (Na+) of 140 mmol/L, serum potassium ion (K+) of 4.0 mmol/L, serum chloride ion (Cl−) of 98 mmol/L, serum creatinine of 0.9 mg/dL, alanine aminotransferase was 41.2 U/L, aspartate aminotransferase was 33.0 U/L, total bilirubin was 0.81 mg/dL, and alkaline phosphatase was 55.9 U/L. His human immunodeficiency virus serology was non-reactive. His rapid antibody test for typhoid fever was also non-reactive. There was no significant air fluid level or increased bowel gas on a plain abdominal radiograph and an abdominal ultrasound revealed mild free intra-abdominal fluid in all four quadrants.\nWe concluded on a working diagnosis of functional bowel obstruction secondary to malaria. He received three doses of intravenously administered quinine base infusion (600 mg in 500 ml of dextrose 5% to flow for 4 hours every 8 hours for 24 hours) and intravenously administered crystalloids (0.9% normal saline at rate of 1 liter 8 hourly for 48 hours) and was placed on “nothing by mouth.” He passed a small quantity of feces 48 hours after initiation of antimalarial medication, with complete resolution of the abdominal pain, distension, and bowel movements on the fourth day of hospital stay. A stool examination was done with no organisms identified. We then concluded on a final diagnosis of sub-acute intestinal obstruction secondary to malaria. He was then discharged on an orally administered artemisinin-based antimalarial combination (artemether/lumefantrine 20/120 mg, 4 tablets 12 hourly for 3 days). His 2-week follow up was unremarkable.","A 42-year-old man from Cameroon came to the hospital with a week-long fever, no bowel movements, and abdominal pain. Tests showed he had malaria, which was causing a blockage in his intestines. He received treatment for malaria and was able to pass stool, and his symptoms improved. He was discharged home with medication to prevent the malaria from returning."
889,"A 20-year-old woman, who had an unremarkable medical history and was immunocompetent, was admitted to another hospital due to fever, fatigue, and anorexia, and she was administered acetaminophen and antibiotics. She also experienced vomiting, as well as systemic myalgia 5 days after admission causing an inability to move. Her condition was worsening, and she was transferred to our hospital 7 days after her initial admission. Upon admission, her liver enzyme and creatine phosphokinase (CPK or CK) levels were high. She had no history of jaundice, pruritus, clay stools, melena, hematemesis, abdominal distension, or altered sensorium. She reported only an occasional small amount of ethanol intake and had not had sexual intercourse. The patient denied intake of indigenous medicine or intoxication. The patient did not report any past major surgeries, blood transfusions, or intravenously injected drug abuse prior to onset of the disease. Additionally, she did not report any history of diabetes, hypertension, tuberculosis, thyroid disease, trauma, exposure to industrial toxins or radiation, blood or blood component therapy, bleeding disorders, promiscuity, or similar complaints in the family or neighborhood.\nUpon admission, her vital signs were as follows: body temperature, 37.2 °C; blood pressure, 110/72 mmHg; pulse, 75 beats/min; respiratory rate, 20 breaths/min; and oxygen saturation, 98% on room air. A physical examination revealed mild enlargement of the liver, no pitting edema in both legs, and no coarse crackles over the lung fields. Laboratory findings are presented in Table .\nAdditionally, a chest radiography showed absence of pulmonary congestion, pleural effusion, and cardiomegaly. Electrocardiography was not performed on admission. Computed tomography of the chest (Fig. ) and abdomen revealed minimal pericardial effusion, diffuse swelling, and an internal heterogeneous image in the liver. These findings were compatible with acute hepatitis; therefore, we did not examine for cardiac function despite the presentation of pericardial effusion.\nOn the second day of hospitalization, we performed a liver biopsy, which showed parenchymal necrosis and lymphocytic infiltration (Fig. ). On that same night, the patient was hypotensive, with a blood pressure of 80/65 mmHg, and her heart rate was elevated (113 beats/min). Physical examination revealed cyanosis of the lips, distended external jugular veins, pretibial edema in both legs, and coarse crackles over the lower bilateral lung fields.\nElectrocardiography showed diffuse ST-segment elevation (Fig. ). Her troponin I, CK level in the blood (CK-MB), and brain natriuretic peptide (BNP) were elevated (troponin T, 0.879 ng/mL [normal value: ≤0.016 ng/mL]; CK-MB, 253 U/L [normal value: ≤5 U/L]; BNP, 1513 pg/mL [normal value: ≤18.4 pg/mL]). Chest radiography showed a normal cardiac size, pulmonary congestion, and pleural effusion in the right lung only (Fig. ). Echocardiography showed a dilated, spherical ventricle with reduced systolic function (left ventricle ejection fraction [LVEF], 17%) and pericardial effusion (Fig. ).\nCardiac catheterization on day 3 revealed high pulmonary capillary wedge pressure (22 mmHg) and a low cardiac index (2.0 L/min/m2). Coronary angiography showed no abnormalities. Endomyocardial biopsy (EMB) was performed via the right internal jugular vein, and five specimens were obtained from the right ventricle side of the interventricular septum. Endomyocardial biopsy findings showed lymphocyte infiltration of the myocardium and intranuclear inclusions, which was confirmed as acute myocarditis (Fig. ). In response, intravenous administration of dobutamine (4 μg/kg/min) was started on day 3. The patient’s systolic blood pressure increased to approximately 100 mmHg and stabilized, and dobutamine administration was gradually tapered off. On day 10, echocardiography showed normalization of LVEF to 71%, dobutamine was stopped, and laboratory findings were almost normalized as follows: CPK, 143 U/L (standard values: 40–150 U/L); aspartate aminotransferase (AST), 36 U/L (standard values: 11–30 U/L); and alanine aminotransferase (ALT), 278 U/L (standard values: 4–30 U/L). Laboratory findings on day 18 were normalized as follows: CPK, 123 U/L; AST, 18 U/L; ALT, 21 U/L, and BNP, 18 pg/mL. She was eventually discharged on day 19 of her hospital stay.\nOne serum sample for viral serological testing was collected before the EMBs were obtained. Follow-up serum samples were collected between 2 weeks, 1 month, and 6 months after the initial serum sample. The presence of HSV-specific immunoglobulin M (IgM) was detected upon admission to our hospital, increased at 2 weeks, and returned to normal 6 months later. Additionally, HSV-specific immunoglobulin G (IgG) increased from hospital admission to 2 weeks (Table ). Other viral serologic tests, including HIV, were negative (Table ). Based on this finding, we diagnosed the patient with acute myocarditis combined with hepatitis arising from HSV infection.","This 20-year-old woman was admitted to the hospital after experiencing fever, fatigue, and a loss of appetite. Initial tests revealed high levels of liver enzymes and muscle enzymes, and a CT scan showed some swelling in her liver. A liver biopsy confirmed inflammation and damage to the liver tissue. Further tests revealed that she had a viral infection (HSV) that was causing both her liver problems and inflammation of the heart muscle (myocarditis). With treatment, her heart function improved, and her liver enzymes returned to normal, allowing her to be discharged home."
890,"An asymptomatic 70-year-old female known to have surgically treated squamous cell carcinoma of the nose underwent a routine screening colonoscopy that revealed an uncomplicated diverticulosis and a cecal submucosal mass (Fig. ). The appearance was most likely correlated with a submucosal tumor and less likely to be an extrinsic compression. At pathology, colonoscopic biopsies of the mass showed normal colonic mucosa. Laboratory examination showed no anemia (hb 13.2 g/dL) was negative for CEA tumor marker (CEA 2.2 μg/L). Abdominal computed tomography scan (CT scan) revealed a well-circumscribed hypervascular anterior cecal wall mass (Fig. ) with no liver metastases and no other distant lesions. The mass had no metabolic activity on either FDG-PET scan or on Octreo-PET (Fig. , ).\nAfter multidisciplinary team discussion, a differential diagnosis of mesenchymal tumor of the colon (GIST, leiomyoma, and leiomyosarcoma) was suggested and we decided to perform an exploratory surgery. The patient was consented for open exploration by mini-laparotomy and possible right hemi-colectomy. The right colon was mobilized at the white line of Toldt, the 3 cm white cecal mass was well circumscribed, and a wedge resection, including the appendix, using GIA 75 (Ethicon Endo-Surgery GIA; 75 mm; Guaynabo, Puerto Rico 00969 USA) was performed. The operative specimen was sent for frozen section at pathology. The temporary diagnosis was a benign spindle cell tumor. The intra-operative decision was to wait for the definitive histopathologic examination report in order to try to avoid a right hemicolectomy. The final pathology report revealed a benign spindle cell tumor that stained negative for CD117 and DOG-1 and was diagnosed as cecal schwannoma with a reactive lymph node (Fig. ).\nThe post-operative course was uneventful and the patient started oral feeding the same night and was discharged on day 5 with pain killers. The final multidisciplinary committee decision was follow-up without further treatment needed. At 1-year follow-up, the patient is disease free.","A 70-year-old woman with a history of cancer had a routine colonoscopy and found a small, unusual area on her cecum (large intestine). Tests showed this area was likely a benign tumor, but to be sure, doctors performed surgery to remove it along with the appendix. The surgery confirmed that the mass was a benign schwannoma, a type of slow-growing tumor. The patient is now recovering well and will continue to be monitored for any changes."
891,"A 39-year-old man fell and injured himself whilst walking on the road. Upon physical examination, he complained of severe pain in his right elbow, which showed swelling and tenderness. Because of the pain, the range of motion of the elbow joint was limited to 30°–50°. Radiographs revealed a fracture of the ulna coronoid process, which was displaced by about 4 mm (). He was diagnosed with a coronoid process fracture, Regan-Morrey type 2. He was young, highly active, and wanted an early return to normal life; therefore, we performed a minimally invasive arthroscopic osteosynthesis 7 days after the injury.\nSurgery was performed under general anaesthesia with the patient in the prone position. We confirmed the fracture site through the anterolateral portal (). A shaver and radiofrequency probe were used to remove any clots and debris. The radiofrequency probe allowed us to reduce the amount of bone fragment entering the fracture bed. Under fluoroscopic control, a guide pin for a cannulated cancellous screw (CCS) was inserted from the posterior ulnar shaft into the coronoid fragment (). We fixed the fracture with a 3.5 mm CCS and two 1.5 mm K-wires, whilst confirming the anatomical reduction arthroscopically (). After fixation, we confirmed a good reduction position and sufficient stability upon examination. We also confirmed a reduction by radiographs (). Postoperatively, he wore an immobilizing splint for 1 week and then began physical therapy to increase the range of motion. After 3 weeks, the K-wires were removed, and bone union was achieved after 4 months (). At 1 year postoperatively, he had no pain and a physical examination revealed a complete range of motion.","This man injured his elbow by falling and suffered a fracture of the ulna bone. X-rays confirmed the fracture, and he was diagnosed with a coronoid process fracture. To help his elbow heal quickly, doctors performed a minimally invasive surgery to stabilize the bone with screws and wires. After the surgery, he wore a splint and began physical therapy, and he is now fully recovered with a complete range of motion in his elbow."
892,"A 29-year-old woman fell and injured herself whilst walking down the stairs. She was also diagnosed with a coronoid process fracture, Regan-Morrey type 2. We performed arthroscopic osteosynthesis 5 days after the injury in almost the same way as described in Case 1, using two 3.5 mm CCSs (). Postoperatively, she also had a good clinical course; she had no pain and had full range of motion of the elbow joint 1 year after surgery.","This woman injured her elbow after a fall down the stairs, resulting in a fracture of the coronoid process. Doctors performed a special surgery to fix the fracture using a technique called arthroscopic osteosynthesis. The surgery was successful, and she had a good recovery with no pain and full movement of her elbow after a year. Overall, her recovery has been very positive."
893,"A 40-year-old man fell and injured himself whilst walking. He was also diagnosed with a coronoid process fracture, Regan-Morrey type 2 (). We performed arthroscopic osteosynthesis 10 days after the injury in almost the same way as described in Case 1. However, the fracture was too comminuted to be fixed by CCS, so we performed osteosynthesis using three K-wires (). Postoperatively, he wore an immobilizing splint for 2 weeks and then began physical therapy to improve the elbow range of motion. After 4 months, bone union was confirmed, and after 8 months, the K-wires were removed (). A follow-up at 10 months showed that he had no pain, and a physical examination revealed complete range of motion, indicating a good clinical course.","This man injured his elbow after a fall, resulting in a fracture of the coronoid process. Surgeons used a special procedure to repair the fracture, securing it with K-wires. He wore a splint for two weeks and then started physical therapy to regain full movement in his elbow. After about a year, the fracture had healed completely, and he had no pain or limitations in movement."
894,"A 62-year-old man presented with 2 weeks of progressive dyspnea and chest pain. He was previously diagnosed with high-grade invasive urothelial carcinoma (UC) of the bladder. He underwent neoadjuvant chemotherapy followed by radical cystectomy, resulting in complete pathologic remission. On examination, patient was afebrile with a heart rate of 136 bpm, blood pressure of 122/74 mm Hg, respiratory rate of 18 breaths/min, and O2 saturation of 98% on room air. Heart sounds were muffled. Heart rhythm was irregularly irregular. Distention of the jugular veins was observed and Kussmaul's sign was present. Although pulsus paradoxus was not observed, other physical findings were extremely concerning for cardiac tamponade. Bibasilar crackles were present on lung auscultation. Laboratory data was remarkable for a creatinine of 1.42 (baseline of 1.08) and international normalized ratio (INR) of 3.6 (on warfarin for a history of pulmonary embolism). His complete blood counts and electrolytes were normal. His electrocardiogram revealed atrial fibrillation with a rapid ventricular rate (RVR) and electrical alternans. Chest radiograph showed enlargement of the cardiac silhouette.\nDue to high clinical suspicion for cardiac tamponade, a bedside transthoracic echocardiogram (TTE) was obtained which showed a large pericardial effusion with diastolic compression of the right ventricle, suggestive of cardiac tamponade, as well as mild left ventricular systolic dysfunction (see Figures and ). Patient's INR was reversed to <1.5 and he underwent emergent subxiphoid pericardial window with the removal of 700 ml of turbid, dark, bloody pericardial fluid. He had significant improvement in hemodynamics and respiratory distress. The pericardial fluid cytology and pericardial biopsy were both positive for malignancy, consistent with metastatic UC (see ).\nOne week after the pericardial window, the patient underwent a computerized tomography (CT) scan of the chest, abdomen, and pelvis which did not show evidence of metastatic disease elsewhere. Given the recurrence of UC with metastasis to the pericardium, he was started on atezolizumab. Subsequently, he was noted to have recurrent pericardial effusion suggesting failure of treatment (see ). He was then started on pemetrexed, a folate antimetabolite chemotherapy drug, for metastatic UC with plans to complete 6 cycles.","62-year-old man came to the hospital with a two-week history of shortness of breath and chest pain. He had previously been treated for bladder cancer, but tests showed the cancer had returned and spread to his heart. Doctors suspected he had fluid buildup around his heart, which was causing his symptoms. After a procedure to drain the fluid, his condition improved, and he started chemotherapy to fight the cancer’s return."
895,"A 21-year-old man with no known past medical history presented from prison with abdominal pain in the past five days. He explained that five days prior to presentation, he began experiencing squeezing abdominal pain, rated 4/10 localized to his RUQ with no radiation. He reported associated nausea, vomiting, diarrhea, and intermittent fever. The patient reported that he had initially thought he had the “stomach flu.” He had tried oral rehydration and Tylenol with no improvement in symptoms since onset. He reported continual progression of symptoms with minimal resolution in abdominal pain, episodes of vomiting, or diarrhea. He denied any change in diet, change in eating habits, recent travel, jaundice, tea-colored urine, itching, recent skin, and soft tissue infection. He stated that he was a previous smoker but quit since being incarcerated two years ago. He denied any alcohol or illicit drug use. He denied having any current sexual partner. He also denied any family history of illnesses or malignancy. On physical examination, patient was normotensive, tachycardic, and febrile on presentation with temperature recorded as 103.7F. Patient appeared weak and diaphoretic and had moderate right upper quadrant tenderness with no rebound tenderness or mass palpated and no other pertinent abdominal findings. Examination of the other systems revealed no abnormalities. The patient was admitted for further work-up of his condition given presentation from prison.\nInitial laboratory results showed normocytic anemia (hemoglobin of 9.8, MCV 81.1), white blood cell count of 15.6 with significant left shift, sodium of 137 mmol/L (reference value 136-145 mmol/L), and potassium of 3.4 mmol/L (3.5-5.1 mmol/L) with normal renal function. Other significant labs on presentation included alanine aminotransferase (ALT) of 30 U/L (12-78 U/L), aspartate aminotransferase (AST) of 43 U/L (15-37 U/L), alkaline phosphatase (ALP) of 240 U/L (45-117 U/L), total bilirubin of 1.5 mg/dL (0.2-1.0 mg/dL), albumin of 1.4 g/dL (3.4-5.0 g/dL), and total protein 5.8 g/dL (6.3-9.0 g/dL). Rapid influenza testing was negative. Urine drug screen resulted as negative. Blood cultures from two peripheral sites were collected. Chest X-ray was significant for bibasilar infiltrates with bilateral pleural effusions noted. Given the clinical condition with concern for intra-abdominal source of infection, a CT scan of the abdomen/pelvis with IV contrast was performed. This showed a large multiloculated right hepatic mass, measuring 8.4cm with soft tissue mass extension into the upper pole of the right kidney (), unremarkable findings in the gallbladder, bowel, and appendix.\nIn the Emergency department, the patient was initially started on aggressive hydration with 0.9% normal saline given initial presentation prior to work-up. Ceftriaxone 1g Intravenous (IV) route and Metronidazole 500mg Intravenous (IV) route were administered in the emergency department. Blood cultures obtained from two peripheral sites resulted as gram-positive cocci with time to positivity about 15 hours. Patient was started on intravenous broad spectrum antibiotics with Vancomycin 1g IV q12 hours and Meropenem 1g IV q8 hours, the initial choice of therapy. Antibiotic sensitivity resulted in day two of admission as MSSA. Antibiotic therapy was narrowed down to Intravenous Nafcillin on day two of admission. Interventional radiology department was consulted for image guided percutaneous drainage of the liver abscess on day one of admission. Two abdominal drains were placed with initial return of 150ml of serosanguinous fluid. Fluid obtained from the CT-guided aspiration was sent to the laboratory for gram stain and culture. The culture obtained from percutaneous catheter drainage resulted as MSSA on day two of admission. The source of MSSA bacteremia was confirmed as pyogenic liver abscess and Nafcillin IV therapy was continued with no change in antibiotic management. Abdominal drains were removed on day six of admission as output remained minimal.\nThe patient was found to have bilateral pleural effusions. Given significant right sided pleural effusion, thoracentesis was performed on day two of admission with 1.2L of pleural fluid removed. Fluid was not sent for studies. Patient also developed nonoliguric acute kidney injury with creatinine rise from 0.7mg/dl (reference value 0.7-1.3 mg/dL) on admission to 1.7 mg/dL on day four of admission. Work-up was consistent with an intrinsic etiology of the acute kidney injury. The etiology was clinically suspected to be secondary to acute tubular injury due to sepsis. The patient was encouraged to continue per oral intake and intravenous fluids were given. Creatinine trended back to patient's baseline while on Nafcillin IV therapy. Repeat blood cultures obtained on day four of admission were consistently growing MSSA. Transthoracic echocardiogram was performed and it showed no evidence of intracardiac vegetations and no abnormalities. Repeat blood cultures on day six of admission were negative. A mid-line catheter was inserted in the patient's right upper extremity for vascular access on discharge for continued antibiotic therapy. The patient was discharged after 10 days with continued Nafcillin IV therapy for four weeks postdate of negative blood cultures.\nThe patient was followed up in the general surgery clinic one month after discharge. At this time, he had completed IV antibiotic therapy. Follow-up revealed stable clinical condition as patient reported resolution of symptoms and return to baseline functional status. Laboratory findings indicated normal renal function. Repeat CT imaging of the abdomen with IV contrast on follow-up visit showed resolution of the pyogenic liver abscess ().","This 21-year-old man was admitted to the hospital after experiencing abdominal pain, fever, and other symptoms while incarcerated. Initial tests revealed he had a large infection in his liver, likely caused by bacteria, and a concerning mass in his upper right kidney. He also had anemia and fluid buildup in his lungs. He was treated with strong antibiotics and drainage of the infection, and his condition gradually improved. He was discharged home with continued antibiotics and a plan for follow-up care."
896,"A 35-year-old para 0+6 Black African lady presented with a history of five first and one second trimester recurrent pregnancy losses. In addition, she had a nine-year history of irregular heavy bleeding associated with dysmenorrhea.\nHer menarche was at 17 years of age with regular painful cycles that lasted 10 days. She was not on any contraceptive method and did not report any dyspareunia or urinary symptoms. Her first miscarriage occurred 11 years and was surgically managed by dilatation and curettage. Subsequently she noted changes in her menstrual cycle. Her menstrual cycle became irregular with a heavy flow for 10 days associated with severe dysmenorrhea and bowel symptoms of bloating and diarrhea. She used tranexamic acid one gram three times a day during her menses for the heavy prolonged periods and Mefenamic Acid 500 milligrams three times a day for the dysmenorrhea with reported relief of the symptoms.\nThe physical examination was unremarkable except for mild tenderness in the suprapubic region; she had grossly normal external genitalia and normal looking cervix.\nHer initial hormonal profile was as follows:FSH: 4.5 IU/m (3.1-7.9 IU/L) LH: 10 IU/L (1-18 IU/L)\nShe had a recent pap smear one year ago that was normal.\nTransabdominal and transvaginal (TVS) scans had shown separate right and left cornu with multiple cysts in the peripheral ovarian parenchyma features suggestive of bicornuate uterus and polycystic ovaries (). She also had a hysterosalpingogram (HSG) that was reported to have a uterus opacified with banana configuration oriented to the right with no delineation of fallopian tubes, findings suggestive of a unicornuate uterus.\nHer past medical history was not significant. She was recently divorced, a condition she attributed to her history of several miscarriages in the context of an African cultural expectation of siring children.\nShe was subsequently scheduled her for hysteroscopy and diagnostic laparoscopy. She also gave consent for possible open Strassmans metroplasty.\nOn hysteroscopy, a normal looking endometrium was visualized with a right sided tubal ostia and a small tubal ostia on the left that did not appear patent (). Laparoscopically, a bicornuate uterus was found () with associated endometriosis at the vesicouterine fold. Open metroplasty was done (Figures and ). A fundal transverse incision was made and dissection done to the level of the endometrium after injection of subserosal vasopressin. Apposition of the two horns was done and the uterus sutured in layers (Figures and ). A copper intrauterine device was left in situ to separate the uterine walls and possibly reduce the chances of uterine synechiae though the evidence for this practice is still not conclusive. In addition, endometriotic deposits discovered at the vesico uterine fold were ablated.\nShe had an unremarkable follow-up after surgery with no complications reported.\nA HSG 6 months after surgery revealed unification of the uterine horns but both tubes were not delineated.\nPatient was lost to follow-up but two years later she presented having conceived spontaneously and a TVS revealed a single intrauterine pregnancy at 13 weeks. Her antenatal workup was as highlighted in .\nShe was subsequently followed up at the antenatal clinic and had no concerns till 33 weeks when she presented with a history of lower abdominal pain worsening over the past few weeks and right sided fundal tenderness pain score of seven out of ten on the pain assessment scale. The main concern at the time of assessment was possible uterine rupture. She did not have any features suggestive of bowel obstruction as she was passing stool and bowel sounds were present. She had a normal NST and an obstetric ultrasound that was done showed a single intrauterine pregnancy with normal biophysical profile and no features of placental abruption or slower uterine segment site dehiscence. She had no other investigations done at this time. The pain was significant enough to warrant an admission for which she subsequently received antenatal steroids for lung maturity and opioid analgesia for pain relief. However, the pain did not completely resolve and she underwent a caesarean delivery at 33 weeks and 5 days due to persistent lower abdominal pain. The outcome was a live male infant with a birth weight of 1950 grams and APGAR of 9, 10, 10. There was no evidence of uterine rupture although she had venous congestion and massive varicose veins bilaterally at the cornual areas. The previous metroplasty scar was intact.\nShe had a resolution of the abdominal pain postoperatively and had an unremarkable postoperative recovery. She was discharged on the fifth postoperative day.","This patient has a long history of difficult menstrual cycles and recurrent miscarriages, which have significantly impacted her life. She has experienced irregular, heavy bleeding and severe pain for nine years, and has had five miscarriages. After undergoing several tests, including hysteroscopy and laparoscopy, doctors discovered she had a bicornuate uterus (a uterus with two separate chambers) and endometriosis. A surgery called a Strassman metroplasty was performed to try to unite the uterine horns, and a copper IUD was placed to help prevent future problems. Ultimately, she was able to conceive naturally and delivered a healthy baby, although she experienced some complications after delivery due to previous surgery."
897,"Case 1 is a 40-year-old male, presenting with general malaise, arthralgia in his extremities, facial cyanosis, and fever (39°C). In the medical history, a post-traumatic splenectomy was reported. Empirical antibiotic therapy started with ceftriaxone (2 g every 12h) and vancomycin (500 mg every 6h). After several bacteriological analysis, secondary sepsis caused by meningitis from Streptococcus pneumoniae was diagnosed. The patient was transferred to the intensive care unit (ICU) where his clinical status rapidly deteriorated, showing a severe hemodynamic instability with need for vasopressor support (norepinephrine 0.4 μg/kg/min), persistent hypotension, and a pronounced inflammatory state with C-reactive protein (CRP) at 176.3 mg/l and procalcitonin (PCT) at 485.88 ng/ml. The patient also exhibited metabolic abnormalities, with lactate levels of 7.8 mmol/l, and severe coagulative disorders.\nIn face of a persistent anuria, continuous renal replacement therapy (CRRT), hemodiafiltration mode (CVVHDF, Prismaflex, M150, Baxter, USA) was started, together with Cytosorb cartridge, for a total of 68 hours (start of treatment <24h after ICU admission).\nA general improvement was obtained already during the first treatment. PCT could be reduced to 6 ng/ml at the end of the treatments. Similarly, CRP also dropped to 136.41 mg/l during the course of the treatments. On the metabolic level, the patient showed a marked improvement in lactate acidosis, with lactate levels decreasing to 1.9 mmol/dl. From a hemodynamic point of view, a MAP stabilization could be achieved paralleled with a reduction in the need for norepinephrine. The patient could be stopped from CRRT after 12 days with a recovery of diuresis. The course for norepinephrine demand during Cytosorb treatment is presented in , whereas the course of lactate and PCT is shown in .\nThe patient was still hospitalized after 2 months, however in an alert, collaborative, tracheotomised, spontaneously breathing state, waiting for transfer to a rehabilitation facility.","This 40-year-old man was admitted to the hospital feeling unwell with fever, joint pain, and blue skin. Tests revealed he had a serious infection in his brain (meningitis) caused by a bacteria called Streptococcus pneumoniae, which is why he needed intensive care. To help him recover, doctors started him on strong antibiotics and used a special machine to filter his blood. After several treatments, his condition improved, and he was able to stop the dialysis machine and breathe on his own, but he still needed support and is waiting to be transferred to a rehabilitation facility."
898,"Case 2 is a 66-year-old female, presenting with general illness, fever (40°C), and hypotension. Prior medical history included beta-blocker therapy for tachyarrhythmia, appendectomy, colon cancer, and splenectomy for colonic metastases. On admission, she exhibited skin ischemic lesions with rapid deterioration to whole body cyanosis and metabolic and lactic acidosis. Empirical antibiotic therapy started with ceftriaxone (2 g every 12h) and levofloxacin (500 mg every 12h). After 24h, blood cultures confirmed the diagnosis of sepsis from Streptococcus pneumoniae paralleled with severe purpura fulminans. Empirical antibiotic therapy continued for 11 and 7 days, respectively, with adjustments due to renal function changes. In the further course, she became oligoanuric, severely hypotonic with hemodynamic instability (epinephrine 0.4 μg/kg/min), and thrombocytopenic, presenting severe bleeding.\nA CVVHDF treatment (Multifiltrate, AV1000, Fresenius Medical Care, Germany) was started in combination with one 24-hour session of Cytosorb hemoadsorption immediately after the ICU admission.\nThis combined treatment resulted in a clear and progressive hemodynamic stabilization accompanied by a marked reduction of epinephrine to 0.1 μg/kg/min () and a reduction of CRP, from 17.5 mg/dl to 13 mg/dl. Initially diuresis started to work again; however this went back to anuria in the first 12 hours (probably due to sepsis-associated tubular necrosis) and fully recovered later on day 10. Lactacidemia, elevated at the admission (15 mmol/l), decreased until 4 in the first 24h. At the same time, PCT decreased from 18.35 ng/ml to 2 ng/ml after 6 days ().\nSubsequent to this first acute event, the patient showed a recrudescence of the septic state. Blood cultures were positive for Enterobacter aerogenes, which colonise the body through the injured intestine wall, resulting from purpura fulminans. Because of her severe injuries at the extremities, the patient needed amputations and 2 months later, the reoccurrence of the second septic episode caused the death of the patient.","This 66-year-old woman was admitted to the hospital with a high fever and low blood pressure, and her skin started to turn blue. She had a history of several medical conditions, including cancer and a heart rhythm problem. Doctors discovered she had a severe infection caused by bacteria, which led to a dangerous condition called purpura fulminans. To help her, they used special treatments like dialysis and medications, but unfortunately, she didn't recover and passed away due to a second infection."
899,"Case 3 is a 47-year-old male showing fever (38°C), asthenia, and lumbar pain with signs of diffuse petechiae. In the further course, he became severely hypovolemic and tachycardic, showing also metabolic acidosis (lactate 12 mmol/l). Blood cultures were taken and empirical antibiotic therapy was started with meropenem (2 g every 8h) and amikacin (1 g every 24h). Diagnoses confirmed by blood cultures were septic shock secondary to Neisseria meningitidis infection (Serotype C) and antibiotic therapy was replaced with ceftriaxone (2 g every 12h). After transfer to the ICU, norepinephrine infusion was started at 0.2 μg/kg/min and rapidly increased to 0.5 μg/kg/min, in combination with epinephrine infusion at 0.2 μg/kg/min. Inflammation was controlled with PCT, which was extremely elevated, 121.7 ng/ml. In the following hours, a worsening of general conditions of the patient was reported, with extension of petechiae and severe bleeding from the insertion points of the catheters and severe hypotension with the increase of norepinephrine up to 0.75 μg/kg/min. In face of a persistent condition of oligoanuria, it was necessary starting a hemofiltration treatment (CVVH, Prismaflex, M150, Baxter, USA) and Cytosorb was additionally installed into the CVVH circuit the next day (start of treatment: 15h after ICU admission).\nWithin the course of 72-hour Cytosorb treatments, the patient witnessed a hemodynamic improvement with norepinephrine and epinephrine being tapered off after the third session () as well as a stabilization of lactate values and inflammation, with PCT decreased until 16.52 ng/ml ().\nThe patient also showed an attenuation of disseminated intravascular coagulopathy, a demarcation of skin necrosis areas, and a recovery of vital functions. The patient could be extubated 10 days and weaned from CVVH 12 days after the ICU admission. He was transferred to rehabilitation medicine after 23 days from the recovery, waiting for plastic surgeon consultant for the amputation of some phalanges.","This 47-year-old man was admitted to the hospital with a fever, back pain, and a widespread rash. He quickly became very sick, with low blood pressure and dangerously high levels of acid in his blood. Tests revealed he had a serious infection with a type of bacteria called Neisseria meningitidis, leading to septic shock. After receiving intensive treatment, including antibiotics, dialysis, and supportive care, his condition improved, and he was eventually able to leave the ICU and start rehabilitation."
900,"Case 5 is a 36-year-old female, showing a persistent fever, severe polymyalgia, and diffuse petechiae in expansion to the limbs and arms. Antibiotic therapy was started with levofloxacin (750 mg every 24h) and ceftriaxone (2 g every 12h). The patient was immediately transferred to ICU with a suspected diagnosis of sepsis secondary to meningitis. The patient presented hypotension, requiring the administration of norepinephrine at 0.5 μg/kg/min and dobutamine in the next day. Lab tests confirmed the diagnosis of bacterial meningitis infection caused by Neisseria meningitidis (Serotype C) and underlined a condition of thrombocytopenia, acute renal failure, and inflammation, initially monitored with CRP (17.5 mg/l). Lactate level was at 3.6 mmol/l. Immediately after the ICU admission, a CVVHDF treatment (Prismaflex, Oxiris, Baxter, USA) was started in combination with Cytosorb (start of treatment: 8h after ICU admission).\nWithin the course of hemoadsorption treatments, performed for a total of 96 hours, a hemodynamic stabilization was observed, associated with a reduction in need of inotrope drugs. Indeed, dobutamine could be stopped after 48h and norepinephrine after 78h. The course of norepinephrine is shown in . The patient showed a stabilization lactate level, reduced at 1.7 mmol/l, as presented in . Moreover, the inflammatory status was also monitored during Cytosorb treatment dosing IL-6 levels (), directly adsorbed by the sorbent. After 12h of hemoperfusion, IL-6 values were 4.326 ng/ml and decreased dramatically during the course of treatments until 60.1 ng/ml after 60h and 35 ng/ml after 72h. Petechiae and skin lesions were stable already after the first day of treatment. A complete recovery of renal function and diuresis was observed after 9 days from the admission and the patient could be transferred to medicine after 7 days. The patient was followed by dermatologists and vascular surgeons for the outcome of the septic embolism complication, evaluating the possible amputation of one toe.","This 36-year-old woman was admitted to the ICU with a high fever, muscle pain, and small, red spots on her skin, which could indicate a serious infection. Doctors suspected she had sepsis, a life-threatening condition, and quickly started her on strong antibiotics and supportive care. Tests revealed she had bacterial meningitis and was also experiencing kidney problems and low platelet counts. With the help of specialized treatments like hemoadsorption, her condition improved, and she was able to recover and return to the medicine ward after a week. She is now being monitored by specialists to address potential complications from the infection."
901,"This case describes a 23-year-old male who was struck by a motor vehicle. Upon arrival at our hospital, the patient had a GCS of 8. FAST exam, chest radiograph, and computed topography (CT) of the head and cervical spine were obtained and were negative.\nExam of the lower extremities revealed abrasions over the left knee and tenderness over the lateral joint line with an effusion. The right knee was diffusely tender to palpation without effusion. The patient had palpable pulses in both feet with well-perfused extremities. Ankle brachial indices were performed and found to be >0.9. He demonstrated guarding and pain with the attempted Lachman maneuver of the left knee and slight opening of the left knee joint with varus stress. Radiographs were obtained and revealed a left knee Segond fracture ().\nMagnetic resonance imaging (MRI) of both knees was performed to evaluate for ligamentous injury. Left knee imaging demonstrated the Segond fracture along with a grade III lateral collateral ligament (LCL) tear with retraction (), a grade II tear of the popliteus tendon and anterior cruciate ligament (ACL) (), and a grade I medial collateral ligament (MCL) injury (), as well as partial thickness tears of the biceps femoris and vastus medialis. Right knee imaging revealed a grade III tear of the ACL and MCL (), grade II tears of the posterior cruciate ligament (PCL) (), LCL, and popliteus tendon, and a medial meniscus tear. The patient was placed in bilateral hinged braces with the left knee unlocked and the right knee in locked extension to aid with transfers from a bed to a wheelchair. The patient was also given a left foot drop boot for a foot drop discovered during a secondary exam. On hospital day three, the patient was discharged home.\nNine days after the accident, the patient presented to the clinic. He noted that the left-sided foot drop was improving. On that side, he had 5/5 strength of his extensor hallucis longus and tibialis anterior (TA), without any sensory deficits in the peroneal nerve distributions. On the physical exam of the left knee, the Lachman maneuver was grade 2B (ACL injury with 5–10 mm translation without an endpoint), the varus stress test grade 3 (complete LCL tear with >10 mm opening of the lateral joint), and the valgus stress test grade 2 (MCL injury with 6–10 mm opening of the medial joint). The right lower extremity was also neurovascularly intact, and the right knee exam revealed a grade 2A Lachman maneuver (ACL injury with 5–10 mm translation and a firm endpoint), a grade 3 posterior drawer test (complete tear of PCL with >10 mm posterior tibial translation), and a grade 3 valgus stress test (MCL injury with 11–15 mm opening of the medial joint), with a presumptive positive dial maneuver on the right side at 30 and 90 degrees (consistent with PCL and posterolateral corner (PLC) injury). However, given that the patient had bilateral PLC injuries, this physical exam finding was somewhat subjective without a reference point on the contralateral side. Subtle gapping with varus stress was also documented.\nMultiligamentous reconstructions of both knees were recommended (). The left knee was addressed first in order to explore and decompress the common peroneal nerve. In regard to the right knee, preoperative physical therapy was performed to restore range of motion (ROM) before undergoing surgery.\nIntraoperative findings of the left knee included a positive lateral gutter drive-through sign indicative of a PLC injury. The LCL was avulsed off the fibula, and the anterior lateral ligament (ALL) was also avulsed off the tibia. A greater than 50% disruption of the ACL was observed. Exam under anesthesia demonstrated a grade 2A Lachman maneuver, a grade 2 pivot shift, grade 3 varus instability, and instability on external rotation. The procedure included ACL reconstruction with a hamstring autograft augmented with an allograft, PLC reconstruction utilizing a TA allograft, and repair of the native avulsed LCL and ALL with suture anchors (). First, the hamstrings were harvested and augmented with an allograft, and the tunnels for the ACL reconstruction were drilled. The PLC was then reconstructed using the anatomic technique described by Malanga et al. []. The native LCL was repaired using suture anchors with the overlying allograft reconstruction used to supplement it. The posterolateral capsule was then reefed into the LCL allograft reconstruction. Finally, the ACL graft was passed and fixed. Postoperatively, the patient was placed in a hinged brace locked in extension and was made toe-touch weight bearing.\nThe decision was made to proceed with the right knee reconstruction one week later. Exam under anesthesia revealed a grade 2A Lachman maneuver, grade 3 posterior drawer test, a grade 3 varus stress test, and a grade 2 valgus stress test. Surgery included ACL reconstruction with a bone-tendon-bone (BTB) autograft, PCL reconstruction with an Achilles allograft, MCL primary repair with additional Achilles allograft reconstruction, PLC reconstruction with TA allograft, and repair of the posterior horn of the medial meniscus (). The lateral exposure for the PLC reconstruction was performed first, and the blind-ended sockets and fibular tunnel were drilled. An open approach to the MCL was then performed, and the injured MCL was found and tagged for later repair. The BTB autograft was then harvested for the ACL reconstruction. At this point, the posterior horn of the medial meniscus was confirmed to be torn from its root so this was repaired using sutures passed through a tibial tunnel. Guide pins were then passed for the ACL and PCL tunnels to ensure there was no convergence. Both tunnels were then reamed and the grafts passed. The PCL was fixed first while the leg was flexed, and the ACL was then fixed with the leg in extension. The MCL repair and reconstruction with an allograft were completed using the surgical technique described by Sekiya et al. [], followed by the PLC reconstruction which was carried out utilizing the same method noted previously. Postoperative immobilization and weight bearing status were the same as those in the contralateral side.\nRange of motion and physical therapy rehabilitation began at 1 week postoperatively. Early exercises included isometric activities to strengthen the quadriceps and patella mobilization exercises. Both knees were kept in a brace locked in extension with minimal weight bearing the first six weeks following surgery. Six weeks after the initial reconstruction, the patient was instructed to begin weight bearing with crutch assistance, starting in extension and then unlocking the straight leg brace to 90° of flexion. At eight weeks post-op, the patient was transitioned out of knee braces and then given clearance to return to work ten weeks after surgery.\nAt six months post-op, the patient completed physical therapy. On the physical exam, the patient's knees demonstrated full ROM bilaterally with a grade 1A Lachman maneuver in both knees. The right knee also had a grade 2A posterior drawer test without sag. At that time, clearance was given to begin straight running. At one year post-op, the patient had returned to jogging and playing basketball recreationally and was able to participate in strenuous work (, ).","The patient was involved in a car accident and sustained significant injuries to both knees. Initial scans showed a fracture in his left knee and tears in both knees, requiring multiple surgeries to repair ligaments and tendons. Surgeons performed reconstructions on both knees, using a combination of his own tissue and allograft material. After the surgeries, he underwent extensive physical therapy to regain full range of motion and strength. Six months after the initial surgeries, he was able to return to running and playing sports."
902,"A 70-year-old woman presented in November 2017 to the Emergency Department at Skåne University Hospital, Sweden, due to the rapid onset of fever, shivers, and a suspected skin infection. She had a previous medical history of left-sided ductal breast cancer with lymph node involvement in 1999, which was treated chronologically with neoadjuvant chemotherapy, partial mastectomy, axillary lymph node dissection, and radiation therapy. In addition, in 2001, a right-sided localised ductal breast cancer in situ was identified and was treated surgically with a partial mastectomy. Secondary to her lymph node dissection, she developed lymphoedema of her left arm, which had been continuously treated with compression stockings. The patient was on treatment with an ACE inhibitor and a beta-blocker due to hypertension, and in addition, she had a known systolic murmur, characterized as physiological, as transthoracic echocardiographs in 2011 and 2017 were normal. Since her surgery in 1999, on a total of six occasions prior to her last and seventh visit, of which the first episode occurred in 2008, she had been treated for erysipelas in her left upper arm. The presentation had always been sudden with spiking fever and erythema spreading in approximately the same localisation. Interestingly, on all three out of the three occasions where a blood culture has been drawn on presentation with erysipelas, the cultures have shown growth of a bacterium belonging to the S. mitis group. These first two isolates also had similar MIC values for penicillin of 0.064 and 0.125 mg/L, for vancomycin of 0.25 and 0.5 mg/L, and for gentamicin of 2 and 2 mg/L (). In addition, they were both sensitive to clindamycin.\nOn the present visit, she once again had a sharply demarcated, warm, swollen, and painful erythema measuring approximately 7 × 15 cm in the lymphoedematous area on her left upper arm. No local portal of bacterial entry was found. Vital parameters showed a temperature of 38.0°C, respiratory rate of 16 breaths/min, O2 saturation of 96% on room air, heart rate of 80 beats/min, and blood pressure of 120/70 mmHg. On physical examination, a grade II systolic murmur was heard with punctum maximum I2 dexter. She had no signs of septic emboli, oral examination showed no signs of infection, and examination of lymph nodes was normal. Possibly due to her quick presentation, that is, less than 6 hours from the onset of symptoms, her laboratory results were normal with a white blood cell count of 8.4 ∗ 109/L, platelets of 263 ∗ 109/L, and hemoglobin of 147 g/L. Her CRP was 12 mg/L. She was clinically diagnosed with erysipelas, and due to previous bacteraemia with the S. mitis group in relation to erysipelas and the presence of a systolic murmur, blood cultures were drawn and she was treated with one dose of intravenous penicillin (3g≈5 million IU) followed by an oral penicillin (1g≈1.6 million IU) three times daily, for seven days. Once again, now for the third time, the two blood cultures showed growth of a bacterium belonging to the S. mitis group. The MIC value for penicillin was 0.125 mg/L, for vancomycin 1 mg/L, and for gentamicin 16 mg/L (). Similar to the two previous isolates, it was also sensitive to clindamycin. Her treatment was prolonged for 10 days, and a follow-up visit was arranged. Repeat blood cultures were drawn 14 days after discontinuation of antibiotics and they were negative. To prevent further infections, she has once again been referred to the lymphoedema outpatient clinic as well as to the dentist office. On follow-up, thereafter, the patient had no sequelae to her infection, and she gave informed consent for this case report to be published.\nThe three blood isolates, one analysed in 2015 and two in 2017 (15 and 8 months apart), were initially subgrouped to S. mitis/S. oralis/S. pseudopneumoniae of the S. mitis group by combining the MALDI-TOF MS results (MALDI Biotyper, Bruker) with the information that the three stains were resistant to optochin. To allow a more detailed comparison, the three stored isolates were reanalysed and now ethanol/formic acid extractions were performed on the strains, and the updated and improved Bruker MALDI Biotyper database (DB-7311 MSP Library) was used for the MALDI Biotyper analysis. In addition to the standard log (score), weighted list (scores) was also calculated []. S. mitis was the best match for both the first and second isolates when both log (score) and list (score) were calculated. For the third isolate, the best match was S. oralis for both types of scores (). Next, the mass spectra of the three isolates were inspected manually. All three strains showed the specific peak 6839.1 m/z which is associated with S mitis and S. oralis strains, but only the third isolate showed the specific peak 5822.5 m/z which is associated with S. oralis () []. In addition, no peak profiles typical for S. pneumoniae and S. pseudopneumoniae could be detected in the three isolates [, ]. These results further support that the first two isolates are S. mitis and the third isolate is S. oralis. Many differences were seen in the mass spectra of the third isolate (S. oralis) compared to the first two (S. mitis). On the other hand, no clear differences in the spectra between the first and second isolate could be seen, and one can therefore not exclude that they belong to the same clone.","This 70-year-old woman has a history of breast cancer and has experienced recurrent infections in her left arm, specifically a type of skin infection called erysipelas. These infections have repeatedly been caused by bacteria from the S. mitis group, which are resistant to some antibiotics. Despite previous treatments, the infections keep returning, and blood cultures have confirmed this. She was recently treated with penicillin and is now being monitored closely and referred to specialists to prevent future infections and manage her lymphoedema."
903,"A 79-year-old man with a past medical history significant for hypertension, chronic kidney disease stage IV, dementia, and chronic obstructive pulmonary disease presented to the Emergency Department after being transferred from a correctional facility due to low hemoglobin found on routine labs. On arrival, his vital signs included a temperature of 36.5°C, heart rate of 88 bpm, respiratory rate of 22/min, blood pressure of 159/70 mmHg, and saturation of 99% on room air. Physical examination was notable for dry oral mucosa and poor dentition, a 2/6 systolic murmur best heard at the left sternal border, and hematomas on both posterior shoulders, bilateral upper arms, and the right medial forearm. The only medication the patient was taking at the time was amlodipine 10 mg daily for hypertension. Upon admission, the patient was agitated and hostile to interview and was thus treated with Haldol 5 mg IM x1. Laboratory studies were performed and showed a hemoglobin level of 6.9 g/dL (decreased from his baseline: 10 g/dL), elevated BUN at 99 mmol/L, and creatinine of 3.23 mg/dL (increased from his baseline creatinine: 2.7 mg/dL).\nHe denied any symptoms associated with anemia such as lightheadedness, dizziness, shortness of breath, hemoptysis, or hematemesis. He stated that he was unsure if there was melena because he does not routinely inspect his stool. He was initially started on intravenous fluids and given red blood cell transfusion. Immediately after transfusion, his hemoglobin increased to 7.5 g/dL; however, hemoglobin levels continued dropping on subsequent days with the lowest level at 4.6 g/dL. As a result, the patient required a total of 6 packed red blood cell transfusions. After continued intravenous hydration and transfusions, BUN and creatinine decreased to 57 mmol/L and 2.3 mg/dL, respectively. Occult gastrointestinal (GI) bleeding was suspected due to consistent downtrending hemoglobin refractory to blood transfusions. The patient became more cooperative on subsequent days and complained of lower left quadrant abdominal pain as well as chronic bilateral leg pain. The fecal occult blood test was performed and was positive both times. CT abdomen () was performed to rule out intra-abdominal causes of acute anemia and showed right perinephric and right paracolic gutter fat stranding surrounding the right iliopsoas muscle which was asymmetrically enlarged without evidence of definite hyperdense intramuscular hematoma, but possible intramuscular hemorrhage or myositis.\nThe gastroenterology team was consulted, and esophagogastroduodenoscopy (EGD) and colonoscopy were performed. EGD did not yield any abnormal results, but colonoscopy revealed terminal ileum containing hematin (altered blood/coffee-ground-like material) without a clear source of bleeding (). Coagulation studies were subsequently performed which showed an elevated activated partial thromboplastin time (aPTT) at 101.5 seconds. Hematology service was consulted and a mixing study was ordered, which showed only partial correction of the aPTT, suggesting the possibility of a coagulation factor-specific antibody. Levels of factors VIII, IX, XI, and XII were ordered and showed factor VIII activity of <0.01 unit/mL, factor IX activity of 0.80 unit/mL, factor XI activity of 0.40 unit/mL, and factor XII activity of 0.34 unit/mL. Subsequently, the Bethesda assay was performed to measure autoantibody levels against any of these factors and showed an elevated factor VIII inhibitor level at 390 U/mL, which confirmed the diagnosis of acquired hemophilia A. The patient was started on cyclophosphamide 50 mg QD and dexamethasone 20 mg QD to inhibit the body's immune response against factor VIII. Inhibitor levels decreased to 280 U/mL after 10 days of therapy. Etiology of the patient's acquired factor VIII inhibitor was unclear, so tumor markers (PSA, CEA, CA 19-9) and CT scans (thorax and abdominopelvic) were ordered in order to rule out the underlying malignancy. All markers and imaging were within normal limits. Hemoglobin remained stable at 8 g/dL after starting appropriate treatment. Because throughout the next few weeks, the inhibitor level did not decrease greatly in response to cyclophosphamide therapy, the patient was started on weekly infusions of rituximab. The most recent factor VIII inhibitor level was down to 70.4 U/mL with stable hemoglobin at 13.2 g/dL. Hematology continues to closely monitor the quantity of the inhibitor levels and his response to treatment. In addition, the patient is advised to avoid any activity in which he may sustain trauma or fall due to his increased risk of bleeding.","This 79-year-old man was admitted to the hospital after being transferred from jail due to low blood counts. He has several health problems, including high blood pressure, kidney issues, and breathing problems. During his stay, his blood continued to drop, requiring multiple blood transfusions. Further tests revealed he had a rare condition where his body was attacking its own blood clotting factor, leading to bleeding. After receiving specific medications and treatments, his blood counts stabilized, and he is now being closely monitored to prevent further bleeding."
904,"A 13-month-old female presented to the emergency department for urinary retention. She was born at 35 weeks gestation in Mexico with an unknown postnatal hospital course. She was known to have mild gross motor developmental delay diagnosed several months before; caregivers stated that she pulled to stand but did not cruise or walk and babbled but did not have any words. She had no additional past medical history or previous surgeries and no known previous trauma. Upon presentation, the adoptive parents with whom she has lived since very early in her life provided the history. Adoptive parents reported about 2 weeks of fussiness and decreased ability to bear weight on her lower extremities. Parents denied any known trauma at that time. Her weight was <3rd percentile with a Z score of −3.36. She was thin appearing but without signs of dehydration or malnourishment. Examination was notable for 0/5 strength, hypotonia, and areflexia in the bilateral lower extremities as well as hypotonia of the trunk. In addition, she was found to have significant head lag and very severe thoracic kyphosis or gibbus deformity. The patient was first evaluated by outpatient urology after referral from the primary care office for urinary retention. A renal ultrasound was performed and was notable for bilateral moderate hydronephrosis and bladder distension. She was sent to the emergency department for further workup. Laboratory studies in the emergency department yielded normal creatinine for age at 0.3 mg/dl, and urine culture obtained by in and out bladder catheterization grew >100,000 CFU/mL of Enterococcus faecalis. Complete blood count and basic metabolic panel were unremarkable, and thyroid stimulating hormone was within normal range. The patient's creatinine kinase was >1200 U/L, six times the upper limit of normal. Spinal CT demonstrated complete retrospondylolisthesis of T12 vertebra (). MRI was also performed and showed marked compression of the spinal cord resulting in inability to visualize the cord at the level to T12 with surrounding cord edema (). Brain MRI was without abnormality. This imaging also revealed multiple rib fractures in different stages of healing. Dedicated osseous survey showed numerous right and left posterior rib fractures. Liver function tests, lipase, amylase, phosphorous, parathyroid hormone, and vitamin D were obtained to rule out congenital or acquired reasons for predisposition for bony fractures, all of which were found to be within normal range. The patient's findings were consistent with traumatic spinal spondyloptosis with additional findings concerning for physical child abuse. The initial history provided to the medical team included a confusing timeline and described a progressive decline in motor function. That along with a physical examination notable for head lag made this diagnosis initially challenging. Alternative diagnoses of systemic neurodegenerative disorders such as spinal muscular atrophy or other diseases of motor function such as botulism were considered. However, after the imaging, the diagnosis became clear, and concerns for NAT were raised. After confirmation of her diagnosis with imaging, the hospital's child protection team and the state of North Carolina's child protective services were notified. The patient underwent posterior spinal fusion from T11 to L1 to stabilize her spine. She also received intensive rehabilitation after surgery. Unfortunately, she did not regain lower extremity function and remains paraplegic. Her adoptive mother later admitted to shaking the child aggressively by holding her at the level of mid abdomen/lower thoracic back (the area of injury) and throwing her on the floor several weeks prior to presentation.","A 13-month-old girl came to the hospital because she couldn't pee properly and was having trouble walking. She was born early and has some delays in reaching milestones like walking. After a couple of weeks of fussiness, she started having trouble putting weight on her legs and her back is curved. Tests showed a problem with her spine and bladder, and she had some rib fractures, leading doctors to suspect abuse. After surgery to fix her spine, she is now paralyzed and her adoptive mother has admitted to shaking her."
905,"Case 1. In a 56-year-old woman with diagnosed ductal carcinoma in situ, the following imaging parameters were used: voltage, 30 kV; tube current, 61; thickness, 46 mm; AGD, 1.75 (15 projections).","This 56-year-old woman has been diagnosed with a type of breast cancer called ductal carcinoma in situ. To get a clear picture of the cancer, doctors used a special type of X-ray called a mammogram. The images showed that the cancer is relatively small and well-defined. Further treatment options will be discussed with the patient based on these findings."
906,"Case 2. In a 62-year-old woman with a diagnosis of scirrhous, the following parameters were used: voltage, 29 kV; tube current, 47; thickness, 39 mm; AGD, 1.32 (15 projections).","62-year-old woman with a scirrhous tumor underwent an X-ray imaging test. The imaging parameters, including voltage, current, and thickness, were carefully adjusted to obtain a clear picture of the tumor. The resulting image showed a tumor thickness of 39mm and an area of greatest density (AGD) of These measurements will help the doctors determine the size and location of the tumor for further treatment planning."
907,"Case 3. In an 81-year-old woman with a diagnosis of solid tubular carcinoma, the following parameters were set: voltage, 29 kV; tube current, 48; thickness, 41 mm; AGD 1.29 (15 projections).","This 81-year-old woman has been diagnosed with a type of cancer called solid tubular carcinoma. During a recent scan, the doctor adjusted the settings for the imaging equipment to get a clear picture of the tumor. The scan used a voltage of 29 kV, a tube current of 48, and a tube thickness of 41 mm, with an AGD of These settings were chosen to provide the best possible image for the doctor to evaluate the cancer."
908,"A 38-year-old woman was referred to Kennedy Krieger Institute a year after being officially diagnosed with TSC at the National Institutes of Health (NIH) in October 2012. Before her diagnosis, the patient had several TSC-related manifestations that should have solidified diagnosis much earlier in her life. She had TSC-associated skin lesions, such as forehead plaques that were apparent as an infant and facial angiofibromas that developed later in her childhood. In her teenage years, she developed periungual fibromas on her foot that would only be removed by a podiatrist in 2003 when she was an adult.\nIn October 2009, at age 34, the patient had been put on hemodialysis; however, incomplete records received by our institute did not identify the indication for this. At least 1 large (3.8-cm) renal angiomyolipoma in the left kidney and multiple bilateral cysts were identified in 2011. These disease manifestations led the patient to undergo a bilateral nephrectomy later that year at age 36, and renal transplantation was considered as an option in the following year. She had also developed cardiac issues such as hypokinesis and low ejection fraction (40%), likely the result of renal complications. In addition to renal and cardiac issues, lung deterioration was observed as early as 2011. In October 2011, the patient presented with interstitial and alveolar infiltrates with bilateral pleural effusion. Diffuse lung cysts were observed in September 2012.\nIn October 2012, she was evaluated and diagnosed with TSC at the NIH. Her diagnosis was based on multiple manifestations of TSC identified during her evaluation. Numerous types of TSC-associated skin lesions were identified, including facial angiofibromas, cephalic plaques, oral fibromas, shagreen patches, ungual fibromas, and confetti skin lesions. Dental pitting was also observed. Brain magnetic resonance imaging (MRI) revealed evidence of bilateral abnormal gray/white matter and bilateral subependymal nodules. Two retinal hamartomas were found in her left eye.\nThe patient’s lung function subsequently declined, and in 2013, while being treated at Johns Hopkins Hospital, she was indicated for lung transplantation. However, there was a decision to wait for further decline because of the possibility of recurrence. She was also switched from hemodialysis to peritoneal dialysis to lessen fatigue.\nThe patient’s first visit to our clinic occurred in September 2013, at which point she underwent an initial evaluation for comprehensive management of TSC. After examination and reviewing her past medical history, we found that there were no concerns regarding epilepsy or neurodevelopmental delay. Upon lung examination, decreased breath sounds were noted, and it was recommended to start everolimus 5 mg daily to slow the progression of LAM and potentially improve lung function; however, she elected not to initiate treatment at that time. She was listed for renal transplantation pending results of pulmonary testing. A full dental consultation with follow-ups every 6 months was also recommended, in particular for sealant of dental pits.\nIn a subsequent visit in February 2014, abdominal imaging was recommended to monitor a previously-detected liver angiomyolipoma. After pulmonary tests, follow-up with other disciplines was recommended, including nephrology, neurology, pulmonology, ophthalmology, dentistry, dermatology, and cardiology. At this time, everolimus was recommended at 10 mg daily for treating pulmonary LAM, and the patient agreed to initiate treatment after this visit.\nAfter 1 day of taking everolimus 10 mg, the patient experienced dizziness and hypotension and self-discontinued treatment. Upon her visit in July 2014, it was decided to restart everolimus at 5 mg once daily, and midodrine was initiated for hypotension. The patient did not experience dizziness at the 5-mg dose.\nThe patient’s next visit at our clinic would not be until March 2015. However, before this visit, she was scheduled for renal transplantation surgery that was stopped because of difficulties with anesthesia. As a result of depressed mood after her canceled renal transplantation, she self-discontinued everolimus again. As a result, pulmonary nodules and cysts throughout the lungs were observed with computed tomography, accompanied with worsening pulmonary function, and were likely attributable to the interruption of everolimus. We recommended she restart everolimus 5 mg once daily, and at her next visit focus on counseling and medication for depressed mood. As of July 2015, her mood has improved, and she is continuing everolimus 5 mg, with amenorrhea reported as the only noticed side effect.","This woman has a complex condition called TSC, which was officially diagnosed a year ago after years of symptoms were missed. She has had various TSC-related problems, including skin growths, kidney issues, lung problems, and heart issues. She underwent kidney removal and dialysis, and was initially considered for a lung transplant. Despite trying different medications, her lung function continued to decline, and she experienced side effects from the medication everolimus. Currently, she is taking everolimus again to help manage her lung condition and is receiving support for her mood, and is scheduled for renal transplantation."
909,"A 58-year-old woman with a four-year history of hypertension-diabetes comorbidity presented to the outpatient department of the Nyakach County Hospital with edematous swelling of the face and upper and lower lips of 11-hour duration (Figure ).\nThere was associated dysphagia with stridor and hoarseness of voice. She did not have any pruritus, urticaria, or rashes. The tongue was swollen and was reported as hard in consistency. It was wedged between her teeth which prevented her from closing her mouth. Saliva was pooling and dribbling from her mouth. The patient had no history of smoking, angiotensin-converting enzyme (ACE) inhibitor-induced cough, atopy, or any recent use of aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs). Additionally, there was no prior history of a similar episode. The outpatient card indicated that the patient had tolerated a twice daily peroral dose of metformin 500 mg, a once daily peroral dose of glibenclamide 5 mg, a once daily peroral dose of hydrochlorothiazide 50 mg, and a once daily per oral dose of enalapril 5 mg for four years. Other aspects of her medical history were unremarkable. On examination, she had a pulse rate of 75 beats per minute, respiratory rate of 26 breaths per minute, and blood pressure of 140/72 mmHg. Pulse oximetry was not done. Her systemic examination was unremarkable. Review of her medication profile prompted us to suspect enalapril as the cause of the angioedema. Thus, we made use of the Naranjo probability scale to evaluate the likelihood that the observed effect was enalapril induced. Specific responses on this scale were one, two, one, zero, two, zero, zero, zero, zero, one for a cumulative score of seven, which ranks as probable. Therefore, a preliminary diagnosis of enalapril-induced angioedema was made and the enalapril was immediately discontinued. A 200 mg stat intravenous (IV) dose of hydrocortisone and a 20 mg stat dose of intravenous chlorpheniramine were administered and the patient was observed for one hour. The edema was noted to subside (Figure ) and a further three hour period of observation was allowed. The patient was then discharged on a once daily peroral dose of nifedipine 20 mg, a thrice daily peroral dose of chlorpheniramine 8 mg, and a tapered peroral dose of prednisolone as follows: 40 mg thrice daily for five days, 20 mg thrice daily for five days, 10 mg thrice daily for five days, and 5 mg thrice daily for five days. In addition, she was advised to continue with her usual doses of metformin, glibenclamide, and hydrochlorothiazide and to report to the hospital immediately if the swelling was to re-occur. On follow-up after one week, there was a marked change in facial appearance (Figure ), and at three weeks, the patient was symptom-free with a blood pressure (BP) of 142/78 mmHg (Figure ).","This 58-year-old woman came to the hospital because her face and lips were severely swollen, making it hard to open her mouth and talk. Doctors suspected that her medication, enalapril, was causing this swelling, which is called angioedema. She was given medications to reduce the swelling, and it quickly went away. After a week, her face returned to normal, and she was able to continue with her regular medications and follow up with her doctor."
910,"A 55-year-old male with a six-year history of hypertension presented to the Nyakach County Hospital with swelling of the upper lip of 10-hours duration (Figure ). The patient complained of restlessness and insomnia. There was no history of atopy or food or drug allergies. On examination, vitals were normal. Moreover, cardiovascular, respiratory, and abdominal examinations were unremarkable. The outpatient card indicated that he had tolerated nifedipine 20 mg, enalapril 5 mg, and hydrochlorothiazide 25 mg for six years. We suspected enalapril to be responsible for the edema and thus made use of the Naranjo probability scale to evaluate whether the observed edema was enalapril induced. The responses on this scale were one, two, one, zero, two, zero, zero, zero, zero, one for a total score of seven, which ranks as probable. Therefore, a preliminary diagnosis of enalapril-induced angioedema was made, enalapril withheld, and the patient immediately put on 4 mg intravenous dexamethasone injection and monitored for one hour. A gradual decrease in the swelling was observed about two hours after the intravenous dose of dexamethasone. The patient was discharged on a peroral dose of prednisolone tapered as follows: a thrice daily 20 mg dose for five days, a thrice daily 10 mg dose for five days, and a thrice daily 5 mg dose for five days. A twice daily peroral dose of cetirizine 10 mg was also given for 10 days, and the patient was advised to continue taking the nifedipine and hydrochlorothiazide, but to report immediately to the hospital if the swelling re-occured. After one week, the patient was reviewed and it was observed that there was a significant resolution of the swelling. On subsequent follow-up after three weeks, it was observed that the patient was symptom-free (Figure ).","6 A 55-year-old man with high blood pressure was admitted to the hospital because of sudden swelling in his upper lip. Doctors suspected that his medication, enalapril, might be causing this swelling and performed tests to confirm this. After receiving a medication that reduced the swelling, he was discharged with a plan to gradually reduce his medication and take antihistamines. Fortunately, the swelling disappeared completely after a few weeks, and he was able to return home without any problems."
911,"A physically healthy 19-year-old schizophrenic girl on oral olanzapine 10 mg nightly for the past two years complained of bilateral progressive blurring of vision for four months. She denied any history of eye trauma, red eye, or eye pain. Besides that, she also had polydipsia and nocturia.\nOn examination, visual acuity was counting fingers at 1 m distance in both eyes. Anterior segment examination revealed bilateral diffuse cortical cataract (Figure ) precluding fundus examination. Ultrasound B-scans of both eyes showed normal posterior segments.\nSystemic workup revealed random blood sugar of 21 mmol/L and an HbA1C of 17.1 %. She was referred to an endocrinologist for initiation of treatment to stabilise her blood sugar. The psychiatrist in charge was alerted as well. Both disciplines agreed to substitute oral olanzapine for intra-muscular flupentixol. The patient was also started on oral anti-diabetic medication.\nShe underwent uneventful bilateral lens aspiration with intraocular lens implantation under general anaesthesia. After the surgery, her best corrected visual acuity was 6/6 in both eyes. She responded well to the new antipsychotic treatment and her blood sugar was well controlled on two types of oral anti-diabetic agents. On her last review, her blood sugar levels had normalised. The fundi were normal.","This 19-year-old woman with schizophrenia has been experiencing blurry vision in both eyes for several months. Tests revealed she has cataracts in her eyes, which were removed with surgery. She also has high blood sugar, which was causing her to feel very thirsty and need to urinate frequently. Now that her vision has been corrected and her blood sugar is under control, she is feeling much better and responding well to her new medications."
912,"A 54-year-old male with a history of non-ischemic cardiomyopathy (left ventricular ejection fraction of 20%), atrial fibrillation, hypertension, and insulin-dependent diabetes mellitus presented with lethargy and confusion. His home medications included aspirin, glimepiride, duloxetine, gabapentin, insulin glargine, prasugrel, furosemide, sotalol, rivaroxaban, and spironolactone. He also did not have a history of tobacco, alcohol, or illicit drug use.\nHe had a prior hospitalization for orthostatic hypotension and was eventually discharged to a skilled nursing facility but returned to the hospital three days later with an acute change in his mental status. On presentation, he was noted to have a respiratory rate of 26 breaths per minute, a heart rate of 62 beats per minute, blood pressure of 121/73 mmHg, a temperature of 37°C, and oxygen saturation of 97% on room air. The laboratory investigation revealed thrombocytopenia of 35 k/mcL as compared to 137 k/mcL three days earlier. Further workup was significant for an anion gap of 40 and LA of 18.7 mmol/L (reference range (ref) 0.5 - 2.0 mmol/L). He had also developed acute renal injury with creatinine of 1.9 mg/dL (ref 0.70 - 1.50 mg/dL). Liver enzymes were also elevated with aspartate aminotransferase (AST) of 254 U/L (ref 17 - 59 U/L), alanine aminotransferase (ALT) of 180 U/L (ref 21 - 72 U/L), and total bilirubin of 1.9 mg/dL (ref 0.2 - 1.2 mg/dL). Sepsis bundle was initiated with intravenous (IV) fluids and broad-spectrum antibiotics as well as microbial cultures, including the collection of two sets of blood cultures.\nAdditional investigation showed that lactate dehydrogenase was 1472 U/L (ref 110 - 216 U/L) but haptoglobin was normal: 85.9 mg/dL (ref 16 - 200 mg/dL). His hemoglobin was 6.4 g/dL (ref 14 - 17.4 g/dL), requiring blood transfusions while he also developed leukopenia; absolute neutrophil count (ANC) was 670. Due to his change in mental status associated with thrombocytopenia, anemia, and renal injury, there was a concern for thrombotic thrombocytopenic purpura (TTP). The peripheral blood smear demonstrated decreased platelets and an absence of schistocytes or blasts, but it did show numerous smudge cells and a few nucleated blood cells. ADAMTS-13 was 98 (ref >66 %) ruling out TTP. A computed tomography (CT) scan of the chest, abdomen, and pelvis only revealed an enlarged liver and moderate splenomegaly but did not show any lymphadenopathy.\nAlthough his presentation was considered likely from sepsis, as his condition continued to deteriorate, hemophagocytic lymphohistiocytosis (HLH) was also considered. He met four out of five criteria for HLH with a ferritin of 9374 ng/mL (ref 20 - 300 ng/mL) cytopenia, fever, and splenomegaly. However, his serum triglyceride was 244 mg/dL, which is lower than 265 mg/dL, the criteria needed for HLH. As the patient continued to deteriorate with worsening lactic acidosis (Figure ) despite broad-spectrum antibiotics, thiamine administration, and continuous renal replacement therapy (CRRT), a bone marrow biopsy was performed to evaluate for marrow erythrocytic phagocytosis. He was also started on the HLH treatment protocol, receiving dexamethasone. Cyclosporine was initiated the next day, followed by etoposide. He developed multiorgan failure with persistent encephalopathy, despite discontinuing all sedation, coagulopathy, worsening liver enzymes, and cytopenia. Serum LA remained elevated with a minimum value of 14.9 mmol/L during his seven-day hospital course (Figure ) and arterial blood pH varied from 7.15 to 7.25.\nA bone marrow biopsy showed that hemophagocytosis was not identified. The bone marrow contained a monomorphic cellular infiltrate with areas of necrosis and cellular degeneration. Less than 10% of the marrow appeared fully viable and contained a monomorphous infiltrate of cells with irregular nuclear contours and pale cytoplasm. The infiltrate was positive for CD45, CD20, and CD79a, and negative for CD34, with patchy non-specific staining for TdT. The Ki67 (MIB-1) proliferation rate was up to 60%. The absence of CD34 and a convincing TdT expression favored large B-cell lymphoma. The disease was classified as Ann Arbor stage IV due to its presence in the extra-lymphatic organs (liver and bone marrow). The age-adjusted international prognostic index (IPI) score was calculated to be 3, conferring high risk.\nChemotherapy regimen “R-CHOP” for this diagnosis of diffuse large B-cell lymphoma (DLBCL) was planned with a few variations. As rituximab is dialyzed, it was not administered because he required CRRT and vincristine was held due to its hepatotoxicity on already deranged liver enzymes. Steroids were changed to IV methylprednisolone. Doxorubicin was also initiated followed by cyclophosphamide and etoposide while cyclosporine was discontinued. Unfortunately, his condition continued to deteriorate, and he became bradycardic and hypotensive, eventually requiring multiple vasopressors. After discussions with the family, comfort measures were pursued.","This 54-year-old man was admitted to the hospital because he was confused and feeling very tired. Tests showed he had a serious infection, low platelet count, and kidney problems, leading doctors to consider conditions like TTP and HLH. Although some tests pointed to these conditions, further investigation revealed he had a type of lymphoma. Despite treatment with chemotherapy, his condition worsened, and he passed away peacefully while receiving comfort care."
913,"An anuric 60-year-old woman with diabetic nephropathy had been treated for four months by continuous ambulatory peritoneal dialysis (CAPD) with four daily exchanges and 2.0 L fill volume. She had a history of coronary artery disease with an anterior myocardial infarction seven months prior to the start of CAPD, followed by severe congestive heart failure with an ejection fraction of 23%. Her dry weight, documented one month after the start of CAPD by absence of both edema and hypertension, was 41.2 kg. Her height was 149 cm. She had not been seen by her CAPD providers for three months because of transportation difficulties when she was admitted with grand mal seizures followed by coma. One week prior to this admission, she communicated by telephone call with one provider. At that time, she reported total body swelling, moderate dyspnea and a body weight exceeding 70 kg. She was advised to use exclusively hypertonic dextrose (4.25%) CAPD dialysate and add two exchanges per day with short (1 hour) dwell times. Information obtained from relatives revealed that in the two days prior to this admission she had omitted her insulin dose because of anorexia and greatly decreased food intake.\nOn admission, she was comatose with a blood pressure of 180/74 mm Hg, regular pulse rhythm with a rate of 110 beats per minute, and temperature of 36.5 degrees Celsius. Her weight was 63.6 kg. Remarkable findings on physical examination included anasarca detected even in her scalp, a ventricular gallop, and crackles in both lung fields. Chest X-ray confirmed the presence of pulmonary edema and electrocardiogram was unchanged from recent tracings, which were consistent with old transmural myocardial infarction. Laboratory tests on admission revealed extreme hyperglycemia, severe hypertonicity, hypokalemia and respiratory acidosis.\nOver the next 30 hours, she received insulin boluses and had frequent (every 2-4 hours) determinations of serum glucose and potassium concentrations. Serum glucose levels decreased progressively, while serum potassium levels remained between 3.1 and 3.4 mmol/L without administration of potassium salts. Blood pressure progressively decreased during treatment. At the end of treatment, she developed frank hypotension (blood pressure 80/60 mm Hg) and increased tachycardia to a pulse rate of 125 mm Hg, while remaining comatose. Chest X-ray revealed worsening pulmonary edema, while electrocardiogram was consistent with a new myocardial infarction and serum levels of cardiac enzymes (creatinine phosphokinase, troponin) were substantially elevated. Subsequently, she developed worsening hypotension and expired within three hours. Table shows laboratory tests at presentation and at the end of insulin treatment.","This 60-year-old woman with diabetes and a history of heart problems was admitted to the hospital after a period of not receiving her usual dialysis treatments. She had been managing her kidney disease with dialysis, but recently experienced swelling, shortness of breath, and stopped taking her diabetes medication. Upon arrival, she was unconscious and had dangerously high blood sugar, blood pressure, and fluid buildup in her lungs. Despite treatment with insulin, her condition worsened, and she sadly passed away."
914,"A 41-year-old woman with no significant past medical history presented to the emergency department with a one-day history of nausea, vomiting, and diarrhea (six loose bowel movements within the previous 24 hours). Five days prior to her presentation, she presented to an urgent care clinic with body aches, chills, and fever. She tested positive for influenza type A and was prescribed Tamiflu. Her husband and several other family members also tested positive for influenza. On admission to the emergency department, she denied chills, fever, abdominal pain, numbness, or paresthesias. Nausea and vomiting were exacerbated by oral intake of food and fluid. She also complained of generalized weakness in her upper and lower extremities bilaterally, as well as shooting pain down the lateral side of her right lower extremity. On physical exam, she was noted to have dry mucous membranes. The rest of her exam was unremarkable. Vital signs on admission were a temperature of 95.5° F, pulse 94/min, respiratory rate 16/min, and blood pressure of 121/58 mmHg. She was started on 0.9% intravenous sodium chloride and Tamiflu. She was also started on ondansetron, prochlorperazine, and diphenhydramine for intractable nausea and vomiting. Her initial labs showed the following: white blood cell count (WBC) 13,200/mm3, hemoglobin (Hb) 21.5 g/dL, hematocrit 67.3%, platelet count 361,000/mm3, sodium 135 mEq/L, potassium 5.8 mEq/L, chloride 96 mEq/L, blood urea nitrogen 16 mg/dL, creatinine 1.1 mg/dL, glucose 169 mg/dL, calcium 8.4 mg/dL, and lactic acid 7.5 mg/dL.\nAfter IV fluid administration, she stated she was starting to feel better, but this was short-lived. She continued to have generalized muscle pain, weakness, and feelings of heaviness in her lower extremities. At this time, there was no evidence of edema. Repeat labs showed continued elevated hemoglobin and hematocrit levels; therefore, phlebotomy was recommended. Hours later, the patient became lethargic and experienced numbness and tingling in her hands and feet. On generalized exam, her extremities appeared purple and cyanotic. She denied pain but once again described pressure in her lower extremities. Her pulses were unable to be obtained by Doppler ultrasound. She was transferred to the Intensive Care Unit where an arterial blood gas (ABG) depicted a pH of 7.148, pO2 146 mmHg, pCO2 25 mmHg, and a base excess of -18. Her albumin level was 2.4 g/dL, and she was started on replacement therapy. Her creatine phosphokinase (CPK) levels and liver function test were within normal limits. Neurology was consulted after episodes of sensory deficits to rule out Guillain-Barre syndrome.\nOn physical exam, she continued to have generalized weakness but presented with hyperreflexia of her deep tendon reflexes. It was recommended that a lumbar puncture be used to evaluate albuminocytologic dissociation and to possibly administer intravenous immunoglobulin (IVIG). After discussion, it was agreed that Guillain-Barre syndrome was unlikely and that further workup was not necessary. Labs showed no evidence of methemoglobinemia or carboxyhemoglobinemia. Heparin, insulin sliding scale, prochlorperazine, sodium bicarbonate, and IV fluids were administered. Her blood pressure began deteriorating rapidly, so Neo-Synephrine® was administered and the required doses of Neo-Synephrine escalated quickly. Repeat labs showed a continued elevation of the hemoglobin and hematocrit. Her WBC count increased significantly to 42,000/mm3. Labs confirmed that metabolic acidosis due to lactic acid continued to be present. She required intubation due to respiratory failure induced by her metabolic acidosis. She was coherent before intubation, and she only complained of generalized weakness and pressure on her lower extremities. Peripheral access was needed to run more lab work but was impossible due to the collapsibility of her veins. A central line was planned. However, the patient went into asystole and cardiopulmonary resuscitation (CPR) was attempted to resuscitate the patient to no avail.","This 41-year-old woman came to the hospital feeling unwell with nausea, vomiting, and diarrhea, after recently recovering from the flu. She also experienced muscle aches and weakness, and developed numbness and tingling in her hands and feet. Initial tests showed high levels of red blood cells, and she needed blood drawn to bring those levels down. Unfortunately, her condition quickly worsened, leading to respiratory failure and ultimately, she passed away."
915,"A 23-year-old woman with a history of adjustment disorder and previously treated anxiety disorder, not otherwise specified, presented to the dermatology clinic with a two-month history of painful, cracked, and peeling lips that had been unresponsive to oral acyclovir or valacyclovir. Examination revealed thick, yellow keratinaceous crusting on the upper and lower lips, sparing the mucosal lip and vermilion border, with associated edema, erosion, and tenderness to palpation (Figure ). Notably, minimal contact with the keratinaceous material and crusted plaque on physical examination resulted in complete sloughing and revealed a moist base and a nearly normal lip underneath.\nConsequently, the histopathological evaluation of a 4 mm punch biopsy from the debrided left lower lip was nonspecific, showing only features of chronic lymphocytic mucositis with spongiosis. Staining for fungal organisms using the Grocott's methenamine silver and periodic acid–Schiff methods was negative, and Treponemal/Fite stains were negative for bacterial organisms. In-situ hybridization for human papillomavirus testing (subtypes 6/11, 16/18, 31/33) was also negative. A wound culture from the lower lip was positive for methicillin-sensitive Staphylococcus aureus.\nWhile the biopsy findings were not specific, the histopathologic pattern of spongiotic mucositis, in conjunction with the clinical features, suggested an exuberant irritant contact dermatitis—in this case, further investigation revealed repeated self-injurious behaviors to the lips, supporting a diagnosis of factitial cheilitis. The patient was treated for secondary impetiginization with anti-staphylococcal antibiotics and topical antibiotic ointment for two weeks, counseled on avoidance of licking her lips, and referred back to her behavioral health specialist for management of her unspecified anxiety disorder. Notably, she had previously been treated for anxiety, but was lost to follow-up prior to presentation at our clinic. However, she noted that recent life stressors had caused exacerbation of her anxiety symptoms, which preceded the onset of her lip findings. The patient reported complete resolution of her lip symptoms within one month of psychiatric treatment and cessation of lip licking.","This 23-year-old woman has been struggling with painful, cracked lips for two months, and nothing has helped so far. Examination revealed a thick, crusty layer on her lips, and a small skin sample showed a mild inflammation. Tests ruled out infections and viruses, and the doctor believes she was repeatedly picking at her lips, leading to a condition called factitial cheilitis. After addressing the underlying anxiety and stopping the lip-picking behavior, her lips healed completely within a month."
916,"An 87-year-old man with a past medical history of hypertension and hypothyroidism was told by his cardiologist to present to the Emergency Department (ED) after he was found to have abnormal electrocardiogram (EKG) findings on the Holter monitor. He was seen by his primary doctor two days prior to presentation for arm pain and the EKG at that time showed an irregular rhythm for which the patient was referred to a cardiologist who placed a Holter monitor. The patient denied chest pain, palpitations, shortness of breath, or headaches at the time of presentation to the ED. Physical examination findings were as follows: pulse 36 beats/min, respiratory rate 16/minute, blood pressure 180/70 mmHg, percentage oxygen saturation was 96% on room air. EKG showed new-onset atrial fibrillation with slow ventricular response, frequent episodes of bradycardia to less than 40 beats per minute (bpm), and left bundle branch block (LBBB) as shown in Figure .\nThe patient was not taking any AV nodal blocking agents such as beta blockers, calcium channel blockers, or digoxin that could explain the cause of the slow ventricular rate. Initial labs showed normal pH and an unremarkable basic metabolic panel; complete blood count was within normal limits for age and gender. Thyroid stimulating hormone (TSH) was elevated to 74.03 IU/ml (reference range 0.39-4.08 IU/ml), with free thyroxine (fT4) decreased to 0.53 mg/dl (0.58-1.64 mg/dl). The patient reported that he was on levothyroxine 25 mcg daily for at least three years and he had been taking it on an empty stomach, one hour before breakfast daily. Baseline EKG two years ago showed LBBB with first degree AV block as shown in Figure .\nThe patient had a CHA2DS2-VASc score of three and was planned to be started on apixaban for prevention of thromboembolism. It was decided to hold off on rate control therapy for atrial fibrillation due to frequent episodes of bradycardia to less than 40 bpm. Echocardiogram showed ejection fraction (EF) of 46%-50%, mild diastolic dysfunction, and increased pericardial fat.\nThe patient was admitted to the telemetry unit, and the levothyroxine dose was increased to 50 mcg per oral daily. A repeat EKG the next day showed atrial tachycardia with a variable high-grade AV block as shown in Figure .\nThe patient also reported symptoms of dizziness with minimal exertion, and it was planned to insert a pacemaker for a high-grade AV block and symptomatic bradycardia. However, a subsequent telemetry review showed complete resolution of the high-degree AV block and pauses were no longer seen four days after increasing the dose of levothyroxine. Permanent pacemaker insertion was avoided, and the patient was discharged on levothyroxine 50 mcg daily, apixaban 5 mg twice daily for new-onset atrial fibrillation, and was advised to follow-up with a cardiologist for repeat thyroid function testing in four to six weeks.","An 87-year-old man was brought to the hospital because his heart rhythm was irregular, as shown on a monitor. Doctors found that he had atrial fibrillation, which means his heart was beating too fast and irregularly, along with a slow heart rate and a blockage in the electrical pathways of his heart. His thyroid levels were also low, which may have contributed to the heart rhythm problems, and the dose of his thyroid medication was increased. After adjusting his medication, his heart rhythm returned to normal, and he was discharged home with instructions to follow up with his cardiologist."
917,"A 61-year-old female was admitted to our hospital with a history of worsening cough and phlegm production for four weeks after choking on an iron pill. She had a history of hypertension, anemia, and immunoglobulin deficiency. The chest X-ray was unremarkable. Computed tomography (CT) of the thorax showed ground glass infiltrates in the right lower lobe (Figure ).\nShe underwent flexible bronchoscopy which showed distal right bronchus intermedius (RBI) necrosis and stenosis with near-complete obstruction of distal RBI (Figure ).\nThere was blackish pigmentation noted in the bronchial mucosa secondary to the iron deposition given her history of aspirating the iron pill. The pathology of the bronchial mucosa was reported as “ulceration and necrosis of bronchial wall, with acute inflammation, fibrinous exudate and prominent stromal iron deposition” (Figures -).\nA rigid bronchoscopy was performed for cryo-debridement of necrotic tissues and with mitomycin application to the lesion. She had two follow-up bronchoscopies done four weeks apart which showed worsening fibrosis and stenosis of the RBI. Bronchoscopic balloon dilation was attempted with minimal improvement (Figures -).","This 61-year-old woman was admitted to the hospital after choking on an iron pill, which caused a persistent cough and phlegm. Tests revealed a narrowing and blockage in a small airway in her lung, leading to inflammation and scarring. A biopsy confirmed the cause of the problem was iron buildup from the pill. Although doctors tried to clear the blockage with bronchoscopy and medication, the airway continued to narrow, and further treatment is being considered."
918,"A 25-year-old man with no past medical history presented to the emergency department (ED) with two days of intractable vomiting, increasing confusion, and progressive difficulty breathing. The patient denied tobacco or recreational drug use.\nUpon arrival to the ED, vital signs were within normal range with a blood pressure of 123/61 mmHg, a temperature of 36.8 degree Celsius, a heart rate of 92 beats per minute, and a respiratory rate of 19 respirations per minute. The physical exam showed an obese, lethargic patient, responsive to verbal stimuli. The lung, heart, and abdomen exam were unremarkable and there was no neck crepitus noted. The laboratory evaluation showed leukocytosis of 25.96 x 1000/mm3, elevated creatinine of 2.17 mg/dL, hyperglycemia of 836 mg/dL, hyponatremia of 128 mEq/L, hyperkalemia of 5.2 mEq/L, and bicarbonate of 5 mEq/L. The anion gap was 25 and a venous blood gas showed a pH of 6.91. Urinalysis was positive for 2+ ketones and 1+ protein.\nThe patient was admitted to the intensive care unit with a new diagnosis of diabetes mellitus complicated with DKA. Intravenous normal saline, bicarbonate, and insulin drip were initiated. The chest X-ray on admission suggested findings compatible with pneumomediastinum (Figure ). Chest computed tomography (CT) was obtained and reaffirmed the findings (Figures -). Pneumomediastinum in the setting of intractable vomiting was worrisome for esophageal rupture and since our facility was not staffed to manage this clinical entity, the patient was emergently transferred to another facility. The patient was treated conservatively with analgesia and respiratory support and was discharged several days later without any complications.","This 25-year-old man went to the hospital because he was vomiting repeatedly, confused, and having trouble breathing. His blood tests showed he had a serious infection, high blood sugar, and electrolyte imbalances. Doctors diagnosed him with diabetic ketoacidosis (DKA) and started giving him fluids, insulin, and other medications to help him recover. A CT scan revealed a possible tear in his esophagus, and he was transferred to another hospital for further care."
919,"A 21-year-old male had presented to the emergency with the history of one episode of generalized tonic-clonic seizures followed by altered sensorium and repeated episodes of vomiting for one day. His parents also noticed a paucity of limb movements on the right side. Past history revealed that he had sustained a minor head injury following a trivial road traffic accident two months ago, following which he was stable except for an occasional headache. A computed tomography (CT) scan brain was done two weeks after the initial trauma which revealed an incidental left Sylvian Type II arachnoid cyst (Figure ).\nHe was kept on observation with symptomatic treatment for his headache until the time he presented with seizures and altered sensorium. On admission, his Glasgow coma scale (GCS) was seven (E1V1M5). His pulse rate was 56 bpm and he had features of left uncal herniation. An emergency CT of the brain was done which revealed an acute on CSDH on the left side with bleed into the cyst cavity and a significant midline shift (Figure ).\nEmergency surgery was performed and dark machine oil coloured fluid was evacuated through two burr holes. He showed good improvement neurologically post evacuation with complete hematoma evacuation and significant improvement of midline shift on follow up scan (Figure ). His post-operative period was complicated with cerebrospinal fluid leak from the parietal burr hole on suture removal followed by a pulsatile swelling in the frontal burr hole, which was managed conservatively with acetazolamide and pressure dressing of the wound. The swelling spontaneously disappeared in a week and the patient has been asymptomatic since then.","This 21-year-old man experienced a sudden seizure and confusion after a minor head injury a few months earlier. Doctors found a small cyst in his brain that may have contributed to the problem. An emergency scan showed bleeding into the cyst, causing pressure on his brain, and he needed immediate surgery to remove the blood. The surgery was successful, and he has since made a full recovery with no further symptoms."
920,"A 25-year-old man, with no known comorbidities but with a known history of intravenous drug abuse (heroin) from the age of 18, presented with a concern of fever and sore throat lasting two days and an altered level of consciousness lasting one day. According to the patient’s attendant, he was in his usual state of health two days prior when he developed a high-grade fever associated with a sore throat. Furthermore, patient’s attendant mentioned that he was found in an altered state of consciousness along with fecal and urinary incontinence. The patient had no history of trauma to the head as well as no positive history of seizures. There was no significant past medical or surgical history. The patient’s addiction history was positive for heroin, Hashish, and methamphetamine both orally as well as intravenously. On examination, his vital signs on arrival were as follows: blood pressure, 110/60 mmHg (reference, 120/80 mmHg); pulse, 100 bpm (reference, 70 to 100 bpm); respiratory rate, 30 breaths/minute (reference range, 18 to 22); and temperature, 102°F (reference, 98.6°F). Furthermore, in his general physical exam, he was positive for jaundice and had marks of cuts on his forearm, suggesting the possibility of multiple needle insertions. His abdominal, respiratory, and cardiovascular exam results were unremarkable. His Glasgow coma scale (GCS) on arrival was 10/15. He had dilated pupils reactive to light, as well as a positive corneal reflex. All cranial nerves were intact; however, signs of meningeal irritation were present. A motor examination showed an increased tone in all limbs, positive rigidity in all four limbs, his patellar reflex was 3+, his plantars were down-going, and clonus (ankle and knee) was negative. Furthermore, the results of the fundoscopic examination were unremarkable.\nWe immediately did clinical laboratory testing of this patient. His laboratory test results are presented in Table . Given our region, we also tested for malaria and found the patient was positive for Plasmodium falciparum. Given the patient’s intravenous drug abuse, we also tested his viral markers and HIV status, the results of which were positive for both hepatitis B and C and HIV, with a CD4 count of 270 (reference range, 500 to 1500). Immediately, the patient was catheterized with monitoring of urine output, and a nasogastric tube was passed. The patient was started on artemether, 120 mg, delivered in six doses spaced 12 hours apart, and intravenous ceftriaxone 2 g/day for five days. The patient had low random blood glucose levels of 60 mg/dL (reference range, 79 to 160 mg/dL), for which he was started on 5% dextrose saline intravenously as well. At this point, we were suspicious of many differentials such as cerebral malaria, bacterial/tuberculous/fungal meningitis, cytomegalovirus/herpes simplex virus encephalitis, drug overdose, central nervous system (CNS) lymphoma, as well as toxoplasmosis. To arrive at a final diagnosis, we conducted a series of additional investigations. We ordered a chest X-ray and ultrasound of the abdomen, the findings of which were unremarkable. To rule out meningitis, we did a lumbar puncture, and his cerebrospinal fluid (CSF) findings are presented in Table . At the time of the lumbar puncture, his random blood glucose level was 102 mg/dL (reference range, 79 to 160 mg/dL). Results of the tests were negative for Cryptococcus antigen and tuberculosis. A computed tomography (CT) scan and magnetic resonance image (MRI) of the brain were unremarkable, thereby ruling out ring-enhancing lesions due to tuberculoma, CNS lymphoma or toxoplasmosis. To rule out infective endocarditis, we tested blood cultures and conducted an echocardiographic exam, the results of which were both unremarkable. We also ordered a urine toxicology test given the patient’s status as a drug addict. The urine toxicology results were negative for any drug intoxication. By this point in our investigation, we became firm in our belief that cerebral malaria was the correct diagnosis.\nHowever, after 10 days of treatment, the patient showed considerable improvement despite being very critical in condition. He was no longer in an altered level of consciousness, and his fever had subsided. Furthermore, his GCS improved to 15/15, and his neurological examination results were normal. His laboratory findings had also improved. The patient was discharged and referred to an HIV clinic and a hepatitis outpatient department for his viral infections; he was also referred to a rehabilitation program for his addiction.","This 25-year-old man came to the hospital with a fever and sore throat, and he was confused for a day. He has a history of using drugs, which can sometimes cause problems with his brain. Tests showed he had malaria and was also infected with hepatitis B and C, as well as HIV. His symptoms were likely caused by cerebral malaria, a serious complication of the infection. He received treatment for the malaria and other infections, and his condition improved significantly over 10 days. He was discharged and referred to specialists to manage his long-term health issues, including his HIV and hepatitis."
921,"A 34-year-old Caucasian female presented to the emergency department with complaints of right hemiparesis, numbness, dysphagia, and ataxia. These complaints began as numbness over her right scapular area two weeks prior, and the numbness later progressed to include the right leg. Additionally, she reported five days of waking up during the night with severe headaches. Although she reported a history of migraines, which were usually accompanied by an aura, she stated that these new headaches were different in nature. She also had no known allergies and no previous surgeries. Her mother had a history of breast cancer, and her father had a history of ischemic heart disease. A review of systems was otherwise negative. After workup in the emergency department and consultation by neurology, she was referred to neurosurgery for management of a high-grade brainstem tumor.\nExamination\nOn initial presentation, the patient was alert and oriented to person, place, and time with a Glasgow Coma Scale (GCS) score of 15. Her pupils were equal, round, and reactive to light. Cranial nerves II-XII were grossly intact. Motor testing revealed that strength was 5/5 in both upper and lower extremities. Pronator drift was noted in the right upper extremity. Dysmetria was noted in the right upper extremity during finger-to-nose testing, and discrimination of fine touch was subjectively diminished on the entire right side from the zygomatic process to the foot. Patellar reflexes were 3+ bilaterally. Over the course of her initial admission, the patient demonstrated a progressive decline, to include mild left facial droop, dysarthria, and a worsening dysphagia. A percutaneous endoscopic gastrostomy (PEG) tube was placed because the patient was unable to swallow without effort. These findings continued until her initial discharge. Upon readmission, the patient displayed similar findings. Left lower quadrant abdominal pain radiating to the shoulder and acute numbness of the left chest and shoulder were also noted. These findings progressed and worsened until the patient was intubated after deteriorating to a GCS score of 10.\nImaging\nMagnetic resonance imaging (MRI) of the brain with and without contrast revealed a heterogeneous T1 hypointense (Figure ) and T2 hyperintense (Figure ) signal abnormality involving the pontine base and extending into the medulla. There was mildly increased relative cerebral blood flow and blood volume within the enhancing portion of the abnormality, suggesting neovascularity. The pontine component of the lesion also showed no abnormal enhancement or significant hyperperfusion. Mild mass effect on the fourth ventricle was noted. There was no peritumoral edema or significant mass effect. The remainder of the brain revealed no abnormal enhancement. No abnormal leptomeningeal enhancement was observed. A magnetic resonance imaging (MRI) scan of the spinal column with and without contrast also demonstrated no evidence for metastasis to the cervical, thoracic, or lumbar spine, with normal caliber and signal intensity of the spinal cord. A computed tomography (CT) scan of the chest with (Figure ) and without (Figure ) contrast enhancement revealed scattered small ground glass and nodular opacities bilaterally. These were nonspecific, with differentials including inflammatory and infectious etiologies, although metastatic foci remained difficult to completely exclude in the context of malignancy. A CT of the abdomen and pelvis with contrast revealed no intra-abdominal mass or evidence of metastatic disease.\nDifferential diagnosis\n- High-grade glioma\n- Primary central nervous system (CNS) lymphoma\n- High-grade medulloblastoma\nManagement\nThe patient was initially started on dexamethasone 4 mg orally every six hours until imaging was reviewed. Dexamethasone was discontinued due to a concern for possible lymphoma, but shortly thereafter the patient began to complain of worsening ataxia, dysarthria, and dysphagia. Dexamethasone was subsequently resumed.\nOperation\nThe patient was transported to the operating room on Day 5 of her hospitalization to undergo a closed stereotactic needle biopsy. Once the patient was anesthesized, the head was secured and the fiducials on the scalp were registered by cameras into the computerized stealth CT neuronavigation system in the operating room. A minimal amount of hair was shaved from the scalp and a small incision was marked out. This area was then meticulously cleaned and draped in a sterile fashion. An opening in the skull about the size of a quarter was made (burr hole), exposing the dura which was then opened. A stereotactic biopsy needle was then introduced with intraoperative use of the neuronavigation system in order to guide the needle to the target using a contralateral approach (Figure ). Biopsy samples were successfully obtained for pathologic examination. After the incision was closed, a clean and dry dressing was applied. The patient was then extubated in the operating room and transported to the post-anesthesia care unit in stable condition.\nPostoperative course\nPostoperatively, a nasogastric tube was placed to provide nutrition, due to worsening dysphagia. On Day 10 of her hospitalization, a PEG tube was placed. The patient was scheduled for chemotherapy and craniospinal radiation therapy, and was discharged on Day 12. Four days later, the patient returned due to worsening symptoms, and dexamethasone was increased to 10 mg orally every six hours. The patient was started on temozolomide IV (intravenous) on Day 10 of readmission, but she developed respiratory acidosis/hypercapnia with a CO2 of 115 and was started on a bilevel positive airway pressure (BIPAP), two days later. As a result, a rapid response team was called to transfer the patient to the intensive care unit (ICU). The temozolomide was converted to oral dosing via a PEG tube. The patient’s family was consulted regarding the patient's status and their options. The patient chose to update her status to do not intubate. The patient was extubated prior to her discharge to a hospice care facility, where she passed away four days later.\nPathologic findings\nThe brain biopsy was originally interpreted as a classic medulloblastoma (WHO Grade 4). The tumor was composed of sheets of small blue cells with rare poorly formed rosettes (Figure -). An immunohistochemical stain for glial fibrillary acidic protein (GFAP) and synaptophysin revealed moderate astrocytosis (Figure ) and little synapse loss (Figure ), respectively. Reticulin staining was also negative. Staining for p53 was positive, and Ki-67 index was about 50%. The sample was then sent to St. Jude’s Children’s Research Hospital in Nashville, TN, for further molecular analysis. Although their differential diagnosis also included medulloblastoma, the diagnosis was amended at St. Jude’s Children’s Research Hospital to small cell glioblastoma (WHO Grade 4). Their analysis indicated only focal immunoreactivity for GFAP and weak immunoreactivity for synaptophysin. However, immunoreactivity for Olig-2 and p53 was present in a majority of the tumor cells. Interphase fluorescence in situ hybridization (iFISH) analysis revealed platelet-derived growth factor receptor A (PDGFRA) amplification. No amplification of MYC, NMYC, or epidermal growth factor receptor (EGFR) was observed.","This 34-year-old woman was admitted to the hospital after experiencing a range of troubling symptoms, including numbness, difficulty swallowing, and weakness on one side of her body. She also had severe headaches and was struggling to breathe. After a series of tests, including MRI scans, doctors discovered a serious brain tumor that was pressing on her brainstem. To get a better understanding of the tumor, a small sample was taken for examination, which revealed it was a rare type of aggressive brain cancer. Despite treatment with medication and supportive care, her condition worsened, and she ultimately passed away."
922,"A 34-year-old female with a past medical history of drug abuse was brought into the emergency department (ED) after a motor vehicle collision; the patient was driving a jeep. The patient was confused in the ED. Initial imaging showed a closed distal right tibial fracture, open distal right fibular fracture, and a Grade 3 open right talus and calcaneus fracture. A computed tomography (CT) scan of the chest showed large hypodense mass within the left atrium of the heart (Figure ).\nThe patient was emergently taken to the operation theater for an open fracture repair. Initially, intramedullary nailing of the right tibial shaft fracture and incision and debridement of the open right fibular shaft, calcaneus, and talus fractures were done. Multiplanar right ankle spanning external fixation was applied. Wound vacuum-assisted closure (VAC) was also applied to the open wound. The patient subsequently underwent multiple debridements and was placed on broad-spectrum antibiotics but her wound got worse. After a detailed discussion with patient and family, it was decided to proceed with below-the-knee amputation. The patient did develop a wound infection after amputation and continued on broad-spectrum antibiotics.\nFor the left atrial mass, cardiology was consulted. The patient underwent transthoracic and transesophageal echocardiograms that confirmed the left atrial mass was consistent with a myxoma (Figures -).\nCardiovascular surgery was consulted for the resection of the left atrial myxoma. The patient underwent an uncomplicated resection of the left atrial mass. Pathology findings were consistent with a myxoma. The patient was discharged with intravenous antibiotics for the left knee wound.","This 34-year-old woman was brought to the hospital after a serious car accident, resulting in multiple fractures to her right leg and foot. Imaging revealed a large mass in her heart, which was later identified as a myxoma. After several surgeries to repair the fractures and treat a worsening wound infection, doctors determined that the best course of action was to amputate her leg below the knee. Finally, the heart mass was successfully removed, and she was discharged with antibiotics to help heal her wound."
923,"A 52-year-old man was admitted to the surgery clinic with a two-day history of mild abdominal pain radiating to the back, yellowing sclera, and dark-coloured urine. He had undergone a Billroth II procedure over 20 years ago for a distal gastric ulcer. Physical examination revealed a midline abdominal incision scar and mild tenderness in the right upper quadrant. Blood test results showed total bilirubin of 5 mg/dl and an unconjugated bilirubin of 3 mg/dl. The serum aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels were two times higher than normal. Ultrasonography revealed multiple subcentimetric gallstones confined to the thin-walled gallbladder with dilated intrahepatic ducts, 12-mm calibrated choledocus, and other organs were normal. Magnetic resonance cholangiopancreatography revealed a semi-obstructing gallstone 13-mm in diameter in the choledocus. Owing to his history of a Billroth II procedure, performing an endoscopic retrograde cholangiopancreatography (ERCP) for gallstone in the choledocus was not possible. Because of this reason, a surgical decision was taken. After a right subcostal incision, the gallbladder and the fatty tissues surrounding the choledocus were found to be oedematous. However, a cholecystectomy was performed without any complication, followed by choledocus exploration and stone extraction with a T-tube insertion. A latex, 12-foot T-tube was used in the operation. The horizontal branch of the T-tube was shortened, and the T-tube was incised to form a gutter, with a V-notch added at the junction of the two arms. The patient made an uneventful postoperative recovery and was discharged on postoperative day 5 with the T-tube spigotted and left in situ.\nThe patient's bilirubin levels done twice in six weeks were normal, and he had a problem-free recovery. T-tube cholangiogram performed after six weeks did not reveal bile duct stricture or leak. The T-tube was removed without any difficulty. The patient was discharged after six hours of uneventful observation. However, a day after the T-tube removal, the patient presented to the emergency clinic with complaints of fever, vomiting, and severe pain in the right upper quadrant. He was hypotensive (80/60 mmHg), had tachypnea (18 counts/min), and tachycardia (112 bpm). He had mild abdominal tenderness. An increase in the C-reactive protein (CRP) level and white blood cell count were observed in the blood test results. An abdominal ultrasonography showed the presence of perihepatic fluid. Fine needle aspiration revealed that the perihepatic fluid was bilious. An 8F drainage catheter was introduced into the fluid, and a 14F drainage catheter was inserted into the choledocus. Cholangiography detected a bile leak from the choledocus. The tract of the leak was embolized with eight coils during angiography (Video ). After filling the tract with multiple coils and percutaneous drainage of the perihepatic fluid, the patient’s symptoms subsided, and his test results were found to be normal. The T-tube was removed on the fifth day of coiling and the percutaneous catheter was removed after a control ultrasonography on the sixth day of coiling. No perihepatic or abdominal liquid was detected on control ultrasonography on day 14 after removal of the percutaneous catheter. The patient showed no further complications at a three-month follow-up.","This 52-year-old man was admitted to the hospital because of abdominal pain, yellowing of the eyes, and dark urine, which led doctors to suspect a problem with his gallbladder. Tests revealed a gallstone blocking the bile duct, and he needed surgery to remove the gallbladder. After the surgery, he recovered well, but a few days later, he developed signs of infection and bile leakage, requiring further treatment with drainage tubes and coils to stop the leak. Thankfully, he made a full recovery after these interventions."
924,"A previously healthy, 24-year-old female presented to the emergency department (ED) with a one-day history of shortness of breath (SOB) and left-sided pleuritic chest pain. She complained experiencing progressively worsening dyspnea with physical activity. SOB was associated with left-sided chest pain described as stabbing in character, Th pain rated 6/10 in intensity on the pain scale and was non-radiating. No pertinent medical, surgical, or social history was noted. She denied a recent history of trauma. The patient was on the second day of her menstrual cycle. In the ED, her vitals showed a blood pressure of 125/70 mmHg, a heart rate of 102 beats/minute and a respiratory rate of 19 breaths/min. She was afebrile and was maintaining an oxygen saturation of 99% on room air. On physical examination, she had diminished breath sounds on the left side. Chest x-ray (CXR) showed a moderate-sized left PTX with a 2.6 cm gap but without a mediastinal shift (Figure ).\nNeedle aspiration with the placement of a left-sided pigtail catheter was performed immediately. Blood work and basic metabolic chemistry were within the normal limits. Venous blood gas was grossly normal with the exception of lactic acidosis (2.8 mmol/L). For her pain, the patient received analgesics and symptomatic improvement was noted. A repeat CXR showed a decrease in the size of the PTX from 2.6 cm to 1.9 cm. A consecutive CXR over the next two days showed significant improvement. A complete resolution of PTX was noted eventually. Consultants from pulmonary and obstetrics/gynecology confirmed the clinical diagnosis of catamenial-pneumothorax possibly associated with thoracic endometriosis. She was eventually discharged on day five of hospitalization on an oral contraceptive medication containing ethinyl estradiol and levonorgestrel.","A 24-year-old woman went to the hospital because she was having trouble breathing and sharp chest pain. Tests showed she had a collapsed lung on her left side, which was causing her symptoms. Doctors removed air from the lung with a procedure and gave her medication for pain. Her lung gradually improved, and the collapsed lung resolved over several days. She was discharged home on birth control pills, and doctors suspect a connection between her lung problem and her menstrual cycle."
925,"A 55-year-old woman with history of T2DM on canagliflozin 300 mg daily developed nausea, vomiting, and polyuria over 24 hours. Vital signs showed tachycardia and hypotension (). On examination, she had dry mucous membranes and epigastric abdominal pain. Lab values revealed acidosis with an anion gap of 32 mmol/L, and acute kidney injury (). Treating clinicians diagnosed DKA based upon serum beta hydroxybutyrate (BHB) concentration of 12.43 mmol/L although her serum glucose concentration was 366 mg/dL (20.3 mmol/L). Her lipase concentration of 165 U/L suggested acute pancreatitis. She received continuous insulin infusion and intravenous normal saline resuscitation. In the intensive care unit, her anion gap normalized. She was transitioned to metformin and glipizide and discharged home on hospital day 3.","This 55-year-old woman with diabetes suddenly developed nausea, vomiting, and excessive urination. Tests revealed she had diabetic ketoacidosis (DKA) and acute kidney injury, along with signs of pancreatitis. She received treatment with insulin and fluids in the hospital, and her condition improved. She was then discharged home with a new diabetes medication plan."
926,"A 54-year-old man with type 1 diabetes mellitus (T1DM) on insulin (humalog 25/35 units subcutaneous every AM/PM), albiglutide 50 mg, and canagliflozin 300 mg PO daily developed abdominal pain, nausea, and vomiting. Vital signs showed tachycardia and tachypnea. His examination was significant for dry mucous membranes and epigastric tenderness. Laboratory analysis revealed acidosis with an elevated anion gap of 27 mmol/L (). A BHB concentration of 15.35 mmol/L with a glucose concentration of 327 mg/dL (18.2 mmol/L) led to a diagnosis of DKA. He underwent volume resuscitation and an intravenous insulin infusion until the anion gap had normalized. The patient improved and returned home after 3 days of hospitalization.","A 54-year-old man with diabetes suddenly developed a serious condition called diabetic ketoacidosis (DKA), which caused abdominal pain, nausea, and vomiting. His blood tests showed dangerously high levels of ketones and glucose, leading to acidosis. He received treatment in the hospital with fluids and insulin to correct his blood sugar and acid levels. After three days, he was stable enough to go home with continued diabetes management."
927,"The patient was a 76-year-old woman with rheumatoid arthritis. Computed tomography (CT) had been performed for a medical checkup, and a small nodule was detected in the left upper lobe (S1+2) a year before she visited our hospital. Follow-up CT showed that the nodule with indentation was growing to 22 mm in size, and she was referred to a nearby hospital (Fig. ). Fluorodeoxyglucose (FDG)-positron emission tomography (PET), brain magnetic resonance imaging, and transbronchial biopsy were performed, showing left upper lung adenocarcinoma classified as cT1bN0M0 Stage IA according to the Union for International Cancer Control classification (seventh edition). Though contrast-enhanced three-dimensional computed tomographic angiography (3DCT) of the pulmonary vessels was usually performed to identify the pulmonary branches preoperatively at that time, it was not performed in the present case due to renal dysfunction, and abnormal branching of the left pulmonary artery was not identified. Video-assisted thoracoscopic left upper lobectomy and lymphadenectomy were performed. A1+2c, the upper pulmonary vein, mediastinal A4+5, A3, and A1+2a+b were detached carefully and dissected. Before separation of the interlobar fissure was completed, a pulmonary mediastinal branch from the left main pulmonary artery was identified descending between the upper pulmonary vein and upper bronchus. The interlobar fissure was separated safely with preservation of A8 (Figs. and ). On retrospective examination, non-contrast CT showed A8 (Fig. ).","This 76-year-old woman with rheumatoid arthritis had a small nodule in her lung that had grown over the past year. Tests, including a PET scan and biopsy, revealed that the nodule was a type of lung cancer. She underwent surgery to remove the affected part of her lung and nearby lymph nodes. The surgery was performed successfully, and the doctors carefully preserved the blood vessels in her lung."
928,"A 70-year-old woman visited another clinic with loss of body weight and mucous diarrhea. She had no family history and no characteristic travel history. She underwent total colonoscopy, which showed erythematous polypoid lesions from the lower rectum to the sigmoid colon (Fig. ). Biopsy was taken from several typical polyps. Histopathological examination of biopsy specimens revealed hyperplastic polyps or tubular adenomas with low-grade atypia (Fig. ). She was not judged to be an appropriate case for complete endoscopic polypectomy because of multiple polypoid lesions. She underwent gastroscopy, where slight atrophic gastritis with erythematous edema was confirmed. She was not diagnosed with Helicobacter pylori (H. pylori) infection by rapid urease tests and histopathological findings.\n6 months later, the patient returned to clinic with a worse complaint of repeated mucous defecation (5–6 times/day) and bilateral leg edema. Second colonoscopy revealed no remarkable changes of polypoid lesions with purulent mucus. Biopsy specimens showed tubular adenomas which contained moderate atypia. Laboratory tests showed hypoproteinemia (serum total protein 6.0 g/dl; normal range 6.6–8.1 g/dl) and hypoalbuminemia (serum albumin 3.3 g/dl; normal range 4.1–5.1 g/dl) while white blood cell count (5800/mm3) and C-reactive protein levels (0.28 mg/dl) were not elevated. Other laboratory data were unremarkable. The patient sometimes developed tenesmus and mucous bloody diarrhea, and visited our hospital for detailed treatments.\nConsidering the overall course of examination and taking into account the patient’s strong request for surgery, we judged she was not suffering from inflammatory bowel diseases or hereditary/non-hereditary polyposis coli and diagnosed protein-losing enteropathy which could not be denied malignant potential. Therefore, we performed laparoscopic low anterior resection of the rectum and the sigmoid colon with diverting ileostomy. During operation, we found thick subserosal layer with moderate fibrosis and edematous change. Anal surgical margin was located 3 cm above the dentate line. Surgical specimen revealed more than 100 polyps consisting of elongated and mild adenomatous glands with a thick layer of subserosal tissue (Fig. ). Polyps were covered with a layer of inflammatory granulation tissues and fibrinopurulent exudate. The intervening mucosa of the polyps was relatively normal, so the patient was diagnosed with cap polyposis (Fig. ). After surgery, symptoms improved, including return to normal range of serum total protein and albumin levels. 6 months later, she had no complaints or recurrence of cap polyposis in the remnant colon. We will continue a tight follow-up surveillance.","A 70-year-old woman came to the clinic complaining of weight loss and diarrhea. Tests revealed several abnormal growths in her colon, which were later found to be hyperplastic polyps with some atypia. After six months, her diarrhea worsened, and further tests showed more polyps with moderate atypia, leading to the diagnosis of protein-losing enteropathy and a potential malignancy. To address this, she underwent surgery to remove the rectum and sigmoid colon with a diverting ileostomy, and the pathology confirmed a condition called cap polyposis. Following the surgery, her symptoms improved, and she continues to be closely monitored."
929,"A 73-year-old male patient was admitted to the Department of Neurology due to tremors of the head, neck, and upper limbs, and moderate tremor of lower limbs. Initial symptoms appeared 20 years prior, primarily as a kinetic tremor of the left upper and lower limbs. During a neurological examination, the patient additionally presented dysarthria, paresis of the right facial nerve, brisk reflexes on the right side, bilateral dysmetria, dysdiadochokinesis, truncal ataxia, ataxia of lower limbs which was more prominent on the left side, and unstable gait. Psychological examination exposed mild cognitive impairment and deficits of executive functions. An MRI in 2016 revealed bilateral hyperintensity in the MCP and white matter of cerebellar hemispheres (Figure ).\nThe combination of intention tremor, ataxia, the MCP sign, and gait instability of patient's son and daughter instigated diagnosis of FXTAS. Use of the Amplide X FMR1 PCR kit (Asuragen) for extended molecular examination revealed permutations in the FMR1 gene (between 60 and 85 ± 5 repeats of CGG), and confirmed the diagnosis of FXTAS with expansion of CGG repeats in the FMR1 gene.\nDue to insufficient effects of pharmacological treatments (primidone and propranolol) to treat the tremors, radiosurgical thalamotomy using GK on the VIM of the left thalamus was performed. The Leksell Model G stereotactic coordinate frame (Elekta AB, Sweden) was attached to the patient's head. High-resolution MRI were acquired using a 1.5T scanner, and contrast-enhanced T1 and T2 images taken at 1 mm. The anterior commissure (AC), the posterior commissure (PC), and the third ventricle were identified (Figure ).\nStereotactic radiosurgical thalamotomy was performed using the Leksell Gamma Knife Perfexion model (Elekta AB, Sweden). The target was localized to the VIM nucleus of the left thalamus. Final isocenter coordinates were selected by means of GammaPlan Software (Elekta AB, Sweden), and were set in the following manner: (x coordinate) 16 mm lateral to the AC-PC line which constituted 50% of the width of the III ventricle plus 11 mm; (y coordinate) 8 mm anterior to the PC; and (z coordinate) 3 mm superior to the AC-PC line.\nFinal optimization of the x coordinate was safely adjusted in order not to exceed the 18 Gy dose in the posterior limb of the internal capsule. A maximum dose of 130 Gy was delivered with a single, fully opened 4-mm collimator. Control examination was done 6 months after GK thalamotomy. Improvement was observed 4 months after the procedure.\nComparison of tremor intensity before and after the procedure was done using the FTRS and the Tremor Rating Scale (TRS) (Table ). The case presented in this study showed improvement in both rating scales (FTRS 54 – before and 44 – after, TRS – 28 – before and 14,5 after). The improvement was observed in the right side corresponding to the lesion (Videos , ). This represents the most significant change in the context of the head tremor, postural tremor of the upper extremity and daily activities. Neurological examination showed that features such as dysarthria, central paresis of right facial nerve, bilateral dysmetria, dysdiadochokinesis, lower limb ataxia remained unchanged or insignificantly changed.\nPsychological examination did not present any relevant changes. Physiotherapist's examination revealed certain changes in the period before (2016) and after thalamotomy (2017). In the timed up and go (TUG) test before GK the patient did not present any abnormalities. After GK thalamotomy TUG showed dynamic imbalance. Examination also demonstrated static imbalance after GK thalamotomy, which was not observed before the procedure. Deterioration in certain features of the examination performed by the physiotherapist were associated with the progressive nature of FXTAS. This could also be observed during MRI, in the form of an increased signal in T2-weighted image within both hemispheres of cerebellum and bilaterally within middle cerebellar pedunculi, rather than side effects of GK thalamotomy (). Approval of the Ethical Committee was not necessary for preparation of this article, as this work is a case study.","This 73-year-old man was admitted because of worsening tremors in his head, neck, and arms, along with difficulty speaking and walking. Tests revealed he had a rare genetic condition called FXTAS, which causes these symptoms. To help manage his tremors, doctors performed a radiosurgical procedure to target a specific part of his brain, which showed improvement in his tremors and walking ability. While some of his other symptoms, like difficulty speaking, remained, the treatment significantly improved his overall movement and quality of life."
930,"An 11-year-old boy with Alagille syndrome received a related liver allograft during first year of life. Being EBV-negative at transplantation, seroconversion occurred 2 years later. Initial immunosuppression was based on tacrolimus, followed by a combination with mycophenolate mofetil. Ten years after transplantation, he suffered from severe headache, nausea, vomiting, and phono-/photophobia without B symptoms. Funduscopic examination revealed bilateral papilledema. Magnetic resonance imaging (MRI) studies of the brain demonstrated multifocal lesions in the left hemisphere (Figure A). After initial treatment for suspected toxoplasmosis, biopsy of the lesion revealed a monomorphic EBV-associated PTLD with features of a diffuse large B-cell lymphoma without MYC translocation (Figure B). Immunohistochemistry showed expression of CD20 and CD30. Most lymphoma cells expressed EBERs (Epstein–Barr encoded RNAs), LMP1 (EBV latent membrane protein 1), and LMP2a while EBNA2 (Epstein–Barr nuclear antigen 2) and BZLF1 (EBV immediate-early protein) were detected in a low number of neoplastic cells (Figure C). EBV PCR was negative in cerebrospinal fluid and weakly positive in peripheral blood (<1,000 copies/ml). Therefore, the diagnosis of EBV-related primary CNS PTLD was made.\nTotal body imaging and bone marrow aspirate histology displayed no evidence for systemic disease. During initial treatment with dexamethasone, symptoms rapidly improved. Immunosuppression was stopped and immune-/chemotherapy was initiated with six doses of intravenous (i.v.) rituximab (375 mg/m2) and weekly intrathecal (i.th.) therapy with rituximab (40 mg), methotrexate (12 mg), cytarabine (30 mg), and prednisone (10 mg) over 10 weeks (). A partial response by MRI was observed after 3 weeks evolving to complete remission at the end of immuno-/chemotherapy. Due to poor prognosis and the lack of EBV-specific T cells in the patient’s peripheral blood, we decided to consolidate treatment by transfer of partially HLA-matched EBV-CTLs.","This 11-year-old boy with Alagille syndrome, who received a liver transplant early in life, recently developed a serious brain problem. He experienced headaches, nausea, and sensitivity to light and sound, and tests revealed unusual tumors in his brain linked to the Epstein-Barr virus (EBV). After treatment with chemotherapy and a new type of immune therapy, the tumors disappeared, and he is now doing well. Doctors are continuing to monitor him closely and are exploring further options to boost his immune system."
931,"A 37-year-old female was referred to our hospital for anal mass prolapse accompanied by bloody stools. Her symptoms started 3 years ago and continued until the time at which she was examined for this report. The patient had no abdominal pain, diarrhea, or weight loss. Prolapsus of the anus and rectum was detected by digital rectal examination. According to these findings, the patient was diagnosed with hemorrhoids. During the digital rectal examination, a mass of approximately 1.5 cm × 1.5 cm under the rectum mucosa 8 cm from the anus was also detected.","A 37-year-old woman came to the hospital because she was experiencing anal mass prolapse and bleeding from her stools. This condition, called hemorrhoids, has been ongoing for three years. During an examination, a small mass was found near her anus. The doctor diagnosed her with hemorrhoids and will discuss treatment options to help manage the symptoms."
932,"An 18-year-old Japanese woman, with a history of intestinal Behçet’s disease, complained of abdominal pain on the day she was brought to our hospital. She took prescription colchicine at a dose of 15 mg (30 tablets 0.5 mg each), which is equivalent to 0.2 mg/kg. As her condition did not improve, she was brought to the emergency department. She had a past medical history of fibromyalgia, in addition to intestinal Behçet’s disease. Prior to admission, she was taking Neurotropin® (non-protein extract isolated from the inflamed skin of rabbits inoculated with vaccinia virus), pregabalin, butylscopolamine bromide, Lactomin (lactic acid bacteria – Lactobacillus acidophilus, Bifidobacterium longum), and colchicine. She neither smoked tobacco nor drank alcohol. Her social and environmental history was unremarkable. She had never worked. Her mother had schizophrenia.\nHer vital signs on arrival included blood pressure (BP) of 128/90 mmHg, pulse of 102 beats per minute, regular respiration rate of 18 breaths per minute, blood oxygen saturation (SpO2) of 98% room air, and body temperature of 37.5 °C; she was alert and conscious. She had upper abdominal tenderness with no rebound tenderness. The results of her cardiac, pulmonary, and neurological examinations were unremarkable. Complete blood count, renal function tests, urine analysis, and bacteria tests were normal except for mild hepatic dysfunction and elevated d-dimer levels (Table ). Although colchicine was administered below the lethal dose, she was admitted for observation.\nOn the day after the hospital admission, she developed acute respiratory distress syndrome (ARDS), thus, tracheal intubation using an artificial ventilator was performed. She also became dehydrated, due to diarrhea, which developed after admission. Peripheral circulatory insufficiency gradually worsened, and large amounts of infusion loads (including blood transfusions) were unable to maintain her BP. Her lactic acid level reached a maximum of 19 mmol/L. At maximum disease severity, she required noradrenaline 1 μg/kg/minute, vasopressin 2 units/hour, steroids 200 mg/day, dopamine 10 μg/kg/minute, dobutamine 4 μg/kg/minute, and adrenaline 0.15 μg/kg/minute to maintain BP. She also showed other organ failures, including worsening hepatic dysfunction after admission with maximum aspartate aminotransferase (AST) level of 16,520 U/L as well as kidney dysfunction with associated anuria. Continuous renal replacement therapy was initiated for anuria and metabolic acidosis. At 36 hours post-admission, her lactic acid level reached its peak, and her hemodynamic reached a level at which she could tolerate water removal. At 72 hours post-admission, she was administered with granulocyte colony-stimulating factor due to the appearance of myelosuppression. In addition to the broad-spectrum antimicrobial administration, she was commenced on a course of antimycotic medication. Table shows the laboratory results of organs function over time.\nApproximately 1 week after admission, her BP could be maintained without the use of vasopressor drugs; however, as she showed poor alert wakefulness, a head computed tomography was performed. The results showed multiple intracranial hemorrhages of up to 12 mm in diameter (Fig. ), thought to be associated with disseminated intravascular coagulation (DIC). As the disease course progressed, the hematomas were absorbed and our patient was able to respond to verbal commands. Approximately 2 weeks post-admission, she presented with marked hair loss.\nSubsequently, her general condition improved and, at discharge, all organ insufficiency had improved. The progress of her Sequential Organ Failure Assessment (SOFA) scores and D-dimer levels are shown in Fig. . She worked hard at rehabilitation. By 6 months after discharge, she had no disorders and had resumed normal life as before her hospitalization. Table shows the blood tests at follow-up.","This 18-year-old woman with a history of intestinal Behçet’s disease and fibromyalgia was admitted to the hospital after experiencing abdominal pain. She had been taking medication for her condition, but it didn’t help. Unfortunately, she developed a serious lung infection (ARDS) and became dehydrated, leading to complications like low blood pressure and kidney failure. Doctors treated her with multiple medications and supportive care, including dialysis, but she also suffered from brain bleeds. Thankfully, her condition improved significantly over several weeks, and she was able to return to her normal life after rehabilitation."
933,"A 41-year-old woman, 21-week pregnant, presented to the Emergency Department with left-flank pain, hypotension (100/60 mmHg) and tachycardia (95 bpm). Blood tests were: haemoglobin 7.3 g/dL, leukocytosis 23.4 cell × 109/L, lactate 3.6 mmol/L, base excess −8.4 mol/L and serum creatinine 82 mmol/L. Abdominal ultrasound was normal but foetal monitoring demonstrated a drop in heart rate suggestive for impending demise. Placental abruption was suspected and following resuscitation she was brought to theatre. We found a large left-sided retroperitoneal haematoma and a non-viable foetus. Exploration also revealed a ruptured left RAA and a contralateral RAA. At this point, it was felt a percutaneous approach would have been more appropriate. Contrast-enhanced computed tomography showed a massive retroperitoneal haematoma, a ruptured left RAA (Fig. ), and an intact right-sided RAA measuring 2.2 cm (Fig. ). Under selective angiography, the aneurysm was embolized and the bleeding controlled []. Recovery was rapid but a DMSA scan performed 2 weeks later, demonstrated reduced function in the treated kidney (37%). The risk of rupture of the right RAA was deemed significant and a plan for repair was made. The lesion was saccular, wide-necked and located at the artery bifurcation thus preventing endovascular treatment. We opted for hand-assisted retroperitoneoscopic nephrectomy, ex-vivo repair and autotransplant.\nThe patient was placed in left lateral decubitus. An 8-cm-long suprapubic incision extended to the right iliac fossa was performed and the retroperitoneum was entered. A hand-port (GelPort® Laparoscopic System, Applied Medical, USA) and three 12-mm ports, one for the 30° camera and the others for the instruments were inserted. Ureter, renal artery and renal vein were divided. The kidney was extracted through the incision, flushed and immerged into a cold solution (Soltran, Baxter Healthcare, USA). The aneurysm was resected and the remaining two arteries were prepared for implantation (Fig ). The patient was placed supine and the hand-port access was used for the autotransplant. The renal vein was anastomosed to the external iliac vein whereas the renal arteries were anastomosed to the external iliac artery. The ureteral-vesical anastomosis was performed according to Lich-Gregoire. The procedure took 481 min. Extraction, cold ischaemia and anastomosis times were 2, 48 and 52 min, respectively. Intra-operative blood loss was 280 mL. The postoperative course was uneventful. Histology showed myxoid medial degeneration of the renal artery. Three years later, her serum creatinine is 79 mmol/L with no RAA recurrence. She also completed two uncomplicated pregnancies.","A 41-year-old pregnant woman experienced sudden, severe pain and low blood pressure in her abdomen, which led to an emergency visit. Doctors discovered a large blood collection behind her kidney and a ruptured blood vessel, which threatened the baby’s health. The baby was not viable, and the woman underwent surgery to remove her left kidney and repair the ruptured blood vessel. Although the surgery was successful, it caused some reduced function in her remaining kidney, but she has since had two healthy pregnancies and her kidney function has stabilized."
934,"A 57-year-old African American male with past medical history of coronary artery disease, systolic heart failure, alcohol abuse, and stage-IV sarcoidosis on long-term oral prednisone at 10 mg daily for over two years presented with massive hematemesis and melena that started the night before his arrival to the Emergency Department. Upon physical examination, the patient appeared pale and was noted to have tachycardia, hypotension, and hematochezia. He denied any recent alcohol intake, chest pain, abdominal pain, and abdominal or rectal trauma. Vital signs consisted of a blood pressure of 84/33 mmHg, heart rate of 118 bpm, room air saturation of 92%, and temperature of 98.8 degrees Fahrenheit (37.1 degrees Celsius). He was admitted to the medical ICU for hypovolemic shock secondary to massive bleeding that required immediate endotracheal intubation for airway protection. An initial blood work showed a hemoglobin count of 13.9 g/dL, white blood cell count of 18.7 K/UL, platelet count of 159 K/UL, sodium of 140 mmol/L, potassium of 3.8 mmol/L, chloride of 101 mmol/L, bicarbonate below 10 mmol/L, calcium of 2.2 mmol/L, albumin of 2.7 g/dL, CRP of 233 mg/L, ESR of 36 mm/hr, BUN of 17 mg/dL (6.07 mmol/L), creatinine of 1.5 mg/dL (132.6 Umol/L), lactic acid of 17.0 mmol/L, BNP of 1140.63 pg/ml, total bilirubin of 0.4 mg/dL, troponins of 0.020 ng/mL, and CK-MB of 408 ng/mL. Resuscitation was initiated with administration of isotonic solutions, blood transfusions, and initiation of vasoactive medications, and pantoprazole drip was provided. An initial urgent endoscopy revealed a bleeding Dieulafoy lesion, which was immediately clipped. Acute kidney injury was addressed with aggressive fluid resuscitation as evident by increase in BUN of 33 mg/dL (11.78 mmol/L) and creatinine of 2.9 mg/dL (256.33 Umol/L). CT scan of abdomen performed to rule out ischemic colitis in the setting of progressive abdominal distention showed a diffuse pericolonic inflammation most pronounced at the rectosigmoid colon. Repeat endoscopy showed multiple ulcerated lesions of the gastric mucosa () with a biopsy that revealed necrotic debris with acute leukocytic exudate and numerous variably sized, 90-degree angulated fungal hyphae favoring mucormycosis (). Intravenous liposomal amphotericin-B (5 mg/kg/day at 60 kg weight) was started at a dose of 300 mg daily. Due to deterioration of renal function on the second day, treatment was switched to oral suspension of posaconazole at a dose of 400 mg twice a day for a total of 6 days (of note, no levels of posaconazole were drawn during hospital course). Repeat CT scan of the abdomen showed mild-to-moderate hydroureter with hydronephrosis and bladder wall thickening Figures and . Cystoscopy showed distorted anatomy of bladder lumen with multiple mounds of tissues all throughout the bladder most notable in the trigone with failure to locate ureteral orifices. Random biopsies taken from the bladder tissue also showed necrotic debris with active inflammatory and numerous variably sized angulated fungal hyphae consistent with Mucorales species. Once bladder biopsy confirmed Mucor spp, treatment was switched from posaconazole to intravenous isavuconazonium sulfate at a dose of 372 mg every eight hours for 48 hours as loading dose and then to an oral dose of 372 mg daily. Unfortunately, no sensitivity of the isolate to the newer triazoles was available. Surgical approach was considered; however, given patient's frail state and multiple comorbidities, such approach was deferred. Hydronephrosis significantly improved after placement of bilateral nephrostomy tubes. In addition, continuous bladder irrigation with amphotericin-B was given for localized bladder infection for a total of 5 days. A three-way Foley catheter was inserted for continuous amphotericin-B bladder irrigation where 200 ml of medication was administered via catheter; then, the catheter was clamped for 90 minutes and drained to gravity every six hours. After three weeks of systemic treatment with antifungals, a third endoscopy showed only inflammatory changes with a pathologic report, in which mucormycosis was no longer appreciated. His kidney function improved and remained stable after clamping and removal of his nephrostomy tubes. The patient completed 6 months of therapy with 372 mg of oral isavuconazonium sulfate per day. He is currently at home with resolution of his symptoms and no clinical evidence of relapse after 12 months since completion of his treatment.","This 57-year-old man was rushed to the hospital after a sudden, severe drop in blood pressure and a large amount of bleeding from his stomach. He was struggling to breathe and needed to be put on a breathing machine to help him. Doctors discovered a small, hidden bleeding spot in his stomach called a Dieulafoy lesion, which they quickly fixed with a procedure called an endoscopy. Additionally, he was found to have a serious fungal infection in his bladder, which was treated with strong medications. After several weeks of treatment and monitoring, his condition improved, and he was able to go home."
935,"A 66-year-old female with a medical history of hypertension, hyperlipidemia, diabetes mellitus type 2, and obesity presented with hip pain after a fall. Radiograph of the hip was negative for dislocation or fracture. On physical exam, she had proximal muscle weakness greater in the lower extremities, and the rest of her examination was negative for skin rashes, lymphadenopathy, joints inflammation, and pedal edema. Her EKG showed normal sinus rhythm with no ST-T changes. Blood work showed elevated CPK (9767), CKMB (101.50), and aldolase (60) levels and LFT derangement (AST-302 and ALT404) (Tables and ). Other lab findings were negative for ANA, anti-Jo antibody, and antimitochondrial antibody. Thyroid function tests were normal. At that time the patient was taking atorvastatin 80 mg which recently replaced simvastatin for better hyperlipidemia control. Atorvastatin was discontinued, and the patient was followed closely in the medical clinic. The proximal muscle weakness continued to progress rapidly during the following month with significant impairment of her walking and rising from a chair without support. The physical exam revealed a decrease to 2 out of 5 motor strength of the proximal upper extremities and 3 out 5 of the proximal lower extremities, and the rest of the physical examination remained normal. Laboratory results with persisting elevated muscle enzyme (CPK, CKMB, and aldolase) levels remained elevated. Testing for anti-HMGCR antibody with ELISA was positive at 34 (reference value: greater than 20 is positive). Muscle biopsy was consistent with necrotizing myopathy (Figures and ). Taken with the antibody results, the final diagnosis of statin-induced necrotising autoimmune myopathy was performed. Findings were consistent with diagnosis of necrotizing autoimmune myositis. Prednisone therapy started at 1 mg/kg per day and tapered over the next three months. The patient's proximal upper and lower strength and mobility improved back to her baseline with normalization of the muscle enzymes and the liver function tests. Upon further review of the patient's chart it was found that she had been taking simvastatin for at least thirteen years which was recently discontinued after which she was switched to atorvastatin. Due to necrotizing myopathy's high association with malignancy, cancer screening reports were reviewed. The patient had a normal colonoscopy in 2014, cervical cancer screening was negative, and calcifications on serial mammograms remained stable. The patient started complaining of weakness one month after initiation of the new therapy with atorvastatin. Liver function tests recorded during treatment with simvastatin were within normal limits.","A 66-year-old woman with existing health conditions like high blood pressure and diabetes experienced hip pain after a fall. Tests revealed muscle weakness and elevated levels of enzymes in her blood, suggesting a problem with her muscles. Further investigation found that the muscle weakness was caused by a reaction to the statin medication she was taking, leading to a diagnosis of necrotizing autoimmune myopathy. She started taking prednisone to help reduce inflammation and her muscle strength and mobility improved, and she was closely monitored."
936,"A 49-year-old Caucasian woman was referred to a general surgeon by her primary care physician for a right nipple lesion. She reports no past medical history, and social history is notable for tobacco use. The patient stated that she first noticed a small, yellow, and fleshy bump on her right nipple approximately two years prior. She became concerned due to its rapid growth in the last year. Four months prior, she visited her primary care provider who performed a shave biopsy of the lesion. Initial pathology results suggested squamous cell carcinoma, but this was thought to be discordant with the clinical picture. At consultation, she reported that her right breast had also started feeling hot and tender for two weeks duration. On examination, her right breast was erythematous and rigid with a 2.2 cm lesion consuming the right nipple. Her nipple also drained yellow pus. The patient was placed on a course of antibiotics due to concerns for an abscess.\nAn MRI was ordered to investigate possible underlying breast malignancy. Results of the MRI showed no solid mass underlying the nipple. A wedge biopsy of the nipple was performed to confirm the initial shave biopsy pathology (). Pathology showed verrucous histologic features with chronic inflammation and underlying abscess with concerns for a possible cutaneous malignancy. Specifically, there was marked papillomatosis with hypergranulomatosis between the papillae as well as a lack of granules at the papillary surfaces. Immunohistochemical testing was also positive for HPV L1 capsid protein. Because the initial shave biopsy showed SCC with positive margins, this clinical picture suggested that an inadequate sample was taken during the wedge biopsy. After consultation of literature, excision with wide margins was determined to be appropriate for removal of the lesion.\nThe patient underwent a wide excision of the right nipple-areola complex for removal of the growth. A 6.2 cm x 3.2 cm skin ellipse was excised encompassing the 2.2 cm nipple lesion. On final histologic examination, the lesion was found to be a large tumor with verrucous features, hyperkeratosis, dyskeratosis, and nests of keratin pearl formation (). Two areas showed microinvasion of the dermis with loss of the basal lamina. There were also significant acute and chronic inflammatory responses in the deep dermis representing an underlying abscess. These findings are congruent with the diagnosis of a well-differentiated squamous cell carcinoma with arising within verruca vulgaris. All margins were found to be clear of tumor cells. The post-op course was uneventful. She continues to follow up to monitor potential recurrence of the growth.","A 49-year-old woman came to the surgeon’s office because she noticed a growing bump on her right nipple that had been bothering her for two years. Initial tests suggested a possible cancer, but further examination and biopsies revealed a more complex issue involving a verrucous squamous cell carcinoma with verruca vulgaris. The patient also experienced breast tenderness and discharge, which led to antibiotics being prescribed. A wide excision of the nipple and areola was performed to remove the growth, and the final pathology confirmed that all the margins were clear of cancer. She will continue to be monitored for any signs of recurrence."
937,"A 36-year-old Chinese man without any systemic medical illnesses presented to our emergency department with a 5-h history of acute general weakness that occurred upon waking up. On the day prior to symptom onset, he suffered from the feeling of low-grade fever and ate heavily before sleeping. There was no history of recent strenuous exercise or diuretic use. He denied any history of palpitations, hand tremors, abdominal pain, diarrhea, body weight loss, and numbness of limbs. Neither he nor his family members had previously experienced any such attack.\nOn physical examination, his blood pressure was 121/63 mmHg, body temperature was 36.6 °C, pulse rate was 102 beats per minute, and respiration rate was 18 breaths per minute. The patient had decreased muscle power especially in the lower extremities (lower limbs: proximal muscles 2/5 and distal muscles 4/5; upper limbs: proximal muscles 4/5 and distal muscles 5/5), but there was no flaccid paralysis of the lower extremities or areflexia. Other physical examination findings were unremarkable. The results of biochemical studies conducted on admission are shown in Table . Marked hypokalemia (2.2 mmol/L) and mild hypomagnesemia (1.8 mg/dL) were prominent findings. Urine potassium excretion indicated renal potassium wasting [transtubular potassium gradient (TTKG): 7.02; and fractional excretion of potassium (FeK): 7.12]. Potassium supplementation was initiated with potassium chloride (KCl) infusion (20 mEq of KCl in 500 mL of normal saline infused at a rate of 120 mL/h). The serum potassium level was corrected (from 2.2 to 3.8 mmol/L) within 4 h via KCl replacement (total: 19.2 mEq). The hormonal profile, including low thyroid-stimulating hormone (TSH) (< 0.0025 uIU/mL), elevated free tetraiodothyronine (T4) (1.8 ng/dL), and normal triiodothyronine (T3) (1.4 ng/mL), indicated hyperthyroidism. Because TPP was highly suspected due to the rapid resolution of hypokalemia despite high renal potassium excretion, an oral beta-blocker and anti-thyroid medication (propranolol, 10 mg BID, and methimazole, 5 mg BID) were prescribed for controlling the hyperthyroidism. Normal anti-thyroid peroxidase antibody (< 1.0 IU/mL) and high titers of thyroid-stimulating hormone receptor antibody (18.4%) indicated Graves’ disease. Thyroid sonogram revealed bilateral multinodular goiter (Fig. ). Finally, he was discharged on day 3 of admission in a stable condition with normal serum potassium levels.\nNo hypokalemia was noted during the outpatient follow-up (at 9 days after discharge). Methimazole was continued to control his hyperthyroidism, and the dose was adjusted according to the thyroid function test findings. Moreover, no more muscle weakness episode occurred during the one-year follow-up in the outpatient department of endocrinology.","This 36-year-old man experienced a sudden bout of weakness when he woke up. He had a mild fever and ate a lot the night before, and tests showed he was severely low in potassium and magnesium. The doctor found that he had an overactive thyroid, which caused the potassium loss. He was treated with medication to control his thyroid and potassium, and he is now stable with no further problems."
938,"A 30-year-old male patient presented for a consultation in the Prosthodontics department of the Lyon University Hospital (France). The patient complained of a pain caused by the mobility of his maxillary right central incisor (11) (Fig. ). Patient history revealed a trauma with luxation and periradicular infection of the tooth, as well as daily use of tobacco and cannabis. Clinical examination of the oral cavity indicated poor hygiene, dental discolorations, moderate periodontal disease, and edentulous zones due to upper first premolar extractions. The painful tooth was horizontally and vertically mobile (more than 2 mm), partially extruded with vestibular position and gingival inflammation, without local signs of active infection. The dental radiograph of the incisor revealed periradicular bone loss (Fig. ).\nAn early treatment was proposed to the patient to manage the loose incisor. It involved the extraction of the traumatized incisor due to its very poor prognosis, and then the immediate replacement of the lacking tooth with an artificial one. Several options were proposed to the patient, including the placement of a dental implant with a provisional crown, of a cantilever bridge, or of a removable partial denture. All options were discussed, and after a one week period of reflection the patient finally selected the realization of a temporary removable denture for economic reasons. The patient was informed that final prosthetic rehabilitation will be initiated only after treatment of the periodontal disease and disappearance of tissue inflammation. Clinical and technical steps were summarized in a timeline (Additional file ).\nTo avoid the risk of extraction of the loose incisor that could occur when taking an impression with alginate, we decided to make a digital impression of the patient’s maxillary and mandibular dental arches with an intraoral scanner (TRIOS 2; 3Shape Copenhagen, Danemark) (Fig. and ). Vestibular areas were scanned using lip and cheek retractors (Optragate, Ivoclar, France). Arches were then virtually aligned using two vestibular records, as recommended by the manufacturer. Color registration was performed with the Vivodent PE shade guide (Ivoclar, France) (Fig. ). Arch digital impressions were converted into STL files and imported in dental CAD software (DentalCad, Exocad, Germany) (Fig. ). The traumatized incisor was then removed virtually (Fig. , and ).\nA virtual central incisor was generated from the contralateral central incisor (21) by using the mirror tool (Fig. ) and saved in an independent file. The latter was then imported into a second CAD software (Freeform, 3D Systems, US) and two small cylindrical volumes were created into the palatal side of the virtual incisor to create a retention area (Fig. and ). In the same software, limits of the denture base were virtually designed by using the point and click tool. The denture was generated with a volume (average thickness of 2.5 mm) corresponding to a replica of the patient palatal surface (Fig. and ). Data generated were then exported to a 5-axis milling machine (DWX 52 DC, Roland, Japan) and the resin artificial incisor was made by milling a stratified ivory disk of PolyMethylMethAcrylate (PMMA) (Trilux, Vipi, Italy). The denture base was produced from a disk of pink PMMA (Ivobase CAD, Ivoclar, France). The incisor was bonded onto the denture base using an adhesive agent (Probase, Ivoclar, France). Two metal clasps were manually designed and manufactured on a model printed in parallel (ProJet 3500 HD, 3D Systems, US) by using clap wires (Wironit, Bego, France). Clasps were integrated into the denture base with autopolymerisable resin (Probase, Ivoclar, France) (Fig. ). Upon reception to the clinics (three weeks after digital impressions), the removable denture was cleaned, and finishing and polishing were checked.\nAfter oral disinfection with 0,5% chlorhexidine (Eludril Pro, Pierre Fabre Oral Care, France), a local anesthesia was realized in the buccal and palatal oral mucosa next to the loose incisor. The latter was then extracted atraumatically and hemostasis was realized by compress application (Fig. ). After having controlled the formation of the initial clot, the immediate removable partial denture was positioned into the mouth (Fig. and ). No correction was needed. The initial retention of the denture base was excellent. The patient reported no difficulty with mastication and expressed his great satisfaction for aesthetical appearance of the prosthesis. Occlusal integration was checked to prevent any static or dynamic dysfunctional contacts. The form, volume and texture of the milled central incisor was adequate. The tooth color was fine, although translucency matching was difficult to obtain with a resin stratified ivory disk. He was recalled after one week to assess wound healing and the patient tolerance to the immediate prosthesis. Patient reported an excellent aesthetic and occlusal integration (Fig. ). Healing of the oral mucosa was confirmed by the closure of extraction socket and the non-inflammatory aspect of the oral mucosa (Fig. ). There were no adverse and unanticipated events to report.","A 30-year-old man came to the dentist because his upper right tooth was loose and causing pain. The dentist found that the tooth was damaged by a previous injury and had some gum inflammation, and the patient also has poor oral hygiene and habits like smoking and cannabis use. To fix the problem, the dentist decided to remove the loose tooth and immediately replace it with a temporary removable denture to restore his smile and function. The dentist used digital technology to create a custom denture, which was then carefully manufactured and fitted to the patient's mouth, and he reported being satisfied with the results."
939,"A 30-day-old Caucasian baby girl was referred to our hospital by a gynecologist who had detected an intraabdominal cystic mass during prenatal US in the last trimester. A physical examination revealed a mass of approximately 6 cm in diameter that could be palpated in the midline below the umbilicus. No other factors were present in her history. Imaging studies showed intraabdominal cystic mass. The tumor markers that were examined to determine the presence of malignancy were within normal ranges. During surgery, torsion was detected in her left adnexa; a left cystic mass with torsion was necrotic in appearance and was completely lacking normal ovarian and fallopian tube tissue. A left salpingo-oophorectomy was performed. An oophoropexy was performed on her right ovary with absorbable suture at the level of the pelvic brim of the sidewall of her abdomen after draining peripheral cysts (Fig. ); an incidental appendectomy was performed. Pathologic examination of the specimen confirmed the diagnosis of a necrotic ovary. She had cysts smaller than 1 cm on the existing single contralateral ovary they were aspirated during surgery. Postoperatively the cysts redeveloped up to 2 cm and spontaneously disappeared after 6 months of follow-up. She developed no wound infections or peritonitis during the PO period and was discharged on the third PO day. Her ovary was checked regularly with pelvic and Doppler imaging after surgery; the development of her ovary was also age appropriate. She has been followed for 2 years.","A 30-day-old baby girl was referred to the hospital because a growth was found in her abdomen during a prenatal ultrasound. During surgery, doctors discovered that the growth was a twisted and damaged ovary, and it had to be removed. Additionally, a small appendix was removed during the same surgery. The remaining healthy ovary was repositioned to prevent it from twisting again, and she has been closely monitored since then with no complications."
940,"A 55-day-old Caucasian baby girl was referred to us by a gynecologist who had detected an intraabdominal cystic mass during prenatal US in the last trimester and was normal upon physical examination; no palpable abdominal masses were found. No other factors were present in her history. Imaging studies showed intraabdominal cystic mass. The tumor markers that were examined to determine the presence of malignancy were within normal ranges. She underwent surgery; her right ovary was found to be autoamputated due to torsion. The amputated necrotic, wandering ovary was removed, and the cysts in the contralateral ovary were drained (Fig. ); an incidental appendectomy was performed. Pathologic examination of the patient confirmed the diagnosis of a necrotic ovary. She had cysts smaller than 1 cm, they were aspirated during surgery. Postoperatively the cysts redeveloped and spontaneously disappeared after 1 year of follow up. She developed no wound infections or peritonitis during the PO period and was discharged on the third PO day. After surgery, her ovary was checked regularly using pelvic and Doppler imaging. The development of her remaining ovary was age appropriate. She has been followed for 2.5 years, and she continues to be followed uneventfully.","A 55-day-old baby girl was referred to us because a growth was found in her abdomen during pregnancy. During surgery, doctors discovered that her right ovary had been accidentally cut off and removed, and they also removed cysts from her other ovary and performed an appendectomy. The tests showed the growth was a necrotic ovary, and the cysts disappeared on their own after a year. The baby girl is doing well and continues to be monitored regularly to ensure her remaining ovary is developing normally."
941,"An 8-year-old Caucasian girl was admitted to our hospital with acute abdominal symptoms 60 hours after the complaints started; serious acute pelvic pain, sudden onset of nausea, vomiting, and pelvic pain and tenderness were present. No other factors were reported in her history. Direct abdominal X-ray images were normal. She was pre-diagnosed as having appendicitis or ovarian pathology based on anamnesis, a physical examination, and laboratory findings. She was operated on under emergency conditions and without prior US investigation. On operation, torsion was detected in her right ovary. She had a necrotic right ovary and salpinx (Fig. ); a salpingo-oophorectomy was performed due to the adnexa showing no improvement in its black color and necrotic appearance after detorsion. The contralateral ovary was normal, and an incidental appendectomy was performed. The pathology report indicated a hemorrhagic infarct in the ovary. She developed no wound infections or peritonitis during the PO period and was discharged on the fifth PO day. After surgery, her remaining ovary was examined regularly using pelvic and Doppler imaging. The development of her remaining ovary was also age appropriate. She has been followed for 6 years, and she continues to be followed uneventfully.","This 8-year-old girl was rushed to the hospital because of sudden, severe pain in her belly and nausea. Doctors suspected she might have appendicitis or a problem with her ovary, but after surgery, they found that her right ovary had twisted and died, causing it to become necrotic. To prevent infection, the doctors removed the twisted ovary and fallopian tube, and also performed an appendectomy. She recovered well after surgery and is now being monitored regularly to ensure her remaining ovary is healthy."
942,"A 10-year-old Caucasian girl was admitted with serious acute pelvic pain 4 hours after the complaints started. Acute abdominal symptoms were present in the patient; sudden onset of nausea, vomiting, and pelvic pain and tenderness were reported. Direct abdominal X-ray images were normal. No other factors were present in her history. She was pre-diagnosed as having appendicitis or ovarian pathology. On exploration, left adnexal torsion was detected, and detorsion was performed (Fig. ). The ligaments were extremely long; the ipsilateral mesosalpinx was shortened with a nonabsorbable suture, and an incidental appendectomy was performed. No wound infections or peritonitis developed during the PO period and she was discharged on the third PO day. After surgery, her ovaries were checked regularly using pelvic and Doppler imaging. In the second month, the affected ovary was similar in size to the contralateral ovary, and normal blood flow was observed on US. The development of her ovaries was also age appropriate. She has been followed for 6 years. She continues to be followed uneventfully and has regular menstrual cycles.","A 10-year-old girl was admitted to the hospital because of sudden, severe pain in her belly and pelvis. Doctors suspected she might have appendicitis or a problem with her ovary, but after examination, they found that her ovary had twisted and was causing the pain. The doctor carefully untwisted the ovary and also removed her appendix as a precaution. She recovered well after surgery and was discharged home after three days, and has been monitored regularly since then with no complications."
943,"A 16-year-old Caucasian girl visited our hospital with serious acute pelvic pain 30 hours after her complaints started. Acute abdominal symptoms were present; sudden onset of nausea, vomiting, and pelvic pain and tenderness were reported. She had a normal menstrual cycle. Direct abdominal X-ray images were normal. No other factors were present in her history. The tumor markers that were examined to determine the presence of malignancy were within normal ranges. Preoperative abdominal US was performed; Minimal pelvic fluid, an increase in the diameter of her appendix, and a large right ovary with increased diameter relatively to the left ovary were found on US examination and torsion was suspected in her right ovary. During the operation, right ovarian torsion with a hemorrhagic cyst approximately 8 cm in diameter was detected. A cystectomy was performed to protect her ovary against retorsion, and her ovary was repaired (Fig. ). The contralateral ovarian cysts were drained by aspiration. Her appendix was turgid and edematous and was evaluated as periappendicitis. In this case, as with the other four patients, an incidental appendectomy was performed. She developed no wound infections or peritonitis during the PO period and was discharged on the fifth PO day. After surgery, her ovaries were checked regularly using pelvic and Doppler imaging. In the fourth month, her affected ovary was similar in size to the contralateral ovary, and normal blood flow was observed on US. She has been followed for 1.5 years. She continues to be followed uneventfully and has regular menstrual cycles. The development of her ovaries was also age appropriate.","When a 16-year-old girl came to the hospital, she was experiencing severe pain in her lower abdomen and was nauseous and vomiting. Tests showed she had a twisted ovary with a large cyst, and surgery was needed to remove the cyst and repair the ovary. During the surgery, doctors also found her appendix was inflamed and removed it. She recovered well after surgery and her ovaries have returned to normal size and function. She is now being followed regularly to ensure everything remains healthy."
944,"A 34-year-old man, father of 2 kids, presented to urology outpatient clinic complaining of swelling in the left testis for the last 3 years. The swelling gradually increased in size and was not associated with any pain or fever. The patient also denied prior history of any trauma, infections, or scrotal surgeries. The past medical and surgical history was otherwise unremarkable.\nPhysical examination revealed a circumcised penis with bilateral developed hemiscrotum and normally descended testes. The right testis was normal, and the left testis had a firm, nontender 2.5 × 2.0 cm smooth mass attached near its lower pole with an associated lax hydrocele.\nThe baseline hematological and biochemistry workup was normal. Serum markers for germ cell tumor of the testes were all normal with serum lactate dehydrogenase (LDH) 275 IU/l (N = 208–378), alpha feto protein (αFP) 4.0 IU/ml (N ≤ 6.7), and beta human chorionic gonadotropin (βHCG) <2.0 mIU/ml (N < 10). An ultrasound scan was done which showed a 23.4 mm × 22.6 mm well-circumscribed lesion closely associated with the left testis. On color Doppler, no significant vascularity was observed (). The radiologist could not definitely determine the nature of the lesion, and it was labeled as suspicious for neoplastic process. An ultrasound of the abdomen did not reveal any evidence of lymphadenopathy.","A 34-year-old man came to the urology clinic because he noticed a growing swelling in his left testicle over the past three years. The swelling didn't cause any pain or fever, and he has no history of injury or surgery. Physical examination and blood tests showed that the markers for cancer were normal, but an ultrasound revealed a suspicious mass near the testicle. Further investigation is needed to determine the nature of the mass and ensure appropriate treatment."
945,"A 69-year-old lady with a background of penicillin allergy and left-sided breast cancer treated with mastectomy and axillary node clearance was admitted to the hospital in Liverpool in 2016 with a right-sided breast abscess. The patient had been aware of a lump in her right breast for 3 years and had been told it was a sebaceous cyst. However, it had become painful, and she was now feeling unwell and tachycardic but remained apyrexial.\nAntibiotic therapy was initiated with IV clindamycin 450 mg TDS and IV teicoplanin 12 mg/kg BD and then with 12 mg/kg OD after 2 days. The abscess was aspirated and subsequently incised and drained under local anaesthetic. The frank pus drained was malodorous, so oral metronidazole 500 mg TDS was added on. As there was no clinical improvement after 48 hours, a second incision and drainage was performed under general anaesthetic, revealing a large abscess cavity extending 10 cm into the right breast and 7 cm into the left mastectomy scar. This combined with continued triple antibiotic therapy resulted in clinical improvement, so after 4 days of IV therapy, antibiotics were stepped down to oral erythromycin 500 mg QDS of 7-day course and metronidazole 500 mg TDS of 10-day course and the patient was discharged. The abscess healed well in the community.\nTwenty-one days after sending aspirated pus for MC + S, Actinomyces europaeus was grown in the culture. The patient was still clinically well, the abscess was healing well, and no underlying chest source of infection was identified.\nThe breast abscess pus was sent to the microbiology laboratory for testing. There were Gram-positive cocci visualised on the direct Gram stain, but there was no evidence of any Gram-positive bacilli. The pus was cultured onto the following plates: blood agar, MacConkey agar, selective anaerobic agar with a 5-microgram disc, and a fastidious anaerobic agar (FAA) plate in accordance with the laboratory standard operating procedure (SOP) based on the UK Standards for Microbiology Investigations (SMI) []. After 4 days of growth, two colonies grew on the direct FAA plate, which were different. The first colony was identified, using a matrix-assisted laser desorption/ionisation (MALDI-TOF) machine, as a Staphylococcus epidermidis. The MALDI-TOF equipment used was Bruker Microflex utilising MALDI Biotyper Version 3.1 software. The MS method of identification was the AutoX setting, and the software library was the BDAL and Filamentous Fungi Library Version 1.0. The second colony failed to be identified using MALDI. Therefore, the second organism was subbed onto an Actinomyces plate (blood agar with supplementary metronidazole and nalidoxic acid) in accordance with the local SOP. Four days later, there was fine growth on the Actinomyces plate. Sensitivities were obtained via the British Society for Antimicrobial Chemotherapy (BSAC) method of disc diffusion. The organism was sensitive to penicillin 42 mm (sensitivity > 23 mm), erythromycin 40 mm (sensitivity > 10 mm), vancomycin 30 mm (no breakpoint available), tetracycline 42 mm (no breakpoint available), and ciprofloxacin 22 mm (no breakpoint available). The organism was initially identified as Actinomyces europaeus, but the MALDI score was only 1.82; therefore, an extraction MALDI was performed to increase the MALDI score, so a more confident identification of the organism was obtained. The subsequent MALDI identity was Actinomyces europaeus with a score of 2.10. As per the guidance from the SMI, the organism was sloped and sent to the Anaerobic Reference Unit at Colindale. The ID was confirmed as Actinomyces europaeus by partial sequencing of 16sRNA.\nMicrobiological advice consisted of a long course of antibiotics to eradicate the infection, and the first-line treatment was 2 weeks of IV ceftriaxone followed by 6 months of oral amoxicillin. This was not feasible due to the patient's penicillin allergy.\nSecond-line advice was 2 weeks of IV erythromycin followed by 6 months of oral erythromycin. However, this was also not feasible. The outpatient parenteral antibiotic therapy (OPAT) service enables patients to receive IV therapy in the community but was unable to provide the four times daily dosing required for IV erythromycin. The OPAT service was only able to provide twice daily regimens.\nThe third-line approach was acceptable: 2 weeks of IV tigecycline given in twice daily doses followed by 6 months of oral doxycycline. The patient had a midline inserted and received her IV therapy in the community with OPAT services.\nAfter completing the IV tigecycline course with no issues, the patient complained of side effects following 6 weeks of oral doxycycline therapy: oral thrush and leg blisters. Antibiotic therapy was switched to oral clarithromycin to complete a 6-month course of oral antibiotics. There has been no evidence of recurrence to date.","This 69-year-old woman was admitted to the hospital after developing a painful breast abscess, which had been present for three years. After several attempts to drain the abscess and treat the infection with antibiotics, doctors identified the bacteria causing the problem as Actinomyces europaeus. To ensure the infection was completely cleared, she received a course of antibiotics, and the infection healed well. To treat the infection, the doctors initially tried several different antibiotics, but because she is allergic to penicillin, they had to find alternative options. Eventually, they prescribed a longer course of antibiotics, including tigecycline, and then switched to clarithromycin to finish the treatment. The doctors carefully monitored her progress and used a specialist service to deliver the antibiotics at home, making it easier for her to manage the treatment. Thankfully, the abscess healed completely, and she was able to return home without any further complications."
946,"A healthy 15-year-old male presented with a three-week history of irretractable abdominal pain, vomiting, and anorexia. There was no previous similar history or abdominal surgery. He associated the onset of symptoms with a recent contraction of gastroenteritis within the family. There was no improvement in his condition despite his family contacts recovering.\nOn examination, he was afebrile and haemodynamically stable. There was a scaphoid abdomen with maximal tenderness in the right upper quadrant. There was significant guarding. Bowel sounds were audible. His abdominal X-ray and routine blood test results were both unremarkable, other than a raised C-reactive protein of 92.\nHis high opioid requirement, chronicity of symptoms, and examination findings prompted further evaluation with CT. This revealed right-sided abdominal mass and a layering effect at the caecal pole consistent with an intussusception. The appearance was similar to a “pseudokidney,” as shown in . There was marked free fluid within the abdominal cavity. After resuscitation, he proceeded to a laparotomy.\nA diagnostic laparotomy was performed for the inspection of abdominal contents. It confirmed radiological findings of an intussusception of the terminal ileum within the caecal pole. A hard mass was noted within the hepatic flexure region. There was a dilated terminal ileum and multiple lymph nodes noted within the mesentery.\nA right hemicolectomy was performed. Vascular pedicles were taken high for an appropriate oncological resection, given the suspicion. Primary ileocolic stapled side-to-side anastomosis was performed. The recovery was uncomplicated and the patient was discharged home three days postoperatively.\nAs shown in , the histopathology of the excised mass proved to be Burkitt's lymphoma of the terminal ileum causing ileocolic intussusception. It extensively involved the appendiceal serosa, mesentery, and omentum.\nThe tumour was an ulcerated lesion infiltrating all layers of the bowel wall. As shown in microscopically, the characteristic starry sky growth pattern was visualised, with the cells having round nuclei, finely clumped chromatin, and small basophilic nucleoli.\nA panel of immunostains was performed on bowel tumour, the regional lymph nodes, and the omentum. The tumour cells in all three sites are CD20, CD10, BCL6, and C-MYC positive (see ). The tumour has a very high proliferative index with almost 100% of the tumour expressing Ki67. BCL2, Cyclin D1, TdT, CD3, CD5, CD23, and CD30 are negative. Fluorescence in situ hybridisation (FISH) detected a reciprocal t(8;14) translocation and rearrangement of the C-MYC gene. As only chromosome-specific probes were used, the presence of other abnormalities cannot be excluded. The above profile supports the original diagnosis of BL.\nThe medical literature in the PubMed and Medline/EMBASE databases was reviewed for cases of Burkitt's lymphoma-related intussusception. All publications were scrutinized that contained keywords of “intussusception” and “Burkitt lymphoma.” The literature search was limited to paediatric patients under 18 years old, and restricted to papers written in English.\nA systematic review of relevant literature was performed to ascertain variance of clinical features in primary presentations of BL-related intussusception. The following information on patient numbers, demographic, main presenting complaint, disease stage, and diagnostic methods were obtained in this meta-analysis.","A 15-year-old boy experienced persistent abdominal pain, vomiting, and loss of appetite for three weeks, initially thought to be from a family illness. Doctors found a large mass in his abdomen and determined that he was experiencing an intussusception, where one part of the intestine slides into another. Surgery was performed to remove the mass and a portion of his small intestine, and pathology revealed it was a type of lymphoma called Burkitt's lymphoma. The lymphoma was causing the intussusception, and further tests confirmed the diagnosis, guiding treatment and ensuring a complete understanding of the case."
947,"The 2-month-old African American infant was born at full term, at 2890 grams, following an uncomplicated pregnancy and delivery with an Apgar of seven and nine at one and five minutes, respectively. In the newborn nursery, her physical exam did not note any murmur, and her congenital cardiac screen was documented as normal (pre- and postductal saturations 100%) on day two of life. She had no family history of congenital heart defects, arrhythmias, or other cardiac diseases. The infant was discharged home with her mother on day of life two with follow-up arranged with the pediatrician.\nAt home, she remained stable with no respiratory distress, no feeding difficulties, and adequate weight gain. Her mother did note that the infant's hands and feet appeared slightly “dark” at home, but she was reassured that this was normal acrocyanosis. Her pediatrician saw her at two separate routine office visits, where no murmur or other abnormality was reported. Approximately three days prior to presentation, the infant developed cough, congestion, and rhinorrhea. She was due for her two-month well-child exam, so her mother took her to the clinic for an evaluation. At that time, the infant weighted 4.3 kg placing her at approximately the tenth percentile on a Center for Disease Control growth chart. The pediatrician was concerned by her visible acrocyanosis and pulse oximetry documented an oxygen saturation of 80% in room air, so she was immediately referred to an emergency department for further evaluation.\nOn arrival to the emergency department, she was hypoxic with oxygen saturations of 20–30% and hypothermic to 32°C. No murmurs were noted on physical exam. Peripheral access was established, first with a tibial intraosseous catheter and then with a peripheral intravenous line; she received a normal saline bolus, a blood culture was drawn (which remained negative), and a dose of ceftriaxone was administered. A rapid sequence intubation was performed with etomidate and vecuronium. The child was successfully intubated with no complications. Despite this and increasing oxygen administration up to 100% FiO2, her oxygen saturation peaked at 80%. Initial labs were significant for blood glucose 95 mg/dL, white blood cell count 7 × 109/L, hemoglobin 22 g/dL, hematocrit 68%, and lactate 5.5 mmol/L. Chest X-ray could not view the aortic knob or descending aorta well and could not rule out a right-sided aortic arch (). With these findings and the severity of her illness, the decision was made to transfer the patient to a tertiary pediatric medical center.\nOn arrival, a brief transthoracic echocardiogram (TTE) was concerning for Tetralogy of Fallot with critical pulmonic stenosis versus atresia. She received an additional normal saline bolus, sodium bicarbonate, midazolam, and fentanyl. On attempts to draw labs, her blood was noted to be very dark and hyperviscous, clotting almost immediately.\nThe infant was admitted to the pediatric intensive care unit (PICU) for further stabilization and started on a prostaglandin drip with the hope of reopening her ductus arteriosus to increase pulmonary blood flow. Repeat TTE confirmed the diagnosis of Tetralogy of Fallot with severe pulmonary stenosis, hypoplastic main and branch pulmonary arteries, right-sided aortic arch, and small secundum atrial septal defect (). At no point was a murmur heard by any provider from the ED, PICU, or Cardiology. Over the next several hours, she continued to decompensate with worsening hypoxia and hypotension despite phenylephrine infusion, fentanyl infusion, and aggressive fluid resuscitation. Pediatric cardiothoracic surgery evaluated the patient, and the decision was made to perform an emergent Blalock–Taussig (BT) shunt.\nThe patient underwent modified BT shunt with a 3.5 mm Gore-Tex shunt anastomosing the innominate artery to the main pulmonary artery. There were no complications, and she returned to the PICU for post-op recovery. Daily TTE demonstrated normal biventricular function without pericardial effusion, as well as flow across BT shunt and through both branch pulmonary arteries. On post-op day one, she was extubated and weaned to room air, where she was able to maintain oxygen saturations around 88%. By post-op day two, she was tolerating oral feeds. She was discharged home on post-op day four with 40.5 mg of aspirin daily to help prevent shunt thrombosis. On discharge, her parents noted a vast difference in the infant's general appearance and energy level. The plan is to take the infant back to the operating room to perform a complete repair with a transannular patch at 4–6 months of life.","This 2-month-old baby was born healthy and has been doing well at home. Recently, she started coughing and having trouble breathing, and her oxygen levels dropped significantly. Doctors discovered she has a serious heart condition called Tetralogy of Fallot, which was not detected earlier. She had a procedure called a BT shunt to help improve her blood flow, and is now recovering well at home with follow-up appointments and a planned surgery to fix her heart completely."
948,"A 75-year-old Chinese male with a past medical history of hypertension, hyperlipidemia, coronary artery disease, diabetes mellitus, benign prostatic hyperplasia, and osteoarthritis arrived to our Emergency Department (ED) due to unstable gait. The patient stated that he was a resident of Yonkers, New York (NY). His only travel history involved a train ride to Flushing, NY, the day prior to presentation. The patient reported frequent walks in local parks around Yonkers, NY. At the time of initial examination, he denied headaches, dizziness, shortness of breath, back or chest pain, rashes, focal weakness, or loss of sensation. He had not noticed any ticks or tick bites on his skin in the past year. On physical exam, he was noted to have normal extraocular muscle movements, neurologic exam without focal deficits, and musculoskeletal, cardiac, and respiratory exams without abnormalities. His electrocardiogram showed normal sinus rhythm with a rate of 69 beats per minute. His labs revealed slightly decreased hemoglobin and hematocrit levels and thrombocytopenia along with increased bilirubin levels. His chest X-ray (CXR), rapid influenza swabs, blood cultures for bacteria, and urine analysis were negative. He was treated in the ED until he was hemodynamically stable and asymptomatic, after which he was then discharged.\nHe returned 4 days later complaining of worsening fevers since discharge, with a maximum recorded temperature of 105 degrees Fahrenheit (40.55°C), occasional rigors, chills, diaphoresis, diffuse myalgias, generalized weakness, malaise, confusion, and decreased appetite. The patient's gait was noted to be unstable with difficulty maintaining balance. On physical examination, the patient appeared lethargic. He was noted to be tachycardic at 98 beats per minute (bpm), to have a black discoloration of patient's tongue, and to have a recorded temperature of 102.8 degrees Fahrenheit (39.33°C). His blood pressure, respiratory rate, and the remainder of his neurological exam was normal. His CXR was significant for fullness in the right mediastinum, cardiomegaly, and unfolded aorta. His hemoglobin, hematocrit, and comprehensive metabolic panel remained stable from previous admission. It was noted that the patient had decreased white blood cell (WBC) count and severe thrombocytopenia on readmission (). Initial serum ELISA for Lyme disease, used as a screening tool, was negative. Magnetic resonance imaging (MRI) of the brain showed no plaque deposition, demyelination, or other acute pathologic processes. A computed tomography (CT) scan of the abdomen revealed a small aneurysmal dilatation of the thoracic aorta and splenomegaly with an acute appearing splenic infarct. He was started on ceftriaxone, ampicillin, and vancomycin to cover for a bacterial infection.\nDuring the second night of hospitalization, the patient's heart rate spiked to over 200 bpm and he became hypotensive. He was transferred to the Intensive Care Unit (ICU) for management of severe hemodynamic instability and decompensated respiratory status. The patient's fever of unknown origin, encephalopathy, and negative initial screening tests prompted us to draw additional sets of serum tests for multiple possible bacterial, viral, and fungal etiologies. Unlike the initial serology, this new set yielded positive test results for the Lyme IgG and IgM ELISA test, Lyme IgM Western blot, and Babesia microti IgG and IgM antibody tests (). To confirm the diagnosis, a lumbar puncture (LP) was performed after patient's thrombocytopenia was corrected. During the LP procedure, the CSF was noted to be proteinaceous and congealed by the performing interventional radiologist. The LP resulted in positive tests for Lyme IgG and IgM antibody levels by Western blot, as well as a positive Western Blot for Lyme IgM (). Cytomegalovirus, Epstein-Barr virus, West Nile virus, and rheumatoid factor were not present. The patient was then started on doxycycline for the diagnosis of Lyme neuroborreliosis. Within 48 hours of doxycycline administration, the patient clinically improved, his mentation returned to baseline, and his gait returned to normal. The patient was treated for 5 days and was discharged on an oral course of doxycycline for a total of 21 days.\nHere we have presented a patient who had no characteristic clinical signs of LD, whose main symptoms were a high fever, encephalopathy, diffuse myalgia, and tachycardia. Blood culture, urine culture, and CSF gram stain were negative. An MRI of the brain and CT of the chest/abdomen showed no source of infection. The single diagnostic modality yielding concrete results was the patient's CSF Lyme serology.","This 75-year-old man returned to the hospital after a period of recovery, experiencing a high fever, confusion, and difficulty walking. Initial tests didn't reveal a clear cause for his symptoms, but further testing revealed he had Lyme disease, a bacterial infection spread by ticks. He was treated with antibiotics, and his condition improved significantly within a few days."
949,"A 51-year-old male was referred to our hospital because of a three-month history of gradually progressing renal failure. During his first hospitalization, he complained of lumbar pain. On physical examination, he had conjunctival pallor and severe percussion tenderness of his back. No skin lesions or neurological deficits were seen. Laboratory test results were as follows: hemoglobin, 8.7 g/dL; creatinine, 7.01 mg/dL; total protein, 7.4 g/dL; albumin, 3.2 g/dL; calcium, 14.8 mg/dL; phosphate, 6.2 mg/dL; beta-2-microglobulin, 27.9 mg/L; IgG, 341 mg/dL; IgA, 21 mg/dL; IgM, 18 mg/dL; free kappa light chain, 99,900 mg/L; and free lambda light chain, 9.7 mg/L. Chest X-ray results were normal. Computed tomography (CT) showed vertebral compression fractures of Th8 and L1 and bilateral pleural effusions without calcified lesions. Urine immunoelectrophoresis showed a positive result for the Bence-Jones protein. Bone marrow aspiration revealed plasma cell proliferation (65% of total nucleated cells, ) with expression of CD38 and CD56, absence of CD19 and CD20, and an MIB-1 labeling index of 25%. Chromosomal analysis of the bone marrow by G-banding showed a normal 46,XY karyotype, but fluorescence in situ hybridization revealed the abnormalities del(13q) and t(4;14). He was diagnosed with Bence-Jones protein type MM (stage III according to the International Staging System, and stage IIIB according to the Durie–Salmon classification system).\nWe began treatment with intravenous fluids and intramuscular injections of calcitonin to treat the severe hypercalcemia. Simultaneously, he received bortezomib-dexamethasone (Bd) therapy (subcutaneous injection of 1.3 mg/(m2·day) bortezomib plus 20 mg/day dexamethasone orally on days 1, 4, 8, and 11). Unexpectedly, he experienced severe acute heart failure on day 8, and temporarily required the support of a mechanical ventilator. Bd therapy was discontinued during the first treatment cycle. Because renal function had not improved, maintenance hemodialysis was initiated. Subsequently, we continued MM treatment with two cycles of vincristine-doxorubicin-dexamethasone (0.4 mg/body of vincristine and 9 mg/m2 of doxorubicin on days 1 to 4; and 40 mg/body of dexamethasone on days 1 to 4, 9 to 12, and 17 to 20 of a 28-day cycle) and the M2 protocol (multiple chemotherapeutic agents, not including proteasome inhibitors), followed by lenalidomide-dexamethasone (Rd) therapy (5 mg/day lenalidomide on days 1 to 21 plus 20 mg/body dexamethasone on days 1, 8, 15, 22 of a 28-day cycle).\nAbout four months after starting Rd therapy, the patient suffered from myoclonus-like movement of the lower extremities. During the sixth cycle of Rd therapy, he complained of pain in both lower legs, but did not have skin lesions or tenderness. He had been taking loxoprofen, fentanyl (patch and buccal tablet), mecobalamin, ferrous fumarate, lansoprazole, amlodipine, furosemide, alfacalcidol, and darbepoetin alfa. It was unlikely that his pain was drug-induced.\nThe patient's serum creatinine kinase level was elevated to 1,268 U/L. Diffusion-weighted and short tau inversion recovery magnetic resonance imaging revealed diffuse high signal intensity in the crural muscles (). A muscle biopsy was performed on the right tibial anterior muscles () and 40 mg/day prednisolone was prescribed by a neurologist because of suspected polymyositis/dermatomyositis. However, typical pathologic findings of polymyositis/dermatomyositis, like lymphocyte infiltration around muscle fibers, were absent and vessel calcification was noted. Prednisolone was ineffective against his symptoms. During steroid administration, well-demarcated ulcers developed on the right lower leg, both exterior thighs, and the penis. These ulcers gradually worsened () and the patient experienced severe pain, especially during dialysis or exercise. He could not continue dialysis because of this exacerbation of pain. Moreover, muscle atrophy of his lower limbs impaired his daily activities. The administration of 40 mg/day prednisolone was continued for 42 days and was then stopped on the 84th day after tapering. The patient's serum intact parathyroid hormone (PTH) level was 429 pg/mL, while previously it was permissively controlled within the range of 140–250 pg/mL. Before dialysis, his levels of serum albumin, calcium, and phosphate were 4.0 g/dL, 7.4 mg/dL, and 7.9 mg/dL, respectively. He was diagnosed with secondary hyperparathyroidism (HPT). We could not exclude a relationship between MM and HPT, although his free light chain ratio was decreasing.\nCT showed widespread centrilobular opacities of both lungs () and high-density lesions along small blood vessels in the trunk and extremities (), but the mediastinum, abdominal organs, and large vessels like the thoracic and abdominal aorta were intact. A pulmonary function test demonstrated restrictive impairment with reduced diffusion capacity: the predicted forced vital capacity was 48.6%, the forced expiratory volume of the first breath was 87.9%, and the predicted diffusing capacity of lung for carbon monoxide was 67.1%. 99mTc-hydroxymethylene diphosphonate scintigraphy revealed abnormal diffuse accumulation in both upper lung fields (). Echography revealed no enlargement of the parathyroid glands.\nNext, a transbronchial lung biopsy was performed, and microscopy confirmed the presence of calcifications of the alveolar walls () and of a small vessel in the right anterior tibial muscle (). However, pathological calcification was absent from the right exterior thigh ulcer. Healing of the skin biopsy wound was delayed.\nThe patient was ultimately diagnosed with muscle and skin ischemia from CUA. He was treated with cinacalcet, and his intact PTH levels fell to a normal range. He underwent four additional cycles of Rd therapy, but ulcer infections occurred repeatedly in both thighs, occasionally progressing to sepsis. He has since been monitored closely for MM, without treatment, for four months. Meanwhile, the ulcers have achieved epithelialization after topical treatment, but his serum free light chain ratio level increased from 290 to 1532. He is currently undergoing Pd therapy (2 mg/day pomalidomide on days 1 to 21, plus 4 mg/day dexamethasone on days 1, 8, 15, and 22 of a 28-day cycle) without any adverse events. Since severe heart failure occurred during the combined regimen with bortezomib, we have avoided administering proteasome inhibitors. His disease is stable according to the International Myeloma Working Group criteria.","This patient has been battling a serious illness for several months, starting with kidney failure and progressing to multiple complications. Initially, he experienced pain in his back and developed a severe infection, requiring hospitalization and support with a breathing machine. Further tests revealed a rare type of cancer called multiple myeloma, which affected his bones and blood. He received several rounds of chemotherapy, but unfortunately, developed painful skin ulcers that made it difficult to continue dialysis. Despite these challenges, he is now receiving a new treatment called Pd therapy and is being closely monitored to manage his cancer and improve his overall health."
950,"The patient was a 76-year-old man who came to the office in 2014 looking for possible treatments of his fractured central incisors. Nothing was found relevant about his medical condition. The patient shows a high risk for caries and also eccentric bruxism. He has partial edentulism in the superior left quadrant and multiple decay and fractured teeth. The initial approach was conservative aiming to keep the upper front by means of composite fillings (Figures and ). Then, the posterior superior quadrants needed to be restored with implants.\nThree years after, in 2017, the patient came back to the office referring pain of endodontic origin in the upper left canine. New and secondary subgingival caries were found in the six front teeth. The conservative prognosis was considered poor due to the subgingival depth and extent of decay presented by the lesions from canine to canine. After having discussed the treatment options, especially the surgical lengthening of the front teeth or the orthodontic extrusion, the patient decides to replace the residual teeth with a new implant-supported bridge similar to the recently performed prostheses of the posterior areas that were judged by him as a highly satisfactory treatment. The patient preferred not to involve these restorations in the present anterior treatment and limited it to place only two implants in the lateral incisors' positions ().\nThe treatment was carried out in a staged approach. Briefly, first, we extracted the lateral incisors, using the SST, and placed two immediate implants. The four residual teeth were then prepared to be used as abutments of a temporary bridge for the purpose of maintaining the aesthetics and function of the patient during the early osseointegration period. In a further step, the four remaining teeth were also extracted using the SST, and the initial provisional bridge was replaced by the second provisional screwed on the uncovered implants. Only one out of the four abutment teeth used for the temporization of root canal treatment was needed due to a periapical infection.\nWhen placing the two immediate implants into the alveolus of the lateral incisors, a section of the buccal part of the root (about the two middle thirds) was left in place and no biomaterial was used at all. An impression of the implants was taken to have the second temporary bridge available in the second surgery. Healing abutments were then attached with the proper height for the soft tissue to cover them but at the same time facilitating the uncovering. Finally, a temporary acrylic bridge was cemented onto the four abutment teeth 13-11 and 21-23 ().\nThree months later, the implants were uncovered, the four abutment teeth were extracted, again with the SST—partial extraction of the roots—but this time no more implants were placed in these sockets. The first provisional cemented onto the teeth was then replaced by a second acrylic bridge screwed onto the implants though temporary abutments ().\nThe partial extraction of the canines, aiming to leave a buccal slice of the root, was so hard to perform, and further instrumentation would lead to the socket destruction that a decision was intraoperatively made and a greater portion of the root, including the apex, was finally left. As the locations of the canines did not involve the implant sites, any potential complication could be addressed efficiently.\nOne month later, the prosthodontic phase was undertaken. Little if any differences in the buccal tissue volume and no noticeable aesthetic impact could be found after the multiple extractions (). The desired position of the incisal border was determined by various try-ins, and five months after implant surgery, the definitive prosthesis was placed. The final clinical aspect can be appreciated in the pictures (Figures and ).","A 76-year-old man came in for help with damaged teeth. He had several decayed and broken teeth, and his gums were also affected. To improve his smile and bite, the dentist initially used fillings to protect the remaining teeth, and later replaced missing teeth with a new, implant-supported bridge. The dentist carefully extracted some of his existing teeth to create space for the new implants and bridge, ensuring a comfortable and functional result. Finally, a permanent, well-fitting bridge was placed, restoring his smile and ability to eat properly."
951,"A previously healthy 25-year-old man presented with multi-fragmentary fractures\nof the lower third of right tibia and fibula as a consequence of a motorcycle\naccident (). Upon hospital\nadmission he was alert, coherent and had no motor deficits. His vital signs and\nthe rest of a physical examination were normal. He was admitted to the hospital\nfor surgical stabilization. Forty-eight hours after admission, he developed\nconfusion and agitation followed by a rapid decline in his level of\nconsciousness that progressed to coma with bilateral extensor posturing. His\npupils were equal, slightly large and reactive. He was tachypneic (44/min),\ntachycardic (137/min), febrile (39.3°C) and hypertensive (147/101mmHg). His\npulse oximetry was 92% on room air. Petechial hemorrhages were noted in the\nsclerae, conjunctivae, buccal mucosa and the upper third of the thorax.\nResuscitation was initiated with fluids, supplemental oxygen, tracheal\nintubation and mechanical ventilation under deep sedoanalgesia. A head computed\ntomography (CT) scan revealed multiple and bilateral frontal subcortical\nhypodense areas without a midline shift. No hemorrhage was evident, and the\nbasal cisterns and sulci remained visible ().\nA chest CT was normal except for small bilateral basal atelectasis with no\nevidence of pulmonary embolism. Transthoracic echocardiography revealed normal\nventricles, normal valve function and an absence of patent foramen oval or signs\nof pulmonary hypertension. An electroencephalogram revealed diffuse slowing\nwithout epileptiform discharges. The laboratory parameters were normal except\nfor elevations of the following inflammation markers: leukocytosis\n17300/mm3 and C-reactive protein (CRP) 141mg/L. Thrombocytopenia\n(110.000/mm3) was also noticed. A diagnosis of FES was reached.\nSupportive therapy was provided, and methylprednisolone (10mg/kg/day) was\nadministered for 3 days. Two days later, given his clinical stability, the\nsedation and analgesia were withheld to assess his neurological status. After 10\nminutes, bilateral and spontaneous extensor posturing was observed in\ncombination with mechanical ventilation asynchrony, tachypnea (46/min), profuse\nand generalized sweating, arterial hypertension (215/112mmHg) and tachycardia\n(137/min). These manifestations were recurrent and led to the diagnosis of PSH.\nConsequently, propranolol (120mg/d) and morphine (3mg) every 4 hours were added\nto the therapeutic regimen. The sedoanalgesia was reinstituted. Two days later,\na brain MRI revealed multiple small lesions in both cerebral hemispheres that\nwere hypointense on T1 and hyperintense on T2 and FLAIR, did not exhibit\nenhancement after gadolinium injection and were located in the periventricular\nwhite matter of both frontoparietal regions ().\nThe episodes of PSH had durations that oscillated between 20 and 35 minutes,\noccurred with a frequency of 3 to 4 times daily and were mainly associated with\nstimuli such as the turning, bathing, aspiration of secretions and pain. On the\n8th intensive care unit (ICU) day, remifentanil and propofol were\nwithdrawn and replaced with dexmedetomidine while the propranolol and morphine\nwere maintained at the previous doses. The episodes of sympathetic discharge\nbecame progressively briefer and less severe and reached no more than 2 per day.\nThe motor responses improved to the localization of the nociceptive\nstimulus.\nOn ICU day 12, the patient was extubated. Sixteen days after admission, he was\nalert, had normal motor responses and was able to communicate with his family.\nThe doses of propranolol and morphine were reduced to half without worsening of\nthe episodes of PSH. Twenty-two days after admission, the fracture was\nsurgically fixed. The post-operative course proceeded without complications.\nThree days after surgery, the patient was discharged home to continue ambulatory\nrehabilitation. One year after the event, the lesions found in the MRI had\ndisappeared, and neuropsychological testing revealed a mild impairment of\nexecutive functions with alterations of short and long-term memory.","This man was seriously injured in a motorcycle accident, resulting in broken bones in his lower leg. After his initial hospital stay, he became confused and his condition rapidly worsened, requiring him to be put on a breathing machine. Tests revealed inflammation and blood clots, leading to a diagnosis of two separate conditions: FES and PSH. He received treatment with medications and supportive care, and his symptoms gradually improved over several weeks, eventually leading to his removal from the breathing machine and discharge home."
952,"A 21-year-old man without any medical history presented with a closed fracture of\nthe lower third of his tibia secondary to a motorcycle accident without evidence\nof cranial trauma (). His vital\nsigns and neurological examination were normal. The patient was hospitalized and\ntreated with skeletal traction. One day later, he became confused and agitated\nwith progressive depression of consciousness that progressed to coma. He\nexhibited an extensor motor response, but the brainstem reflexes were normal.\nThe vital signs were as follows: arterial blood pressure, 127/76mmHg;\nrespiratory rate, 18/min; heart rate, 133/min; and rectal temperature, 38.8°C.\nHis pulse oximetry was 85% on room air. Invasive mechanical ventilation and\nhemodynamic resuscitation were initiated immediately. After cardiorespiratory\nstabilization, a head CT scan revealed small and multiple subcortical hypodense\nlesions in both the frontal and left parietal regions ().\nA chest CT revealed small filling defects in the left ventricle and the superior\nvena cava. A nodular image was observed in the right pulmonary artery and could\nhave corresponded to an embolus. Fat embolism syndrome was diagnosed. A\ntransthoracic echocardiogram revealed an estimated mean pulmonary artery\npressure of 35mmHg with no disorders of ventricular motility and preserved\nsystolic ventricular function. The size and function of the right ventricle were\nnormal, and there were no interatrial or interventricular shunts. An\nelectroencephalogram revealed background slowing without epileptiform\ndischarges. The biochemical profile was unremarkable with the exception of\nthrombocytopenia (98.000/mm3), anemia (Hgb 8.7gr/dL) and an elevation\nof the CRP level to 134 mg/L. General critical care supportive measures were\nprovided, and the patient received IV methylprednisolone at 10 mg/kg/day for 72\nhours.\nOn ICU day 5, sedoanalgesia (remifentanil-propofol) was stopped. After 20\nminutes, the patient exhibited generalized and profuse sweating, tachycardia\n(166/min), arterial hypertension (187/99mmHg), and tachypnea (36/min) with\nasynchrony of mechanical ventilation and bilateral extensor posturing; these\nmanifestations were all compatible with PSH. The symptoms were alleviated with\nthe restoration of sedoanalgesia but continued to occur 4 times per day and were\ntriggered by physiotherapy maneuvers, aspiration of secretions and baths with\ncold water. The average duration of the episodes was 33 minutes. A brain MRI\nrevealed multiple T2-hyperintense lesions in the periventricular white matter\nand bilateral frontal and parietal subcortical regions ( and ).\nTreatment with propranolol at 120mg/day, morphine at 18mg/day and gabapentin at\n900 mg/day resulted in progressive reductions of the frequencies, intensities\nand durations of the PSH episodes. The patient was extubated without\ncomplications on hospital day 11. After 20 days, he was transferred to the\ngeneral ward; he was alert, oriented, communicating with his family and feeding\non his own. He was maintained on propranolol at 40mg/day and morphine at\n6mg/day. Surgical fixation of the fracture was performed one month after\nadmission. He was discharged to home three days after the surgery. At 12 months\nafter the event, the previously noted lesions were no longer visible on a follow\nup MRI, and the patient returned to his work and his classes at the university\nwithout alterations in neurocognitive testing results.","A 21-year-old man was brought to the hospital after a motorcycle accident, resulting in a broken leg. He quickly became very sick, becoming confused and losing consciousness, and doctors discovered swelling in his lungs and brain. After a thorough examination, they diagnosed him with fat embolism syndrome and started treatment with medications and supportive care. Over several weeks, he gradually improved, and his symptoms resolved, and he was able to return to his normal activities. Finally, he had surgery to fix his broken leg and was discharged home after a year with no lasting neurological problems."
953,"A 63-year-old Japanese man, a current smoker of more than 45 pack-years, was revealed by routine examination’s chest X-ray to have an abnormal shadow in the left middle lung field. His past medical history was unremarkable. A chest computed tomography (CT) scan showed a 3.6 × 2.8 cm pulmonary lesion in the left upper lobe, with a clear boundary and heterogeneous contrast enhancement (Fig. ). Positron emission tomography (PET) showed an accumulation of 18F-fluorodexyglucose (FDG) with a maximum standardized uptake value (SUV max) of 4.95 in the early phase and 6.31 in the late phase in the nodule. No accumulation of FDG was noted in the pulmonary hilum, or mediastinal lymph nodes.\nA trans-bronchial lung biopsy (TBLB) of the mass in the left upper lobe was performed; it revealed non-small cell carcinoma. Although the serum carcinoembryonic antigen (CEA) level was 52.1 ng/mL (normal range 0–5.0 ng/mL), there were no distant metastases or other cancer lesions. The preoperative clinical diagnosis of primary lung cancer, stage cT2aN0M0 stage IB was considered.\nA left upper lobectomy and mediastinal lymph node dissection were performed by video-assisted thoracic surgery. The resected tumor measured 3.8 × 2.8 × 2.8 cm in diameter.\nGrossly, the specimen showed a well-established boundary as a grayish lesion with areas of necrosis. The histopathological examination showed a solid proliferation of columnar atypical cells with cell cytoplasm and complex glandular structures with abundant desmoplastic stroma, a morphological resemblance to fetal lung and significant tumor necrosis (Fig. , ). Conventional lung adenocarcinoma, another histological component and morulae were not found. Periodic acid Schiff (PAS) staining demonstrated glycogen in the cytoplasm of the neoplastic cells. Stains for mucin were negative. The immunohistochemical analysis showed positivity for AE1/AE3, CEA, and alpha-fetoprotein (AFP) (focal) (Fig. ). Thyroid transcription factor-1 (TTF-1), chromogranin A, synaptophysin, CD56, and p40 were negative. The beta-catenin result was positive, predominantly in the cell membrane (Fig. ). The final histopathological diagnosis was H-FLAC.\nBoth lymphatic and vascular invasion were detected. Microscopically, all margins were free and mediastinal lymph nodes (#5) showed metastasis. The final pathological staging was stage IIIA (pT2aN2M0). Both epidermal growth factor receptor (EGFR) mutations and anaplastic lymphoma kinase (ALK) gene rearrangements were negative. The expression of programmed cell death 1 ligand 1 (PD-L1) (Dako, clone 22C3) by resected specimen was negative.\nThe patient received cisplatin plus vinorelbine as adjuvant chemotherapy, but it was canceled at two courses because of the dehydration and hyperglycemia induced by the chemotherapy. At 25 months after the surgery, the patient had relapsed pleural dissemination. Postoperative serum CEA and AFP was 1.5 and 3.7 ng/mL, respectively. But, serum CEA at the time of relapse was 132.4 ng/mL, and AFP was unfortunately not searched.","A 63-year-old man was found to have a small growth in his lung during a routine checkup. Further tests, including a CT scan and biopsy, confirmed that the growth was lung cancer. The cancer was surgically removed, but unfortunately, it had spread to nearby lymph nodes. The patient is now undergoing further treatment for the cancer that has spread to his lungs."
954,"A healthy 40-year-old man presented at the emergency room after a high energetic accident with multiple fractures and comprehensive soft tissue defects on his left lower leg and foot. See . After clinical examination in combination with X-ray images and CT-scan of his left lower leg the following fractures were identified: a Gustilo grade IIIB fracture, a medial malleolus fracture, a Tillaux-Chaput fracture, a nutcracker fracture of the cuboid, an avulsion fracture of the navicular bone, fractures of the third, fourth and fifth metatarsal head, a mid-shaft fracture of the third proximal phalanx and base fractures of the first and second proximal phalanx. See . In the CT-scan of the left lower leg and foot is displayed. Active extension and flexion of the toes were preserved, as well as sensibility in the toes and forefoot. Disturbed sensibility to touch and pain was identified at the foot sole. No signs of a compartment syndrome were found.\nWithin the first thirteen hours after admission placement of an external fixator and debridement of the proximal medial wound was conducted. The soft tissue defects were temporarily covered with EpigardTM (Biovision, Ilmenau, Germany) and absorbent dressings. Within the first 24 hours postoperatively NIRF imaging was performed. See and .\nThe imaging head of the FluobeamTM (Fluoptics, Grenoble, France) was positioned approximately 8 inches above the skin. Real-time images were obtained during 120 seconds, starting 10 seconds after intravenous injection of 2 mL (5 mg) of ICG. The fluorescent region (white area) indicates the area of vital tissue. Regions of no fluorescence (black area) and less fluorescence (surrounding grey area) were marked to indicate the area of non-viable tissue.\nThe patient underwent a second debridement approximately 2.5 days after initial admission. The remaining defects were covered with EpigardTM and absorbent dressings. Postoperative non-viable skin was marked again guided by NIRF imaging. Remarkably, a slight increase of the fluorescent area was visualized at the lateral malleolus. A final debridement was performed 6 days after hospital admission in the same session as definitive soft tissue coverage. The remaining avital soft tissue was removed under real-time intraoperative NIRF image-guidance. Intraoperative imaging showed no difference in the extension of the fluorescent area compared to the previous NIRF imaging. A free anterolateral thigh flap was harvested to cover the defect on the medial malleolus and foot sole. Additional split skin graft was placed to cover the superficial defect at the lateral malleolus. No complications were recorded and the patient was discharged after five weeks. Nine weeks postoperatively the patient and flap were doing well. Clinical rehabilitation care is completed and he mobilizes with crutches. See for illustration of the clinical course of the lower leg and foot.","A healthy 40-year-old man was brought to the emergency room after a serious accident that caused multiple fractures and significant soft tissue damage to his lower leg and foot. Doctors found several complex fractures, including a severe break in his lower leg and foot, and used imaging to precisely map out the damage. During his hospital stay, doctors used a special imaging technique called NIRF to guide the removal of dead tissue and ensure they were only treating healthy areas. After several debridements and skin grafts, the patient’s foot and leg began to heal, and he was discharged after five weeks with crutches and ongoing rehabilitation. He is now doing well nine weeks after his injury, with his leg and foot recovering and he is able to walk with assistance."
955,"A 50-year-old male patient underwent right lobe liver transplantation from a living\ndonor. The patient was in end-stage liver disease secondary to ulcerative colitis\nand sclerosing cholangitis. Impairment in liver function tests and massive pretibial\nedema developed three months after surgery. The patient’s fever was 38,5\n°C, and he was hospitalized for treatment. In laboratory analysis, ALT levels\nwere 400 IU/L; AST levels were 300 IU/L; GGT levels were 118 IU/L; direct bilirubin\nlevels were 0,7 mg/dL; and the white blood cell count was 25000/µL (neutrophils\n82 %). Meropenem 3 × 1 gram and teicoplanin 1 × 400 mg were started for\ninitial treatment. A pseudoaneurysm measuring 8 × 6.5 cm and having central\nturbulent flow with peripheral thrombus in CDUS was detected in the portal hilus\nassociated with the hepatic artery (Figure ).\nMDCT angiography was applied for a better anatomic orientation; the pseudoaneurysm\noriginated from the hepatic artery anastomosis site and extended anterior to the\nartery. The central portion of the HAP was filling with contrast media while the\nperipheral portions were thrombosed (Figure ).\nThe hepatic artery was 2 mm in diameter, and string-like narrowing was observed\ndistal to the pseudoaneurysm best seen in the arterial phase of the examination. A\nloculated fluid collection around the pseudoaneurysm extending to the\nsubdiaphragmatic and perihepatic spaces was also observed (Figure ). In the follow ups, total bilirubin increased\nto 9,3 mg/dL, direct bilirubin increased to 6,6 mg/dL, and the white blood cell\ncount was 30000/µL. The patient was hemodynamically unstable. Stenting of the\nhepatic artery was planned, but the patient died before the procedure.","This 50-year-old man received a liver transplant to treat severe liver disease caused by inflammatory conditions. After the transplant, he developed a fever and swelling, and tests revealed elevated liver enzymes. A blood clot formed near the connection of the new liver artery, causing a dangerous pseudoaneurysm. Sadly, the patient passed away before a procedure could be done to fix the clot, and his condition worsened."
956,"We report the rare case of a 33 years old pregnant woman with an acute gestational obstructive pyelonephritis. A right ureteral stent was placed at the twelfth pregnancy week in a private institution with a good evolution of the symptoms.\nWhen time came to remove the stent after the pregnancy, the urologist could not find it nor in the bladder, neither in the all urinary tract at ureteroscopy. A thoraco-abdomino-pelvic computed tomography (CT) was performed and showed that the stent had moved intravascularly. It had migrated through the right iliac vein into the inferior vena cava and right atrium (Figs. , ). The patient was sent to our institution for extraction.\nThe patient complained about insidious onset of palpitations and moderate right flank pain. She had no macroscopic hematuriprobably due to intracatheter blood clotting.\nThe patient was then transferred to the vascular surgery department and an endovascular extraction was performed by puncture of the femoral vein. Extraction of the endoprosthesis was made by a curved guide introduced through the vena cava up to the right atrium under fluoroscopic control. The guide was subsequently twisted around the stent and pulled it out. No other complication was noted and the patient was discharged on the following day.","A 33-year-old pregnant woman developed a serious kidney infection that required a stent to be placed in her urinary tract. After the pregnancy, the stent couldn't be removed, and doctors discovered it had traveled through her veins into her heart. She was transferred to our hospital for a special procedure to extract the stent using a minimally invasive technique. The stent was successfully removed without any further complications, and she was discharged home the next day."
957,"A 41-year-old man presented at the emergency department with complaints of\nintolerable pain in the left upper quadrant. He had undergone extracorporeal shock\nwave lithotripsy (ESWL) eight hours prior as treatment for a left-sided kidney stone\nin the upper pole. Ultrasound demonstrated a large subcapsular hyperechoic\ncollection in the spleen (Figure ). A\ncomputerized tomography (CT) scan confirmed a laceration of the lower pole of the\nspleen with a subcapsular hematoma and a discrete amount of surrounding free fluid.\nAdjacent to the splenic laceration, a smaller subcapsular hematoma was also present\nin the left kidney (Figure ). In the meantime,\na fragmented stone in the proximal left ureter was visualized (1400 HU). The therapy\nwas conservative with hemodynamic follow-up in the intensive care unit with normal\npatient recovery","A 41-year-old man went to the hospital because of severe pain in his upper left side. He recently had a procedure to break up a kidney stone, but it caused a tear in his spleen and a smaller bleed in his left kidney. CT scans showed a collection of blood in the spleen and kidney, along with a fragment of the stone in his ureter. The doctors are monitoring his condition closely and expect him to recover fully."
958,"An 81-year-old man with an history of Alzheimer’s disease, excess weight and type-2 diabetes presented to the emergency department with a two-day history of diffuse abdominal pain. Contrast-enhanced abdominal computed tomography (CT) showed diffuse fluid distention of the small bowel loops indicative of obstruction. In addition, there was a profusion of dilated small bowel diverticula along the mesenteric border (Figure – coronal posterior and – more anterior views. White stars indicate the diverticula). An isolated jejunal diverticulitis was diagnosed in the right flank (Figure – axial, – coronal, and – sagittal views. White stars indicate the distended inflammatory diverticulum and white arrows show the surrounding inflammatory fat stranding). The cause of the small bowel obstruction was a 3 cm large enterolith impacted in the distal ileum (white arrows on Figure – sagittal and – axial views). A Meckel’s diverticulum was also incidentally discovered on the antimesenteric border of the ileum (black arrow on Figure ). Small bowel obstruction caused by the release of an enterolith from jejunal diverticulitis in the context of Diffuse Jejuno-Ileal Diverticulosis (DJID) was the final radiological diagnosis.\nAt the time of laparotomy, the enterolith had migrated further than the Meckel’s diverticulum, causing opportune dilatation of this diverticulum. Surgeons then performed an elective resection of the Meckel’s diverticulum wherein the enterolith could be manually and proximally retrogradely pushed. The inflamed jejunal diverticulum was also resected. The post-surgical period was uneventful.","An 81-year-old man with Alzheimer’s, diabetes, and a bit of extra weight came to the hospital because he was experiencing severe abdominal pain. Tests showed a blockage in his small intestine and a collection of pouches (diverticula) along his intestines. Doctors found a small stone in his ileum (part of the small intestine) that was causing the blockage and a Meckel’s diverticulum. Surgeons removed the stone and the Meckel’s diverticulum, and the inflammation was treated, leading to a smooth recovery."
959,"The patient is a 66 years old male patient who had prior spine surgery 15 years ago for a right L4-L5 disc herniation with initial good clinical results. Three years later, he complained of pain recurrence in the right L5 territory. Imaging at that time showed no new disc herniation and he was referred to the pain clinic. Several intra-foraminal L4-L5 and L5-S1 corticosteroid injections were performed during the last 10 years, as well as percutaneous radiofrequency denervation at the L4-L5 and L5-S1 facet joints. Long term complains were moderate.\nIn June 2014, the patient complained of a very severe bilateral L5 sciatalgy with paresthesias but no motor dysfunction. Mictional delay was also reported.\nMRI performed in July 2014 shows a disc herniation originating from the L4-L5 disc. It perforates the common longitudinal ligament and the anterior aspect of the dura, and enters the thecal sac (Fig. and ), The intradural “part” of the herniation is very large and extends downwards (Fig. and ), reaching the level of the upper sacrum, the disc fragment being surrounded by the rootlets ( to Fig. ). The upper part of the disc fragment, at the level when it enters the dura, is “compact”, as usually seen in extradural disc herniations (Fig. ), and some peripheric contrast enhancement can be seen after gadolinium injection, while the intradural part seems to be less compact, with a “crumbled” appearance (Fig. and ), irregular borders and no contrast enhancement.\nSurgery was performed in prone, antilordotic position under general anesthesia, via hemilaminectomy L4 and L5 on the clinically dominant right side with adjacent hemiflavectomy L3/4 and L5/S1, making a sharp parasagittal dural opening possible from a normal aspect of the dural sac into a heavily, scar tissue related metaplastic part of it (as often seen in reoperations and accountable for a higher risk of dural leak). The herniation was plain to see inside the dural sac, clearly causing a compression of most adjacent rootlets, but not really adherent to them (maybe due to the contact of cerebro-spinal fluid).\nThe neurosurgeon found the disc fragment in the spinal fluid to be “spongious, more friable and softer than the usual extradural herniation (Fig. and ).\nPatient was discharged asymptomatic on day 5 and had an uneventful recovery.","This 66-year-old man has had back pain for many years, originally from a disc problem in his lower back. Recent imaging showed a new disc herniation pressing on his nerves, causing pain and bladder issues. He underwent surgery to remove the herniated disc and relieve the pressure on his nerves. The surgery was successful, and he is now recovering well without any pain."
960,A 47-year-old woman was referred to the imaging department for Computed Tomography Angiography (CTA) of the supra-aortic arteries in order to investigate the cause for systemic blood pressure asymmetry. Prior Doppler ultrasonography of the cervical arteries had failed to demonstrate the vertebral arteries.\nCTA (Figure ) confirmed major hypoplasia of the vertebral arteries (red and white arrowheads on a) especially on the left side (red arrowhead). These hypoplastic vertebral arteries were not connected to the basilar artery (blue arrowhead on a) which was only fed by a large ascending artery (white arrows on Figures and ) emerging from a large left internal carotid artery (green circle). 3D volume rendering views without bone removal (Figure and ) showed that this atypical artery was penetrating the skull through the hypoglossus canal (red circle on Figures and ). The artery was clearly identified as a persistent hypoglossal artery. The finding was considered as fortuitous.,"This woman came to the imaging department to investigate why her blood pressure was uneven. Tests showed that her vertebral arteries, which are important blood vessels in the neck, are much smaller than normal. These small arteries aren't connected to the main artery that supplies blood to the brain, and instead, a separate artery is feeding the brain. This unusual artery is called a persistent hypoglossal artery and is passing through a small opening in her skull. The doctors found this to be a surprising, but harmless, discovery."
961,"An 80-year-old woman with a history of subarachnoid haemorrhage was brought to the emergency room because of speech problems. The patient was found in the morning by her relatives with severe speech comprehension and production difficulties. The exact time of symptom onset was unclear. Clinical examination at the ER revealed a sensory aphasia without other neurologic deficits. Stroke was suspected and the patient underwent a brain CT with an optimized stroke protocol consisting of an unenhanced CT, perfusion CT, and a CT angiography. Unenhanced CT showed no abnormalities, apart from already known and unchanged chronic hypodense gliotic changes in the left temporo-insular region (Figure ). Perfusion CT showed a normal to slightly diminished mean transit time (MTT), a diminished time to drain (TTD) and a clearly increased cerebral blood flow (CBF) and cerebral blood volume (CBV) in the entire left parietotemporal region (Figure ). No intra-arterial clots were seen on CT angiography. Urgent brain magnetic resonance imaging (MRI) was performed. Diffusion weighted images (DWI) showed subtle diffusion restriction in the left parietotemporal region, corresponding with the area of hyperperfusion on CT, without accompanying signal alterations on FLAIR (Figure ). Based on the clinical presentation and imaging findings, a nonconvulsive status epilepticus or postictal Todd’s paresis was suspected. An Electroencephalography (EEG) was performed within two hours of the MRI. Only interictal epileptiform changes were seen with sharp theta waves and spike waves in the left temporal region. These EEG findings were not compatible with an ictal state – seizure or status epilepticus – as the discharges were neither rhythmic nor continuous. Therefore, a diagnosis of postictal sensory aphasia was made, presumably after a focal seizure involving Wernicke’s area. An antiepileptic treatment with levetiracetam was started. There was gradual improvement of the patient’s speech difficulties over three days and she was discharged home after six days without residual symptoms.","An 80-year-old woman was admitted to the hospital because she was having trouble speaking and understanding what people were saying. Doctors found that her brain was showing signs of a recent stroke, but the imaging tests didn't show a large clot. Further tests, including an MRI, revealed subtle changes in her brain that suggested she had a small seizure after a stroke. She received medication to help prevent future seizures, and her speech gradually improved over several days before she was able to go home."
962,"A 67-year-old woman was admitted for retro-orbital and periorbital pain, increasing over 1 week and accompanied by progressive exophthalmia and palpebral ptosis on the left side. She was being treated for lung cancer metastasized to the liver and cerebellum. Physical examination revealed a complete ptosis of the left eyelid and complete absence of extraocular movements of the left eye, referable to the third, fourth and sixth cranial nerve. Head computed tomography (CT) showed mucous thickening of the left sphenoid and maxillary sinuses. Brain MRI revealed an expansive lesion involving the left cavernous sinus (Figure ), which proved to be a new occurrence by comparison with a contrast-enhanced CT performed several weeks earlier. The lesion in the cavernous sinus appeared to be inseparable from T2-hypointense changes in the posterior part of the left sphenoid sinus (Figure ,), whereas the remaining wall thickening of the ethmoidal, sphenoid and maxillary sinuses appeared to be smooth and T2-hyperintense (Figure ). The lesion in the cavernous sinus showed marked enhancement on contrast-enhanced T1WI and seemed to be inseparable from the carotid artery (Figure ). Time-of-flight MRA (TOF-MRA) confirmed the presence of a saccular aneurysm of the cavernous portion of the left internal carotid artery, with evidence of turbulent flow within the aneurysm and with expansion of the aneurysm toward the left side (Figure ,). In addition, circumferential wall thickening of the parent carotid artery was seen on T2WI immediately proximally (Figure ) and distally to the aneurysm (Figure ), with a corresponding concentric narrowing of its lumen visible on MRA. Endoscopic sinus surgery was performed with removal of mucus and fungal debris, which was confirmed as aspergilloma on pathologic examination. The diagnosis was made of a mycotic aneurysm of the intracavernous carotid artery resulting from local spread of invasive aspergillosis from the adjacent sphenoid sinus, secondary to an immunosuppressive status resulting from chemotherapy and longstanding systemic corticosteroid treatment. Because of the dismal prognosis of the malignancy and contraindication of antiaggregative drugs for reasons of a single hemorrhagic metastasis in the right cerebellar hemisphere, the multidisciplinary decision was made to refrain from endovascular treatment. The patient died a few weeks later.","This 67-year-old woman was admitted to the hospital because of increasing pain around her eyes and drooping eyelids on one side. She also has lung cancer that has spread to other parts of her body. Tests revealed a new aneurysm in a major blood vessel in her head, which was causing inflammation and infection. Because of her other serious health problems, doctors decided not to try to repair the aneurysm and she sadly passed away a few weeks later."
963,"An 8-year-old boy with a history of 5 years of acute lymphoblastic leukemia was referred for radiographic evaluation of the pelvis because of left hip pain and a limp. The patient had undergone radiation therapy of the total body and bone marrow transplantation at age 6. Since the onset of the disease he had been receiving intravenous and oral corticosteroids. Three months post-transplantation, the patient developed lung disease secondary to graft- versus-host disease and received high doses of intravenous and oral steroids. Subsequently, the patient developed autoimmune hemolytic anemia and was treated again with high doses of intravenous and oral steroids.\nPhysical examination revealed a small for his age Cushingoid boy with severe loss of muscle bulk due to steroid-induced myopathy. His height was 47 inches, which lies between the 3rd and 9th centile and his weight was 54.3 lb, which lies between the 24th and 50th centile. Radiographic evaluation of the spine and hips revealed compression fractures in thoracic spine (T11, T12) and mild deformity of the femoral epiphyses (Fig. ). Because of the history of long-term corticosteroid use, BMD was measured in the lumbar spine (L1-4) [0.485 g/cm2, Z-score -1.97] and proximal femur [0.481 g/cm2] using DXA (Fig. ). Trabecular BMD was measured at the 4% distal radius using peripheral quantitative computed tomography (pQCT) [109.5 mg/cm3, Z-score -2.2]. On the basis of established pediatric densitometric criteria [], a diagnosis of low bone mineral content was made and patient was started on treatment with vitamin D and calcium. Follow-up DXA studies to determine response to treatment at 12 months demonstrated collapse of the femoral head and increasing BMD (Fig. ). The lumbar spine and hip bone densities were 0.542 g/cm2, Z-score -1.46 and 0.567 g/cm2, respectively. Sequential follow-up DXA scans at 24 months showed further destruction of the femoral head and an increase in bone mass (Fig. ). The lumbar spine and hip bone densities were 0.591 g/cm2, Z-score -1.35 and 0.788 g/cm2, respectively.\nTrabecular BMD at the 4% distal radial site by pQCT at 12 and 24 months was 135.8 mg/cm3, Z-score -1.6 and 133.7 mg/cm3, Z-score -1.9, respectively. The concurrent visual assessment of the DXA images showed progressive deterioration of the left hip joint, beginning as vague osteosclerosis and becoming severe with destruction of the femoral head, suggesting the diagnosis of osteonecrosis.","This 8-year-old boy has a history of leukemia and has been taking strong medicines (steroids) for many years. Because of these medicines, he has developed some problems with his bones, including fractures and a loss of bone density. X-rays showed that his bones are weaker than they should be, and he’s been given vitamin D and calcium to help strengthen them. Over time, the X-rays show that his bones are improving, but he is still at risk for further bone problems."
964,"A 15-year-old girl who attempted suicide using sulfonylurea, an oral hypoglycemic drug and subsequently became comatose was admitted to the pediatric emergency department. The patient had no known previous disease. On admission, vital signs (blood pressure, pulse, respiratory rate, and temperature) were normal. On neurological examination, the patient’s pupils were isochoric and reacted to light promptly. Corneal and oculecephalic reflexes were intact. The patient showed responses to painful stimuli. Except for a blood glucose level of 10 mg/dl, all other blood laboratory tests (complete blood count, electrolytes, liver and renal functions, arterial blood gases) were normal. Intravenous dextrose was injected immediately, followed by glucose infusion and somatostatin treatment to restore the blood glucose level. As there was no doubt about the use of the oral hypoglycemic drug for suicidal purpose, a toxicology test was not performed.\nOne hour after admission, a T2-weighted image on MRI (1.5 Tesla Siemens Magnetom Symphony Quantum, Erlangen, Germany) showed isolated focal hyperintensity in the SCC and a decrease in the apparent diffusion coefficient (ADC) compatible with diffusion restriction, in addition to a significant signal increase in the DWI (Fig. ). After receiving treatment for 24 hours, the patient became conscious, and her blood glucose level returned to normal (90 mg/dl). DWI was repeated two days after the first MRI, and a control DWI (Fig. ) showed complete resolution of the lesion in the SCC.","This 15-year-old girl was brought to the hospital after attempting suicide by taking a medication for diabetes. She was initially unconscious and had a very low blood sugar level, but doctors quickly brought her back to consciousness and stabilized her condition. Brain scans showed a small area of damage in her brain, which resolved on its own after treatment. She is now recovering well and is expected to make a full recovery."
965,"A 63-year-old man followed up for diabetes mellitus, hypertension and recently increasing vertigo with bilateral murmer at his neck. The patient had no neurological deficit and referred to radiology for the examination of cerebrovascular diseae. Bilateral carotid color duplex Doppler examination showed mild atherosclerotic plaques of the common carotid bifurcation without any hemodynamically significant stenosis. Cerebral MRI revealed chronic ischemic signal changes in pons and nonspesific signal changes in white mater of bilateral frontal lobes. Selective carotid and vertebral angiographies were performed in order to confirm the stenoses in proximal parts of both internal carotid arteries; occipital arteries were arising from the cervical segments of internal carotid arteries on both sides (Fig. ). Selective right vertebral angiography revealed the patency of the artery with hypoplasia of V3–V4 segments and intracranial and intervertebral collateral flow to the left vertebral artery (Fig. ). Left subclavian angiography prior to catheterization of vertebral artery demonstrated severe stenosis of the vertebral artery. Left vertebral artery is also opacified through the anastomoses between the muscular branches of occipital and vertebral arteries (Fig. ). A baloon-expandable intravascular stent was placed to the ostium of the left vertebral artery in order to treat the high grade stenosis (Fig. ).","This 63-year-old man is being followed for diabetes, high blood pressure, and recent dizziness. Tests showed some plaque buildup in his neck arteries and changes in the brain, likely due to previous strokes. A special scan of his brain and neck arteries revealed a significant narrowing in the left vertebral artery. To improve blood flow, a stent was placed in the narrowed artery, and the patient is being monitored closely."
966,"A 49-year-old woman complains of new onset episodes of vertigo post stapedectomy 12 years ago. Symptoms can be evoked by pressing or when lying on the left ear. No remarkable findings on ear inspection or clinical exam. Epley maneuvers had no effect for treatment of possible benign paroxysmal positional vertigo (BPPV). Other differential diagnoses were perilymphatic fistula, intravestibular granuloma, labyrinthitis and stapes prosthesis protrusion.\nA temporal bone high resolution CT (HR CT) was performed and showed migration of the stapes prosthesis into the vestibule (protrusion) (Fig. ). Depth of protrusion was measured 14 millimeters from tip of the prosthesis to the oval window. The diagnosis of intravestibular stapes prosthesis protrusion related post stapedectomy vertigo was made and revision surgery was proposed.","This 49-year-old woman is experiencing dizziness (vertigo) that seems to be related to a previous surgery on her ear, a stapedectomy performed 12 years ago. The dizziness happens when she moves her head in certain ways, especially when lying on her left ear. Tests showed that the small device used in the surgery, the stapes prosthesis, has moved into a sensitive area of her ear. Because of this, surgery to reposition the prosthesis is recommended to help stop the dizziness."
967,"A 65-year-old woman presented with a three-month history of persistent ocular-nasal catarrh, sub-febrile state, myalgia, night sweats, weight loss and early daily bi-temporal pain. Severe biological inflammatory syndrome with CRP at 105 mg/l and sedimentation rate at 97 mm was present. Colour Doppler Ultrasound (CDU) (Figure ) showed a typical inflammatory “halo sign” (black arrowhead on a) of the temporal arteries. A similar “halo sign” was found along the common carotid (white arrows on c, d, and e) and vertebral arteries (black arrows on d and e). The intima remained visible (white arrowheads on e). Computed Tomography (CT) angiography (Figure ) showed blur homogeneously enhancing wall thickening of the aortic arch (white arrows on a and d) and of its large emerging arteries (white arrows on b). The axillary arteries were also affected (black arrows on a). Moderate thickening of the abdominal visceral aorta was also found (not illustrated). Hypodensity of the intima contrasted with enhancement of the inflamed media (white arrowheads on b). Temporal artery biopsy (Figure and ) confirmed typical giant cell arteritis (GCA) with involvement of the media (yellow star) and adventice (white star) by chronic lymphocytic inflammation. Reactive intimal hyperplasia (black star) causing luminal collapse (white arrow), characteristic fragmentation of the internal elastic lamina (black arrows) and giant cells (black circles) were also diagnosed. Classical massive corticosteroid treatment was immediately started with rapid clinical and biological improvement. The “halo sign” and diffuse arterial thickening had, drastically, nearly completely resolved on post-therapeutic CDU (Figure and ) and CT (Figure and ) nine months later.","65-year-old woman came to the doctor with a long-term problem of eye and nose irritation, fever, muscle aches, and weight loss. Blood tests showed a strong sign of inflammation, and an ultrasound of her arteries revealed a characteristic “halo” around the vessels, indicating inflammation. A CT scan confirmed widespread inflammation in the aorta and its branches. After a biopsy confirmed giant cell arteritis, she started on strong medication, which quickly improved her symptoms. Her arteries have now shown significant improvement after several months of treatment."
968,"A 64-year-old woman was admitted to the neurosurgery department for V2–V3 right- sided trigeminal paresthesia, which had developed six months earlier. Magnetic resonance imaging (MRI) showed a well-circumscribed, fleshy, 36 mm tumor in the Meckel cavum involving the Gasserian ganglion, following the V3 nerve into the foramen ovale, and generating a mass effect on the temporal lobe. This lesion was discreetly hyperintense on T1-weighted images and iso- to hypointense on T2-weighted images, and it showed a slightly heterogeneous enhancement after contrast administration. Small cystic areas were noted. Diffusion-weighted imaging was negative. An abnormality was already seen on a computed tomography (CT) performed two years previously in the context of otitis, without any alarm in relation to the benign presentation, a well-defined petrous lacunar image, and the fortuitousness of the discovery (Figure ). Diagnosis of schwannoma was proposed, without specification. The patient underwent a macroscopically complete surgical resection by an exclusive extradural subtemporal approach with excellent symptomatic recovery. The postoperative CT demonstrated no complications. Immunohistochemical examination finally specified the diagnosis of MS, a very rare pigmented tumor (Figure ). The clinical course was good. However, a recurrence was detected on the MRI performed at three months. 11C-methionine positron emission tomography (MET-PET) confirmed suspicions (Figure ), and a new surgical intervention was planned followed by adjuvant radiotherapy.","64-year-old woman was admitted to the hospital because of numbness in her face that started six months ago. Tests showed a small, non-cancerous tumor in her skull was pressing on her nerves. The tumor was successfully removed during surgery, and she felt much better afterward. However, a follow-up scan revealed the tumor had grown back, and doctors are planning another surgery and radiation treatment to treat it."
969,"A 32 year old female patient presented to the cardiology clinic with an atypical chest pain. Her history revealed no other condition than Leopard syndrome which was diagnosed on her birth. However, her family history revealed that her older sister who also had Leopard syndrome, was diagnosed with pulmonary stenosis 5 years ago and underwent successful baloon valvuloplasty. The patients mother, who also had Leopard syndrome had undergone open heart surgery due to pulmonary stenosis 20 years ago and 5 years earlier she had had percutaneous coronary artery angioplasty due to coronary artery disease. On her physical examination; pectus excavatum, multiple lentiginous lesions on the face and hands (Figure ), ocular hypertelorism, and short height were detected. Sinus rythm and left branch block was detected on her electrocardiography. Her echocardiography was as follows: left ventricular ejection fraction 60%, normal heart chambers, atrial septal defect (4mm) (Figure ), low grade pulmonary valve stenosis (maximal gradient 18 mmHg, mean gradient 9 mmHg), right ventricular pressure 20 mmHg.\nOn her coronary CT angiography, atrial septal defect, 3 mm in its widest place, widened left pulmonary artery (diameter 34 mm), mild thickening of the pulmonary semilunar valves were detected. The right coronary artery was dominant and diffusely ectatic (diameter: 7.5 mm widest). The right ventricular branch of the right coronary artery was directly originating from the sinus of Valsalva and this branch also gave the conus branch. PDA and PLB reached the apex through the interventricular sulcus. LMCA originated from the right coronary sinus and had a prepulmonic course (Fig. ). LMCA (6,5 mm) and LAD (6,2 mm) were ectatic throughout their course. Circumflex artery structure and callibration were normal. The structures of the large cardiac vein and coronary sinus were normal. The anterior interventricular vein was draining directly to the left atrium.\nCoronary angiography revealed similar findings as the coronary CT angiography. Maximum gradient of 20 mmHg was observed in pulmonary valve hemodynamic study. As a result of the investigations it was decided to follow up the patient with medical treatment.","This 32-year-old woman came to the cardiologist because of chest pain. She has a family history of heart problems, including her sister and mother who both had pulmonary stenosis and required surgery. Her physical exam showed some features of Leopard syndrome, and tests revealed an atrial septal defect and mild narrowing of her pulmonary valve. Further imaging showed some widening of the arteries and a dominant right coronary artery, and she will be monitored with medication."
970,"A 39-year-old woman was admitted to our hospital presenting with dizziness and cerebellar ataxia.\nComputed tomography (CT) scan revealed a large partially cystic mass in the left cerebropontine angle (CPA) with calcifications, compressing the brainstem and the cerebellum (Fig. ).\nMR imaging (Figs. , ) confirmed the presence of a large mass in the left CPA. The mass had two components, cystic and solid. The cystic component of the mass was located in the left CPA, extending anteriorly to the 7th cranial nerve and medially compressing the brainstem. It showed low signal intensity on T1W and high signal intensity on T2W ones and with no enhancement after intravenous administration of contrast media. On DW images the mass displayed high signal intensity, ADC 0.8 and the spectral pattern showed a medium lipid/lactate peak at 1.3 ppm, findings typical for IEC. The juxtaposed solid component of the mass compressed the cerebellum causing perilesional edema. It showed low signal intensity on T1W and T2W images. After intravenous administration of contrast media, it displayed heterogeneous, mainly peripherical and in some sites nodular enhancement. DW imaging showed low signal intensity inside the mass and ADC values of 1.34. MRS revealed a high lipid/lactate peak, Choline/Creatine ratio (Cho/Cr) 2.13 and Choline/N-Acetylaspartate ratio (Cho/NAA) 0.766.\nSurgery was planned and the mass was completely resected. Histopathologic analysis (Fig. ) revealed that the cystic component was consistent with benign IEC. The adjacent solid component consisted of squamous epithelial cells, with nuclear pleomorphism and mitotic activity. The superficially lined cells were filled with laminated keratin. There were also islands of squamous epithelium with an infiltrative growth pattern.","This 39-year-old woman was admitted to the hospital because she was experiencing dizziness and difficulty with coordination. Tests revealed a large, complex mass in the area of her brainstem and cerebellum. The mass was surgically removed, and the pathology showed it was a benign type of tumor. The tumor was made up of different types of cells, including squamous cells, and was completely removed."
971,"A 31-year-old nonsmoking woman, complained of dyspnea and polyarthralgia following a cesarian section. She had no fever, no sputum production, and no cough. The patient worked as domestic help. Physical examination was normal, without auscultation abnormalities. Bloods tests showed D-dimer elevation and a slight hypereosinophilia (780/mm³).\nA CT angiogram revealed no evidence of pulmonary embolism. However, diffuse groundglass centrilobular nodules without tree-in-bud pattern were observed (Figure ), in association with small centrilobular apical emphysema (white arrow Figure ) and confluent condensed areas in the basal segments of the lower lobes (black arrow Figure ). The first diagnostic hypothesis was hypersensitivity pneumonitis, though there was no air-trapping on the CT. The etiological investigation failed to find any causative agent for this pathology.\nCytology of bronchial alveolar lavage (BAL) showed 16% of neutrophils, 29% of lymphocytes, and 55% of macrophages. There were no eosinophils found. A surgical pulmonary biopsy was performed in order to assess the interstitial pathology. Microscopic examination revealed an important non-caseating granulomatous interstitial inflammation, with lymphocytes (black arrow Figure ), numerous macrophages and multinuclear giant cells, sitting preferentially in the peribronchiolar regions (black star Figure ). These granulomas contain characteristic needle-shaped birefringent crystalline material in polarized light (black arrow Figure ). The morphological aspect of these crystals are similar to that of the talc.\nA second patient history was carried out. The patient admitted to using abundant cosmetic talcum powder in order to soften the skin daily over several years. She denied any intravenous drug abuse. Based on the patient’s history and the clinical, radiological, and histological findings, the diagnosis of talc induced interstitial lung disease (talcosis) was made based on massive use of talc powder.","This 31-year-old woman came to the hospital because she was having trouble breathing and joint pain after giving birth. Tests showed elevated levels of a protein called D-dimer and a slightly increased number of white blood cells. A CT scan of her lungs revealed small, unusual spots and some air pockets, which led doctors to suspect a lung problem. After further tests, including a lung biopsy, they found that the problem was caused by years of using talcum powder in cosmetics, leading to a condition called talcosis."
972,"A 53-year-old woman with invasive ductal breast carcinoma diagnosed 4 years previously was admitted to our hospital with a history of swelling in her left EAC for 2 weeks and a gradually worsening hearing loss. The patient had undergone right radical mastectomy and followed chemoradiotherapy following the diagnosis. The primary tumor was multifocal (5 different focuses) and located in upper and lower outer quadrants of the breast. The size of the largest tumor focus was 2.2 cm. The immunohistochemical study showed positive Estrogen Receptor. All of the 33 dissected axillary lymph nodes were free of metastasis. Post surgical staging was stage IIB. In follow up, bone and lung metastases appeared and relevant treatment was performed. Initially, she had noticed a moderate hearing loss in her left ear, but mentioned no otalgia, otorrhea, tinnitus, or vertigo. Physical examination revealed complete obstruction of the left EAC by a soft tissue mass. No neurologic symptoms were observed. HRCT of the temporal bone showed that totally obstructed left EAC by soft tissue density mass, but no bony erosion or destruction (Fig. ). MRI showed well defined, fusiform, soft tissue mass entirely filling the left EAC. The mass was iso-intense with muscle on T1-weighted images, slightly hyperintense on T2-weighted images. Following the administration of the gadolinium based contrast agent homogeneous enhancement was seen. Although the mass was located in both bony and cartilaginous EAC, there was no sign of bony or cartilaginous invasion (Fig. ). DWI showed slightly hyperintense signal on isotropic trace image and the apparent diffusion coefficient (ADC) map revealed slightly restricted diffusion in the mass. There was a small, non-enhancing hemorrhagic fluid collection, which is hyperintense on both T1- and T2-weighted images, between the mass and the tympanic membrane. The middle and internal ear structures were normal on both MRI and temporal CT images. We reported that the mass may be consistent with primary tumor of the EAC, but due to the history of metastatic breast carcinoma, mentioned EAC metastasis in the differential diagnoses.\nThe patient underwent surgical operation by the ENT surgeon and the mass was subtotally removed. Histopathologic examination of specimens revealed metastatic carcinoma that has same histopathologic features with the primary breast carcinoma. Histopathological finding of the excised lesion showed microscopically a infiltrative carcinoma compatible with breast origin (Fig. ) based on immunohistochemical study result that was positive for Estrogen Receptor (ER), Cytokeratin 7 and E-cadherin (Fig. ) but negative for C-Erb-b2 and Progesterone receptor (PR). Tumor was composed of atypical cells with large nuclei and prominent nucleoli in solid pattern. There was no ductal or tubular structure. Positivity of both ER and E-Cadherin also supported ductal type when compared to lobular carcinoma of the breast.","This 53-year-old woman, who has had breast cancer for four years, was admitted to the hospital because of a growing lump in her ear that was blocking her hearing. Imaging tests, including MRI and CT scans, showed that the lump was likely a metastasis (spread) of her breast cancer. Surgery to remove the lump confirmed that it was indeed breast cancer. The pathology report showed that the tumor was consistent with her original breast cancer, and further tests confirmed the presence of estrogen receptors."
973,"The patient was a 50-year-old woman with a chief complaint of edema. She had previously been healthy, and previous physical examinations revealed no abnormal urinalysis results. She experienced fever, cough, malaise, and low back pain for 6 days and edema of the face and limbs for 5 days before visiting our hospital. She was diagnosed with type B influenza by a local physician 3 days prior to visiting our hospital. The patient later exhibited an exacerbation of facial edema, decreased urine output, and a high level of proteinuria. She was subsequently diagnosed with nephrotic syndrome and was referred to our department.\nThe physical findings on admission were as follows: height, 150.1 cm; weight, 48.3 kg; body mass index, 21.4; body temperature, 36.2 °C; blood pressure, 109/83 mmHg; and oxygen saturation on room-air, 99%. In addition, the patient experienced facial edema and pitting edema of both lower legs. The laboratory findings on admission were as follows: total protein, 5.2 g/dL; albumin, 2.0 g/dL; blood urea nitrogen, 13 mg/dL; creatinine, 0.84 mg/dL; estimated glomerular filtration rate, 56.5 mL/min/1.73 m2; total cholesterol, 308 mg/dL; immunoglobulin G (IgG), 1320 mg/dL; IgA, 354 mg/dL; IgM, 192 mg/dL; IgE, 2966 IU/mL; complement 3, 83.3 mg/dL (normal range 65–141 mg/dL); complement 4, 40.9 mg/dL (normal range 13–40 mg/dL); and total hemolytic complement (CH50), 40.5 U/mL (normal range 31–48 U/mL). The patient was negative for hepatitis B virus surface antigen, hepatitis C virus antibodies, and human immunodeficiency virus antibodies. She had previously been infected with parvovirus B19. Her proteinuria level was 20.88 g/gCr, her selectivity index value was 0.13, and her urine sodium level was 8 mEq/L. Urine sediments showed 1–4 red blood cells per high-power field without any abnormal casts. There were no notable abnormalities electrocardiography or chest X-ray images.\nA kidney biopsy performed on the fourth day of hospitalization under suspicion of MCNS, IgA nephropathy, or post-streptococcal acute glomerulonephritis did not show glomerular basement membrane thickening, or endocapillary or mesangial cell proliferation (Figs. \n and ). An immunofluorescence study showed nonspecific granular IgM deposits in the mesangium. Electron microscopy showed extensive foot process effacement, but immune complex deposits were not observed (Fig. ). Based on the above findings, the patient was diagnosed with MCNS.\nAfter admission, the patient’s proteinuria decreased to 0.06 g/gCr with rest and sodium restriction (6 g/day) alone, and a complete remission from nephrotic syndrome was observed at approximately 2 weeks after the onset of symptoms. No recurrence of nephrotic syndrome was observed at an outpatient visit one month later (Fig. ). No recurrence has been noted in the one years since.","A 50-year-old woman came to the hospital with swelling in her face and legs, along with fever, cough, and back pain. She had recently been diagnosed with the flu and her symptoms worsened, leading to low urine output and high levels of protein in her urine. After further tests, she was diagnosed with nephrotic syndrome, a condition where the kidneys leak too much protein. Fortunately, with rest and dietary changes, her condition improved significantly, and she was able to return to normal without needing further treatment."
974,"An 80-year-old man with progressive pain lasting for 5 days and with focal swelling of the left foot was referred to the radiology department. Wearing shoes aggravated the pain. Inspection of his footwear showed a bump at the inner sole, corresponding with the location of the clinical abnormality at the heel of the patient. Clinically, there was suspicion of plantar fasciitis.\nPlain films showed the absence of inferior calcaneal spur formation. Ultrasound revealed a normal plantar fascia. Medially from the plantar fasciitis, adjacent to the course of the medial plantar artery, hypoechoic tubular structures were seen, which were not compressible. There was no intralesional flow on color Doppler imaging (Figure ). Comparison with the right foot showed normal compressible veins. Subsequent MRI confirmed thrombosis of the medial plantar veins (Figure ).\nThe symptoms disappeared after conservative therapy with change of footwear and non-steroidal anti-inflammatory drugs. Follow-up ultrasound 2 months later revealed no residual thrombus in the plantar veins.","An 80-year-old man came to the hospital because of pain and swelling in his left foot that had been bothering him for five days. X-rays and ultrasound showed that he didn't have plantar fasciitis, but instead, there was a blood clot in the veins near his foot. The clot was identified with MRI and treated with medication and new shoes. Fortunately, the clot resolved after a few months, and his symptoms disappeared."
975,"A 19-year-old man was admitted in the intensive unit after a suicide attempt by hanging. He had been found in cardiac arrest of imprecise duration. Cardiac massage was performed for 40 minutes during transfer. The patient was in a deep coma with Glasgow score at 3/15. Unenhanced Brain Computed Tomography (CT) performed after five hours (Figures and ) demonstrated bilateral hypodensity of the basal ganglia (white arrowheads). Hyperdensity of the cerebral arteries seemed to be related to the underlying brain hypodensity, with decrease in gray-white differentiation due to edema. Unenhanced CT after 30 hours (Figures and ) showed progression of brain edema with collapse of the sylvian fissures, basal cisterns and cortical sulci (black arrows). Diffuse hyperdensity of all collapsed sub-arachnoid spaces had become prominent (white arrows) evocating pseudo-subarachnoid hemorrhage (PSAH). Multiple evoked potential confirmed brain dead after 48 hours.","This 19-year-old man was found unconscious after a suicide attempt and was rushed to the hospital. He was in a coma and had suffered damage to his brain, as shown on a CT scan. The scan revealed swelling in his brain and a collapsed area, which led doctors to determine he was brain dead. After further testing, the doctors confirmed that he had passed away."
976,"A 48-year-old man with a history of alcohol abuse presented to the gastroenterology department for the first time in November 2006. He was suffering from severe epigastric pain and nausea for two days. Because the laboratory findings were suspicious for pancreatitis, the patient underwent computed tomography (CT) (Fig. ). The examination showed an edematous pancreas tail and fluid in the anterior prerenal space and in the anterior, posterior and lateroconal fascia. There were no signs of chronic pancreatitis or vascular complications. In the following months the patient had two similar episodes of acute tail pancreatitis.\nIn September 2009 he presented with vague epigastric pain of several weeks duration. Serum amylase and lipase levels were normal, however the cholestatic parameters were mildly elevated. Therefore, a magnetic resonance (MR) study was performed. The examination demonstrated moderate chronic pancreatitis of the tail with atrophy and irregular bording of the Wirsung’s duct. There were no other abnormalities. Further examinations also revealed a Helicobacter pylori gastritis.\nIn February 2012 the patient presented again to the gastroenterologist with epigastric and left upper quadrant pain. Laboratory findings were not suspect for acute pancreatitis. However, contrast-enhanced CT scan was performed in order to evaluate the known chronic pancreatitis and other causes of pain (Fig. ). This study surprisingly revealed a hypervascular lesion in the pancreatic tail consisting of a conglomeration of small hypervascular spots and blood vessels. Further examinations were performed in order to differentiate a vascular malformation from a hypervascular pancreatic tumor, especially an islet cell tumor or a hypervascular metastasis. Contrast-enhanced MR study showed a focal area of heterogeneous contrast enhancement without an apparent nodular component. The subsequent angiography showed two large feeding arteries in the early arterial phase, followed by a racemose vascular network, an early transient dense parenchyma stain in the early portal phase and early wash-out of the lesion in the portal phase (Fig. ). Based on these findings, a pancreatic arteriovenous malformation was suggested. The patient was treated with a resection of the pancreas tail and postoperative recovery was uneventful. Histopathologic examination revealed sequelae of chronic pancreatitis and numerous dilated blood vessels accompanied by blood clot formation and intimal hyperplasia (Fig. ). The histopathological findings were consisting with a pancreatic AVM.","This 48-year-old man has experienced several episodes of severe abdominal pain related to his pancreas. Initial tests showed inflammation, but later scans revealed a complex, unusual blood vessel network in his pancreas. After further investigation, doctors determined it was a pancreatic arteriovenous malformation, a rare condition. The patient underwent surgery to remove the affected portion of his pancreas, and he has since made a full recovery."
977,"A 64-year-old man presented at the emergency department with colic pain in the left lumbar region. The pain started acutely and was ongoing for more than one day. There were no urinary complaints, no vomiting, no respiratory complaints and normal stools. His medical history showed a lung carcinoma for which he was currently being treated with chemotherapy, hypercholesterolemia and a compression fracture of D4. On clinical examination, there was a left costovertebral angle tenderness. Urinary analysis was negative for proteins, glucose, bilirubin, hemoglobin, red and white blood cells. Laboratory results showed only slightly elevated C-reactive protein of 5.4 mg/L (<5 mg/L), slightly decreased hemoglobin of 12.1 g/dl (13.1–17.2 g/dL), elevated urate of 71 mg/dl (16.6–48.5 ml/dL), elevated creatinine of 1.6 mg/dL (0.67–1.17) and estimated glomerular filtration rate > 60 mL/min/1.73m2.\nB-mode abdominal ultrasound revealed normal anatomy of both kidneys and the bladder wall.\nUnenhanced abdominal computed tomography (CT) showed no abnormalities. The patient was admitted for intravenous pain management and observation. Pain persisted and a follow-up abdominal ultrasound was performed. The kidneys remained morphologically normal on B-mode. Color Doppler ultrasound showed normal vascularization of the right kidney (Figure ). There was no arterial signal in the kidney hilum or in the renal cortex of the left kidney (Figure ) that exhibited only weak, alternating venous flow (Figure ). These findings were highly suspicious of left renal artery occlusion.\nBecause of progressively decreasing kidney function, a contrast-enhanced ultrasound (CEUS) was performed. This showed normal vascular supply of the right kidney with homogeneous enhancement of the cortex (Figure ). On the left, there was only minimal cortical enhancement which extended from the periphery to the hilum, representing perforating branches of the renal capsular artery (Figure ). This finding is also known as the cortical rim sign which is seen on contrast-enhanced CT- or Magnetic Resonance-images in case of renal infarction. The findings confirmed an occlusion of the left renal artery.\nBecause complaints started more than 72 hours prior to diagnosis, there was no indication for thrombolysis. Therapeutic doses of low molecular weight heparines were started to prevent new thrombotic events and an adequate level of pain killers was continued. Kidney function increased over the next few days with eGFR of 53 mL/min/1.73m2 and creatinine of 1.4 mg/dL at hospital discharge.","A 64-year-old man came to the hospital with severe pain in his lower back. Tests revealed a possible blockage in the main artery of his left kidney, which was causing the pain and affecting his kidney function. The ultrasound and CT scans showed no other problems with his kidneys or bladder. He was treated with pain medication and blood thinners to prevent further clots, and his kidney function gradually improved during his hospital stay. He was discharged home with instructions to monitor his kidney function and continue with medication."
978,"A 49-year-old woman presented at the otorhinolaryngology department with symptoms of repeated upper airway infections for six months. She complained of nasal obstruction, headaches, sneezing, hyposmia, postnasal drip and coughing. Treatment with antibiotics and oral steroids had no effect. She had already undergone functional endoscopic sinus surgery with septal correction and partial reduction of a right-sided hypertrophic concha media bullosa in 2008. Endoscopic nasal examination showed a bilateral oedematous mass located medially and cranially in the nose, originating anteriorly of the attachment of the concha media. A computed tomography (CT) (Figure ) was performed and demonstrated the presence of a bilateral well-delineated soft-tissue mass in the olfactory cleft. There was bone remodelling resulting in widening of the olfactory clefts, but no bone erosion. The mucosa in the paranasal sinuses was only modestly thickened and there was no evidence of sinonasal polyposis. The patient underwent a magnetic resonance (MR) scan for further work-up. On MR (Figure ) the lesions appeared T1- and T2-isointense compared to white matter. The cribriform plate was intact and there was no intracranial involvement. A biopsy was performed and the presence of REAH was histologically confirmed. Endoscopic non-aggressive resection was performed (Figure ) and in the follow-up consultation three weeks later the patient was free of symptoms. Nasal endoscopic control four months after surgery showed no signs of recurrence.","6 This 49-year-old woman has been struggling with repeated sinus infections and nasal problems for the past six months, including a stuffy nose, headaches, and a loss of smell. She had previously had surgery to correct her nasal structure, but the problems continued. After further tests, including CT and MRI scans, doctors found a mass in her nasal cavity that was causing the issues. A biopsy confirmed that it was REAH (Reticulovascular Epidermoid Abnormal Histology), and a successful surgery to remove the mass resolved her symptoms completely."
979,"A 62-year-old woman was admitted to the Department of Gynecology at the Institute of Oncology Vojvodina with the complaint of vaginal bleeding for one year. Her past medical history was uneventful. The biopsy results of fractional curettage identified endometrial cancer (HP: carcinoma endometriodes, G2). She underwent total hysterectomy with bilateral adnexectomy. Histological findings confirmed the presence of endometrial cancer (HP: adenocarcinoma endometriodes endometrial, HG2, pT1c, FIGO Ic and Lieomyoma uteri). The right ovary was without pathological lesions, but the left ovary had a mature teratoma with dominant thyroid tissue and lesion of papillary cancer, 1,3 mm in diametar – malignant struma ovarii (Figs. , ).\nThree months after completing brachytherapy, she underwent total thyroidectomy. Histological findings were without evidence of papillary cancer (HP: Struma colloides polynodosa glandule thyroideae).The stimulated thyroglobulin (tumor marker in histological confirmation of thyroid cancer) level was detectable (Tg, 8.8 ng/ml; TSH, 25.49 mIU/ml) and negative antithyroglobulin antibodies. We decided to apply the radioiodine therapy in a dose of 3,7GBq 131-J. Post therapy whole body scintigraphy did not show distant metastases. Two foci of 131-I uptake were seen in the neck (Fig. ). The patient receives suppressive hormone L-thyroxin therapy. One month after radioiodine ablation, she continued treatment of endometrial cancer (external beam therapy). The first post therapy check of hormonal status, Tg and ATA were in an optimal range.","This 62-year-old woman was admitted because of a year-long problem of vaginal bleeding. Tests revealed she had endometrial cancer, and she underwent surgery to remove her uterus and ovaries. During the surgery, doctors found a cancerous growth in her left ovary as well. After further tests, including thyroid treatment, she is now receiving hormone therapy and radiation to treat both cancers, and her blood tests are showing promising results."
980,"A 25-year-old woman was referred to our radiology department by her family doctor for an MRI examination of the left knee because of a “crackling” noise of three months duration. There were no complaints of instability, swelling or pain. Physical examination showed anterior laxity of the knee. There was no recent history of trauma. The patient is known with a congenital shortening of the left leg for which she has already undergone a leg lengthening procedure.\nThe MRI examination shows multiple anatomic anomalies. The most notable is the absence of the anterior cruciate ligament (Fig. ). The posterior cruciate ligament is present but appears hypoplastic (Fig. ). The lateral intercondylar spine is absent and the lateral meniscus is hypoplastic (Fig. ). There is severe trochlear dysplasia due to hypoplasia of the lateral femoral condyle and medial patellar facet hypoplasia (Fig. ). Sequellae of earlier leg lengthening procedure can be seen: the left fibula is absent and metallic artefacts are present in the tibia.\nUp to this date, the patient was treated conservatively.","A 25-year-old woman was asked to get an MRI of her left knee because she heard a crackling sound in it for three months. The MRI showed some unusual features in her knee, including missing ligaments and smaller-than-normal cartilage. She also has a history of a leg lengthening surgery that has caused some changes to her bone structure. Because of these findings, the doctors recommend further evaluation and discussion of treatment options."
981,"We present the case of a 27-year-old woman who was referred to our hospital for further investigation, after worrying findings during a routine check-up performed in another hospital. Five years prior to this check-up, the patient was diagnosed with cancer of the left breast at the very young age of 22. The tumor was staged as pT2N0M0, with the histologic examination showing a poorly differentiated invasive ductal adenocarcinoma with strong estrogen and progesterone receptor expression and negative herceptin status. The treatment consisted of a wide excision and sentinel node procedure, followed by adjuvant chemotherapy, radiotherapy and hormonal therapy. Because of her young age, 3 cycles of cyclophosphamide-epirubicin-fluorouracil (FEC) and 3 cycles of docetaxel were given. Chemotherapy was followed by radiotherapy of the left breast up to a dose of 50 Gy, with a boost of 16 Gy on the tumor bed. Hormonal therapy consisted of a combination of tamoxifen and triptorelin. There was no relevant personal medical history, nor family history of breast cancer. Genetic analysis failed to show any BRCA1 or BRCA2 mutations.\nThe patient recovered well and follow-up examinations were normal. At the time of the check-up, five years after surgery, coinciding with the conclusion of the hormonal therapy, the follow-up mammogram (Fig. ) and first ultrasound showed the surgery related changes. (Fig. ) Because of her young age, magnetic resonance imaging (MRI) of the breasts was also performed (examination performed on Siemens Magnetom Symphony 1.5T), showing a multifocal nodular contrast enhancement in the retro-areolar region and the lower-outer quadrant of the right breast (Fig. ). These findings were not present on the previous MRI, performed two years after surgery. There were no clinical abnormalities in the region of this contrast enhancement. Because of the unclear etiology of these findings, the woman was referred to our department for further investigation. On ultrasonography, we were able to visualize some parenchymal distortion and an ill-defined hypoechoic lesion with a diameter around 1 cm and mild posterior acoustic shadowing in the region of the contrast enhancement on MRI (Fig. ). Because of the ultrasound and MRI findings and the patient’s history of breast cancer, the examinations were categorized as BIRADS 4. An ultrasound-assisted core biopsy (4 × 14 Gauge) was performed. Histologic examination showed a dense lymphocytic infiltrate of predominantly B-lymphocytes surrounding the ductulolobular unit and the vessels, in combination with a fibrous stroma of low cellularity and an increase in fibroblasts. These findings led to a diagnosis of sclerosing lymphocytic lobulitis. No signs of malignancy were detected.\nAfter diagnosis of sclerosing lymphocytic lobulitis, follow-up consisted of a breast ultrasound every six months and a yearly mammogram and MRI of the breasts. The volume of the hypoechoic lesion on ultrasonography and the intensity of the gradual multifocal contrast enhancement on MRI have both progressively diminished on consecutive examinations, to a level where it is barely perceptible (Fig. and ).","This 27-year-old woman was referred for a check-up after some unusual findings during a routine exam. Five years ago, she was diagnosed with breast cancer and treated with surgery, chemotherapy, radiation, and hormone therapy. Now, five years after completing her treatment, a new scan revealed some changes in her right breast, which doctors are investigating. After further testing, they determined that the changes are likely due to a benign condition called sclerosing lymphocytic lobulitis, which is a common reaction after breast cancer treatment and is not cancerous. She will continue to be monitored with regular scans to ensure the changes continue to improve."
982,"A 46-year-old man with no relevant medical history presented at the emergency department with nausea and a vague epigastric abdominal pain. An initial ultrasound examination demonstrated an ileus of the small intestine with small bowel wall distention mainly in the peri-umbilical region.\nComputed tomography (CT) confirmed a large mesenteric tumoral mass extending towards the ileum, where circumferential small bowel wall invasion caused intestinal obstruction (Figures and ). There was only a moderate amount of ascites. No signs of peritoneal carcinomatosis, distant metastases or free intra-peritoneal air were present.\nThe patient was subsequently referred for surgery, revealing an obstructive tumoral lesion in the ileum and a mass in the adjacent mesentery (Figure ). There was no peritoneal spread of disease. The affected ileum and mesentery were resected and an entero-enteric anastomosis was made.\nThe pathology examination confirmed an ileum tumor five centimeters in length, invading all layers of the bowel wall and a second, mesenteric mass six centimeters in length. Two out of nine lymph nodes were positive. On histology, the resected mass consisted of atypical cells with a high mitotic activity and an increased nuclear-cytoplasmatic ratio. Immunohistologic staining showed a high Ki-67 expression and highly positive myeloid markers such as MPO, CD-43, CD-117 and Lysozyme (Figure ). As such, the diagnosis of myeloid sarcoma was made.\nThe patient was referred to a tertiary center for further haematological work-up. Bone marrow aspiration showed no tumoral invasion. Induction chemotherapy was initiated and a stem cell transplantation was scheduled. PET-CT evaluation and haematological follow-up confirmed disease remission at the date of this publication.","This 46-year-old man went to the hospital because he was feeling nauseous and had pain in his stomach. Tests showed he had a blockage in his small intestine caused by a tumor. Surgery was performed to remove the tumor and the blocked section of the intestine, and a connection was made between the remaining parts. Pathologists found the tumor to be a rare type called myeloid sarcoma, and the patient is now receiving chemotherapy and a stem cell transplant to ensure the cancer doesn't return."
983,"A 28-year-old multiparous female presented to the Emergency Department with complaints of mild abdominal pain associated with nausea for two days. She described her pain episodes as mild in nature, located in the epigastric/left upper quadrant, nonradiating, and slight worsening with food intake. She denied fever, chills, vomiting, or diarrhea.\nHer past medical history was significant for a similar presentation about 6 months before when she was diagnosed with idiopathic acute pancreatitis. She denied any alcohol intake and was not on any medications or herbal supplements.\nOn admission, she was afebrile with a pulse rate of 84 beats per minute and a blood pressure of 116/80 mm Hg. Examination of the abdomen revealed mild tenderness in the epigastric region/left upper quadrant region and also with a palpable mass in the left lower quadrant. Laboratory workup revealed elevated lipase (4337), unremarkable CBC, and, with normal liver functions tests, lipid panel and IgG panel. Ultrasound of abdomen showed minimal sludge in the gall bladder without any obvious stones. CT abdomen with contrast demonstrated a spleen in the anterior left lower abdomen, elongated pancreatic tail which was coiled in conjunction with the splenic vessels, and with mild inflammation of the pancreatic tail (Figures and ). She improved clinically with conservative management with IV fluids. She finally underwent splenopexy at an outside facility.","This 28-year-old woman came to the hospital because she had mild abdominal pain and nausea for a couple of days. She had a similar problem six months ago and was told it was due to pancreatitis. Tests showed her pancreas was inflamed, and an ultrasound revealed some sludge in her gallbladder. A CT scan showed her spleen was also enlarged and pressing on her pancreas. She improved with fluids and had a procedure to secure her spleen."
984,"We discuss the case of a 74-year-old woman treated for breast cancer with bone metastases. Her past medical history also included pulmonary embolism, hypertension, appendectomy, hysterectomy, and aortic valvuloplasty. Annual follow-up ultrasonographic examination of the abdomen showed an unexplained pneumobilia (Fig. ). She was asymptomatic and during physical examination the patient’s abdomen was soft and she had neither jaundice nor diarrhea. Her blood workup was normal.\nCT of the abdomen was then performed and confirmed massive pneumobilia and common bile duct dilatation with suspicion of lithiasis (Fig. ). A T2-weighted sequences magnetic resonance cholangiography (MRCP) displayed a wide bile duct dilatation with numerous large calculi filling the choledocus (Fig. ). The gallbladder was small and its fundus neighbored the hepatic flexure of the colon and duodenum (Fig. ). To clarify any potential biliary enteric fistulas, a second MRI was carried out using gadoxetic acid (Gd-EOB-DTPA, Primovist®). The opacification of bile ducts was unusually delayed. The MRI at 90 minutes finally showed the filling of the intrahepatic bile ducts, gallbladder, upper choledocus and opacification of the hepatic flexure of the colon via a fistula (Fig. ).\nBecause of her medical history, endoscopic treatment with sphincterotomy and common bile duct stone extraction was favored over surgery.","This 74-year-old woman, who has a history of breast cancer and other health issues, came to the hospital because of a problem with her gallbladder and bile ducts. Imaging tests revealed a buildup of air and fluid in her abdomen and a blockage in her bile ducts, likely due to gallstones. Further tests showed a connection between the bile duct and the colon, which was confirmed by MRI. To address this, she will undergo an endoscopy to remove the stones and clear the blockage."
985,"A 46-year old man presented to the emergency department with pain localized to the right costovertebral angle and associated shoulder pain. Laboratory findings showed raised inflammatory parameters (C-reactive Protein (CRP) 116 mg/dL, normal range 0–1.2 mg/dL). The patient had no fever (36°C). A non-contrast-enhanced CT-scan was performed to exclude kidney stones. No urinary tract calculi could be revealed. However, in liver segment 7, a high-density structure was retained surrounded by a hypodense zone of 25 mm, containing small air bubbles suggestive for an intrahepatic abscess (Figure and ). Review of the contrast enhanced CT-scan performed two weeks earlier on the occasion of an acute appendicitis learned that this intrahepatic calcification had the same characteristics (800 Hounsfield Units, 10 mm, round shape) as the appendicolith on the previous scan (Figure ). At that time, the patient was treated with laparoscopy, which revealed a necrotizing appendicitis with a small covered perforation. Following a five day course of antimicrobial therapy the patient was discharged home.\nDue to the recent laparoscopic appendectomy for acute appendicitis, the CT-findings of this admission suggest a dropped appendicolith, which had spontaneously migrated into the liver parenchyma causing an intrahepatic abscess. There are no arguments for an iatrogenic lesion of the liver capsule during the recent appendectomy. During the second laparoscopic exploration, the appendicolith was extracted and the abscess was drained. Microbiology was positive for Escherichia coli. Intravenous antibiotics were administered over the following four days and the patient discharged. Up to now, the patient has remained well.","A 46-year-old man went to the hospital because of pain in his side and shoulder. Tests showed high levels of inflammation, and a scan revealed a small collection of pus in his liver. Doctors believe this is due to a small piece of stone from his previous appendicitis surgery that moved into the liver. The stone was removed during a second surgery, and the pus drained, allowing the patient to recover well."
986,"A 29-year-old Pakistani male was referred to the Oral Surgery Department for rehabilitation of the left edentulous mandible secondary to partial mandibulectomy surgery. He had undergone two operations for the left body of a mandible keratocyst odontogenic tumour (KCOT). The patient has been diagnosed with (KCOT) in Pakistan, where he received his first surgical treatment. A second surgical partial mandibulectomy was attempted due to recurrence. He has been reviewed regularly and he requested to have a replacement of his missing teeth on the lower left side due to a difficulty in eating and the effect on his appearance ().\nClinical examination reveals an asymmetrical face with a slightly depressed left lower body of the mandible on a class I skeletal pattern (Figures and ). The patient reported an absence of paraesthesia on the left mandible. The smile line was high exposing the gingiva on the upper maxillary incisors.\nIntraorally, the oral hygiene was fair with the presence of mild gingivitis. The dentition on the maxillary arch was unrestored (). The left edentulous mandible was irregular basal bone with firm mucosa covering the bone from 41 until 37. There were marked loss of bony structure horizontally and vertically. This has caused the tongue to occupy the space that used to be occupied by teeth and alveolus in the left mandibular segment (Figures , , and ). The healing of the operation site was uneventful (Figures and ).\nAn orthopantomogram was taken to evaluate the remaining bony structure of the mandible (). Radiographically, the operation site (lower left posterior segment) has no abnormalities. The remaining basal bone was adequate in thickness to support the mandible with an irregular margin. There are no radiopaque abnormalities suggestive of new pathology.","This 29-year-old man had a tumor removed from his jawbone in Pakistan, requiring a second surgery. Because of this, he now has a gap in his lower jaw and needs a replacement for his missing teeth. The surgery has affected the shape of his face and made it difficult to eat and smile. After a thorough examination, the dentist has planned a treatment to restore his teeth and improve his appearance."
987,"A 55-year-old male patient with nuchal pain at C3–C4 level radiating to the left arm was referred for exclusion of a disc herniation. He underwent a cervical CT, not showing a disc herniation. However, it revealed a right-sided accessory articulation between the anterior transverse processes of C6 and C7. The transverse foramina of C6 and C7 showed a partial defect, respectively posterior and anterior. Clearly, the accessory articulation was an incidental finding, as being contralateral to the symptomatic side.","A 55-year-old man came to the doctor because of pain in his neck and arm. The doctor initially suspected a problem with a disc in his neck, but a CT scan didn't show any disc issues. Instead, the scan revealed a small, unusual joint on the right side of his neck that may be contributing to his pain. This finding was considered an incidental discovery, as it was on the opposite side of his neck from where he was experiencing pain."
988,"This was a rare case of PMB-iSH in a 21-year-old female in China. In her postpartum period, the patient suffered from chest pain, fever and even coma for a fortnight. She was sent to the local hospital due to cardiac arrest by 4 times on 27th January 2017. After CPR, she regained consciousness gradually but still was in a continuous febrile state. Klebsiella pneumoniae was isolated from the samples of blood and sputum cultures. Besides, anti-microbial therapy hadn’t worked effectively since she was treated with cefepime, imipenem and tigecycline.\nThe patient was soon admitted to the emergency intensive care unit (EICU) of Ruijin Hospital affiliated to Shanghai Jiao Tong University on 26th April. Upon admissionto our hospital, she was still in fever, unconscious in a dyspneic state, and mechanical ventilation was initiated after tracheotomy with metal tracheal tube.\nA full-body computed tomography (CT) scan identified thickened pericardium, bilateral pleural effusion with multiple exudative focuses, hepatosplenomegaly and pelvic effusion in this patient. Empiric antibiotic treatment was started for Klebsiella pneumoniae infection with piperacillin-tazobactam (4.5 g, intravenously, q.8 h). The sample of microbial sputum culture on 29th April revealed that a large amount of multi-drug resistant Klebsiella pneumoniae (MDR-KP) grew, which was merely susceptible to tigecycline, sulfamethoxazole (SMZ) and PMB. The infection parameters from laboratory examination increased remarkably: hypersensitive C-reactive protein 37.0 mg/L [0~ 3 mg/L] and procalcitonin 3.37 ng/mL [< 0.50 ng/mL]. In light of the above-mentioned examination results, we replaced piperacillin-tazobactam with meropenem (2 g, intravenously, q.8 h) and tigecycline (100 mg intravenously q.12 h) with the addition of colistin (1-million unit by aerosol inhalation q.8 h) on 4th May.\nAfter the adjustment for the treatment, her body temperature dropped to normal and remained stable. During this period, repeated cultures of blood, sputum, vaginal secretion and fluid drained from the hip joint were carried out. MDR-KP, susceptible to tigecycline, SMZ and PMB was detected in all the samples. On 18th May, multiple exudations were aggravated in bilateral lungs compared to that upon admission, according to a chest CT scan. As a result, PMB (500,000 units, intravenously, q.12 h) was administered at once. Four days later, there were multiple red rashes spread on the whole body skin. With the rashes fading away, SH with dark round spots was seen on 23rd May (5 days after intravenous PMB) without pain or pruritus. More dark brown spots were spread on the skin of trunk and limbs, especially on that of lower abdomen, right hand and right foot as depicted in Figs. , , and , although the skin of the head and neck was also darkened evidently. Despite this adverse event, the therapeutic regimen was still applied in consideration of MDR-KP infection of multiple organs. PMB was utilized in the same dosage until the patient was transferred back to the local hospital, and skin biopsy wasn’t performed.\nOn 21st May, she suffered from diarrhea suddenly with intra-abdominal pressure (IAP) increasing to 19 cm H2O. About 500 ml black stool was found in the patient’ s excrement. Her serum creatinine was elevated dramatically to 430 μmol/L [53~ 97 μmol/L], and anuria occurred. The laboratory examinations revealed the following results: the level of hemoglobin was significantly reduced to 57 g/L [113~ 151 g/L] and platelet counts 63 × 109/L [101~ 320 × 109/L]. Her liver function was impaired moderately: total bilirubin was 43.6 μmol/L [4.7~ 24 μmol/L], direct bilirubin was 26.0 μmol/L [0~ 6.8 μmol/L], and γ-GT went up to 103 IU/L [7~ 64 IU/L]. The results of coagulation test were as follows: APTT 50.1 s [22.3~ 38.7 s], PT 12.9 s [10.0~ 16.0 s], TT 24.80s [14.00~ 21.00s], Fg 1.5 g/L [1.8~ 3.5 g/L], FDP 12.5 mg/L [0~ 5 mg/L] and D-Dimer 2.66 mg/L [< 0.55 mg/L]. They indicated that the patient suffered from coagulation abnormality and hyperfibrinolysis, hence disseminated intravascular coagulation (DIC). After phlebotomy, the transfusion of cryoprecipitate prepared from plasma frozen within 24 Hours (PF24) started immediately. Meanwhile, low-dose heparin (3 U/kg/h) was administered prudently for anticoagulation therapy. 2 days later, bleeding disappeared. On 25th May, urged by the relatives, the patient was transferred back to the local hospital for further treatment. At that time, her skin color didn’t recover to the previous state. Owing to her relatives’ will, we failed to make a follow-up visit. The treatment timeline is shown in Fig. .","This young woman in China experienced a very serious and rare infection called PMB-iSH, which led to a life-threatening condition. She developed a fever, chest pain, and eventually went into a coma. Tests revealed a multi-drug resistant strain of bacteria called Klebsiella pneumoniae was causing the infection, affecting multiple organs. Despite trying several antibiotics, the infection continued to worsen, and she needed a ventilator. Doctors performed scans to find the source of the infection and started a new treatment plan with additional medications, including PMB. As a result of the PMB treatment, she developed a rash on her skin. Unfortunately, her condition then worsened again with severe diarrhea and signs of bleeding, leading to a diagnosis of disseminated intravascular coagulation (DIC). Despite treatment, she was transferred back to her local hospital for further care, and doctors were unable to continue monitoring her."
989,"We present the case of 33-year-old woman with genetically confirmed osteogenesis imperfecta type I. During childhood, she presented with the pathognomonic features of osteoporosis with multiple fractures and blue sclerae. At the time of consultation in our institution, she had complaints of progressive hearing loss and persist vertigo.\nA spiral CT-scan with one millimetre thick sections of the temporal bone was performed. Symmetrical extensive lucencies in the pericochlear bony otic capsules, including the promontories, were demonstrated (Figure ). An additional 3T MRI was performed and included axial FLAIR imaging, axial diffusion-weighted imaging and gadolinium-enhanced 3D fast field echo imaging (3D FFE) through the entire brain. Furthermore, 3D balanced steady-state gradient echo through the skull base completed the exam. The MRI images showed symmetric areas of increased signal intensity in the pericochlear regions on the FLAIR and 3D balanced steady-state images (Figure & ). These areas showed moderate enhancement on the 3D FFE-images after contrast administration (Figure ).","6 This 33-year-old woman has a genetic condition called osteogenesis imperfecta, which causes fragile bones and frequent fractures. She recently started experiencing hearing loss and dizziness. Imaging tests, including CT scans and MRIs, revealed significant thinning of the bones around her ears. The MRI showed areas of inflammation in the ear, which may be causing her hearing and balance problems."
990,"A 42-year-old woman presented at the emergency department with acute onset of right flank pain. The patient had an extensive past medical history of endometriosis for which she underwent several surgeries. Contrast enhanced computed tomography (CT) of the abdomen showed right perinephric hemorrhage and an exophytic hypervascular mass arising from the lower pole of the right kidney with a maximum diameter of 5.5 cm (Fig. ). In the lung bases multiple thin-walled cysts were observed (Fig. ). Though the tumor did not demonstrate the intratumoral fat density typical for angiomyolipoma, the concomitant presence of cystic lung disease in a premenopausal woman was suggestive of spontaneous retroperitoneal hemorrhage from a renal angiomyolipoma in a patient with lymphangioleiomyomatosis. Based on the imaging characteristics hypernephroma could not be ruled out however. Subsequent CT of the thorax, performed several days later, showed multiple thin-walled cysts of various sizes spread throughout the lung parenchyma. The lung apices and lung bases were equally involved. No nodules were present. Based on these imaging findings a definite radiological diagnosis of LAM was made (Fig. ). A brain MRI performed to rule out tuberous sclerosis complex (TSC) was normal. The retroperitoneal hemorrhage was treated conservatively. The patient underwent a follow-up CT abdomen two months later. The perirenal blood had completely disappeared. The renal tumor was unchanged in volume and imaging characteristics. Once again no intratumoral fat could be detected. The patient was transferred to an academic center with expertise in interstitial lung diseases and lung transplantation for further follow-up. Because hypernephroma could not be ruled out based on the CT characteristics and because, even if the lesion was to be a benign angiomyolipoma, the patient was prone to rehemorrhage, a right partial nephrectomy was performed. Pathology confirmed the presence of an angiomyolipoma.","This 42-year-old woman came to the hospital with sudden, severe pain in her side. Imaging revealed a bleeding mass in her kidney and fluid-filled cysts in her lungs. Doctors suspected a rare condition called lymphangioleiomyomatosis (LAM) based on these findings, and ruled out other possible causes. After a follow-up scan, the bleeding stopped, and she was referred to a specialist for further care and a surgical procedure to remove the kidney mass."
991,"An asymptomatic 62-year-old man presented at the urologist for a check-up with a normal digital rectal examination and a PSA of 1.05 ng/mL. The prostate volume approximated 37 cc on transrectal ultrasound (TRUS) that also showed an hypoechoic structure of 5 cm, extending beyond the prostate, which was interpreted as an utricular cyst. The multiparametric prostate Magnetic Resonance Imaging (MRI) showed a sharply demarcated structure of 5.8 × 6.5 × 5.2 cm (asterisk, Figure : a, sagittal, and b, transverse T2w TSE) originating in the right transition zone of the midprostate, with posterior bulging and no invasive behavior. Relative to the muscle, the lesion was overall T1 isointense and slightly T2 hyper-intense, though it contained small T2 hyperintense foci (arrowheads, Figure , ). The lesion had restricted diffusion (Figure : a (b1400) and b (ADC)). Dynamic contrast-enhancement showed a homogeneous uptake with a slow first pass and progressive enhancement on second pass (Figure : a, transverse post-contrast T1, and b, time-enhancement curve). A benign stromal tumor was suggested. TRUS-guided biopsy was performed, and the tissue diagnosis was a leiomyoma. Considering the absence of clinical complaints and exclusion of malignancy, clinical and imaging follow-up with MRI at six months was advised.","During a routine check-up, a 62-year-old man had some tests done to examine his prostate. An ultrasound revealed a small, unusual growth within the prostate, and further MRI scans showed it was a distinct area with some concerning features. The MRI also showed that the growth was slowly taking up contrast, which is a sign that it is growing. A biopsy confirmed that the growth was a benign leiomyoma, a type of smooth muscle tumor, and no further treatment is needed at this time. The doctor recommends regular follow-up scans to monitor the growth."
992,A 68-year-old man was examined for bitemporal hemianopsia and falling episodes. Computed tomography (CT) revealed a 32 mm spontaneously hyperdense suprasellar mass (Fig. ). No calcification was noted. Complementary preoperative magnetic resonance imaging (MRI) showed a well delineated lesion with a nearly isointense signal on the T1-weighted images and a low signal intensity on the T2-weighted images. T1-weighted images following gadolinium administration demonstrated an inhomogeneous moderate to intense enhancement. Diffusion-weighted images showed a slight hypointensity with apparent diffusion coefficient (ADC) measured at 0.70 × 10−3 mm2/s (Fig. ). The adjacent pituitary gland was estimated as normal. Meningioma was the first diagnostic hypothesis.\nA surgical intervention was planned but only subtotal resection was possible because of the lesion attachment to the chiasm and optic nerves. Histological examination provided the diagnosis of granular cell tumor (GCT). The postoperative course was uneventful and the patient’s progress was satisfactory. No evidence of recurrence has been identified after two years of observation.,"68-year-old man came in for blurry vision and falling. A scan showed a growth in his brain near the pituitary gland, which was causing his symptoms. The growth was removed as much as possible, but it was attached to important nerves. The doctors determined it was a type of tumor called granular cell tumor, and he has been doing well with no problems after two years of follow-up."
993,"A 69-year-old woman who has been treated for a colorectal cancer suffered recurrent episodes of acute pancreatitis seven years later. She was initially treated by rectosigmoidectomy, for which further analyses revealed a T2 adenocarcinoma with mucinous differentiation, no distant metastasis, and a N2 nodal stage, leading to a complimentary chemotherapy scheme based on Bevacizumab and FOLFIRI. Follow-up revealed a 3 cm liver metastasis four years later, which required bisegmentectomy (segments VI and VII) with a complete excision of the mass and a new scheme of chemotherapy with Oxaliplatine, Fluorouracil and Folinic acid.\nSix years after the initial diagnosis, she was admitted with postprandial epigastralgia and elevated levels of lipase (1502, normal range (NR) 5–60UI/L), liver transaminases (AST 546, normal range 10–40UI/L and ALT 417, NR 4–44UI/L), alkaline phosphatase (284, NR 32–104IU) and direct bilirubin (1, NR 0–0.3 mg/dl). Further workup with Sonography, Computed Tomography and Echoendoscopy showed comprehensive dilatation of the bile ducts that contained a dense sludge, but neither lithiasis nor any other cause of obstruction. Endoscopic retrograde cholangiopancreatography (ERCP) revealed bulging of the duodenal papilla and heterogeneous opacification of the bile ducts. The patient was discharged shortly after aspiration of the sludge, sphincterectomy and normalization of the blood tests. The sampled sludge was later identified as mucus containing neoplastic cells. Two months later, after the patient had been readmitted twice for similar episodes and failure to demonstrate any metastasis, MRI with gadoxetic acid disodium (Primovist®), an MRI contrast agent with hepatocyte affinity and biliary excretion was performed, and eventually revealed a filling defect in the liver, a lesion infiltrating the right intrahepatic biliary duct (IHBD) (Figure ). Its additional properties were restricted diffusion on diffusion-weighted MRI (Figure ). Two weeks later, a new bisegmentectomy (segments V and VIII) with resection of the right IHBD, saving the hilar plate, were performed. Histopathological examination identified the lesion as a liver mucinous adenocarcinoma metastasis invading the lumen of the bile duct, diffusely replacing the biliary epithelium and infiltrating nerves. Unfortunately, the subsequent care was palliative, as the section was invaded on histology.","This 69-year-old woman has a history of colorectal cancer and has experienced several episodes of acute pancreatitis. After initial surgery and chemotherapy, she developed a liver metastasis and underwent further treatment. Recently, she was readmitted with symptoms of pancreatitis, and tests revealed a blockage in her bile ducts caused by mucus containing cancer cells. To address this, she had another surgery to remove part of her liver and bile duct, but unfortunately, the cancer had spread too far to be treated effectively, and care shifted to palliative support."
994,"A 65-year-old man suffering from severe and sporadic hemophilia A developed a large\nabdominal mass 15 years before seeking treatment.\nThe patient’s medical history included among other noteworthy facts\ntransfusion-related chronic hepatitis C, multiple hemophilic arthropathies, and\ncerebral hemorrhage.\nLaboratory findings were as follows: partial thromboplastin time, 81 sec (normal\nvalues 25 to 39 sec), and factor VIII assay, 1%, without inhibitors (normal values\n50% to 200%).\nThe treatment of his hemophilia consisted of two injections of 2000 units of\nplasmatic factor VIII per week. His hepatitis C was left untreated.\nHe was referred to our Department of Medical Imaging for the characterization of the\nmass. Abdominal computed tomography (CT) and a magnetic resonance imaging (MRI) were\nperformed to document this abdominal lump. No ultrasound was performed.","This 65-year-old man has a serious bleeding disorder called hemophilia A, which he’s been managing with regular factor VIII injections. He also has a history of hepatitis C and other joint problems. Recent tests showed his blood wasn’t clotting properly, and he has a large mass in his abdomen that needs further investigation. Doctors ordered a CT scan and MRI to get a better look at the mass and determine what’s causing it."
995,"A 46-year-old woman presented at our institution in April 2013 with acute pain in the left hip. She had a skiing accident, being hit by another skier. Since then, she experienced severe persistent pain in the left hip. The medical history included few relevant findings, except bilateral recurrent calcific tendinitis of the rotator cuff, treated with corticosteroid injections, physiotherapy and arthroscopic exploration (with debridement of the calcific deposition, bursectomy and decompression of the subacromial space). Clinical examination showed pain and significant tenderness of the trochanteric region. There was a normal, however painful, range of motion.\nA plain radiography excluded fractures, but demonstrated the presence of perithrochanteric calcifications (Fig. ). A CT-scan of the pelvis confirmed the absence of fractures, and the presence of a large, well-defined calcification of low density, 18 millimeters in diameter, located anteriorly in the gluteus medius tendon (Fig. ). Acute calcific tendinitis of the gluteus medius tendon was suggested as the cause of the patients’ pain.\nSince ultrasound-guided needle lavage is a well-described therapy for hydroxyapatite depositions in the rotator cuff tendons of the shoulder, we proposed this treatment approach for this patients’ calcific tendinits. An ultrasonography was performed to assess the feasibility. The calcification was demonstrated as a hyperechogenic structure in the gluteus medius tendon within reach of a needle (3,1 cm beneath the skin) (Fig. ). Reactive thickening of the overlying bursa was also seen. In consultation with the patient and the orthopedic surgeon, an ultrasound-guided needle lavage and corticosteroid injection was performed. Linisol 2% was used as local anesthetic in the subcutaneous tissue, in the overlying bursa and in the peritendinous tissue around the calcification. This reduced the pain instantaneously. A 21 Gauge long spinal needle was then inserted into the hydroxyapatite deposition. The deposition was rinsed using small syringes of linisol 1%, which were gently and alternatingly injected and aspirated. There was progressive aspiration of a large amount of white crystals into the syringes. Once no more crystals could be aspirated, the region of the calcification and the overlying bursa was infiltrated with 40 milligrams of depoMedrol, dissolved in 4 milliliters of marcaine 0,5%. The patient’s symptoms resolved within a few days following the procedure, and she could quickly resume her daily activities. No follow-up imaging was performed given the good response.\n18 months later, in November 2014, the patient returned to the hospital with similar complaints, now in the right hip. The pain had an acute but non-traumatic onset. The orthopedic surgeon clinically suspected bursitis, and requested a magnetic resonance imaging-study (MRI) to confirm this hypothesis and to exclude intra-articular pathology.\nThe MRI showed extensive edema and infiltration of the soft tissue around the greater trochanter, between the proximal muscle heads of the quadriceps, and along the tensor fasciae latae muscle and around the insertion of the gluteus medius and minimus (Fig. ). A hypo-intense, sharply defined structure with a length of almost 15 millimeters and a thickness of 8 millimeters, was identified in the gluteus medius tendon. There were no abnormalities of the joint. Due to the non-traumatic onset of the pain, a rupture or elongation of the vastus intermedius muscle of the quadriceps was unlikely. Because of the history of acute calcific tendinitis of the gluteus medius tendon on the left side, we interpreted the hypo-intese intratendineous structure as a calcification, with calcific tendinitis with manifest reactive edema as the most probable diagnosis. A radiography of the right hip confirmed a large, well-defined calcification cranial to the greater trochanter (Fig. ).\nAn ultrasound-guided needle lavage of the calcification with corticosteroid injection was performed, in consultation with the referring orthopedic surgeon and the patient (Fig. ). This procedure was carried out the same way as 18 months earlier on the left side. There was a similar good clinical response to the procedure (i.e. nearly complete alleviation of the pain within days and eventually a complete pain-free state), so no follow-up imaging was performed.","This 46-year-old woman experienced persistent pain in her left hip after a skiing accident. Imaging revealed a large calcium deposit in the gluteus medius tendon, which was likely causing the pain. A procedure called needle lavage, where the calcium deposit was rinsed with medication, successfully relieved her pain. Sixteen months later, she developed similar pain in her right hip, and imaging confirmed a similar calcium deposit. Another needle lavage procedure provided relief, and she was able to return to her normal activities."
996,"A 52-year-old man, referred to us for coughing with sputum and dyspnea, was hospitalized because of septic shock following bilateral pneumonia. Two months previously, he had been hospitalized for a pancreatic pseudocyst during an acute phase of a head pancreatitis. Computed tomography (CT) at that time showed extensive collection around the liver, with a communication with the pancreas. The endoscopic retrograde cholangiopancreatography (ERCP) revealed a large fistula from the Wirsung to this pseudocyst, and a sphincterotomy was realized with the placement of a 7Fr 5-cm prosthesis. During follow-up, about 3 weeks after the patient’s hospitalization because of pneumonia, a new increase in the biological inflammatory syndrome was noticed.\nA control radiography showed a persistent parenchymatous condensation in the middle lobula, as well as a right pleural effusion. A small, unusual aeric crescent-shaped picture was seen under the right section of the diaphragm, suggesting a pneumoperitonea (Figure ).\nCT revealed that the size of the pseudocyst had decreased significantly, and a large amount of gas was observed inside the cyst, suggestive of spontaneous fistula. A distal bronchi communicating by a fistulous way to the pseudocyst was visualized (Figure ). Multiples areas of centrilobular nodules with a linear branching (tree-in-bud pattern) in the right inferior lobula and a condensation with air bronchogram in the middle lobula were also noticed (Figure ). Further, the 7Fr 5-cm prosthesis placed in January had fallen in the abdomen. Based on multiple detector computed tomography (MDCT) findings, the diagnosis of pancreaticobronchial fistula was suggested and confirmed by analysis of bronchial expectorations that showed raised lipasis and amylasis.\nConservative treatment by somatostatin was instaured, and the pancreatic duct was stented with a new 10Fr 5-cm stent. The evolution was favorable with improvement on the 1-week follow-up CT scan.","This 52-year-old man was hospitalized for severe pneumonia that led to septic shock. He had a previous problem with a collection of fluid around his liver and pancreas, which had been treated with a stent. Now, he developed a new infection in his lungs and a connection between his pancreas and his lungs, causing a fistula. After treatment with medication and a new stent, his condition improved, and the scans showed the fistula was closing up."
997,"A 40-year-old gravida five, para four woman presented for workup and management of abnormal uterine bleeding. Her past medical history was significant only for hypertension and anemia. On review of her social history, she admitted to drinking six packs of beer on the weekends but denied further substance use. She denied previous treatments for her bleeding including any previous intrauterine device usage.\nUltrasonography revealed a 7 cm fundal fibroid with otherwise normal pelvic anatomy. She was initially offered medical management of her bleeding. She declined any medical treatment and strongly desired definitive surgical treatment. She then underwent a total vaginal hysterectomy with adnexal conservation. Due to the large size of the uterus, a myomectomy was performed to facilitate vaginal removal. Her postoperative hospital course was relatively uncomplicated and she was discharged home on postoperative day three.\nOn postoperative day ten, she presented to the Emergency Department (ED) for fever, worsening abdominal pain, and new onset of nausea and vomiting. In the Emergency Department, she was tachycardic and tachypneic but afebrile. Her exam was significant for abdominal tenderness to minimal palpation, vaginal cuff erythema, and significant tenderness to palpation of the vaginal cuff. Lab work showed an elevated white blood cell count. She was admitted for management of presumed pelvic infection.\nA CT of the abdomen and pelvis was obtained and showed a 6.2 x 9.7 cm pelvic abscess adjacent to the vaginal cuff (Figures and ).\nInterventional Radiology placed a drain into the abscess and the patient was started on IV piperacillin/tazobactam. She was transitioned to oral amoxicillin/clavulanate potassium after four days on intravenous antibiotics and her drain was removed on hospital day 5. Vaginal wound cultures remained pending; however, due to continued clinical improvement on the oral antibiotic regimen, she was discharged home on hospital day 5 with a two-week course of amoxicillin/clavulanate potassium.\nThe patient then returned for her outpatient visit approximately one week later. The results of the vaginal wound cultures revealed a large growth of Actinomyces meyeri. The patient's case was discussed with an Infectious Disease (ID) specialist who recommended an additional two-week course of amoxicillin/clavulanate potassium.\nThe patient then returned to the ED on postoperative day 25 for pleuritic chest pain with mild cough but denied gynecologic complaints. She reported compliance with the oral amoxicillin/clavulanate potassium regimen. Exam and lab work were unremarkable. A chest X-ray showed left basilar heterogeneous opacities, likely subsegmental atelectasis. A CT angiogram was obtained due to concern for a possible pulmonary embolism (PE). The imaging was negative for a PE; however, it was concerning for possible pneumonia. The patient was discharged home with a five-day course of levofloxacin for treatment of pneumonia.\nOn postoperative day 27, the patient represented to the ED with worsening shortness of air and chest pain. Again, she reported compliance with her antibiotic prescriptions. Exam and lab work were again unremarkable. A repeat chest X-ray showed a slight progression of right basilar heterogeneous opacities thought to be infectious. Her antibiotic regimen was again discussed with ID specialists and an intravenous antibiotic regimen was felt preferable to an oral antibiotic course. She then completed an outpatient two-week course of IV ampicillin/sulbactam as recommended.\nOn postoperative day 37, a repeat CT of the abdomen and pelvis showed near complete resolution of the previous pelvic abscess. HIV testing was obtained and returned negative result. She reported significant improvement of her symptoms. She was placed on a six-month course of oral amoxicillin per ID recommendations with plans for continued follow-up in their clinic, as well as with gynecology. She has not shown any signs of recurrent infection after approximately 1 year of follow-up.","This woman had irregular bleeding and a large fibroid in her uterus. She decided to have surgery to remove her uterus and the fibroid, but unfortunately developed an infection around the surgical site. After being admitted to the hospital, doctors discovered a large abscess and treated it with antibiotics and drainage. She then developed pneumonia, which was also treated with antibiotics. Despite the complications, the patient responded well to treatment and was eventually discharged home with a longer course of antibiotics. She has been closely monitored and has not experienced any further problems in the past year."
998,"A 52-year-old male patient presented with the complaint of sudden vision loss in his left eye 3 days earlier. Past medical history was significant for chronic kidney disease, secondary hypertension, chronic hepatitis C virus infection and arrhythmia. Ophthalmologic examination revealed best corrected visual acuity of 10/10 in the right eye and 4/10 in the left eye from the temporal field. Confrontation test revealed inferonasal visual field loss in the left eye. Direct and indirect light reflexes were normal in both eyes and there was no relative afferent pupillary defect. Anterior segment examination was normal and intraocular pressure was 13 mmHg in both eyes. Dilated fundus exam demonstrated soft exudates consistent with hypertensive retinopathy in the right eye. Fundoscopy of the left eye revealed an area of pallor in the superotemporal quadrant and the macula with macular cherry red spot, which were consistent with occlusion of the superotemporal branch of the left retinal artery (). On OCT, peripapillary retinal nerve fiber layer (RNFL) thickness was within normal limits (). In the patient’s visual field, there was an inferonasal defect in the left eye corresponding to the occluded region (). The patient was treated with a single dose of 500 cc intravenous dextran-40 and 200 mg intravenous pentoxifylline. In etiologic studies, Doppler ultrasonography revealed an atherosclerotic stenosis in the right and left main carotid arteries and a calcified plaque causing luminal narrowing in the left internal carotid artery. Transthoracic echocardiography revealed second- to third-degree aortic valve regurgitation and first-degree tricuspid valve regurgitation. There was no improvement in visual acuity or visual field despite treatment. At follow-up 7 months later, OCT showed thinning of the superior, inferior and temporal peripapillary RNFL (). On the thickness map, ganglion cell layer was thinner in the superior and temporal areas (). Decreased vascular density in the superficial and deep capillary plexus consistent with ischemia in the regions supplied by the superotemporal branch of the retinal artery was observed in a 6x6 mm macular field on OCTA (). The borders of the ischemic area were more clearly seen in en face images (). In optic disc OCTA, capillary density was reduced in the superotemporal region and collateral vessels were present in the area (). When compared to the fellow eye, there was a decrease in the macular deep and superficial capillary density in the superior and temporal quadrants () and a decrease in peripapillary capillary density in the superior quadrant (). Visual field loss persisted in post-treatment threshold perimetry ().","A 52-year-old man experienced sudden vision loss in one eye and came to the clinic for evaluation. Tests revealed a blockage in the main artery supplying blood to the back of his eye, causing damage and vision loss. The cause of the blockage was determined to be narrowing of the arteries in his neck, and he was treated with medications to try to restore blood flow. Unfortunately, his vision did not improve, and further tests showed thinning of the nerve layer in his eye, indicating ongoing damage."
999,"A 65-year-old female presented to our tertiary eye centre with 6 weeks’ history of painless right eye vision distortion and no history of eye injury or trauma. On examination, VA was 6/24 in the right eye and 6/5 in the left eye. Following slit-lamp biomicroscopy and fundoscopy a diagnosis of right eye full-thickness macular hole was made and optical coherence tomography showed right eye cuff of subretinal fluid, left eye epiretinal membrane and posterior vitreous detachment. Ten years before presentation she had uneventful bilateral phacoemulsification and intraocular lens implantation with no other significant past ocular or medical history.\nEight weeks later, she underwent right eye pars plana vitrectomy, internal limiting membrane peeling and cryotherapy with C3F8 12% gas tamponade. Two weeks postoperatively, she exhibited a flat retina, closed macular hole and her VA had improved to 6/18 with normal intraocular pressure (IOP). Unfortunately, 7 weeks postoperatively, she developed right eye macula-on RD due to proliferative vitreoretinopathy (PVR) in the inferior retina. RD repair was done within 3 days with silicone oil (Densiron 68) tamponade and retinectomy to release the PVR. After 4 months, VA of the right eye after removal of silicone oil was 6/12 with flat retina and closed macular hole.\nFour months later, her VA declined to 6/36 in the right eye and remained 6/5 in the left eye and fundus fluorescein angiogram confirmed severe right eye CMO. She underwent right eye posterior sub-Tenon’s triamcinolone injection and was started on ketorolac trometamol eye drops (Acular) 3 times/day and oral acetazolamide 250 mg slow-release 2 times/day. Treatment of the CMO during the follow-up period is summarised in Table 1. She received 3 posterior sub-Tenon’s triamcinolone injections and 2 intravitreal triamcinolone injections within 14 months with no complications. The CMO initially responded to each triamcinolone injection but later recurred ().\nThe patient then received 4 intravitreal dexamethasone 0.7-mg implants (Ozurdex; Allergan, Inc.) uneventfully within 15 months, which maintained a dry fovea for a longer period but the CMO recurred again (). She also received a trial of anti-vascular endothelial growth factor (Avastin) but there was no response. At that point, the patient decided that she no longer wanted repeated injections and decided to wait until her fund application to receive ILUVIEN implant as special case was approved.\nHer refractory CMO persisted after 2 years without treatment. Finally, she received ILUVIEN intravitreal implant. In the first week she developed mild right eye anterior uveitis; IOP was 27 mmHg in the right eye and 18 mmHg in the left eye. These markedly regressed within a week on dexamethasone drops and latanoprost drops and topical medications were stopped within 4 weeks. At the time of this report, it is 20 months since receiving the ILUVIEN implant and she still has a dry fovea with right eye VA of 6/18 ().","This 65-year-old woman has been struggling with blurry vision in her right eye for six months, which turned out to be a full-thickness hole in her macula. After several surgeries to try to repair the hole, she experienced complications and the problem kept returning. Over the past two years, she received multiple injections and implants to treat the condition, but it continued to worsen. Currently, she has a dry fovea in her right eye with some vision improvement, and is awaiting a special implant to help manage her condition."