The application of clinical knowledge to a individual patient is often filled with nuance and is hardly straightforward. In the Clinical Pearls series, we aim to use a case-based format to learn how broader concepts within hepatology can be applied in various unique settings.
A 60-year-old male with a history of HBV exposure, HCV with spontaneous clearance, and myelofibrosis with transfusion-dependent pancytopenia presents with tense ascites and coffee ground emesis. On exam he has massive splenomegaly and a nontender abdomen. Labs are notable for a hemoglobin of 6 gm/dL, platelet count of 12 K/L, INR of 1.2, and normal liver profile. Fluid studies from paracentesis reveal a serum-ascites albumin gradient (SAAG) of 1.8 gm/dL and total protein of 1.7 gm/dL, consistent with portal hypertension. EGD shows multiple columns of non-bleeding large esophageal varices with stigmata of recent bleeding, gastric varices, and portal hypertensive gastropathy. A transjugular liver biopsy with pressure measurement shows a hepatic venous pressure gradient (HVPG) of 10 mmHg. Pathology reveals minimal fibrosis, CD34+ cells within the sinusoids, and reticulin stain shows diffuse nodules surrounded by a compressed reticulin network.
In addition to extramedullary hematopoiesis due to myelofibrosis and increased portal flow, what other entity is contributing to this patient’s portal hypertension?
A 66-year-old male with a past medical history of primary biliary cholangitis s/p orthotopic liver transplant 10 months ago, ESRD s/p kidney transplant 2 months ago, presents with shortness of breath and dyspnea on exertion. His symptoms have gradually worsened over 2 weeks, and he is now short of breath walking across a room. He denies fevers, chills, and sick contacts. He is compliant with his medications and currently on prednisone, mycophenolate mofetil, and tacrolimus for immunosuppression. Liver chemistries are within normal limits and chest imaging shows a large right pleural effusion and right anterior chest wall mass. What is the most likely underlying diagnosis?
Learn about the diagnostic approach to cholestasis in pediatrics, focused on a case of a 5-day-old baby with persistence jaundice and hyperbilirubinemia.
A 63-year-old female presents to the ER with right upper quadrant pain and jaundice. History is notable for a hepatic abscess thought to be secondary to acute cholecystitis three months ago at an outside hospital. At that time, AST was 300 U/L, ALT 200 U/L, alkaline phosphatase 113 U/L, and total bilirubin of 3.5 mg/dL. Hospital course was complicated by Escherichia coli bacteremia. The hepatic abscess was ultimately drained and culture results were positive for Klebsiella pneumoniae and Escherichia coli. The patient was discharged on antibiotics and told to follow up with surgeons for a cholecystectomy. The patient now re-presents with pain and jaundice. On exam, the patient is febrile to 38.9°C, BP 120/60, heart rate 96 bpm. Labs are now significant for conjugated hyperbilirubinemia of 8.8 mg/dL, alkaline phosphatase of 1844 U/L, AST 418 U/L, and ALT 215 U/L. No leukocytosis or eosinophilia is present. An ultrasound in the ER reveals signs of chronic cholecystitis and a large fluid collection in the right lobe of the liver.
A 5 month old FT baby boy presented with a five day history of abdominal distention, jaundice and was found to have significant ascites and elevated AFP. Laboratory evaluation was significant for hypoalbuminemia (albumin-1.8 g/dL), coagulopathy (INR-3.5) and elevated alpha-feto protein (23,300 ng/mL). Whole exome sequencing was non-diagnostic and he was diagnosed with cryptogenic cirrhosis. During his admission his ascites is refractory to both medical therapy and repeat paracentesis. He is listed for liver transplant. Of the following, what is the best predictor of post-transplant mortality and graft survival?
25-year-old female with hyperthyroidism who is 30 weeks pregnant initially presented with costovertebral tenderness, pelvic pain and nausea. Pain resolved after passing a kidney stone, but initial labs are concerning for abnormal liver chemistries with AST of 118 U/L, ALT of 162 U/L, alkaline phosphatase of 200 U/L, Total Bilirubin of 2.0 mg/dL and INR of 1.0. What is the next best step?
A 22-year-old male with a past medical history of anxiety and depression presents with acute psychosis after ingesting 3.5 grams of marijuana. He takes vortioxetine at home. On presentation, he was found agitated, throwing himself against walls. AST and ALT were 187 U/L and 44 U/L on admission. Five days later, AST and ALT increased to 1,402 U/L and 402 U/L. All other liver chemistry tests, hemoglobin, white blood cell count, and INR are normal. What is the most likely cause of his abnormal liver chemistry tests?
75-year-old male presents with diuretic refractory hepatic hydrothorax. No overt hepatic encephalopathy but family has noted some episodes of intermittent confusion. He is independent in his activities of daily living. MELD is less than 10. No history of congestive heart failure, chronic kidney disease or hyponatremia. What do you recommend?
A 38-year-old male presents with new-onset shortness of breath, abdominal pain, and ascites.
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