A 40 year-old female with a past medical history of dermatomyositis, interstitial lung disease (ILD) and hypothyroidism is referred to the hepatology clinic for elevated liver enzymes. Labs reveal total bilirubin 0.3 mg/dL, alkaline phosphatase 177 U/L, ALT 237 U/L, AST 156 U/L, INR 1.0, platelet count 250. Medications include Rituximab Q4 weeks, Mycophenolate sodium 720 mg BID, Prednisone 10 mg daily, Bactrim 800-160 QMWF and Synthroid 88 mcg daily. Serologic workup reveals positive Hep E RNA PCR. What is the next best step?

A 57-year-old woman presents with an acute history of fevers to 101°F, RUQ abdominal pain, fatigue, arthralgias, and elevated liver enzymes. Her symptoms first started two weeks prior to presentation. She does not drink alcohol or use illicit drugs. She has not had any recent travel. She does not take NSAIDs or Tylenol. Her labs on admission are WBC 6.5, Hct 42, Plts 222, ALT 348, AST 225, ALP 185, Tbili 4.5, INR 1.1. A Tylenol level is undetectable. Hepatitis A, B, and C testing are negative. COVID-19 testing and tickborne panel are negative. A doppler ultrasound of the liver is normal. Additional lab testing revealed a positive antinuclear antibody, an elevated anti-smooth muscle antibody at a titer of 1:320 and an elevated IgG at 2052. What is the next best step in evaluation and/or management? 

A 45-year-old female underwent colonoscopy for iron deficiency and chronic diarrhea with associated flushing and was found to have an erythematous region in her terminal ileum. Biopsies revealed a neuroendocrine tumor. She undergoes an abdominal MRI showing multiple hepatic lesions and a mass near the terminal ileum. She was referred to oncology and started on octreotide depot injections for symptom control. Liver biopsy confirmed metastatic well-differentiated neuroendocrine tumor with synaptophysin and chromogranin stains positive. An octreotide scan demonstrated multiple avid foci in the liver but no extrahepatic lesions. An echocardiogram showed normal LV and RV function without valvulopathies.  She now presents to Hepatology clinic for assessment.

What is the next best step?

An 18 year old young man with chronic migraines is referred to hepatology clinic for evaluation of hepatosplenomegaly and thrombocytopenia. Neurology obtained a brain MRI showing calvarial thickening with marrow signaling but otherwise was unremarkable. His AST and ALT are 138 U/L and 150 U/L respectively, platelets 74,000/uL and hemoglobin 10.2 g/dL. At the visit, he complains of chronic leg pain that has been attributed to “growing pains”. He denies alcohol or drug use. On physical exam, liver is palpable 4 cm below costal margin at mid clavicular line with soft contour, spleen is palpable 8 cm below costal margin at mid axillary line. There is tenderness to palpation above the left femur with decrease passive range of motion secondary to pain. What is the best diagnostic test to determine etiology?

A 40 year-old female with a history of recurrent nosebleeds presents to hepatology clinic for persistently elevated liver enzymes. Lab work-up reveals total bilirubin 1.0 mg/dL, alkaline phosphatase 247 U/L, ALT 90 U/L, AST 53 U/L, INR 1.0, hemoglobin 7.5 g/dL and platelet count 250 K/cmm. Iron studies reveal ferritin 12 ng/mL, iron 20 ug/dL, transferrin saturation 5%.

On physical exam she has multiple small telangiectasias on her trunk, upper extremities, lips and oral mucosa. Non-tender hepatomegaly is noted on abdominal exam.

Her mother also suffers from recurrent nosebleeds, iron deficiency anemia and has similar telangiectasias on her lips and trunk.

What is not recommended as part of the work-up for this condition?

A 47-year-old male presents with painless jaundice.  He also reports decreased appetite and energy, along with diffuse itching, pale stools, and dark urine. He denies any weight loss or history of liver disease. His physical exam is notable for jaundice and his labs show cholestasis (total bilirubin 6.3, primarily conjugated, and alkaline phosphatase of 347) and elevated transaminases (AST 160 and ALT 547). A MRCP shows intra-hepatic biliary dilation and retroperitoneal fibrosis without a mass or filling defect

What is the most likely underlying diagnosis?

A 60 year old male with a history of HTN, arthritis, diabetes and stroke presents to hepatology clinic for evaluation of fatigue and a positive hepatitis C antibody. Additional lab work reveals a total bilirubin of 1.0 mg/dL, alkaline phosphatase of 90 U/L, AST of 100 U/L, ALT of 80 U/L, INR 1.2, platelet count of 160, hemoglobin of 13 g/dL. ASMA, ANA, Hep B serologies were negative. Hepatitis C viral load could not be processed in the lab due to an ‘inadequate sample.’ Iron studies were obtained which revealed a ferritin of 1481 ng/mL, iron of 184 ug/dL, and transferrin saturation of 60.5%.  Patient denies alcohol use, denies new medications.

On physical exam, there is no evidence of volume overload or hepatosplenomegaly. His second and third metacarpophalangeal joints appear swollen and enlarged bilaterally. No skin lesions or hyperpigmentation noted.

Family history is notable for his sister who was recently found to have abnormal liver chemistries, which the patient thinks is also due to Hepatitis C

A 28-year-old woman presents with progressive right upper quadrant pain, fever, and non-productive cough. She is 32 weeks pregnant. On admission she is febrile to 39°C and her physical examination is notable for right upper quadrant tenderness as well as a gravid uterus. She has no scleral icterus. Her lungs are clear. She has no visible oral or genital lesions or skin rashes on examination. She is not encephalopathic. She only takes folic acid and denies taking any other medications. She does not drink alcohol or use illicit drugs. She has not had any recent travel. Her labs on admission are WBC 2.8, Hct 30, Plts 85, ALT 9678, AST 8756, ALP 150, Tbili 1.4, INR 1.8. A Tylenol level is undetectable. Hepatitis A, B, and C testing are negative. A pelvic ultrasound shows a viable fetus. A doppler ultrasound of the liver is normal. What is the next best step in management? 

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?