65 year old female with a history of steroid dependent pemphigus vulgaris would like a second opinion on treatment options. Upon review of her medical records, the rheumatologist discovers she has a history of positive hepatitis B core antibody and refers her to hepatology clinic for further workup. 

Her rheumatologist would like to start her on rituximab and would like to know if this can be initiated immediately. What do you do next?

A 39 year-old female was referred for an abnormal liver profile after she noted scleral icterus, fatigue, and pruritus. She denies alcohol, substance, herbal supplement, or recent antibiotic use. Labs demonstrate an alkaline phosphatase of 462 u/l, AST 277 u/l, ALT 281 u/l, total bilirubin 1.9 mg/dl, direct bilirubin 1.0 mg/dl, INR is normal. Viral serologies were notable for immunity to HAV and HBV and negative HCV antibody. Ultrasonography revealed hepatomegaly and coarsened echotexture. Her primary care provider obtained an ANA in evaluation of a silvery plaque which was elevated to >1:2560 titer. You order several studies which are remarkable for significantly elevated IgG level, positive anti-mitochondrial antibody, and positive anti-smooth muscle antibody.

A 19-year-old male with past medical history of hypoplastic left heart syndrome s/p Fontan surgery 15 years ago is referred to be seen in hepatology clinic for screening for Fontan associated liver disease (FALD). Which of the following is the best clinical predictor of FALD?

61 year old male with alcohol-related cirrhosis presents with severe ascites. He had a therapeutic paracentesis with improvement in his ascites and underwent a surveillance EGD which showed large varices that were then banded. The following day, he developed tachycardia, hypotension, and hematochezia. A nasogastric tube was placed to suction without blood return.

What is the most likely etiology of the bleed?

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?