Pathophysiology and Natural History Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease, thought to be immune-mediated in nature, characterized by inflammation and fibrosis…

A 61 year old male with a history of recently diagnosed autoimmune hepatitis presents with fatigue and generalized weakness for 1.5 weeks. He denies fevers, chills, abdominal pain or swelling, and confusion. On exam, he has scleral icterus but no ascites or asterixis. Labs are notable for a total bilirubin of 8.1, AST 1,412, ALT 1020, and INR of 1.5. What is best next step in management?

A 20 year-old healthy female with no PMHx presents with lower extremity edema, abdominal distention, and a 12 pound weight-gain over 3 months. She was seen by her OB/GYN after 2 months of missed menstrual periods, and found to have elevated aminotransferases and bilirubin prompting her to present to the emergency room. full liver-work up noted an ANA screen that was positive, ferritin 176.1, hepatitis serologies normal, ASMA negative, Liver Kidney Microsomal Ab negative, normal serum alpha-1-antitrypsin.

Which gene mutation does this patient most likely have?

A six-month-old full term healthy female born via NSVD, attends a well child check, the pregnancy and birth were uncomplicated.

A 37 year-old man with a recent history of severe alcohol-associated hepatitis treated with 28 days of prednisolone presents to your outpatient clinic. How would you diagnose and manage his alcohol use disorder?

The Real Deal Behind Alagille Syndrome Background Alagille syndrome is an autosomal dominant, multisystem disorder with characteristic hepatic, cardiac, vascular, renal, skeletal, ocular, and facial…

A 46-year-old woman with a history of Crohn’s disease presents with encephalopathy. She is somnolent and unable to follow commands. Her exam is notable for scleral icterus and RUQ tenderness. Labs show AST 3,694 IU/L, ALT 6,158 IU/L, International Normalized Ratio (INR) 2.1, and total bilirubin 6.6 mg/dL. Her acetaminophen level is 46 mcg/mL; the timing of her ingestion is not clear.

What is the most appropriate treatment for this patient?

60-year-old male with history of HCV cirrhosis s/p deceased donor liver transplant with duct-to-duct biliary anastomosis six months ago presents with jaundice, dark urine, pruritus, and pale stools. Exam is notable for icterus. Labs revealed a total bilirubin of 10 mg/dL, direct bilirubin 5 mg/dL, ALP 538 u/L, AST 36 u/L, and ALT 41 u/L. INR is 1.0. Labs from three weeks prior were notable for a normal bilirubin and mildly elevated ALP to 181 u/L which has been rising. Doppler ultrasound reveals coarsened hepatic echotexture, patent vessels, and intrahepatic biliary dilatation to 1cm.

What is the next best step?

A 66 year old male with a history of compensated alcohol-related cirrhosis presents with abdominal swelling and a ten pound weight gain but denies any other symptoms. His exam is notable for normal vital signs and abdominal distension, and labs show an increase in his MELD-Na from 11 to 17, primarily due to an increased bilirubin and INR. AST and ALT are 17 and 13, respectively. What is the most likely cause of his acute decompensation?

A 44 year-old female with a past medical history of hypothyroidism and obesity presents with acute onset right upper quadrant abdominal pain radiating to the back and sternum. Associated symptoms include nausea and lightheadedness with standing. Her medications include Levothyroxine and an oral contraceptive pill (OCP).

Lab work-up reveals total bilirubin 0.2 mg/dL, alkaline phosphatase 150 U/L, ALT 113 U/L, AST 104 U/L, INR 0.9, WBC 14.3 K/uL, hemoglobin 9.3 g/dL, platelet count 488, Lactate 4.5 mmol/L.

On physical exam hepatomegaly is palpable and she is significantly tender to palpation in the epigastrium and right upper quadrant.

What is the next best step?