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 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?
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.
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