Clinicopathology conferences

✍ Scribed by Fenton Schaffner; Swan N. Thung

• Book ID: 102237561
• Publisher: John Wiley and Sons
• Year: 1994
• Tongue: English
• Weight: 482 KB
• Volume: 19
• Category: Article
• ISSN: 0270-9139
• DOI: 10.1002/hep.1840190235

✦ Synopsis

An 18-yr-old woman was referred to the Department of Pediatrics of the Mount Sinai Medical Center for evaluation for possible liver transplantation. She had been abandoned by her mother, who reportedly was an intravenous drug user, and the patient had spent several years in a group home. A routine examination at age 15 yr revealed increased aminotransferase activity and a low platelet count. However, she was asymptomatic. She never received blood transfusions. A bone marrow aspiration showed decreased megakaryocytes. She was not treated, and the abnormalities in her liver function continued. Increasing abdominal girth and fatigability developed. Three years later, she had an open liver biopsy with fresh-frozen plasma coverage because her prothrombin time was 15.7 sec (normal = 11.0 to 13.2 sec). CAH and micronodular cirrhosis were found. At that time she had a serum albumin level of 2.5 gm/dl (normal 3.7 to 5.7 gm/dl), an anti-smooth muscle antibody titer of 1 : 80, normal serum a,-antitrypsin and serum ceruloplasmin levels and a normal 24-hr urine copper level. She began receiving 40 mg prednisone and 50 mg azathioprine per day. Because no response to therapy was noted after 3 mo, she was referred for transplantation.

On initial examination at the Mount Sinai Medical Center, she was obese, her weight was in the ninetieth percentile for her age and her height was in the ninety-fifth percentile. Ascites and splenomegaly were noted. She had a draining abscess at the site of her liver biopsy, from which Staphylococcus aureus was cultured. The initial serum bilirubin level was 3.3 mg/dl (normal = 0.1 to 1.2 mg/dl), ALT 200