A young woman with cirrhosis: Autoimmune hepatitis vs. α1-antitrypsin deficiency
✍ Scribed by Anna S. F. Lok; Marc G. Ghany; Michael A. Gerber
- Publisher
- John Wiley and Sons
- Year
- 1994
- Tongue
- English
- Weight
- 975 KB
- Volume
- 19
- Category
- Article
- ISSN
- 0270-9139
No coin nor oath required. For personal study only.
✦ Synopsis
CASE HISTORY
Y.J., a 22-yr-old black woman, was referred in February 1990 for evaluation of an asymptomatic increase in serum aminotransferase level and thrombocytopenia. She had no history of hepatitis or jaundice. Her only serious childhood illness was bronchial asthma. She had no history of surgical procedure, blood transfusion, ethanol or intravenous drug use. There was no family history of liver disease.
Physical examination was unremarkable. She had no spider nevi, jaundice, lymphadenopathy, purpura, peripheral edema, hepatosplenomegaly or ascites.
Blood tests showed hemoglobin of 14 gm/dl (normal, 12 to 16 gm/dl), WBC count 6.6 x 103/pl (normal, 4.5 to 11 x 103/~1), platelet count 85 x 103/pl (normal, 160 to 420 x 103/p.l), albumin 2.9 gm/dl (normal, 3.7 to 5 gm/dl), total globulin 4.5 gm/dl (normal, 2.6 to 3.2 gm/dl), gamma-globulin 2.7 gm/dl (normal, 0.6 to 1.8 gm/dl), AST 114 U L (normal, <40 UL), ALT 116 U L (normal, <46 U/L), alkaline phosphatase 131 U L (normal, 41 to 116 U/L), total bilirubin 1 mg/dl (normal, 0.1 to 1.2 mg/dl), prothrombin time 14.8 sec (control 11.5). She was negative for HBsAg, antinuclear antibody (ANA) and antimitochondrial antibody (AMA) but positive for smooth muscle antibody (SMA) at a titer of 1 : 60.
Bone marrow examination revealed normal findings and adequate megakaryocytes. Liver biopsy performed in March 1990 showed CAH. She was started on prednisone (40 mg/day).
Her liver profile showed mild improvement. In July 1990, her serum albumin was 3.8 gm/dl, total globulin 2.8 gm/dl, AST 75 UL, ALT 98 U/L and total bilirubin 1.5 gm/dl. She discontinued prednisone treatment at this point and was lost to follow-up.
The patient returned to the Liver Clinic in June 1993 with complaints of weight gain, increasing pedal edema and amenorrhea. Physical examination revealed multiple spider angiomata, pitting leg edema extending to midtibia, a firm liver edge palpable just below the right costal margin with a liver span of 8 cm, a palpable spleen tip and no ascites. Gynecological examination was normal.
Blood tests showed hemoglobin 12 gm/dl, WBC count 4.6 x 103/pl, platelet 65 x 103/~1, albumin 2.5 gm/dl, total globulin 5 gm/dl, gamma-globulin 3.5 gm/dl, AST 214 U/L,