Abstract
In a 20-yr period we saw 33 children (7 boys, 26 girls), 1 to 18 yr old, with chronic active hepatitis. Presenting signs and symptoms were hepatomegaly (91%), jaundice (82%), splenomegaly (76%), bleeding diathesis (50%), anorexia (48%), and lethargy (47%). Laboratory abnormalities (mean values) included increased levels of SCOT (595 IU), bilirubin (7.1 mg/dl), and γ-globulin (3.7 g/dl), prolonged prothrombin time (16 sec), and positive anti-smooth muscle and anti-glomerular basement membrane antibodies. Pi type (protease inhibition) MZ was found in 3 patients, MS in 1, and MM in the other 25 tested. All patients were HBsAgnegative. Average follow-up (FU) has been 49 mo; 4 are not taking medications (FU 95 mo), 12 are well and taking steroids or azathioprine (or both) (72 mo), whereas 10 still have clinical features (34 mo). Only 4 patients died, 3 of whom had cirrhosis at onset. The remaining 3 patients were seen only in the past few months. Therapy was similar in all groups. Medication was titrated, based on SGOT level; attempts to discontinue treatment failed in 41%. In 80% of patients, SGOT, bilirubin, and albumin levels were normal within 1 yr and remained so, but γ-globulin levels were increased (>1.4 g/dl) in 39% at 1 yr and 63% at 4 yr. Histologic pattern at onset (except for cirrhosis, seen in 7) or abnormal Pi type did not influence the course. γ-Globulin rather than SCOT level might be the most useful parameter.
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