Özet:

Clinical and laboratory features and the outcome of ten cases (nine female, one male) (age range 7-14 years, mean 10.7±2.2 years) with autoimmune hepatitis are described. The diagnosis was established by the laboratory features of elevated serum transferases, hypergammaglobulinemia, presence of autoantibodies, liver histology, excluding viral and metabolic etiologies and by a prompt response to corticosteroid therapy. One patient was associated with celiac disease. Chronic active hepatitis was present histologically in seven children while in three, cirrhosis with portal hypertension had already been established. All patients received steroids. Azathioprine was instituted in three patients in addition to steroid therapy. The patients were followed up for 1- 9 years (mean 4.1±3.0 years). One patient relapsed during maintenance therapy, and two patients died within less than one year probably because of poor compliance to treatment. In one patient treatment was withdrawn after three years and no relapse occurred during the six months of follow-up. Control liver biopsies were done in six patients after two years of treatment in which five showed histological improvement. We conclude that autoimmune hepatitis in childhood has a wide spectrum of clinical features including the absence of symptoms, acute hepatitis and established cirrhosis with portal hypertension. Autoimmune hepatitis should be kept in mind in the differential diagnosis of both acute and chronic liver diseases of children. Autoimmune hepatitis carries a high mortality if left untreated but has a favourable outcome when treatment is initiated early in the course of the disease.

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