autoimmune hepatitis in children, clinical features and biochemical of iranian children

background: there are only a very small number of reports which discuss subtype, sex distribution, clinical features and laboratory characteristics of autoimmune hepatitis in children. the aim of this study was both to define the clinical features, biochemical and histological findings and also to determine the age and sex related distribution of autoimmune hepatitis (aih). materials and methods: data of 30 children presenting with aih (20 girls and 10 boys) have been analyzed for their clinical, serological, and histological profile. the most common presenting signs or symptoms were jaundice (60%), abdominal mass (23.4%) and constitutional symptoms (weakness, anorexia and paleness) (6.7%). results: about 10% of patients had an acute hepatitis like clinical presentation. twenty two children (73.3%) (15 girls, 7 boys, 2.1:1) had aih type 1 and 4 patients (13.3%) type 2 due to specific autoantibodies. four children could not be classified. in liver biopsy, 100% of patients had interface hepatitis and fibrosis with or without cirrhosis were found in 60%. conclusions: in our cohort the prevalence of aih was 2:1 in girls. type 1 was the most frequent diagnosis (73.3%) and was more prevalent in older children. patients with type 2 were younger. the clinical presentation of aih in children was unspecific and each type could only be differentiated by the determination of the specific autoantibodies.