Childhood autoimmune hepatitis in a paediatric unit of a tertiary care hospital

INTRODUCTION Although childhood autoimmune hepatitis (AIH) has been extensively investigated in the West, data on AIH in the East is lacking. We aimed to investigate AIH's clinical, biochemical and histological features, as well as its outcomes, in one of Singapore's two major paediatric units. METHODS This was a retrospective study of children diagnosed with AIH in the paediatric unit of National University Hospital, Singapore, over the last 12 years. Children with de novo AIH after liver transplantation were excluded. The demographic and clinical features of the patients, and their laboratory, treatment and clinical outcomes were reviewed. RESULTS This study comprised ten patients (six females, four males), with a median age of 5.1 (range 2.1-13.8) years at diagnosis. Five patients had inflammatory bowel disease (IBD). Seven patients had type 1 AIH, and three had autoimmune sclerosing cholangitis (ASC) and IBD; none had type 2 AIH. The median level of aspartate aminotransferase at diagnosis was 183 (range 45-2,649) U/L. Prednisolone 1 mg/kg/day was prescribed at diagnosis for eight patients. Two patients were lost to follow-up and were treated symptomatically when they re-presented with end-stage liver disease. Azathioprine or mycophenolate mofetil was prescribed after 3-7 months of treatment. Normalisation of aminotransferase levels took an average of 5.3 (range 1-39) months. CONCLUSION AIH is a rare but important cause of liver pathology. Children in this region with elevated aminotransferases or unexplained hepatomegaly should be screened for AIH.