P0355 AUTOIMMUNE HEPATITIS IN MALAYSIAN CHILDREN

Abstract

Introduction: Autoimmune hepatitis (AIH) is a recognized cause of chronic childhood hepatitis. It is more commonly described in population of northern European Caucasoid origin. We described the clinical features and outcome of 8 Malaysian children with AIH. Methods: Children with abnormal liver enzymes and/or deranged liver function from 2000 onwards were included. AIH was diagnosed based on the criteria according to Alvarez et al, after known viral, drugs-related and metabolic causes of hepatitis were excluded. They were classified as AIH type 1 (positive SMA and/or ANA), AIH type 2 (positive LKM1 antibody), or AIH / primary sclerosing cholangitis overlap syndrome (AIH/PSC) if features suggestive of primary sclerosing cholangitis were present as well. Results: 8 children (5 girls, 3 boys; 5 Chinese, 2 Malays, 1 Indian; median age at referral: 6.5 years, range 2–12 years) fulfilled the diagnostic criteria. Six had AIH type 1, one had AIH type 2, and one had AIH/PSC overlap syndrome. The father of one child who has AIH type 1 has AIH type 1 himself, while another child (with AIH type 1 and thyrotoxicosis) had family history of thyrotoxicosis. Of the 7 children with AIH, 5 presented with jaundice and hepatomegaly, one had vague constitutional symptoms, abdominal pain and enlarged liver, while another child had abnormal liver enzymes on routine screening. All had raised liver cell enzymes. One child with AIH type 1 developed grade 1 hepatic encephalopathy during the course of illness. The girl with AIH/PSC had prolonged jaundice, steatorrhoea, and enlarged liver. Magnetic resonance cholangiography showed irregularity and attenuation of hepatic ducts. Liver histology ranged from mild hepatitis, chronic active hepatitis to cirrhosis. All children with AIH alone responded to prednisolone +/− azathioprine, with resolution of jaundice within one month and normalization of liver enzymes two months after therapy. All are in remission. The child with AIH/PSC had resolution of jaundice with a course of prednisolone, but the liver enzymes remained persistently elevated. Conclusion: AIH is a known cause of acute liver dysfunction in children from Asian population. It should be excluded in any children who have recent onset of jaundice, or constitutional symptoms with hepatomegaly.