Share this Article
Low JM, Tan M, Garcia A, Aw M, Quak SH
Correspondence: Dr Tan Li Nien Michelle, email@example.com
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.
Keywords: aminotransferases, childhood autoimmune hepatitis, hepatomegaly, immunosuppressant therapy, liver pathology
Singapore Med J 2014; 55(12): 648-651; http://dx.doi.org/10.11622/smedj.2014179
| 1. Lee YM, Teo EK, Ng TM, Khor C, Fock KM. Autoimmune hepatitis in Singapore: a rare syndrome affecting middle-aged women. J Gastroenterol Hepatol 2001; 16:1384-9. |
| 2. Boberg KM, Aadland E, Jahnsen J, et al. Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population. Scand J Gastroenterol 1998; 33:99?103. |
| 3. Boberg KM. Prevalence and epidemiology of autoimmune hepatitis. Clin Liver Dis 2002; 6:635-47. |
| 4. Delgado JS, Vodonos A, Malnick S, et al. Autoimmune hepatitis in southern Israel: a 15?year multicenter study. J Dig Dis 2013; 14:611-8. |
| 5. Deneau M, Jensen MK, Holmen J, et al. Primary sclerosing cholangitis, autoimmune hepatitis, and overlap in Utah children: epidemiology and natural history. Hepatology 2013; 58:1392-400. |
| 6. Krawitt EL. Autoimmune hepatitis. N Engl J Med 2006; 354:54-66. |
| 7. Roberts EA. Autoimmune hepatitis from the paediatric perspective. Liver Int 2011; 31:1424-31. |
|8. Waldenstrom J. Leber, Blutproteine und Nahrungseiweiss. Dtsch Ges Verdau Stoffwechselkr 1950; 15:113-119.|
| 9. Roberts EA. Autoimmune hepatitis from the paediatric perspective. Liver Int 2011; 31:1424-31. |
| 10. Mieli-Vergani G, Vergani D. Autoimmune liver diseases in children - what is different from adulthood? Best Pract Res Clin Gastroenterol 2011; 25:783?95. |
| 11. Yeh SH, Ni YH, Jeng YM, et al. Emerging importance of autoimmune hepatitis in children in Taiwan, an endemic area for viral hepatitis. Pediatr Neonatol 2009; 50:65-9. |
| 12. Lee WS, Saw CB, Sarji SA. Autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome in a child: diagnostic usefulness of magnetic resonance cholangiopancreatography. J Paediatr Child Health 2005; 41:225-7. |
| 13. Basir N, Yew TG, Telisinghe PU, Chong VH. Autoimmune hepatitis in children: a report of two cases. Singapore Med J 2011; 52:e27-30. |
| 14. Gregorio GV, Portmann B, Reid F, et al. Autoimmune hepatitis in childhood: a 20-year experience. Hepatology 1997; 25:541-7. |
| 15. Olbjørn C, Sanengen T. [Autoimmmune hepatitis in children]. Tidsskr Nor Laegeforen 2007; 127:19-22. Norwegian. |
| 16. Li DY, Schwarz KB. Autoimmune hepatitis. Adolesc Med Clin 2004; 15:131-43, x. |
| 17. Czaja AJ. Autoimmune liver disease. Curr Opin Gastroenterol 2004; 20:231-40. |
|18. Czaja AJ, Manns MP. Advances in the diagnosis, pathogenesis, and management of autoimmune hepatitis. Gastroenterology 2010; 139:58-72.e4.|
| 19. Yokosawa S, Yoshizawa K, Ota M, et al. A genomewide DNA microsatellite association study of Japanese patients with autoimmune hepatitis type 1. Hepatology 2007; 45:384-90. |