Abstract

BRETT, Ana et al. Autoimmune hepatitis in paediatrics: 20-year experience. J Port Gastrenterol. [online]. 2013, vol.20, n.5, pp.191-198. ISSN 0872-8178.  https://doi.org/10.1016/j.jpg.2013.03.002.

Introduction: Autoimmune hepatitis (AIH) is a rare disease in paediatrics. Since it is a treatable liver disease, an early diagnosis, allowing for an adequate treatment, is essential. Aim: To describe the clinical presentation, laboratory features, personal and family history of autoimmune disease, diagnostic delay and outcome of the children followed in a Hepatology Unit with the diagnosis of AIH. Methods: Descriptive retrospective study of the clinical records of children diagnosed with AIH in a Hepatology Unit of a Paediatric Hospital, during 20 years (1992-2011). Results: The study included 33 children, 69.7% female, with an average age of 9.0±3.1 years. At presentation 63.6% had acute hepatitis. Personal and family history of autoimmune disease was present in 18.2% and 21.1%, respectively. Laboratory analysis showed hyperglobulinaemia in 93.9% and major cytolysis in 63.6%. The diagnosis was of AIH type 1 in 66.7%, AIH type 2 in 27.3% and 6% had negative auto-antibodies. All had their diagnosis confirmed by liver biopsy. The duration of disease prior to diagnosis was longer than one month in 66.7% of cases. After 2 months of treatment with immunosuppressants, 89.7% had a decrease of more than 80% in the levels of baseline ALT and AST. Three cases required liver transplantation, one of whom died. Conclusions: The diagnosis of AIH should be considered when a child, usually a girl and over 8 years-old, presents with acute hepatitis, hyperglobulinaemia and important cytolysis. An early referral for liver biopsy, so that adequate treatment can be started, is strongly recommended.

Keywords : Children; Autoimmune hepatitis; Diagnosis.

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