Perfil clínico de pacientes pediátricos con diagnóstico de hepatitis autoinmune en un centro de alta complejidad del suroccidente colombiano

Abstract

Introduction. Autoimmune hepatitis is a progressive inflammatory liver disease that is rare in children, characterized by inflammation in liver histology, the presence of autoantibodies and high levels of immunoglobulin G. There are few data in children and in some studies performed in Latin America, but limited in Colombia. The aim of this study is to describe the demographic, clinical, paraclinical and therapeutic data as well as, the outcome of paediatric patients diagnosed with autoimmune hepatitis in a tertiary care centre in south-western Colombia. Methods. This is a descriptive study of a retrospective cohort of paediatric patients (≤ 18 years) with a diagnosis of autoimmune hepatitis, attended in a third-level hospital in Colombia between 2011 and 2019. Statistical analyses were performed using the program Stata 14.0. Results. A total of 40 patients were included, 57.5% were female, with a median age of 10.5 years (IQR 4-13). The 62.5% presented with acute hepatitis, 20% had liver failure, a 27.5% with cirrhosis at diagnosis; 75% presented compatible liver biopsy, and 82.5% were classified as type 1 autoimmune hepatitis. All patients received a steroid plus immunomodulator; presenting biochemical remission in 75% relapse in the 22.5%. Four of the patients required liver transplantation. Conclusions. Autoimmune hepatitis is an infrequent entity, although not negligible, in children in our environment. A higher prevalence of type I autoimmune hepatitis was found. Most patients were female and presented with symptoms of acute hepatitis at the initial diagnosis. The characteristics and clinical responses, in this cohort, are similar to those described in different series in the world population.