https://pubmed.ncbi.nlm.nih.gov/33500120/ Autoimmune Hepatitis
J Pediatr. 2021 Feb;229:95-101.e3.
doi: 10.1016/j.jpeds.2020.10.009. Epub 2020 Oct 23.

Autoimmune Hepatitis: Predictors of Native Liver Survival in Children and Adolescents

Gilda Porta 1, Elisa de Carvalho 2, Jorge L Santos 3, Jorge Gama 4, Jorge A Bezerra 5, Brazilian Group for the Study of Pediatric Liver Diseases

Abstract

Objective: To determine predictors of native liver survival (NLS) in children and adolescents with autoimmune hepatitis (AIH).
Study design: The medical records of children and adolescents with AIH were reviewed. A questionnaire was used to collect data on clinical presentation, biochemical and histologic findings, and treatment.

Results: A total of 819 patients were included, 89.6% with AIH-1 and 10.4% with AIH-2. The median age (months) at onset was 108 (min 6; max 210; IQR 59). The female sex was predominant (75.8%). The overall survival was 93.0%, with an NLS of 89.9%; 4.6% underwent liver transplantation. The risk of death or liver transplantation during follow-up was 3.2 times greater in patients with AIH-1 (P = .024). Greater levels of aspartate aminotransferase, alanine aminotransferase, serum albumin, platelet, and normal international normalized ratio at the initial presentation were associated with longer NLS (P = .046, P = .006, P < .001, P = .001, and P = .019, respectively). Normal C3 levels was associated with longer NLS (P = .017), with a chance of death or liver transplantation during follow-up being 3.4 times greater in patients with C3 below normal. Death or liver transplantation during follow-up was 2.8 times greater in patients with associated sclerosing cholangitis (P = .046). Complete remission favored NLS (P < .001), with a risk of death or liver transplantation 11.7 times greater for patients not achieving remission.

Conclusions: The best predictors of NLS in children and adolescents with AIH were the AIH-2 subtype, a normal C3 at diagnosis, remission during treatment, and normal a cholangiogram during the disease course.

Published on: 
Jan-2021

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