Pediatric Acute Liver Failure as Presentation of Autoimmune Hepatitis: Learning from a Fatal Case.

Abstract

Severe acute liver failure (SALF) is a rare condition in children. Up to 50% of the cryptogenic causes of SALF are associated with autoimmune hepatitis (AIH). This report presents a 5-year-old girl with progressive jaundice for 10 days. Her 1999 AIH diagnostic score totaled 11 points, compatible with probable AIH. She fulfilled the SALF criteria and the King's College criteria for liver transplantation, despite treatment with corticosteroids, and underwent the transplant, but died in the immediate postoperative period due to massive bleeding. Subsequently, the liver-kidney microsome type 1 result was 1:80, increasing the AIH score to 13 points. The final diagnosis was probable AIH type 2, associated with SALF. The biopsy of the explanted liver was compatible with fulminant hepatitis. This report highlights the difficult diagnosis of AIH in SALF, limitations of the diagnostic criteria for SALF in indications for emergency transplantation, and the uncertain therapeutic response produced by corticosteroids.