[Aetiology, outcomes and prognostic indicators of paediatric acute liver failure]

Abstract

Introduction: Acute liver failure (ALF) is a multisystem disease with severe impairment of liver function of acute onset. The Paediatric End-stage Liver Disease (PELD) score is used as a predictor of mortality in chronic liver disease, however experience is limited in ALF.

Objectives: To evaluate the aetiology and outcomes of children with ALF in a Children's Liver Transplant Centre, and to investigate the validity of PELD as a prognostic indicator.

Patients and methods: A retrospective study was conducted on patients diagnosed with ALF in our hospital from 2000 to 2013 using the criteria of the Paediatric ALF Study Group.

Results: The study included 49 patients with an age range 0-14years. The most frequent aetiologies were: indeterminate (36.7%) and metabolic (26.5%). Liver transplant (LT) was required by 42.8%, and there were 16.3% deaths. Patients with higher levels of bilirubin, INR, or encephalopathy were more likely to require a liver transplant, yielding an OR for INR 1.93. A cut-off of 27 in the PELD score according to the ROC curve showed a sensitivity of 86% and a specificity of 85%, predicting a worse outcome (AUC: 0.90; P<.001). The survival of patients with ALF without transplantation seems more likely in those who have low values of PELD and absence of encephalopathy, with a RR of 0.326.

Conclusions: ALF patients with a high PELD score and the presence of encephalopathy had worse outcomes. The PELD score could be a useful tool to establish the optimum time for inclusion in the transplant list, however further studies are still needed.

Keywords: Acute liver failure; Children; Fallo hepático agudo; Niños; PELD.