Characterization of acute liver failure and development of a continuous risk of death staging system in children

Background/Aims: Acute liver failure (ALF) in children has been associated with an overall mortality of approximately 70% in the pretransplant era and 50-80% survival in those undergoing orthotopic liver transplantation. There is currently no system for staging severity of ALF in children. The aim of this study was to characterize pediatric ALF in a tertiary hospital and to derive a scoring system to stratify severity of ALF based on predicted mortality. Methods: Prospective data collection of 81 children from December 1993-2003 who presented with ALF. Data recorded included peak laboratory values, clinical characteristics, and survival. Results: Transplant-free survival was 56% with overall survival including those undergoing OLT of 72%. Transplantation rate was 22% with transplant survival of 72%. Of the peak laboratory values analyzed, total bilirubin, prothrombin time/INR, and ammonia were the most predictive of death or a need for liver transplant. A simple risk staging system was developed based on the ability of these three laboratory measurements to predict mortality. Conclusions: The survival in pediatric ALF has improved in recent years. Risk staging for ALF could potentially be used in clinical research for risk-adjusted outcomes analysis and to help stratify patients for clinical studies according to mortality risk.