Early life predictive markers of liver disease outcome in an International, Multicentre Cohort of children with Alagille syndrome

摘要

Background & AimsLiver disease in Alagille syndrome is highly variable. Many of the patients presenting with severe cholestasis early in life improve spontaneously; 10-20%, however, have progressive disease. It is currently not possible to predict long-term hepatic outcomes in Alagille syndrome. This international, multicentre study was aimed at identifying early life predictors of liver disease outcome.