P79 Plasma succinylacetone is raised after liver transplantation for tyrosinaemia type 1 and is associated with reduced porphobilinogen synthase activity suggesting it is functional

Abstract

IntroductionTyrosinaemia type 1 (TT1) is a rare disorder of tyrosine metabolism leading to accumulation of toxic metabolites such as succinylacetone (SA) and a high risk of hepatocellular carcinoma. Children with...