Liver Transplantation in a Child with Severe Hypercholesterolaemia in Alagille Syndrome

Abstract

Introduction: Liver transplantation is a curative treatment modality in children with end stage liver disease in Alagille syndrome. Clinical Picture: We report a 3-year-old child with this condition who had severe hypercholesterolaemia, pruritus and extensive xanthomatosis. Treatment: Liver transplantation was performed in this patient. Outcome: He recovered well with normalisation of his lipid profile. This procedure also resulted in resolution of the disfiguring xanthomatosis.