Orthotopic liver transplantation for hepatic-based metabolic disorders

Abstract

Abstract Between January 1989 and June 1993, a total of 470 liver transplantations were performed at King's College Hospital. Thirty-seven transplantations were performed in 34 patients with liver-based metabolic disease. There were 16 females and 18 males with a median age of 19 years (range 1 month to 62 years). There were 14 patients under 16 years of age. The indications for liver transplantation were Wilson's disease (n= 16), alpha 1-antitrypsin deficiency (n= 10), tyrosinaemia (n= 2), primary hyperoxaluria type 1 (PH1; n= 2), congenital haemochromatosis (n= 1), familial amyloidotic polyneuropathy (FAP; n= 1, familial hypercholesterolaemia) (n= 1) and Crigler-Najjar syndrome type I (CNS1; n= 1). These included two patients who received combined heart-liver grafts for familial hypercholesterolaemia and FAP, respectively. Two patients received combined liver-kidney transplants for PH1. There were four deaths: from sepsis (n= 2), acute hepatic vein obstruction in a left lateral segment graft (n= 1) and portal vein thrombosis with liver necrosis (n= 1). Three patients were re-transplanted, one for chronic rejection and two for hepatic artery thrombosis, giving an overall graft survival of 81% and patient survival of 88% (30/34), at a mean follow-up of 34 months (range 10–64 months).