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 retransplanted, 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
展开▼
