title: Orthotopic liver transplantation for hepatic-based metabolic disorders.
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 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).
authors.affiliation: Liver Transplant Surgical Service, Kings College Hospital, Camberwell, London, UK.
authors.forename: M
authors.initials: M
authors.lastname: Rela
authors.forename: P
authors.initials: P
authors.lastname: Muiesan
authors.forename: N D
authors.initials: ND
authors.lastname: Heaton
authors.forename: M
authors.initials: M
authors.lastname: Corbally
authors.forename: H
authors.initials: H
authors.lastname: Hajj
authors.forename: A P
authors.initials: AP
authors.lastname: Mowat
authors.forename: R
authors.initials: R
authors.lastname: Williams
authors.forename: K C
authors.initials: KC
authors.lastname: Tan
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D005260:Female; D006084:Graft Rejection / Q000601:surgery; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D016030:Kidney Transplantation; D017093:Liver Failure / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D008661:Metabolism, Inborn Errors / Q000601:surgery*; D008875:Middle Aged; D012086:Reoperation