title: Outcome of liver transplantation in hereditary hemochromatosis.
abstract: Hereditary hemochromatosis (HH) is a genetic disorder of iron metabolism. It is an uncommon indication for liver transplantation (LT). It has been suggested that patients who undergo LT for cirrhosis related to HH have higher morbidity and mortality from cardiac, infectious and malignant complications. The purpose of this retrospective review was to determine whether these observations hold true in the current era. We analysed the data of 22 patients who had LT for HH from 1996 to 2007 at our center. Thirteen patients had LT for complications of end-stage liver disease, seven for hepatocellular carcinoma (HCC) and two for subacute liver failure. Cofactors promoting liver disease were identified in 15 patients. Ten patients had iron reduction with venesection before transplantation. Patient and graft survival at 1 and 5 years were 80.7%, and 74% respectively. There were seven deaths after a median follow up of 46 months either because of multiorgan failure, or caused by HCC recurrence. Bacterial infections were the commonest cause of morbidity. Patients with HH remain at a higher risk of developing HCC. Infectious complications are common. Iron reduction with preoperative venesection reduces the risk of cardiac and infection complications postoperatively. Improved survival post-LT reflects changes in selection, disease modification through venesection, and improvement in immunosuppression.
authors.affiliation: Kings College London School of Medicine, Institute of Liver Studies, Kings College Hospital, London, SE5 9RS, UK.
authors.forename: Faisal S
authors.initials: FS
authors.lastname: Dar
authors.forename: Walid
authors.initials: W
authors.lastname: Faraj
authors.forename: Muhammad B
authors.initials: MB
authors.lastname: Zaman
authors.forename: Adam
authors.initials: A
authors.lastname: Bartlett
authors.forename: Adrian
authors.initials: A
authors.lastname: Bomford
authors.forename: Adrian
authors.initials: A
authors.lastname: O'Sullivan
authors.forename: John
authors.initials: J
authors.lastname: O'Grady
authors.forename: Michael
authors.initials: M
authors.lastname: Heneghan
authors.forename: Mohamed
authors.initials: M
authors.lastname: Rela
authors.forename: Nigel D
authors.initials: ND
authors.lastname: Heaton
mesh_terms: D000328:Adult; D000368:Aged; D006528:Carcinoma, Hepatocellular / Q000628:therapy; D005260:Female; D005355:Fibrosis / Q000628:therapy; D006085:Graft Survival; D006432:Hemochromatosis / Q000235:genetics / Q000628:therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D019190:Iron Overload; D008113:Liver Neoplasms / Q000628:therapy; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents