title: Long term results of liver transplantation for Wilson's disease: experience in France.
abstract: OBJECTIVE
  Liver transplantation (LT) is the therapeutic option for severe complications of Wilson's disease (WD). We aimed to report on the long-term outcome of WD patients following LT.
  
  
  METHODS
  The medical records of 121 French patients transplanted for WD between 1985 and 2009 were reviewed retrospectively. Seventy-five patients were adults (median age: 29 years, (18-66)) and 46 were children (median age: 14 years, (7-17)). The indication for LT was (1) fulminant/subfulminant hepatitis (n = 64, 53%), median age = 16 years (7-53), (2) decompensated cirrhosis (n = 50, 41%), median age = 31.5 years (12-66) or (3) severe neurological disease (n = 7, 6%), median age = 21.5 years (14.5-42). Median post-transplant follow-up was 72 months (0-23.5).
  
  
  RESULTS
  Actuarial patient survival rates were 87% at 5, 10, and 15 years. Male gender, pre-transplant renal insufficiency, non elective procedure, and neurological indication were significantly associated with poorer survival rate. None of these factors remained statistically significant under multivariate analysis. In patients transplanted for hepatic indications, the prognosis was poorer in case of fulminant or subfulminant course, non elective procedure, pretransplant renal insufficiency and in patients transplanted before 2000. Multivariate analysis disclosed that only recent period of LT was associated with better prognosis. At last visit, the median calculated glomerular filtration rate was 93 ml/min (33-180); 11/93 patients (12%) had stage II renal insufficiency and none had stage III.
  
  
  CONCLUSIONS
  Liver failure associated with WD is a rare indication for LT (<1%), which achieves an excellent long-term outcome, including renal function.
authors.affiliation: Centre National de Référence de la Maladie de Wilson/Fédération des Spécialités Digestives, Hôpital Édouard Herriot, Hospices Civils de Lyon, Lyon, France. Electronic address: olivier.guillaud@chu-lyon.fr.
authors.forename: Olivier
authors.initials: O
authors.lastname: Guillaud
authors.affiliation: Centre National de Référence de la Maladie de Wilson/Fédération des Spécialités Digestives, Hôpital Édouard Herriot, Hospices Civils de Lyon, Lyon, France.
authors.forename: Jérôme
authors.initials: J
authors.lastname: Dumortier
authors.affiliation: Centre National de Référence de la Maladie de Wilson/Centre Hépato-Biliaire, Hôpital Paul Brousse, AP-HP, Villejuif, France; UMR 785, INSERM, France; UMR-S 785, Univ Paris-Sud, Villejuif, France; DHU Hepatinov, Villejuif, France.
authors.forename: Rodolphe
authors.initials: R
authors.lastname: Sobesky
authors.affiliation: Service d'Hépatologie Pédiatrique, Hôpital Necker-Enfants Malades, AP-HP, Paris, France.
authors.forename: Dominique
authors.initials: D
authors.lastname: Debray
authors.affiliation: Service de Chirurgie générale et Transplantation, Hôpital Hautefeuille, CHU Strasbourg, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Wolf
authors.affiliation: Service d'Hépatologie, Hôpital Jean Minjoz, CHU Besançon, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Vanlemmens
authors.affiliation: Service d'Hépatologie, Hôpital Beaujon, AP-HP, Clichy, France.
authors.forename: François
authors.initials: F
authors.lastname: Durand
authors.affiliation: Service de Chirurgie, Hôpital Cochin, AP-HP, Paris, France.
authors.forename: Yvon
authors.initials: Y
authors.lastname: Calmus
authors.affiliation: Service d'Hépatologie, Hôpital Henri Mondor, AP-HP, Créteil, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Duvoux
authors.affiliation: Service d'Hépato-Gastroentérologie, Hôpital Claude Huriez, CHRU Lille, Lille, France.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Dharancy
authors.affiliation: Service de Néphrologie-Hypertension artérielle-Dialyse-Transplantation, Hôpital Rangueil, CHU de Toulouse, France.
authors.forename: Nassim
authors.initials: N
authors.lastname: Kamar
authors.affiliation: Service de Chirurgie Hépatobiliaire et Digestive, Hôpital de Pontchaillou, CHU de Rennes, France.
authors.forename: Karim
authors.initials: K
authors.lastname: Boudjema
authors.affiliation: Service d'Hépatologie et de Gastroentérologie, Hôpital Pellegrin, Bordeaux, France.
authors.forename: Pierre Henri
authors.initials: PH
authors.lastname: Bernard
authors.affiliation: Fédération Médico-Chirurgicale des Maladies de l'Appareil Digestif, Hôpital Saint-Eloi, Montpellier, France.
authors.forename: Georges-Philippe
authors.initials: GP
authors.lastname: Pageaux
authors.affiliation: Service de Chirurgie Digestive, CHU Bretonneau, Tours, France.
authors.forename: Ephrem
authors.initials: E
authors.lastname: Salamé
authors.affiliation: Service de Chirurgie Digestive, Hôpital L'Archet (2), CHU Nice, Nice, France.
authors.forename: Jean
authors.initials: J
authors.lastname: Gugenheim
authors.affiliation: Centre National de Référence de la Maladie de Wilson/Service de Pédiatrie, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Bron, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Lachaux
authors.affiliation: Centre National de Référence de la Maladie de Wilson/Service d'Hépatologie et de Transplantation Hépatique Pédiatriques, Hôpital Bicêtre, AP-HP, Le Kremlin-Bicêtre, France; DHU Hepatinov, Villejuif, France.
authors.forename: Dalila
authors.initials: D
authors.lastname: Habes
authors.affiliation: Service d'Hépatologie, Hôpital de la Croix-Rousse, Hospices civils de Lyon, Lyon, France.
authors.forename: Sylvie
authors.initials: S
authors.lastname: Radenne
authors.affiliation: Service de Chirurgie Digestive, Hôpital la Conception, Marseille, France.
authors.forename: Jean
authors.initials: J
authors.lastname: Hardwigsen
authors.affiliation: Service d'Hépatologie, Hôpital St Antoine, AP-HP, Paris, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Chazouillères
authors.affiliation: Centre National de Référence de la Maladie de Wilson/Service de Neurologie, Hôpital Lariboisière, AP-HP, Paris, France.
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Trocello
authors.affiliation: Centre National de Référence de la Maladie de Wilson/Service de Neurologie, Hôpital Lariboisière, AP-HP, Paris, France.
authors.forename: France
authors.initials: F
authors.lastname: Woimant
authors.affiliation: Centre National de Référence de la Maladie de Wilson/Centre Hépato-Biliaire, Hôpital Paul Brousse, AP-HP, Villejuif, France; UMR 785, INSERM, France; UMR-S 785, Univ Paris-Sud, Villejuif, France; DHU Hepatinov, Villejuif, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Ichai
authors.affiliation: Centre National de Référence de la Maladie de Wilson/Service d'Hépatologie et de Transplantation Hépatique Pédiatriques, Hôpital Bicêtre, AP-HP, Le Kremlin-Bicêtre, France; DHU Hepatinov, Villejuif, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Branchereau
authors.affiliation: Service de chirurgie hépatobiliaire et transplantation hépatique, Hôpital St Antoine, AP-HP, Paris, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Soubrane
authors.affiliation: Centre National de Référence de la Maladie de Wilson/Centre Hépato-Biliaire, Hôpital Paul Brousse, AP-HP, Villejuif, France; UMR 785, INSERM, France; UMR-S 785, Univ Paris-Sud, Villejuif, France; DHU Hepatinov, Villejuif, France.
authors.forename: Denis
authors.initials: D
authors.lastname: Castaing
authors.affiliation: Centre National de Référence de la Maladie de Wilson/Service d'Hépatologie et de Transplantation Hépatique Pédiatriques, Hôpital Bicêtre, AP-HP, Le Kremlin-Bicêtre, France; DHU Hepatinov, Villejuif, France.
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Jacquemin
authors.affiliation: Centre National de Référence de la Maladie de Wilson/Centre Hépato-Biliaire, Hôpital Paul Brousse, AP-HP, Villejuif, France; UMR 785, INSERM, France; UMR-S 785, Univ Paris-Sud, Villejuif, France; DHU Hepatinov, Villejuif, France.
authors.forename: Didier
authors.initials: D
authors.lastname: Samuel
authors.affiliation: Centre National de Référence de la Maladie de Wilson/Centre Hépato-Biliaire, Hôpital Paul Brousse, AP-HP, Villejuif, France; UMR 785, INSERM, France; UMR-S 785, Univ Paris-Sud, Villejuif, France; DHU Hepatinov, Villejuif, France.
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Duclos-Vallée
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D005260:Female; D005602:France; D006085:Graft Survival; D006527:Hepatolenticular Degeneration / Q000401:mortality / Q000601:surgery*; D006801:Humans; D007165:Immunosuppression Therapy; D016031:Liver Transplantation / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D012086:Reoperation; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: ALF; BMI; BP; CLD; CNI; FHF; HAT; HCC; LT; MMF; PNF; SFHF; WD; Wilson’s disease; acute liver failure; blood pressure; body mass index; calcineurin-inhibitor; chronic liver disease; fulminant hepatic failure; hepatic artery thrombosis; hepatocellular carcinoma; liver transplantation; mycophenolate mofetil; n.a.; not available; primary non function; subfulminant hepatic failure