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J Hepatol. 2014 Mar;60(3):579-89. doi: 10.1016/j.jhep.2013.10.025. Epub 2013 Nov 6.

Long term results of liver transplantation for Wilson's disease: experience in France.

Author information

1
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.
2
Centre National de Référence de la Maladie de Wilson/Fédération des Spécialités Digestives, Hôpital Édouard Herriot, Hospices Civils de Lyon, Lyon, France.
3
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.
4
Service d'Hépatologie Pédiatrique, Hôpital Necker-Enfants Malades, AP-HP, Paris, France.
5
Service de Chirurgie générale et Transplantation, Hôpital Hautefeuille, CHU Strasbourg, France.
6
Service d'Hépatologie, Hôpital Jean Minjoz, CHU Besançon, France.
7
Service d'Hépatologie, Hôpital Beaujon, AP-HP, Clichy, France.
8
Service de Chirurgie, Hôpital Cochin, AP-HP, Paris, France.
9
Service d'Hépatologie, Hôpital Henri Mondor, AP-HP, Créteil, France.
10
Service d'Hépato-Gastroentérologie, Hôpital Claude Huriez, CHRU Lille, Lille, France.
11
Service de Néphrologie-Hypertension artérielle-Dialyse-Transplantation, Hôpital Rangueil, CHU de Toulouse, France.
12
Service de Chirurgie Hépatobiliaire et Digestive, Hôpital de Pontchaillou, CHU de Rennes, France.
13
Service d'Hépatologie et de Gastroentérologie, Hôpital Pellegrin, Bordeaux, France.
14
Fédération Médico-Chirurgicale des Maladies de l'Appareil Digestif, Hôpital Saint-Eloi, Montpellier, France.
15
Service de Chirurgie Digestive, CHU Bretonneau, Tours, France.
16
Service de Chirurgie Digestive, Hôpital L'Archet (2), CHU Nice, Nice, France.
17
Centre National de Référence de la Maladie de Wilson/Service de Pédiatrie, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Bron, France.
18
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.
19
Service d'Hépatologie, Hôpital de la Croix-Rousse, Hospices civils de Lyon, Lyon, France.
20
Service de Chirurgie Digestive, Hôpital la Conception, Marseille, France.
21
Service d'Hépatologie, Hôpital St Antoine, AP-HP, Paris, France.
22
Centre National de Référence de la Maladie de Wilson/Service de Neurologie, Hôpital Lariboisière, AP-HP, Paris, France.
23
Service de chirurgie hépatobiliaire et transplantation hépatique, Hôpital St Antoine, AP-HP, Paris, France.

Abstract

BACKGROUND & AIMS:

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.

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

PMID:
24211743
DOI:
10.1016/j.jhep.2013.10.025
[Indexed for MEDLINE]

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