title: Re-evaluation of the indications for liver transplantation in Wilson's disease based on the outcomes of patients referred to a transplant center.
abstract: The aim of this study was to re-evaluate the indications and timing of LT for WD. From 2000 to 2009, eight patients with WD who had been referred to our institution for LT were enrolled in this study. The mean patient age was 15.9 yr (range, 7-37 yr). Four patients could not receive LT, because there were no available donors. All four patients were treated with chelating agent medication. Three of them (two of two patients with fulminant WD and one of two with cirrhotic WD) who did not undergo LT are still alive and doing well with stable liver functional tests. Only one of the patients with cirrhotic WD who did not undergo LT died of hepatic failure. Even among the four patients who underwent LT, one with fulminant WD recovered from hepatic encephalopathy with apheresis therapy and chelating agent. He later required LT because of severe neutropenia from d-penicillamine. The other three patients who underwent LT recovered and have been doing well. Some of the patients with WD can recover and avoid LT with medical treatment. Even when WD has progressed liver cirrhosis and/or fulminant hepatic failure at the time of diagnosis, medical treatment should be tried before considering LT.
authors.affiliation: Department of Transplantation and Pediatric Surgery, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan. akarise@fc.kuh.kumamoto-u.ac.jp
authors.forename: Yuki
authors.initials: Y
authors.lastname: Ohya
authors.forename: Hideaki
authors.initials: H
authors.lastname: Okajima
authors.forename: Masaki
authors.initials: M
authors.lastname: Honda
authors.forename: Shintaro
authors.initials: S
authors.lastname: Hayashida
authors.forename: Hiroko
authors.initials: H
authors.lastname: Suda
authors.forename: Shiro
authors.initials: S
authors.lastname: Matsumoto
authors.forename: Kwang-Jong
authors.initials: KJ
authors.lastname: Lee
authors.forename: Hidekazu
authors.initials: H
authors.lastname: Yamamoto
authors.forename: Takayuki
authors.initials: T
authors.lastname: Takeichi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Mitsubuchi
authors.forename: Katsuhiro
authors.initials: K
authors.lastname: Asonuma
authors.forename: Fumio
authors.initials: F
authors.lastname: Endo
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Inomata
mesh_terms: D000293:Adolescent; D000328:Adult; D002614:Chelating Agents / Q000627:therapeutic use; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006501:Hepatic Encephalopathy / Q000209:etiology / Q000628:therapy; D006527:Hepatolenticular Degeneration / Q000628:therapy*; D006801:Humans; D017114:Liver Failure, Acute / Q000628:therapy; D008111:Liver Function Tests; D016031:Liver Transplantation / Q000379:methods* / Q000592:standards; D008297:Male; D012017:Referral and Consultation; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D002614:Chelating Agents