title: Long-term outcomes for 32 cases of Wilson's disease after living-donor liver transplantation.
abstract: BACKGROUND
  Long-term outcomes after living donor liver transplantation (LDLT) in Wilson's disease (WD) with heterozygous donors for WD gene are unknown.
  
  
  METHODS
  LDLT was performed for 32 patients with WD (15 men, 17 women; mean age 16 years; range, 6-40 years) at Kyoto University Hospital from 1992 to 2006. The mean follow-up time from LDLT was 6.7+/-4.0 years (0.04-15.0 years). Donors were mainly parents (90.6%), who were obligatory carriers of the WD mutation. The present study examined retrospectively the copper metabolism, recurrence of WD, survival rate, and neurologic outcomes after LDLT.
  
  
  RESULTS
  Mean ceruloplasmin at the time of LDLT was 9.7+/-7.3 mg/dL and increased to 22.3+/-5.3 mg/dL (normal, 18-37 mg/dL). Urinary copper decreased from 2704+/-901 to 73.7+/-5.2 microg/day (normal, <50 microg/day). Serum copper improved from 72.9+/-33.9 to 81.0+/-14.9 mg/dL (normal, 78-131 mg/dL). Although six patients died and two received retransplantation, the remaining 24 remain alive without recurrence, with overall survival rates of 90.6%, 83.7%, and 79.9% at 1, 5, and 10 years, respectively. Patients with chronic liver failure had a poorer prognosis (P<0.05).
  
  
  CONCLUSIONS
  Use of liver grafts from heterozygous donors has been considered safe. Good improvements in copper metabolism were obtained without evidence of recurrence in long-term follow-up. Neuropsychologic presentations of WD improved or remained unchanged. Indications for liver transplantation in patients with WD with neurologic symptoms must be considered carefully based on the stage of neurologic damage and its irreversibility.
authors.affiliation: Department of Surgery, Graduate School of Medicine, Kyoto University, Shogoin, Sakyo-Ku, Kyoto, Japan. elenayy@yahoo.com
authors.forename: Elena Y
authors.initials: EY
authors.lastname: Yoshitoshi
authors.forename: Yasutsugu
authors.initials: Y
authors.lastname: Takada
authors.forename: Fumitaka
authors.initials: F
authors.lastname: Oike
authors.forename: Seisuke
authors.initials: S
authors.lastname: Sakamoto
authors.forename: Kohei
authors.initials: K
authors.lastname: Ogawa
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Kanazawa
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Ogura
authors.forename: Shinya
authors.initials: S
authors.lastname: Okamoto
authors.forename: Hironori
authors.initials: H
authors.lastname: Haga
authors.forename: Mikiko
authors.initials: M
authors.lastname: Ueda
authors.forename: Hiroto
authors.initials: H
authors.lastname: Egawa
authors.forename: Mureo
authors.initials: M
authors.lastname: Kasahara
authors.forename: Koichi
authors.initials: K
authors.lastname: Tanaka
authors.forename: Shinji
authors.initials: S
authors.lastname: Uemoto
mesh_terms: D000251:Adenosine Triphosphatases / Q000235:genetics; D000293:Adolescent; D000328:Adult; D027682:Cation Transport Proteins / Q000235:genetics; D002570:Ceruloplasmin / Q000378:metabolism; D002648:Child; D003300:Copper / Q000097:blood / Q000652:urine; D000073840:Copper-Transporting ATPases; D005260:Female; D006085:Graft Survival; D006527:Hepatolenticular Degeneration / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism / Q000401:mortality / Q000601:surgery*; D006579:Heterozygote; D006801:Humans; D053208:Kaplan-Meier Estimate; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D009154:Mutation; D009483:Neuropsychological Tests; D012008:Recurrence; D012086:Reoperation; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D027682:Cation Transport Proteins; D003300:Copper; D002570:Ceruloplasmin; D000251:Adenosine Triphosphatases; D000073840:Copper-Transporting ATPases