Long-term outcomes for 32 cases of Wilson's disease after living-donor liver transplantation.
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.
Yoshitoshi
Elena Y
EY
Department of Surgery, Graduate School of Medicine, Kyoto University, Shogoin, Sakyo-Ku, Kyoto, Japan. elenayy@yahoo.com
Takada
Yasutsugu
Y
Oike
Fumitaka
F
Sakamoto
Seisuke
S
Ogawa
Kohei
K
Kanazawa
Hiroyuki
H
Ogura
Yasuhiro
Y
Okamoto
Shinya
S
Haga
Hironori
H
Ueda
Mikiko
M
Egawa
Hiroto
H
Kasahara
Mureo
M
Tanaka
Koichi
K
Uemoto
Shinji
S
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
D027682:Cation Transport Proteins; D003300:Copper; D002570:Ceruloplasmin; D000251:Adenosine Triphosphatases; D000073840:Copper-Transporting ATPases