Outcomes of living-related liver transplantation for Wilson's disease: a single-center experience in China.
BACKGROUND
Although orthotopic liver transplantation provides a therapeutic option for patients with Wilson's disease (WD) presenting fulminant liver failure or drug resistance, it is still unclear whether the living-related liver transplantation (LRLT) can result in long-term therapeutic effect on WD.


METHODS
Here, we report a retrospective analysis of LRLT for 36 cases of WD patients. The indications for LRLT were fulminant hepatic failure in two patients and chronic advanced liver disease in 32 patients including 13 patients with Wilsonian neurologic manifestations. Two patients presented with severe Wilsonian neurologic manifestations even though their liver functions were stable.


RESULTS
Results revealed that the survival of posttransplant patients or grafts at 1, 3, and 5 years was 91.7%, 83.3%, 75%, or 86.1%, 77.8%, 75%, respectively. Pretransplant intensive care unit-bound and model for end-stage liver disease score were indicated as independent factors predictive of patient survival. Patients with neurologic abnormalities showed significant improvement after liver transplant.


CONCLUSIONS
Our results indicate LRLT is an excellent therapeutic modality for WD patients with end-stage liver disease. Better pretransplant conditions appeared to be advantageous in gaining better survival outcomes of patients undergoing LRLT.
Cheng
Feng
F
Key Laboratory of Living Donor Liver Transplantation, Ministry of Public Health, People's Republic of China.
Li
Guo-Qiang
GQ
Zhang
Feng
F
Li
Xiang-cheng
XC
Sun
Bei-cheng
BC
Kong
Lian-bao
LB
Pu
Li-yong
LY
Wang
Ke
K
Qian
Xiao-feng
XF
You
Wei
W
Wang
Xue-hao
XH
D000293:Adolescent; D000328:Adult; D002570:Ceruloplasmin / Q000378:metabolism; D002648:Child; D002681:China; D003300:Copper / Q000097:blood; D005260:Female; D006498:Hepatectomy / Q000379:methods; D006527:Hepatolenticular Degeneration / Q000601:surgery*; D006801:Humans; D016031:Liver Transplantation / Q000401:mortality / Q000502:physiology*; D019520:Living Donors; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016019:Survival Analysis; D017741:Survivors; D016896:Treatment Outcome
D003300:Copper; D002570:Ceruloplasmin