[Living related liver transplantation for Wilson's disease: clinical study of 32 cases].
OBJECTIVE
To summarize the experience in donor selection and surgical outcomes of living related liver transplantation (LRLT) for Wilson's disease (WD).


METHODS
Thirty-two WD patients, 29 children and 3 being over 14, underwent LRLT because of chronic advanced liver disease (29 cases) or fulminant hepatic failure (3 cases) among which 13 cases showed neurological dysfunction. The donors, including 7 fathers and 25 mothers, had their serum ceruloplasmin levels within normal limit or slightly lower and their 24-hour urine copper < 100 microg. The modes of operation included orthotopic partial liver transplantation (31 cases) and orthotopic accessory partial liver transplantation (1 case). The patients were followed p for 3-63 months postoperatively.


RESULTS
Two patients die in the intraoperative period, and 2 died of bile leakage and severe rejection combined with infection. Twenty-eight patients survived, of which 2 had hepatic artery thrombosis and underwent retransplantation of cadaveric liver, one had anatomic stenosis 8 months after the original transplantation and underwent Roux-en-Y hepaticojejunostomy, 2 had chronic rejection 22 and 28 months after the LRLT respectively and were successfully rescued by switching their primary immunosuppressor from cyclosporine A to FK506. Eleven patients with neurological dysfunctions all showed long-term survival and their extrapyramidal signs and speech disorders, and dyskinesia all were alleviated.


CONCLUSIONS
LRLT is the treatment of choice for WD patients with chronic advanced liver disease and fulminant hepatic failure. After LRLT the disorders of the nervous system are alleviated.
Wang
Xue-hao
XH
Department of Liver Surgery, Liver Transplantation Center, First Hospital of Nanjing Medical University, Nanjing 210029, China.
Zhang
Feng
F
Li
Xiang-cheng
XC
Cheng
Feng
F
Li
Guo-qiang
GQ
Sun
Bei-cheng
BC
Kong
Lian-bao
LB
D000293:Adolescent; D002648:Child; D005500:Follow-Up Studies; D006085:Graft Survival; D006527:Hepatolenticular Degeneration / Q000601:surgery*; D006801:Humans; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D010290:Parents; D014184:Transplantation, Homologous