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  • Title: [Living related liver transplantation for Wilson's disease: clinical study of 32 cases].
    Author: Wang XH, Zhang F, Li XC, Cheng F, Li GQ, Sun BC, Kong LB.
    Journal: Zhonghua Yi Xue Za Zhi; 2006 Dec 12; 86(46):3290-3. PubMed ID: 17313814.
    Abstract:
    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. CONCLUSION: 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.
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