title: [Probing into indication of living-related liver transplantation for Wilson's disease].
abstract: OBJECTIVE
  To probe into indication of living-related liver transplantation (LRLT) for Wilson's Disease.
  
  
  METHODS
  From January 2001 to February 2007, thirty-seven living-related liver transplants were performed. A retrospective analysis was carried on outcome of those patients. The indications for LRLT were acute hepatic failure in 3 patients and chronic advanced liver disease in 32 patients including 13 patients with Wilsonian neurological manifestations. Two patients presented with severe Wilsonian neurological manifestations even though their liver functions were stable. According to the scoring system for evaluation of the neurological impairment in Wilson disease based on neurological signs and functions (total score was 30), the pre-transplantation score of those patients with neurological manifestations was 15.9 +/- 4.3 (n = 15).
  
  
  RESULTS
  Thirty-seven patients were followed up for 20 - 93 months. The survival rates of post-transplant patients and grafts at 1, 3, and 5 year were 91.9%, 83.8%, 75.7%, and 86.5%, 78.4%, 75.7%, respectively. Postoperative surgical complications occurred in 2 donors with bile leakage required drainage, in 2 recipients with hepatic thrombosis underwent retransplantation of cadaveric liver and in 1 recipient with hepatic stenosis required balloon dilatation. Neurological function was improved in all recipients and the score of posttransplantation at 6, 12, 18, 24, and 30 month was 17.5 +/- 3.7 (n = 13); 21.0 +/- 4.3 (n = 12); 23.9 +/- 3.9 (n = 10); 26.6 +/- 2.2 (n = 10) and 28.1 +/- 1.9 (n = 7) respectively.
  
  
  CONCLUSIONS
  Patients with acute hepatic failure or patients with severe liver disease unresponsive to chelation treatment should be treated with LRLT. Early transplantation in patients with an unsatisfactory response medical treatment may prevent irreversible neurological deterioration even though their liver function is stable.
authors: Liver Transplantation Center, First Hospital of Nanjing Medical University, Key Laboratory of Living Donor Liver Transplantation, Ministry of Public Health, Nanjing 210029, China.
authors: Feng
authors: F
authors: Cheng
authors: Xue-Hao
authors: XH
authors: Wang
authors: Feng
authors: F
authors: Zhang
authors: Xiang-Cheng
authors: XC
authors: Li
authors: Guo-Qiang
authors: GQ
authors: Li
authors: Bei-Cheng
authors: BC
authors: Sun
authors: Lian-Bao
authors: LB
authors: Kong
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000601:surgery*; D006801:Humans; D017093:Liver Failure / Q000209:etiology / Q000601:surgery; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D009422:Nervous System Diseases / Q000209:etiology; D012189:Retrospective Studies; D014184:Transplantation, Homologous; D016896:Treatment Outcome; D055815:Young Adult