title: Living-related liver transplantation for Wilson's disease.
abstract: Liver transplantation with liver grafts from deceased donors is the treatment of choice for patients suffering from Wilson's disease (WD) with end-stage liver disease. There are few reports, however, on the use of liver grafts from living-related donors for WD. Five (two pediatric and three adult recipients) underwent living-related liver transplantation (LRLT) for WD at the University of Tokyo. Two patients presented with fulminant hepatic failure with hemolysis, and the other three presented with decompensating cirrhosis, one with an overlapping neurologic WD. All recipients had a low serum ceruloplasmin level (median: 18 mg/dL), high urinary copper level (mean: 1119 microg/d), and presented with Kayser-Fleischer rings before transplantation. Although one patient died from early graft thrombosis unrelated to WD, the other four patients have shown an excellent long-term prognosis. Following successful transplantation, there was a significant reduction in urinary copper excretion (median: 64 microg/d) in all patients. The neurologic symptoms of WD in one patient, however, worsened after 2 months and gradually subsided, but not completely, over the 2-yr follow-up. For advanced liver failure in WD, we consider LRLT a valuable life-saving option. The improvement of neurologic symptoms, however, requires further evaluation.
authors: Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, Japan.
authors: Sumihito
authors: S
authors: Tamura
authors: Yasuhiko
authors: Y
authors: Sugawara
authors: Yoji
authors: Y
authors: Kishi
authors: Nobuhisa
authors: N
authors: Akamatsu
authors: Junichi
authors: J
authors: Kaneko
authors: Masatoshi
authors: M
authors: Makuuchi
mesh_terms: D000293:Adolescent; D000328:Adult; D002570:Ceruloplasmin / Q000032:analysis; D002648:Child; D003300:Copper / Q000652:urine; D005260:Female; D006527:Hepatolenticular Degeneration / Q000601:surgery*; D006801:Humans; D008103:Liver Cirrhosis / Q000601:surgery; D017114:Liver Failure, Acute / Q000601:surgery; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D016896:Treatment Outcome
chemical_list: D003300:Copper; D002570:Ceruloplasmin