Living-related liver transplantation for Wilson's disease.
Orthotopic liver transplantation has been applied to the treatment of Wilson's disease (WD), living-related liver transplantation (LRLT) has also been indicated for WD with increasing frequency. Between January 2001 and November 2003, 22 LRLTs were performed on patients (19 pediatric, three adults) with WD in liver transplantation center. Two patients were transplanted because of a presentation coexistent with fulminant hepatic failure. Twenty presented with chronic advanced liver disease with (n = 9) or without (n = 11) associated neurologic manifestations. All the recipients had low serum ceruloplasmin levels with a mean value of 12.8 +/- 3.2 mg/dl before transplantation and increased to an average of 26.0 +/- 3.6 mg/dl after LRLT at the latest evaluation. The survival patients with neurologic manifestations such as tremor, dysarthia, dysphagia, dystonia and sialorrhea had improved after LRLT. This suggests that LRLT not only resolves the hepatic but also ameliorates the neurologic consequences of WD.
Wang
Xue-hao
XH
Liver Transplantation Center, First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu Province, China. docchengfeng@sohu.com
Cheng
Feng
F
Zhang
Feng
F
Li
Xiang C
XC
Kong
Lian B
LB
Li
Guo Q
GQ
Li
Jun
J
Qian
Xiao F
XF
D000293:Adolescent; D000328:Adult; D002648:Child; D003300:Copper / Q000378:metabolism; D005260:Female; D006085:Graft Survival; D006527:Hepatolenticular Degeneration / Q000378:metabolism / Q000401:mortality / Q000601:surgery*; D006801:Humans; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D019520:Living Donors; D008297:Male
D003300:Copper