OBJECTIVE: To investigate living donor liver transplantation for Wilson disease with neurologic features. METHODS: From Jan 2001 to Mar 2003, fifteen cases of living donor liver transplantation were performed for Wilson Disease (WD), five of those were complicated with neurologic features. A retrospective analysis was given for cooper metabolism and neurologic features. RESULTS: All operation were living related liver transplantation and donors were mothers. Four left lobes with hepatic middle vein and one right lobe without hepatic middle vein were harvested from donors, and graft volume to recipient body weight ratio was 0.79 approximately 1.08. One patient occurred hepatic artery thrombosis and performed retransplantation later, the other recipients recovered satisfactorily. All patients showed Extrapyramidal sign and three patients companying with language handicap and dyskinesia alleviated postoperation follow-up between 2 and 16 months. All recipients are alive and remain well, and none have developed signs of recurrent WD. CONCLUSION: Living donor liver transplantation is effective treatment for WD complicated with nervous system symptom, ceruloplasmin is normal and Kayser-Fleischer ring and nervous system symptom are to various extents.
OBJECTIVE: To investigate living donor liver transplantation for Wilson disease with neurologic features. METHODS: From Jan 2001 to Mar 2003, fifteen cases of living donor liver transplantation were performed for Wilson Disease (WD), five of those were complicated with neurologic features. A retrospective analysis was given for cooper metabolism and neurologic features. RESULTS: All operation were living related liver transplantation and donors were mothers. Four left lobes with hepatic middle vein and one right lobe without hepatic middle vein were harvested from donors, and graft volume to recipient body weight ratio was 0.79 approximately 1.08. One patient occurred hepatic artery thrombosis and performed retransplantation later, the other recipients recovered satisfactorily. All patients showed Extrapyramidal sign and three patients companying with language handicap and dyskinesia alleviated postoperation follow-up between 2 and 16 months. All recipients are alive and remain well, and none have developed signs of recurrent WD. CONCLUSION: Living donor liver transplantation is effective treatment for WD complicated with nervous system symptom, ceruloplasmin is normal and Kayser-Fleischer ring and nervous system symptom are to various extents.