Application Of Living Donor Liver Transplantation In The Treatment Of Hepatolenticular Degeneration

Objective: To summarize and discuss the therapy efficacy and surgical experience of LDLT in the treatment of WD in our hospital in order to guide clinical practice better.Methods: Between 2005.1 and 2008.2, we finished three LDLT to treat WD. The patients were all children, and one of them had neurological dysfunction. The donors, including two fathers and one mother, had their serum celuroplasmin levels within normal limit or slightly lower and their 24-hour urine copper<100μg. The methods of operation were orthotopic partial piggyback liver transplantation with left liver.Results: The three donors are still alive. Two of the recipient are still alive,one died in the intraoperative period due to AHF as a result of portal vein thrombosis and hepatic vein stenosis. The bilirubin rised up in one mixed type,major neurological manifestation patient after the operation, and declined after three balloon dilation. Patient with neurological dysfunctions shows long-term survival and her extrapyramidal signs and speech disorders and dyskinesia alleviats. There was no rejection.Conclusions: Living donor liver transplantation for hepatic complications of Wilson'sdisease can cure and correct the underlying metabolic defect. It is a life saving therapy inchildren with WD. Patient with neurological manifestation can be alleviated after operation.