Clinical effect of liver transplantation in the treatment of hepatolenticular degeneration

Objective To investigate the clinical effect of liver transplantation in the treatment of hepatolenticular degeneration ( HLD) and the prognosis of HLD patients. Methods A retrospective analysis was performed for the clinical data of 15 HLD patients who underwent liver transplantation in Changzheng Hospital of Second Military Medical University from June 2003 to January 2017. Friedman test of K related samples test was used for comparison of data between groups, and Wilcoxon one-sample test of 2 related samples test was used for comparison of data between two groups. Results Of all patients, 13 underwent standard orthotopic liver transplantation, and 2 underwent modified piggyback liver transplantation. One patient died of multiple organ failure during the perioperative period and 2 were lost to follow-up at half a year after surgery. Of all patients, one lived for 14 months, one lived for 5 years and 8 months, and the other ten patients were still alive up to now. Six patients suffered from acute rejection reaction ( AR) , among whom four were improved after conventional hormone pulse therapy, one was improved and discharged after antiviral therapy with ganciclovir for cytomegalovirus infection, and the other one patient was improved and discharged after the second liver transplantation at 5 months after surgery due to poor response to hormone pulse therapy and chronic rejection reaction. Ten patients had varying degrees of improvement in positive Kayser-Fleischer ring, and three patients achieved varying degrees of improvement in severe neuropsychiatric symptoms and had a basically normal neuropsychiatric state at half a year to one year after surgery.One patient died in the perioperative period, and the other 14 patients had a significant improvement in liver function at 1 month after surgery, and the level of ceruloplasmin gradually increased to a normal level. Conclusion Orthotopic liver transplantation can effectively improve copper metabolism, liver function, and neuropsychiatric symptoms in HLD patients and increase their quality of life and long-term survival rates.