Liver transplantation in Wilson disease: a single-center experience.

Abstract

Background: Wilson disease is a complex genetic disorder due to copper accumulation, mainly in the liver and brain. It is associated with severe liver disease, which is effectively cured by liver transplantation (LT). This study aimed to analyze the outcome of Wilson disease after LT from a single center in Iran.

Methods: In this study, we analyzed data from Wilson patients and donors who received LT from March 2018 to December 2022 at Shiraz University of Medical Sciences, Shiraz. Long-term follow-up and post-LT outcomes for both deceased donor LT (DDLT) and living donor LT (LDLT) were measured. The Kaplan-Meier survival analysis was used to test the survival.

Results: 106 recipients with LT (LDLT, n = 22; DDLT, n = 84) were included (mean age of adult and pediatric: 33.1 and 10.8 years respectively; male: 58% and female 42%). The average serum ceruloplasmin and urinary copper levels improved in most patients, with values of 15.6 mg/dL and 32.3 µg per 24 h, respectively. For pediatric patients with Wilson's disease, the survival rates at 6 months, 1 year, and 3 years were 97.0%, 96%, and 94.5%, and in adult patients achieved survival rates of 100%, 100%, and 75% at 6 months, 1 year, and 3 years, respectively.

Conclusions: LT is considered as a principal therapeutic option with good long-term results in Wilson patients, even in those presenting with hepatic failure. Neurologic manifestations have been improved post-LT; however, de novo neuropsychiatric symptoms begin in some cases after successful liver transplantation.