Copper metabolism after living related liver transplantation for Wilson's disease

Abstract

Aim: Liver transplantation is indicated for Wilson's disease (WD) patients with the fulminant form and end-stage liver failure. The aim of this study was to review our experience with living-related liver transplantation (LRLT) for WD.

Methods: A retrospective review was made for WD undergoing LRLT at our hospital from January 2001 to February 2003.

Results: LRLT was carried out in 15 patients with WD, one of them had fulminant hepatic failure and the others had end-stage hepatic insufficiency. The mean age of the patients was 14.5 +/- 2.5 years (range 6 to 20 years). All the recipients had low serum ceruloplasmin levels with a mean value of 126.8 +/- 34.8 mg/L before transplantation. The serum ceruloplasmin levels increased to an average of 238.6 +/- 34.4 mg/L after LRLT at the latest evaluation, between 2 and 27 months after transplantation. A marked reduction in urinary copper excretion was observed in all the recipients after transplantation. Among the eight recipients with preoperative Kayser-Fleischer (K-F) rings, this abnormality resolved completely after LRLT in five patients and partially in three. All the recipients are alive and remain well, and none has developed signs of recurrent WD after a mean follow-up period of 15.4 +/- 9.3 months (range 2-27 months) except one who died of severe rejection. The donors were 14 mothers and 1 father. The serum ceruloplasmin levels were within normal limits in all the donors (mean: 220 +/- 22.4 mg/L). The mean donor age was 35.0 +/- 4.0 years (range, 30 to 45 years). Two donors had biliary leakage and required reoperation. Grafts were harvested as follows: four right lobe grafts without hepatic middle vein and eleven left lobe grafts with hepatic middle vein. The grafts were blood group-compatible in all recipients. Two patients had hepatic artery thrombosis and underwent retransplantation.

Conclusion: LRLT is a curative procedure in Wilson's disease manifested as fulminant hepatic failure and/or end-stage hepatic insufficiency. After liver transplantation, the serum ceruloplasmin level can increase to its normal range while urinary copper excretion decreases. Grafts chosen from heterozygote carriers do not appear to confer any risk of recurrence in recipients.