Eighteen living related liver transplants for Wilson's disease: a single-center

Abstract

The aim of this study was to review our experience with living related liver transplantation (LRLT) for Wilson's disease. From January 2001 to July 2003 LRLT were performed in 18 patients with Wilson's disease, seven of whom had neurologic complications. The mean age of the patients was 13.5 +/- 2.3 years (range 6 to 20). Before transplantation all recipients displayed low serum ceruloplasmin levels (mean value = 118.4 +/- 32.6 mg/L). The serum ceruloplasmin levels increased to an average of 236.8 +/- 38.5 mg/L after LRLT at the latest evaluation, which ranged between 2 and 32 months after transplantation. A marked reduction in urinary copper excretion was observed in all recipients after transplantation. All recipients are alive and remain well. None have developed signs of recurrent Wilson's disease after a mean follow-up of 18.2 +/- 9.4 months (range 2 to 32 months). The donors were 17 mothers and one father of mean age 34.0 +/- 3.0 years (range 30 to 45 years). The serum ceruloplasmin levels were within normal limits in all donors (mean 226 +/- 27.8 mg/L). Two donors had biliary leakage and percutaneous drainage Grafts included four right lobes without the hepatic middle vein and 14 left lobe grafts with the middle vein. LRLT is a curative procedure for Wilson's disease presenting with fulminant hepatic failure and the others with end-stage hepatic insufficiency. After liver transplantation, the serum ceruloplasmin level increases to the normal range, urinary copper excretion decreases, and neurological manifestations improve to various extents.