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

AIM:Liver transplantation is indicated for Wilson’s disease(WD)patients with the fulminant form and end-stage liverfailure.The aim of this study was to review our experiencewith living-related liver transplantation(LRLT)for WD,METHODS:A retrospective review was made for WDundergoing LRLT at our hospital from January 2001 toFebuary 2003.RESULTS:LRLT was carried out in 15 patients with WD,one of them had fulminant hepatic failure and the othershad end-stage hepatic insufficiency.The mean age of thepatients was 14.5±2.5 years(range 6 to 20 years).All therecipients had low serum ceruloplasmin levels with a meanvalue of 126.8±34.8 mg/L before transplantation.The serumceruloplasmin levels increased to an average of 238.6±34.4mg/L after LRLT at the latest evaluation,between 2 and 27months after transplantation.A marked reduction in urinarycopper excretion was observed in all the recipients aftertransplantation.Among the eight recipients with preoperativeKayser-Fleischer(K-F)rings,this abnormality resolvedcompletely after LRLT in five patients and partially in three.All the recipients are alive and remain well,and none hasdeveloped signs of recurrent WD after a mean follow-upperiod of 15.4±9.3 months(range 2-27 months)except onewho died of severe rejection.The donors were 14 mothersand 1 father.The serum ceruloplasmin levels were withinnormal limits in all the donors(mean:220±22.4 mg/L).Themean 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 graftswithout hepatic middle vein and eleven left lobe grafts withhepatic middle vein.The grafts were blood group-compatiblein all recipents.Two patients had hepatic artery thrombosisand 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 ceruoplasmin 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.