Wilson’s病患者亲体部分肝移植和全肝移植术后血清铜蓝蛋白及尿铜水平的变化

Changes of Serum Ceruloplasmin and Urinary Copper Excretion in Wilson’s Disease after Liver-Related Liver Transplantation and Orthotopic Liver Transplantation

目的 总结Wilson’s病患者亲体肝移植和全肝移植术后血清铜蓝蛋白及尿铜水平的恢复情况。方法 自 2 0 0 0年 9月至 2 0 0 3年 11月我院为 2 6例Wilson’s病患者施行了肝移植术 ,均并发终末期肝硬变 ,其中 3例发生急性肝功能衰竭。术前血清铜蓝蛋白和尿铜水平分别为 (12 4 .8± 2 2 .8)mg/L和 (15 2 4 .8± 32 8.6 ) μg/ 2 4h ,其中行活体部分肝移植 2 2例 ,全肝移植 4例 ,亲体肝移植供体术前血清铜蓝蛋白水平为 (2 30 .4± 2 9.6 )mg/L ,尿铜水平均 <5 0μg/ 2 4h。结果 所有患者手术顺利 ,全肝移植患者术后 1、3、6及 12个月血清铜蓝蛋白和尿铜水平分别为 (32 0 .2±36 .8)mg/L、(380 .4± 4 5 .6 )mg/L、(36 0 .5± 37.6 )mg/L、(35 6 .2± 2 7.6 )mg/L和 (2 4 0 .4± 2 2 .8) μg/ 2 4h、(86 .5± 10 .6 ) μg/ 2 4h、(5 4 .2± 6 .8) μg/ 2 4h及 (46 .8± 3.4 ) μg/ 2 4h ;亲体肝移植患者术后 1、3、6及 12个月血清铜蓝蛋白和尿铜水平分别为 (2 16 .8± 2 0 .4 )mg/L、(2 4 8.5± 32 .6 )mg/L、(2 85 .4± 4 4 .3)mg/L、(2 6 0 .2± 36 .6 )mg/L和(380 .8± 37.6 ) μg/ 2 4h、(15 0 .6± 2 4 .5 ) μg/ 2 4h、(75 .5± 9.6 ) μg/ 2 4h及 (6 0 .3± 5 .8) μg/ 2 4h。

Objective To summarize the changes of serum ceruloplasmin levels and urinary copper excretion in Wilson’s disease (WD) after living-related liver transplantation(LRLT) and orthotopic liver transplantation(OLT). Methods From September 2000 to November 2003, 140 cases liver transplantation were performed in our Liver Transplantation Center, LRLT and OLT were carried out in 26 patients with WD, three of them had fulminant hepatic failure and the others had end-stage hepatic insufficiency. All the recipients had low serum ceruloplasmin levels 〔(124.8±22.8) mg/L〕 and high urinary copper excretion 〔(1 524.8±328.6) μg/24h〕 before transplantation. The serum ceruloplasmin levels and urinary copper excretion were within normal limits in 22 donors 〔(230.4±29.6) mg/L〕 and <50 μg/24h〕. Results All recipients recovered satisfactorily. After operation 1, 3, 6,12 months, in OLT group, serum ceruloplasmin level and urinary copper excretion were (320.2±36.8) mg/L, (380.4±45.6) mg/L, (360.5±37.6) mg/L, (356.2±27.6) mg/L and (240.4±22.8) μg/24h, (86.5±10.6) μg/24h, (54.2±6.8) μg/24h, (46.8± 3.4) μg/24h; While in LRLT group, serum ceruloplasmin levels and urinary copper excretion were (216.8±20.4) mg/L, (248.5±32.6) mg/L, (285.4±44.3) mg/L, (260.2±36.6) mg/L and (380.8±37.6) μg/24h, (150.6±24.5) μg/24h, (75.5±9.6) μg/24h, (60.3±5.8) μg/24h. Conclusion OLT and LRLT are curative procedure in WD 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.