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.
Citation：
ZHANG Feng,CHENG Feng,WANG Xuehao,LI Xiangcheng,KONG Lianbao,LI Jun,LI Guoqiang,QIAN Xiaofeng,TANG Qiyun,XIA Yongxiang. Changes of Serum Ceruloplasmin and Urinary Copper Excretion in Wilson’s Disease after Liver-Related Liver Transplantation and Orthotopic Liver Transplantation. CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY, 2004, 11(2): 145-147. doi:
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- 1. Diaz J, Acosta F, Canizares F, et al. Does orthotopic liver transplantation normalize copper metabolism in patients with Wilson’s disease? [Ｊ]. Transplant Proc, 1995; 27(4)∶ 2306.
- 2. Sutcliffe RP, Maguire DD, Muiesan P,et al.Liver transplantation for Wilson’s disease: longterm results and qualityoflife assessment [Ｊ]. Transplantation, 2003; 75(7)∶1003.
- 3. 王学浩，张峰，李相成，等.活体部分肝移植12例次临床报告 [Ｊ]. 中华医学杂志， 2002; 82(5)∶435.
- 4. 王学浩，李相成，张峰，等.活体肝移植若干关键技术 [Ｊ]. 中华外科杂志， 2003; 44(1)∶13.
- 5. Asonuma K, Inomat Y, Kasahara M, et al. Living related liver transplantation from heterozygote genetic carriers to children with Wilson’s disease [Ｊ]. Pediatr Transplant, 1999; 3(3)∶201.
- 6. Komatsu H, Fujisawa T, Inui A,et al.Hepatic copper concentration in children undergoing living related liver transplantation due to Wilsonian fulminant hepatic failure [Ｊ]. Clin Transplant, 2002; 16(3)∶227.
- 7. Kobayashi S, Ochiai T, Hori S, et al.Copper metabolism after living donor liver transplantation for hepatic failure of Wilson’s disease from a gene mutated donor [Ｊ]. Hepatogastroenterology, 2001; 48(41)∶1259.
