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.
ObjectiveTo evaluate and summarize the perioperative management experience of living related liver transplantation (LRLT). MethodsA retrospective analysis was made in perioperative management of 13 cases undergoing LRLT (15 times operation, including 2 cases of liver retransplantation) in our department from January 2001 to December 2002. ResultsAll the operations were successful. All the 13 donors were followed up regularly, 12 donors were uneventfully recovered after operation and 1 donor suffered from bile leakage due to T tube. The survival rate of recipients, who achieved longterm survival at 2 months to 2 years, was 92.3%(12/13); the survival rate of graft was 86.7%(13/15). One adult recipient with Wilson’s disease died of serious rejection on the 72nd day postoperatively. Two cases suffered from embolism of hepatic artery, one case received reduced size cadaveric liver retransplantation, the other case received liver retransplantation from cadaver, and they both achieved longterm survival after retransplantation. The other complications included: 1 case of serious rejection, 2 cases of ARDS, 6 cases of infection of microbe, 7 cases of serious hydrothorax, 1 cases of leakage of biliary tract, and so on. ConclusionPerfect preoperative management, which composes one of the key parts of LRLT, is critically important for both donor and recipient.