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.
【Abstract】Objective To investigate the result of liver transplantation for end stage liver disease. Methods A retrospective analysis was made for 7 cases orthotopic liver transplantation(OLT) and 4 cases living related liver transplantation (5 patients with hepatitis B cirrhosis and 6 with Wilson’s disease),cirrhosis group was treated with lamivudine plus low dose anti-HBV-Ig. Results Ten patients were completely recovered discharged(including 4 cases LRLT) and only 1 died of ARDS.The complications after operation were: 2 cases of abdominal hemorrhage,3 cases of acute respiratory distress syndrome; and 4 cases of hepatitis B cirrhosis were HBV-DNA(-) after operation.Copperoxidase in all with Wilson’s disease became normal. Conclusion Liver transplantation is effective measure for end stage liver disease and living related liver transplantation is suitable for the present medical condition in China.Surgical technique is crucial for reducing perioperative complications.
目的 探讨脾动脉栓塞治疗亲体肝移植术后脾动脉窃血所致小肝综合征的疗效。方法 我院2007年4月至2009年9月期间共完成亲体肝移植25例，其中有2例发生脾动脉窃血致小肝综合征，均采用介入技术经皮行脾动脉栓塞治疗。结果 行脾动脉栓塞术后，2例患者腹水逐渐减少，转氨酶水平、血小板及白蛋白水平及肝功能较快恢复正常。栓塞术后1个月复查，肝功能仍基本正常。结论 脾动脉栓塞是治疗肝移植术后脾动脉窃血所致小肝综合征的有效措施。
Objective To analyze the surgical techniques for the procurement and back-table surgery of the graft in living-related small bowel transplantation. Methods Eligible donor was chosen according to the donor selection criteria of living-related small bowel transplantation, and preoperative plan was designed. A segment of ileum of 120 cm was procured 20 cm proximal to the ileocecal valve which was preserved in the donor. The techniques for the procurement and back-table surgery of the graft were summarized, which included measurement of entire small bowel length from Treitz to ileocecal valve, palpation and transillumination to identify the distal branch of the superior mesenteric artery， and transient blockage of isolated blood vessels with vascular clamps in order to observe the influence on the blood circulation of graft and residual ileum. The detailed manipulation techniques in processing the graft blood vessels were discussed. Results The operations were successful both on the donor and the recipient. The functions of implanted segment of bowel were well. The donor had no other complications, such as mesenteric thrombi and anastomosis leakage of intestine, except for transient moderate diarrhea. She was discharged 14 days after operation. In the next 8 months of following-up, the donor has not experienced significant alteration in bowel habits or weight loss. Now she is in good appetite, without any changes in the habit and amount on diet. No changes have been found in lifestyle, work habits, or psychosocial conditions after the small bowel donation. Conclusion The procurement of a segment of ileum as graft and preservation of 20 cm proximal to the donor ileocecal valve may be ideal protocol. Using a standardized technique with attention presents little recent or long-term risks for the donor and brings satisfied effect for the recipient.
目的总结1例亲体部分肝移植的临床体会。方法受者为5.5岁男童，诊断为先天性肝脏巨大肿瘤; 供者为患儿母亲，34岁; 切取供者肝脏的左叶并原位移植给患儿。术后给予抗感染、抗免疫排斥反应等支持治疗。结果患儿手术历时9 h，失血250 ml; 供者手术历时3 h，失血450 ml。术后供、受者均健康存活，肝功能正常。结论活体肝部分移植技术是可行的，良好的手术及术后处理是确保肝移植手术成功的关键。