儿童心脏死亡捐肝移植治疗儿童肝糖原累积症  

Liver Transplantation in Treatment of Children with Glycogen Storage Disease by Using Liver Graft from Pediatric Cardiac Death

作　　者：蔡秋程[1] 杨芳[1] 吕立志[1] 江艺[1] 

机构地区：[1]全军器官移植研究所,福州总医院肝胆病中心肝胆外科,福州

出　　处：《亚洲儿科病例研究》2014年第2期13-19,共7页Asian Case Reports in Pediatrics

基　　金：福建省自然科学基金重点项目资助,(资金编号:2011Y0046)。

摘　　要：目的:总结儿童心脏死亡捐肝治疗儿童肝糖原累积症的体会。方法:供者为男孩,3岁8个月,因严重病毒性脑炎致脑死亡继发心脏死亡行器官捐献;受者为男孩,4岁2个月,体重15 kg,生长发育迟缓,因肝糖原累积症继发肝功能不全、严重低血糖。手术采用经典原位肝移植术式,供肝热缺血时间6 min,冷缺血时间约180 min,术中留置T管和经胃空肠造瘘管。术后密切监测生命体征和移植肝功能,同时给予抗感染、改善微循环及营养支持等处理,采用他克莫司 + 甲泼尼龙片二联免疫抑制方案。结果:受者手术时间约420 min,其中无肝期约55 min,术中出血量400 ml。患儿术后2 h血糖恢复正常,6 h清醒并拔除气管插管,24 h后开始经胃空肠造瘘管进行肠内营养,术后7天下床,移植肝功能恢复正常,术后18天痊愈出院。术后1个月出现明显的生长追赶,术后6个月开始参加幼儿园学习,至今已健康生存18个月。结论:儿童心脏死亡供肝是儿童肝移植的供肝来源的理想选择,取得良好的移植效果;肝移植治疗儿童肝糖原累积症效果确切。Objective:To sum the experience of liver transplantation from pediatric cardiac deathdonated (DCD) in the treatment of children with glycogen storage disease.Methods: The donor was a 3 years and 8 months old boy, who was brain death ofsevere viral encephalitis and secondary to cardiac death, implementation oforgan donation;the recipient was a 4 years and 2 months old boy with a bodyweight of 15 kg and growth retardation, who suffered from glycogen storage disease,secondary to liver dysfunction, severe hypoglycemia. The surgical wasclassically orthotopically liver transplantation, the warm ischemia time was 6-min,the cold ischemia time was 180-min. T-tube and gastrojejunostomy fistula wasplaced during operation. The vital signs and transplanted liver function of therecipient were monitored after operation, and the recipient was giventreatments of anti-infection, improving microcirculation and nutritionalsupport. The recipient was treated with immunosuppression protocol oftacrolimus and prednisone to prevent rejection. Results: The operation time ofthe recipient was 420-min, the anhepatic phase was 55-min, and the blood losswas 400 ml. The recipient’s blood sugar became normal at 2-h, the endotrachealintubation was removed at 6-h, Enteral nutrition was given throughgastrojejunostomy fistula at 24-h, the liver function returned to normal andthe recipient began to get out of bed at 7 days, the recipient was dischargedon the 18th day. The child grew fast postoperation after 1 month, and went tokindergarten after 6-month. The recipient has healthy survival of 18 months topresent. Conclusion: The pediatric DCD liver is a better graft for pediatricliver transplantation, which could gain a good result of transplantation. Livertransplantation is a good way to treat a child with glycogen storage disease.

关 键 词：肝移植 糖原累积症 心脏死亡 器官捐赠 

分 类 号：R6[医药卫生—外科学]