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。
Liver Transplantation in Treatment of Children with Glycogen Storage Disease by Using Liver Graft from Pediatric Cardiac Death
Objective: To sum the experience of liver transplantation from pediatric cardiac death donated (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 of severe viral encephalitis and secondary to cardiac death, implementation of organ donation; the recipient was a 4 years and 2 months old boy with a body weight of 15 kg and growth retardation, who suffered from glycogen storage disease, secondary to liver dysfunction, severe hypoglycemia. The surgical was classically orthotopically liver transplantation, the warm ischemia time was 6-min, the cold ischemia time was 180-min. T-tube and gastrojejunostomy fistula was placed during operation. The vital signs and transplanted liver function of the recipient were monitored after operation, and the recipient was given treatments of anti-infection, improving microcirculation and nutritional support. The recipient was treated with immunosuppression protocol of tacrolimus and prednisone to prevent rejection. Results: The operation time of the recipient was 420-min, the anhepatic phase was 55-min, and the blood loss was 400 ml. The recipient’s blood sugar became normal at 2-h, the endotracheal intubation was removed at 6-h, Enteral nutrition was given through gastrojejunostomy fistula at 24-h, the liver function returned to normal and the recipient began to get out of bed at 7 days, the recipient was discharged on the 18th day. The child grew fast postoperation after 1 month, and went to kindergarten after 6-month. The recipient has healthy survival of 18 months to present. Conclusion: The pediatric DCD liver is a better graft for pediatric liver transplantation, which could gain a good result of transplantation. Liver transplantation is a good way to treat a child with glycogen storage disease.
文章引用: 蔡秋程 , 杨 芳 , 吕立志 , 江 艺 (2014) 儿童心脏死亡捐肝移植治疗儿童肝糖原累积症。 亚洲儿科病例研究， 2， 13-19. doi: 10.12677/ACRP.2014.22004
 中华医学会器官移植学分会 (2010) 中国心脏死亡器官捐献工作指南. 中华器官移植杂志, 31, 436-437.
 Ausems, M.G., Verbiest, J., Hermans, M.P., et al. (1999) Frequency of glycogen storage disease type II in the Nether- lands: Implications for diagnosis and genetic counselling. European Journal of Human Genetics, 7, 713-716.
 Thomas, E.S., Anthony, J.D., Massimo, T., et al. (1993) Chimerism after liver transplantation for type IV glycogen storage disease and type I gaucher’s disease. The New England Journal of Medicine, 328, 745-749.
 Bartlett, A., Vara, R., Muiesan, P., Mariott, P., et al. (2010) A single center experience of donation after cardiac death liver transplantation in pediatric recipients. Pediatric Transplantation, 14, 388-392.
 Bellingham, J.M., Santhanakrishnan, C., Neidlinger, N., et al. (2011) Donation after cardiac death: A 29-year expe- rience. Surgery, 150, 692-702.