标题:
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, 杨芳, 吕立志, 江艺
关键字:
肝移植, 糖原累积症, 心脏死亡, 器官捐赠Liver Transplantation, Glycogen Storage Disease, Cardiac Death, Organ Donation
期刊名称:
《Asian Case Reports in Pediatrics》, Vol.2 No.2, 2014-05-02
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 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.