迟发型鸟氨酸氨甲酰转移酶缺乏症并发肝性脑病急性期患儿的护理

Nursing care of a child with acute hepatic encephalopathy caused by late-onset ornithine carbamoyltransferase deficiency

总结1例迟发型鸟氨酸氨甲酰转移酶缺乏症并发肝性脑病急性期患儿的护理体会。针对患儿持续高氨血症经药物治疗无效、严重中枢神经系统损害、营养不良风险高等问题,紧急行连续血液净化联合氨清除剂经旁路代谢方式降血氨,严密监测过量氨蓄积导致的肝性脑病,做好预警与急救;根据血氨值动态调整营养策略,尤其是蛋白质与氨基酸的摄入;给予患儿与家长心理支持,增强战胜疾病信心。经过28 d的治疗及护理,患儿病情稳定出院,1个月后随访状况良好。

To summarize the nursing experience of a child with acute hepatic encephalopathy caused by lateonset ornithine carbamoyltransferase deficiency.In view of the ineffectiveness of drug treatment in the child with persistent hyperammonemia,serious central nervous system damage,and high nutritional risk,emergency continuous blood purification combined with ammonia scavenger through bypass metabolism to reduce blood ammonia,closed monitoring of encephalopathy caused by excess ammonia accumulation,early warning and first aid of encephalopathy should be done for the patient;nutritional strategies were dynamically adjusted according to blood ammonia levels,especially protein and amino acid intake,and psychological support for child and parents was provided to enhance confidence to overcome the disease.After 28 days of treatment and nursing,the child was discharged in stable condition,and the condition was good during one month of follow-up.