摘要目的 观察胆道闭锁患儿肝移植术前、术后心理运动行为发展状况,分析肝移植术对患儿神经认知功能的影响.方法 收集2016年1月到2016年12月择期行亲属活体肝移植术的胆道闭锁患儿50例,年龄5～12个月,男女不限,于术前1d采用贝利婴幼儿发展量表(Bayley Scale of Infant Development-BSID)测量评估患儿术前的心理运动行为发展状况,计算智力发展指数(Mental Development Index,MDI)及运动发展指数(Psychomotor Development Index,PDI),记录患儿术前年龄、体重及平均血氨、胆红素、白蛋白、白细胞水平,分析其与术前神经认知功能的关系.分别于术后2周、3周、1个月采用同样方法测量其术后的发展指数,分析患儿术前术后神经认知变化情况.并统计分析术后胆道并发症、二次手术及肺炎感染等各并发症对术后1个月发展指数的影响.结果 50例婴幼儿术前心理运动发育均低于标准水平,平均智力发展指数MDI及运动发展指数PDI均处于中下水平;术前平均血氨、胆红素和白蛋白水平与发展指数相关性分析比较,差异具有统计学意义(P＜0.05),血氨、胆红素与发展指数呈负相关性,白蛋白水平与发展指数呈正相关性;与术前1d比较,术后2周患儿MDI、PDI均有所下降,术后3周MDI上升,PDI与术前无明显变化,术后1个月MDI、PDI较术前均明显上升;与术后2周比较,术后3周与1个月MDI、PDI均明显上升.术后持续存在的肺炎感染可影响患儿的MDI,胆道并发症可影响患儿的PDI(P＜0.05).结论 胆道闭锁患儿术前心理运动行为发展普遍处于中下水平,患儿术前血氨、胆红素、白蛋白与之密切相关;肝移植可明显改善患儿的神经认知障碍.

abstractsObjective To observe the mental and motor development of children with biliary atresia before and after liver transplantation,and analyze the effect of liver transplantation on neurocognitive functions.Methods Fifty children with biliary atresia who experienced living related liver transplantation were selected from Jan.2016 to Dec.2016 in our hospital,with an average age of 5 to 12 months regardless of gender.All the children were assessed by Bayley Scale of Infant Development (BSID) one day before surgery,to obtain the mental and motor development conditions.The results were indicated with Mental Development Index (MDI) and Psychomotor Development Index (PDI).The levels of preoperative ammonia,bilirubin,albumin and white blood cells were recorded,and their relationship with preoperative neurocognitive development was analyzed.The development indexes were obtained at 2nd week,3rd week and 1st month after surgery by the same method,and the neurocognitive changes were analyzed in children before and after the surgery.The effects of postoperative biliary complications,secondary operations,pneumonia infection and other complications on the postoperative development indexes one month after surgery were assessed.Results The preoperative mental and motor development was both below the standard level in 50 infants with average MDI and PDI in middle and lower state.The correlation analysis among the levels of preoperative blood ammonia,bilirubin and albumin showed statistically significant on development index (P ＜ 0.05).The levels of blood ammonia and bilirubin were negatively correlated with development index,while albumin levels showed a positive correlation.As compared with one day before liver transplantation,MDI and PDI were decreased at 2nd week after operation;the MDI increased,and PDI had no significant change at 3rd week after operation.MDI and PDI were significantly higher at 1 st month after operation than preoperation.As compared with 2nd week after operation,PDI and MDI were significantly increased at 3rd week and 1st month after operation.Postoperative persistent pneumonia infection could affect children's MDI,and biliary complications could affect PDI (P＜0.05).Concision The mental and motor development conditions of children with biliary atresia were in medium/low level,and the levels of preoperative blood ammonia,bilirubin and albumin may have significant influence.Liver transplantation may obviously improve the patient's neurocognitive function.