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目的分析二氮嗪试验性治疗先天性高胰岛素血症(CHI)的疗效。方法回顾性分析2006-2008年本院收治并经实验室检查诊断为CHI,并试用二氮嗪治疗的7例患儿(男5例;女2例;起病年龄2d～4个月;出生体质量2.65～4.60kg)的临床资料,并对其方法及疗效进行分析。结果 7例患儿确诊CHI后,均应用二氮嗪进行7～10d的试验性治疗。其中5例均为新生儿期以后起病,经治疗后,血糖逐渐恢复至正常水平,对二氮嗪治疗有效。2例分别于出生2d和出生1个月起病,治疗10d后,仍呈现严重而持续的低血糖症,对二氮嗪治疗无效。结论因部分CHI患儿的细胞具有功能正常的ATP敏感性钾通道,应用二氮嗪治疗有效,因此对确诊CHI的患儿应首先试用二氮嗪治疗。
Objective To analyze the curative effect of diazoxide in the treatment of congenital hyperinsulinemia (CHI). Methods Retrospective analysis of 7 cases (5 males; 2 females; onset age 2d ~ 4 months) born in our hospital from 2006 to 2008 and diagnosed as CHI by laboratory test and diazoxide test. Body weight 2.65 ~ 4.60kg) of clinical data, and its methods and efficacy analysis. Results After the diagnosis of CHI in 7 cases, diazoxide was used for experimental treatment of 7-10 days. Five of them were onset after neonatal period. After treatment, blood glucose gradually returned to normal level and was effective for diazoxide treatment. Two cases were onset at 2 days and 1 month after birth, respectively. After 10 days of treatment, severe and persistent hypoglycemia was still present, which was not effective for diazoxide treatment. Conclusion Because some children with CHI have normal-functioned ATP-sensitive potassium channel and diazoxide is effective, diazoxide should be the first trial in children with CHI.