| 4 | 因K_(ATP)通道突变所致的无亮氨酸敏感性高胰岛素血症患儿口服蛋白质可诱导低血糖症显示文摘Objective:Because children with congenital hyperinsulinism(HI) caused by recessive loss of function mutations in the adenosine triphosphate (ATP)-dependent potassium channel(KATP-HI) are not leucine sensitive,we evaluated for protein induced hypoglycemia with oral protein tolerance tests. Study design:Blood glucose and insulin concentrations were measure devery 15 minutes for 3 hours after an oral protein loadin children with KATP-HI (n=11) and compared with those of children with glutamate dehydrogenase HI (n=12) and control subjects (n=12). Results:Similar to children with glutamate dehydrogenase HI,patients with KATP-HI displayed protein-induced hypoglycemia (10/11)with blood glucose concentrations declining by 17 to 69 mg/dL. In contrast,oral proteinhad little effect on blood glucose concentrations in control subjects.Conclusions:Protein induced hypoglycemia is a feature of KATP-HI,despite the absence of leucine sensitivity. The results indicate that amino acids can stimulate insulin secretionvia a glutamate dehydrogenase and KATP channel-independent pathway. | Fourtner S.H. Stanley C.A. Kelly A. 张振 | 2006 | 世界核心医学期刊文摘(儿科学分册)2006，0，12： | 1 |
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