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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.
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 measured for 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. 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 that that amino acids can stimulate insul in secretionvia a glutamate dehydrogenase and KATP channel-independent pathway.