Protein-sensitive hypoglycemia without leucine sensitivity in hyperinsulinism caused by KATP channel mutations - 10/08/11
Reprint requests: Dr Andrea Kelly, Children’s Hospital of Philadelphia, Pediatrics, 8416 Main, Philadelphia, PA 19104.Résumé |
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 every 15 minutes for 3 hours after an oral protein load in 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 protein had 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 secretion via a glutamate dehydrogenase- and KATP channel-independent pathway.
Le texte complet de cet article est disponible en PDF.Abbreviations : ATP, BG, GDH, HI, KATP, Kir6.2, OPTT, SUR1
Plan
| Supported in part by a grant from the Lawson Wilkins Pediatric Endocrine Society (A.K.), and grants from the National Institutes of Health (RO1 DK 56268 [C.A.S.], K-12 DK 06368 [C.A.S. and A.K.], and GCRC MOI-RR002400. |
Vol 149 - N° 1
P. 47-52 - juillet 2006 Retour au numéro
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