Author: ["Charlotte Kane","Ruth M. Shepherd","Paul E. Squires","Paul R.V. Johnson","Roger F.L. James","Peter J. Milla","Albert Aynsley-Green","Keith J. Lindley","Mark J. Dunne"]
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Abstract
Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is a disorder of childhood associated with inappropriate hypersecretion of insulin by the pancreas. The pathogenesis of the condition has hitherto remained controversial. We show here that insulinsecreting cells from a homogeneous group of five infants with PHHI lack ATP–sensitive K+ channel (KATP) activity. As a consequence, PHHI β–cells are spontaneously electrically active with high basal cytosolic Ca2+ concentrations due to Ca2+ influx. Our findings define the pathogenesis of this disease as a novel K+ channel disorder.
Cite this article
Kane, C., Shepherd, R., Squires, P. et al. Loss of functional KATP channels in pancreatic β–cells causes persistent hyperinsulinemic hypoglycemia of infancy. Nat Med 2, 1344–1347 (1996). https://doi.org/10.1038/nm1296-1344