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An experimental approach based on genetic studies has offered new insights into the pathophysiology of persistent hyperinsulinemia of infancy. This review highlights these advances, with special emphasis on the forms of hyperinsulinemia that are associated with defects of the adenosine triphosphate (ATP)-dependent potassium (KATP) channel, a key regulator of insulin secretion in the pancreatic islets.

Persistent hyperinsulinemia of infancy has been known as nesidioblastosis, persistent hyperinsulinemic hypoglycemia of infancy, and familial hyperinsulinism since its initial recognition as a clinical entity in the 1950s. More prevalent forms of hypoglycemia that are transitory and occur in the immediate newborn period must be differentiated from persistent hyperinsulinemia of infancy, which is defined by inappropriate insulin secretion in the presence of profound hypoglycemia. The characteristic associated clinical findings (Table 1), including inappropriate glucose-to-insulin ratios, suppression of ketone body production, glucose utilization rates...

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