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Diazoxide-resistant focal hyperinsulinism

ORPHA:79298

Definition

A form of congenital diazoxide-resistant hyperinsulinism characterized by recurrent episodes of profound hypoglycemia caused by an excessive/uncontrolled insulin secretion (inappropriate for the level of glycemia) due to a focal adenomatous hyperplasia of pancreas, that is unresponsive to medical treatment with diazoxide.

Prevalence
Unknown
Age of onset
All ages