Abstract
Hyperinsulinemic Hypoglycemia (HH) is the most common cause of permanent hypoglycemia, especially in the neonatal period. Childhood HH is mostly related to genes encoding proteins in the insulin secretion pathways, and may also be seen in syndromes such as Beckwidth Wiedemann, Kabuki, and Turner. The majority of congenital HH cases are the result of KATP channel gene defect. Most of these cases are unresponsive to diazoxide treatment. In this review, recent genetic studies and recent updates in treatment options in childhood HH are reviewed.
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