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Clinical features and insulin regulation in infants with a syndrome of prolonged neonatal hyperinsulinism

Francis M Hoe1, Paul S Thornton, Laura A Wanner

  • 1Divisions of Endocrinology and Neonatology, Department of Pediatrics, Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA.

The Journal of Pediatrics
|February 24, 2006
PubMed

Insights

This study defines a syndrome of prolonged neonatal hyperinsulinism in infants, characterized by spontaneous resolution and diazoxide responsiveness. Insulin regulation tests indicate normal function of key channels, distinguishing it from genetic forms.

Area of Science:

  • Pediatric Endocrinology
  • Neonatal Medicine
  • Metabolic Disorders

Background:

  • Neonatal hypoglycemia is a critical condition requiring precise diagnosis.
  • Prolonged hyperinsulinism presents unique clinical challenges in neonates.
  • Understanding the underlying mechanisms of neonatal hyperinsulinism is crucial for effective management.

Purpose of the Study:

  • To characterize clinical features of prolonged neonatal hyperinsulinism.
  • To assess insulin regulation in affected infants.
  • To differentiate this syndrome from genetic forms of hyperinsulinism.

Main Methods:

  • Retrospective data collection on 26 infants with resolved hypoglycemia due to hyperinsulinism.
  • Acute insulin response (AIR) testing using calcium, leucine, glucose, and tolbutamide in 11 neonates.
  • Comparison of AIR test results with genetic hyperinsulinism cohorts (KATP channel and GDH mutations).

Main Results:

  • A syndrome of prolonged neonatal hyperinsulinism was identified, predominantly in males and small-for-gestational-age infants.
  • Hyperinsulinism onset was median 13 days, resolving median 181 days; diazoxide effective in 19/21 treated infants.
  • AIR tests in affected neonates were similar to controls, differing from KATP channel and GDH genetic mutations.

Conclusions:

  • A distinct syndrome of prolonged, diazoxide-responsive, and spontaneously resolving neonatal hyperinsulinism is defined.
  • AIR tests suggest normal KATP channel and GDH function in this non-genetic hyperinsulinism.
  • This syndrome represents a significant subgroup of neonatal hyperinsulinism requiring specific clinical characterization.
Abstract

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