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Updated: Jul 8, 2025

Hyperinsulinemic-euglycemic Clamps in Conscious, Unrestrained Mice
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Hyperinsulinemic-euglycemic Clamps in Conscious, Unrestrained Mice

Published on: November 16, 2011

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Congenital hyperinsulinism

Christoffer Drabløs Velde1, Hallvard Reigstad2, Erling Tjora3

  • 1Klinisk institutt 1, Universitetet i Bergen.

Tidsskrift for Den Norske Laegeforening : Tidsskrift for Praktisk Medicin, Ny Raekke
|December 13, 2023
PubMed

Insights

Congenital hyperinsulinism, a rare genetic disorder, causes persistent neonatal hypoglycemia due to excessive insulin. Early diagnosis and treatment are vital to prevent severe neurological damage and ensure better outcomes for affected infants.

Area of Science:

  • Pediatrics
  • Endocrinology
  • Genetics

Background:

  • Congenital hyperinsulinism is the most frequent cause of persistent hypoglycemia in neonates.
  • It is a rare monogenic disorder resulting from mutations in genes controlling insulin secretion from pancreatic beta cells.
  • Uncontrolled insulin secretion leads to detrimental effects on the central nervous system, potentially causing brain damage or death.

Purpose of the Study:

  • To provide clinicians managing pediatric and neonatal patients with an overview of congenital hyperinsulinism.
  • To cover the diagnostic and therapeutic strategies for this condition.

Main Methods:

  • This is a clinical review.
  • It synthesizes current knowledge on diagnosis and treatment.

Main Results:

  • The review details the pathophysiology of congenital hyperinsulinism, emphasizing the role of genetic mutations and insulin's effects.
  • It highlights the critical impact of low glucose and ketone bodies on the central nervous system.

Conclusions:

  • Prompt diagnosis and intervention are essential for improving the prognosis of congenital hyperinsulinism.
  • Understanding the genetic basis and clinical presentation is key for effective management.

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