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Hyperinsulinism in infants and children

C A Stanley1

  • 1Division of Endocrinology, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, USA.

Pediatric Clinics of North America
|April 1, 1997
PubMed
Summary

Hyperinsulinism, a common cause of infant hypoglycemia, stems from genetic defects or transient factors. Prompt, aggressive treatment combining medication and surgery is crucial to prevent brain damage.

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Area of Science:

  • Pediatrics
  • Endocrinology
  • Genetics

Background:

  • Hyperinsulinism is the leading cause of hypoglycemia in neonates.
  • Congenital hyperinsulinism arises from genetic defects affecting beta-cell regulation.
  • Transient neonatal hyperinsulinism can be linked to perinatal issues or maternal diabetes.

Purpose of the Study:

  • To summarize the causes and management of infantile hyperinsulinism.
  • To highlight the importance of timely intervention in preventing neurological sequelae.

Main Methods:

  • Review of genetic defects causing congenital hyperinsulinism (sulfonylurea receptor disorders, hyperinsulinism-hyperammonemia syndrome).
  • Identification of risk factors for transient neonatal hyperinsulinism (perinatal asphyxia, SGA, maternal diabetes).
  • Overview of treatment strategies including medical (diazoxide, octreotide) and surgical (subtotal pancreatectomy) options.

Main Results:

  • Genetic defects are a primary cause of persistent hyperinsulinism.
  • Transient forms are associated with specific perinatal conditions.
  • Effective management requires a multi-faceted approach.

Conclusions:

  • Infantile hyperinsulinism necessitates prompt and aggressive treatment.
  • A combination of medical and surgical interventions is often required.
  • Preventing hypoglycemia is key to avoiding long-term brain damage.

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