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Neonatal hyperinsulinemic hypoglycemia. Two case reports.

M Zaffanello1, G Zamboni, C Maffeis

  • 1Regional Centre for Neonatal Congenital Errors of Metabolism, University of Verona, Verona, Italy.

Minerva Pediatrica
|July 20, 2002
PubMed
Summary
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Prompt diagnosis and treatment of neonatal hyperinsulinemic hypoglycemia are crucial for preventing neurological damage. Early identification and intervention can lead to positive outcomes, as illustrated by two case studies.

Area of Science:

  • Neonatology
  • Pediatric Endocrinology

Background:

  • Neonatal hyperinsulinemic hypoglycemia requires prompt diagnosis and treatment to prevent neurological sequelae.
  • Understanding the diverse clinical presentations and underlying causes is essential for effective management.

Observation:

  • Case 1 presented with birth asphyxia, transient hypoglycemia, and hyperinsulinism, resolving within 8 days.
  • Case 2 exhibited persistent hypoglycemia, hyperinsulinism, and severe neurological damage despite initial diagnosis of cerebral injury.

Findings:

  • Hyperinsulinism can manifest differently, with some neonates experiencing transient forms and others developing severe neurological deficits.
  • Diazoxide treatment proved effective in managing hyperinsulinism and achieving euglycemia in one case.
  • Genetic testing excluded glutamate dehydrogenase deficiency and specific channelopathies (SUR1, KIR6.3) in the treated patient.

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Implications:

  • Timely diagnosis and intervention for neonatal hyperinsulinemic hypoglycemia are critical to avert long-term neurological impairment.
  • Persistent hypoglycemic or seizure-like episodes in newborns warrant thorough investigation for underlying hyperinsulinism.
  • This highlights the importance of considering hyperinsulinism in neonates with unexplained hypoglycemia and neurological symptoms.