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Ketotic hypoglycaemia in children with diazoxide responsive hyperinsulinism of infancy

Khalid Hussain1

  • 1The Institute of Child Health, Unit of Biochemistry, Endocrinology and Metabolism, University College London, 30 Guilford Street, WC1N 1EH, London, UK. K.Hussain@ich.ucl.ac.uk

European Journal of Pediatrics
|March 18, 2005
PubMed

Insights

Recurrent hypoglycemia in infants treated with diazoxide may not indicate treatment failure. New cases show "ketotic" hypoglycemia, suggesting alternative mechanisms beyond diazoxide unresponsiveness.

Area of Science:

  • Pediatric Endocrinology
  • Metabolic Disorders
  • Pharmacology

Background:

  • Hyperinsulinism of infancy (HI) causes recurrent hypoglycemia, potentially leading to brain damage.
  • Diazoxide is the primary medical treatment, acting as a KATP channel agonist to inhibit insulin secretion.

Observation:

  • Two children on diazoxide therapy for persistent HI experienced recurrent "ketotic" hypoglycemia.
  • Biochemical analysis revealed appropriate insulin suppression and counter-regulatory hormone response.

Findings:

  • Despite diazoxide treatment, patients exhibited appropriate metabolic and hormonal responses during hypoglycemic episodes.
  • The observed
  • ketotic
  • hypoglycemia suggests mechanisms other than typical diazoxide unresponsiveness or non-compliance.

Implications:

  • Recurrent hypoglycemia in patients on diazoxide does not automatically imply treatment failure or non-compliance.
  • Further research is needed to elucidate the precise mechanisms underlying this novel presentation of hypoglycemia.
Abstract

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