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Related Experiment Videos

Familial hyperinsulinism: successful conservative management.

Z Horev1, M Ipp, P Levey

  • 1Department of Pediatrics, University of Toronto, Mount Sinai Hospital, Canada.

The Journal of Pediatrics
|November 1, 1991
PubMed
Summary

Conservative therapy with diazoxide is recommended for persistent hyperinsulinemic hypoglycemia to avoid pancreatectomy and potential cognitive deficits. This approach allows for spontaneous remission and better long-term outcomes in affected children.

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Area of Science:

  • Pediatric Endocrinology
  • Metabolic Disorders
  • Genetics

Background:

  • Hyperinsulinemic hypoglycemia (HH) is a significant cause of persistent hypoglycemia in infants.
  • Genetic factors contribute to variable severity and long-term outcomes in affected families.
  • Surgical intervention, such as pancreatectomy, is often considered for severe cases.

Observation:

  • A large family with four affected children with hyperinsulinism of varying severity was studied.
  • The oldest child required subtotal pancreatectomy, while three younger siblings were managed with conservative diazoxide therapy.
  • Affected school-age children exhibited deficits in visuomotor integration and short-term memory.

Findings:

  • Prolonged conservative therapy with oral diazoxide successfully managed three younger children with hyperinsulinemic hypoglycemia.

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  • The oldest child, post-pancreatectomy, presented with low-average intelligence (IQ 80).
  • Three affected school-age children demonstrated cognitive deficits, unlike their unaffected siblings.

    • Implications:

    • Conservative management with diazoxide should be prioritized for persistent hyperinsulinemic hypoglycemia to prevent pancreatectomy.
    • Early and prolonged conservative treatment may mitigate the risk of cognitive impairments associated with hyperinsulinism.
    • Avoiding pancreatectomy in infants with persistent hyperinsulinemic hypoglycemia is crucial for preserving cognitive function and overall development.