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Congenital Hyperinsulinism: An Historical Perspective.

Paul S Thornton1, Charles A Stanley2, Diva D De Leon2

  • 1Congenital Hyperinsulinism Center, Cook Children's Medical Center, Fort Worth, Texas, USA.

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|November 29, 2022
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Summary

Congenital hyperinsulinism causes persistent hypoglycemia in children. Advances in diagnosis and treatment, like 18F-DOPA PET scans, offer cures for focal forms, but nonfocal cases still need better options.

Keywords:
Beta cellsGlucoseHypoglycemiaInfantsInsulinPancreas

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Area of Science:

  • Pediatric Endocrinology
  • Metabolic Disorders
  • Genetics

Background:

  • Congenital hyperinsulinism (CHI) is a leading cause of persistent hypoglycemia in pediatric populations.
  • Historical advancements include insulin radioimmunoassay, diazoxide treatment, and provocative fasting challenges for diagnosis.
  • Molecular genetics has identified various genetic subtypes of CHI.

Purpose of the Study:

  • To review the historical progression and current state of congenital hyperinsulinism research.
  • To highlight key diagnostic and therapeutic milestones in managing pediatric hypoglycemia.
  • To underscore the ongoing challenges and future directions in CHI treatment.

Main Methods:

  • Literature review of historical case descriptions and scientific advancements.
  • Discussion of diagnostic tools such as insulin radioimmunoassay and 18F-DOPA PET.
  • Analysis of treatment evolution from diazoxide to emerging therapies.

Main Results:

  • Diazoxide remains the sole approved treatment, though its efficacy is limited in nonfocal cases.
  • 18F-DOPA PET enables precise localization of focal CHI, leading to surgical cures.
  • Despite progress, significant rates of neurological sequelae persist in affected children.

Conclusions:

  • Early diagnosis and effective treatment are crucial for preventing neurological complications in congenital hyperinsulinism.
  • While focal CHI has seen transformative treatment advances, nonfocal, diazoxide-unresponsive forms require novel therapeutic strategies.
  • Ongoing drug development offers hope for improved outcomes in the near future.