The hyperinsulinaemic hypoglycaemias in infancy: a study of six cases

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Hyperinsulinemic-euglycemic Clamps in Conscious, Unrestrained Mice

Published on: November 16, 2011

P Amendt1, K D Kohnert, J Kunz

  • 1Kinderklinik, Humboldt Universität, Berlin, German Democratic Republic.

European Journal of Pediatrics
|November 1, 1988
PubMed

Insights

Functional tests struggle to differentiate causes of hyperinsulinaemic hypoglycaemia in infants. The blood glucose/serum insulin ratio is key for diagnosing hyperinsulinism, but suppression tests don't distinguish adenoma from nesidioblastosis.

Area of Science:

  • Pediatric Endocrinology
  • Metabolic Disorders
  • Histopathology

Background:

  • Persistent hyperinsulinaemic hypoglycaemia is a critical condition in infants.
  • Distinguishing between islet cell adenoma and pancreatic nesidioblastosis is crucial for treatment.
  • Existing functional tests require careful evaluation for diagnostic accuracy.

Purpose of the Study:

  • To assess the efficacy of various functional tests in differentiating causes of hyperinsulinaemic hypoglycaemia.
  • To analyze pathophysiological and histological findings in affected infants.
  • To determine the diagnostic value of the blood glucose/serum insulin ratio.

Main Methods:

  • Evaluation of six infants (2-7 months) with persistent hyperinsulinaemic hypoglycaemia.
  • Measurement of blood glucose, immunoreactive insulin, and C-peptide under basal, stimulation, and suppression conditions.
  • Performance of oral glucose tolerance, diazoxide infusion, somatostatin infusion, and C-peptide suppression tests.

Main Results:

  • Islet cell adenoma identified in four infants; pancreatic nesidioblastosis in two.
  • The blood glucose/serum insulin ratio was the primary diagnostic criterion for hyperinsulinaemia (patients: 0.3-5.1 vs. controls: 4.1-13.3).
  • At least four low ratio determinations (<2.6) were needed for diagnosis.

Conclusions:

  • The blood glucose/serum insulin ratio is a critical diagnostic marker for hyperinsulinaemic hypoglycaemia.
  • Functional tests, particularly suppression tests, do not reliably differentiate between adenoma and nesidioblastosis.
  • Further refinement of diagnostic strategies for hyperinsulinaemic hypoglycaemia is warranted.

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