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Updated: Jun 12, 2026

Hyperinsulinemic-euglycemic Clamps in Conscious, Unrestrained Mice

Hyperinsulinemic-euglycemic Clamps in Conscious, Unrestrained Mice

Published on: November 16, 2011

Congenital hyperinsulinism.

Jean-Baptiste Arnoux¹, Pascale de Lonlay, Maria-Joao Ribeiro

¹Hospital Necker-Enfants Malades, Paris, France.

Early Human Development

|June 17, 2010

Summary

This summary is machine-generated.

Congenital hyperinsulinism (CHI) causes dangerous hypoglycemia due to excess insulin. Early diagnosis and treatment, including surgery for focal forms, are crucial to prevent brain damage.

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Published on: January 4, 2018

Area of Science:

Pediatric Endocrinology
Medical Genetics
Surgical Gastroenterology

Background:

Congenital hyperinsulinism (CHI) is characterized by excessive insulin secretion from pancreatic islet beta cells, leading to recurrent hypoglycemia.
Key features include high glucose requirements to correct low blood sugar and responsiveness to glucagon.
While often isolated, CHI can be associated with genetic syndromes like Beckwith-Wiedemann and Sotos syndromes.

Purpose of the Study:

To outline the diagnostic and management strategies for congenital hyperinsulinism.
To differentiate between focal and diffuse forms of CHI and their respective treatment implications.
To highlight the importance of preventing neurological damage through glycemic control and timely intervention.

Main Methods:

Evaluation of HI severity based on glucose infusion rates and treatment responsiveness.
Diagnostic investigations including pancreatic (18)F-fluoro-L-DOPA PET-CT and molecular genetic analysis.
Assessment of treatment efficacy with oral diazoxide, somatostatin analogues, and calcium antagonists.

Main Results:

Neonatal onset CHI is typically severe, whereas late-onset and syndromic forms are generally responsive to medical management.
Focal CHI, characterized by focal adenomatous hyperplasia, can be cured by partial pancreatectomy.
Diffuse CHI resistant to medical therapy may necessitate subtotal pancreatectomy with unpredictable outcomes.

Conclusions:

Maintaining normal glycemia is paramount to prevent brain damage in CHI patients.
Screening for focal CHI is essential as surgical resection offers a definitive cure.
Understanding the genetic basis, including ABCC8/KCNJ11 mutations in focal and various genes in diffuse forms, guides management and genetic counseling.