[Endocrine pancreas function in mucoviscidosis]

Area of Science:

• Pediatric Endocrinology
• Metabolic Disorders
• Cystic Fibrosis Research

Context:

• Cystic Fibrosis (CF) patients have a tenfold higher risk of diabetes mellitus.
• Long-term pancreatic dysfunction in CF affects glucose metabolism.
• Understanding endocrine complications in pediatric CF is crucial.

Purpose:

• To investigate glucagon and insulin secretion in pediatric CF patients.
• To differentiate between alpha and beta cell function in CF-related diabetes.
• To characterize the pattern of insulin release in response to various stimuli.

Summary:

• CF patients demonstrate normal pancreatic alpha cell function and glucagon release.
• A specific defect in beta cell function was identified: delayed insulin release in response to glucose.
• Insulin output is diminished in response to both oral glucose and intravenous arginine, even with normal carbohydrate tolerance.

Impact:

• The findings reveal a distinct pattern of insulin secretion defect in CF, similar to chemical diabetes in adults.
• Reduced insulin secretion and potential peripheral insulin insensitivity contribute to hyperglycemia in CF.
• This research highlights the specific endocrine challenges in managing pediatric CF patients.