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Glucose intolerance in children with cystic fibrosis

Melinda P Solomon1, David C Wilson, Mary Corey

  • 1Departments of Genetics and Population Health Sciences, The Hospital for Sick Children, University of Toronto, Ontario, Canada.

The Journal of Pediatrics
|February 14, 2003
PubMed

Insights

Screening adolescent cystic fibrosis (CF) patients with pancreatic insufficiency for glucose intolerance is recommended. This approach identified more cases than previously suspected, highlighting the need for routine testing in this population.

Area of Science:

  • Endocrinology
  • Genetics
  • Pulmonology

Background:

  • Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
  • Glucose intolerance and CF-related diabetes are known complications.
  • The relationship between CF genotype, pancreatic status, and glucose metabolism requires further investigation.

Purpose of the Study:

  • To investigate the associations between glucose intolerance, CF transmembrane conductance regulator (CFTR) genotype, and exocrine pancreatic function in pediatric CF patients.
  • To determine the prevalence of abnormal glucose tolerance in asymptomatic adolescents with CF.

Main Methods:

  • Retrospective analysis of data from 335 pediatric CF patients.
  • Modified oral glucose tolerance tests (OGTT) were administered to 94 asymptomatic patients aged 10-18 years without diagnosed CF-related diabetes.
  • CFTR mutation analysis and assessment of exocrine pancreatic status were performed for all participants.

Main Results:

  • Among 94 tested adolescents, 17% had impaired glucose tolerance and 4.3% had CF-related diabetes, despite normal fasting glucose levels.
  • Abnormal glucose tolerance was significantly associated with severe CFTR mutations (classes I-III) and exocrine pancreatic insufficiency.
  • No correlation was found between glycosylated hemoglobin (HbA1c) levels and glucose tolerance test results.

Conclusions:

  • Routine screening for glucose intolerance using OGTT in pancreatic-insufficient adolescent CF patients is recommended.
  • Early detection of glucose metabolism abnormalities can be achieved through this screening, exceeding clinical suspicion.
  • HbA1c is not a reliable biomarker for screening CF-related glucose intolerance in this population.
Abstract

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