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Cystic fibrosis-related diabetes in childhood

Stephen M P O'Riordan1, Mehul T Dattani, Peter C Hindmarsh

  • 1Developmental Endocrinology Research Group, Clinical and Molecular Genetics Unit, Institute of Child Health, University College London, London, UK. s.oriordan@ich.ucl.ac.uk

Hormone Research in Paediatrics
|March 2, 2010
PubMed

Insights

Cystic fibrosis-related diabetes (CFRD) is the most common complication, significantly impacting survival. Early detection and management are crucial for improving outcomes in children and adolescents with CFRD.

Area of Science:

  • Pediatric Endocrinology
  • Pulmonology
  • Metabolic Disorders

Background:

  • Cystic Fibrosis (CF) management has improved survival significantly since the 1950s.
  • Cystic Fibrosis-Related Diabetes (CFRD) is the most prevalent complication, affecting 50% of CF patients by age 30.
  • CFRD negatively impacts survival, increasing morbidity and mortality sixfold.

Purpose of the Study:

  • To review current literature on CFRD in pediatric populations.
  • To comprehensively report on the incidence, prevalence, and pathophysiology of CFRD.
  • To address survival, prognosis, diagnosis, monitoring, and treatment strategies for CFRD.

Main Methods:

  • Systematic literature review using Medline and Pubmed databases.
  • Analysis of studies focusing on CFRD in children and adolescents.
  • Synthesis of data on pathophysiology, clinical outcomes, and management.

Main Results:

  • CFRD is often asymptomatic and can be undetected for up to four years.
  • Pathophysiology involves both insulin deficiency and insulin insensitivity.
  • CFRD is associated with significantly reduced survival rates.

Conclusions:

  • CFRD is a critical complication requiring vigilant monitoring and management in pediatric CF patients.
  • Improved understanding of CFRD pathophysiology can guide therapeutic interventions.
  • Proactive diagnosis and treatment are essential for enhancing long-term outcomes in CFRD.

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