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Cystic Fibrosis-Related Diabetes: Pathophysiology, Spectrum of Glycemic Abnormalities, and Evolving Approaches to
Jennifer Apsan1, Kajal Gandhi2, Juan Pablo Perdomo Rodriguez3
1Department of Pediatrics, Division of Endocrinology, Hofstra Northwell School of Medicine, Northwell Health, Cohen Children's Medical Center, New Hyde Park, New York.
Insights
Cystic Fibrosis Related Diabetes (CFRD) is a growing complication in cystic fibrosis patients, impacting health and survival. This review summarizes CFRD causes, screening methods, and challenges, especially with new CF treatments.
Area of Science:
- Endocrinology
- Pulmonology
- Metabolic Disorders
Background:
- Cystic Fibrosis Related Diabetes (CFRD) is a common complication in cystic fibrosis (CF).
- CFRD negatively impacts pulmonary function, nutritional status, and survival rates.
- It typically develops in adolescence and adulthood.
Purpose of the Study:
- To summarize the pathophysiology of CFRD, including preceding glycemic abnormalities.
- To review current evidence and guidelines for CFRD screening.
- To discuss screening challenges and considerations for emerging CF therapies and increased longevity.
Main Methods:
- This is a narrative review.
- It synthesizes existing literature on CFRD pathophysiology and screening.
- Evidence for various screening approaches is critically evaluated.
Main Results:
- CFRD pathophysiology involves a spectrum of glycemic changes.
- Current screening guidelines exist, but evidence for optimal approaches varies.
- Screening is challenged by evolving CF treatments and patient longevity.
Conclusions:
- Understanding CFRD pathophysiology is crucial for early detection.
- Optimizing CFRD screening strategies is essential for improved patient outcomes.
- Adapting screening to new CF therapeutics and increased lifespan is a key future direction.
Abstract:
Cystic fibrosis-related diabetes (CFRD) is a well-recognized and increasingly prevalent complication of cystic fibrosis (CF), most frequently developing in adolescence and adulthood. CFRD is associated with pulmonary function decline, poor nutritional status, and increased infection risk, and contributes to increased morbidity and mortality. The objective of this review is (1) to provide a summary of CFRD pathophysiology, highlighting the spectrum of glycemic abnormalities preceding development of CFRD, and (2) to review CFRD screening guidelines, including a narrative review of the current evidence for different CFRD screening approaches. We also review challenges to screening and important considerations in the setting of novel CF therapeutics and increasing longevity in this population.
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