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Updated: Jun 11, 2026
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Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
Published on: August 29, 2025
Updates in Cystic Fibrosis-Related Diabetes (CFRD)
Henry Zelada1, Katie L Kaput2, Shauna Runchey3
1Division of Endocrinology, Diabetes & Metabolism, Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, California.
Summary
Cystic Fibrosis Related Diabetes (CFRD) presents unique glycemic challenges due to pancreatic dysfunction. New therapies are improving CFTR function, but CFRD management requires personalized approaches considering metabolic shifts.
Area of Science:
- Endocrinology
- Metabolic Disorders
- Genetics
Background:
- Cystic Fibrosis Related Diabetes (CFRD) stems from pancreatic exocrine and endocrine dysfunction.
- It causes distinct glycemic abnormalities, including post-meal hyperglycemia and variability, differing from type 1 and type 2 diabetes.
- Recent advancements in CFTR modulators have improved CF patient outcomes but altered their metabolic profiles, increasing insulin resistance.
Purpose of the Study:
- To review the unique characteristics of CFRD.
- To discuss the impact of novel CFTR modulator therapies on CFRD.
- To explore current and future management strategies for CFRD.
Main Methods:
- Review of current literature on CFRD pathophysiology and management.
- Analysis of the effects of highly effective CFTR modulator therapy (HEMT) on metabolic profiles.
- Evaluation of diagnostic tools like oral glucose tolerance tests and continuous glucose monitoring.
Main Results:
- CFRD exhibits distinct glycemic patterns due to disrupted insulin and glucagon responses.
- HEMT improves CFTR function and overall health but can lead to weight gain, adiposity, and insulin resistance.
- Insulin therapy remains crucial, with potential roles for advanced delivery systems and select non-insulin agents.
Conclusions:
- CFRD management needs personalization, adapting to evolving nutritional status and insulin requirements in patients aging due to HEMT.
- Continuous glucose monitoring aids in detecting dysglycemia.
- Future research should focus on HEMT's long-term impact on CFRD and personalized treatment strategies.
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