SynthesisFrontiers in endocrinology2025
Effect of CFTR modulators on glucose homeostasis in children and young adults with cystic fibrosis-related diabetes: a systematic review.
Synthesis in Frontiers in endocrinology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
4 citing papers in PubMed.
- Beyond BMI: Nutritional Recovery and Functional Implications of CFTR Modulators in Cystic Fibrosis.Biology · 2026Review
- Cystic Fibrosis of the Pancreas: In Vitro Duct Models for CFTR-Targeted Translational Research.International journal of molecular sciences · 2026Review
- Evolving Cystic Fibrosis Care: Lung Immunology and Emerging Health Challenges in the Era of CFTR Modulators.Biomolecules · 2025Review
- Cystic fibrosis-related diabetes in the era of modern treatment using CFTR modulators in pediatric patients-a systematic review.Frontiers in pediatrics · 2025Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
7 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Introduction: Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the CFTR gene, leading to impaired chloride transport, thickened mucus, and multiorgan dysfunction. Among its complications, cystic fibrosis-related diabetes (CFRD) is a major concern, characterized by progressive b-cell dysfunction and insulin deficiency. The advent of CFTR modulators, including ivacaftor, lumacaftor/ivacaftor, and elexacaftor/tezacaftor/ivacaftor (ETI), has revolutionized CF management by improving pulmonary function, nutritional status, and overall survival. However, their effects on glucose metabolism remain under investigation. Methods: This systematic review (systematic review registration: PROSPERO 2025 CRD420251021499) analyzes recent evidence on the impact of CFTR modulators on CFRD in children and young adults. Results: Ivacaftor demonstrates potential benefits in glucose regulation, enhancing insulin secretion and glucagon control, particularly in patients with gating mutations. Conversely, lumacaftor/ivacaftor exhibits inconsistent effects, with some studies indicating glucose tolerance improvements while others report insulin sensitivity decline. Discussion: ETI therapy shows modest but generally positive effects on glycemic control, with reductions in HbA1c and fasting glucose, though without significant changes in insulin secretion. While CFTR modulators improve systemic health, their role in directly preventing or reversing CFRD remains unclear. Further longitudinal studies are needed to optimize therapeutic strategies and elucidate the long-term metabolic effects of CFTR modulation in CF patients. Systematic review registration: https://www.crd.york.ac.uk/PROSPERO/, identifier CRD420251021499.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.