ArticlePediatric pulmonology2024
Effects of highly effective modulator therapy on the dynamics of the respiratory mucosal environment and inflammatory response in cystic fibrosis.
Article in Pediatric pulmonology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 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.
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Who cites it
13 citing papers in PubMed, 15 citations in OpenAlex.
- How cystic fibrosis modulator therapy could transformMicrobiology (Reading, England) · 2026Review
- Antiviral defenses are diminished at birth in cystic fibrosis pig airways.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2026Article
- Respiratory viral infection is associated with increasedERJ open research · 2026Article
- Clinical Outcomes in Patients with Cystic Fibrosis-Related Chronic Rhinosinusitis Treated with Functional Endoscopic Sinus Surgery or Triple Highly Effective Modulator Therapy: A Monocentric Retrospective Experience.Journal of clinical medicine · 2025Article
- Elexacaftor-Tezacaftor-Ivacaftor Improves Sinonasal Outcomes in Young Children With Cystic Fibrosis.International forum of allergy & rhinology · 2025Article
- Beyond the Lung. Impact of Elexacaftor/Tezacaftor/Ivacaftor on Sinonasal Disease in Children With Cystic Fibrosis.International forum of allergy & rhinology · 2025Observational
- Progress of personalized medicine of cystic fibrosis in the times of efficient CFTR modulators.Molecular and cellular pediatrics · 2025Review
- The impact of cystic fibrosis transmembrane conductance regulator (CFTR) modulators on the pulmonary microbiota.Microbiology (Reading, England) · 2025Review
- The Role of Triple CFTR Modulator Therapy in Reducing Systemic Inflammation in Cystic Fibrosis.Lung · 2025Article
- IL-22Ra2 Levels Remain Elevated in People with Cystic Fibrosis despite Modulator Therapy.American journal of respiratory and critical care medicine · 2024Article
- Persistence and evolution ofmBio · 2024Article
- CFTR dysfunction leads to defective bacterial eradication on cystic fibrosis airways.Frontiers in physiology · 2024Review
- Modulator-refractory cystic fibrosis: Defining the scope and challenges of an emerging at-risk population.Therapeutic advances in respiratory diseaseReview
Corrections and comments
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Authors and funding
15 authors at 4 institutions in 1 country.
Funding
Abstract
backgroundWhile the widespread initiation of elexacaftor/tezacaftor/ivacaftor (ETI) has led to dramatic clinical improvements among persons with cystic fibrosis (pwCF), little is known about how ETI affects the respiratory mucosal inflammatory and physiochemical environment, or how these changes relate to lung function.
methodsWe performed a prospective, longitudinal study of adults with CF and chronic rhinosinusitis (CF-CRS) followed at our CF center (n = 18). Endoscopic upper respiratory tract (paranasal sinus) aspirates from multiple visit dates, both pre- and post-ETI initiation, were collected and tested for cytokines, metals, pH, and lactate levels. Generalized estimating equations were used to identify relationships between ETI and upper respiratory tract (URT) biomarker levels, and between URT biomarkers and lung function or clinical sinus parameters.
resultsETI was associated with decreased upper respiratory mucosal cytokines B-cell activating factor (BAFF), IL-12p40, IL-32, IL-8, IL-22 and soluble tumor necrosis factor-1 (sTNFR1), and an increase in a proliferation-inducing ligand (APRIL) and IL-19. ETI was also associated with decreased URT levels of copper, manganese, and zinc. In turn, lower URT levels of BAFF, IL-8, lactate, and potassium were each associated with ~1.5% to 4.3% improved forced expiratory volume in 1 s (FEV
conclusionsOur observations suggest a dampening of inflammatory signals and restriction in microbial nutrients in the upper respiratory tract with ETI. These findings improve our understanding of how ETI impacts the mucosal environment in the respiratory tract, and may give insight into the improved infectious and inflammatory status and the resulting clinical improvements seen in pwCF.
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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.