Observational studyThe European respiratory journal2024
Impact of elexacaftor/tezacaftor/ivacaftor therapy on lung clearance index and magnetic resonance imaging in children with cystic fibrosis and one or two
Observational study in The European respiratory journal, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.
What it found
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The trial behind it
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Who cites it
28 citing papers in PubMed.
- Long-term effectiveness and safety of cystic fibrosis transmembrane conductance regulator modulators and their impact on the lives of people with cystic fibrosis.American journal of respiratory and critical care medicine · 2026Article
- Effects of elexacaftor/tezacaftor/ivacaftor on the nasal microbial metagenome in cystic fibrosis.Microbiology spectrum · 2026Article
- Effectiveness of elexacaftor/tezacaftor/ivacaftor therapy in children and adolescents with cystic fibrosis and normal spirometry.Pediatric research · 2026Article
- Persistence of lung hyperinflation and small airway dysfunction in school-aged children with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor: results from the real-world MODUL-CF study.ERJ open research · 2026Article
- Off-label treatment with elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: real-world evidence from the German CF Registry.ERJ open research · 2026Article
- Tracking early cystic fibrosis in the new era.ERJ open research · 2026Article
- ENHANCE: a community partnership charting the evolution of early-life cystic fibrosis disease manifestations in a new era of management.ERJ open research · 2026Article
- Advances in cystic fibrosis: CFTR modulator triple combinations.The European respiratory journal · 2026Article
- Type 3 innate lymphoid cells dominate the ILC compartment in endstage lung disease.Frontiers in immunology · 2026Article
- Reduction of systemic inflammation by elexacaftor/tezacaftor/ivacaftor correlates with lung function improvement in cystic fibrosis.The European respiratory journal · 2025Observational
- Comprehensive Evaluation of Elexacaftor/Tezacaftor/Ivacaftor in Paediatric Cystic Fibrosis: Nutritional, Pulmonary, and Quality-of-Life Outcomes.Journal of clinical medicine · 2025Article
- Early life elevations of methionine oxidation and ornithine track and predict cystic fibrosis structural lung disease.ERJ open research · 2025Article
- Real-World Outcomes of Elexacaftor/Tezacaftor/Ivacaftor in Children with Cystic Fibrosis.Annals of the American Thoracic Society · 2025Article
- Quantitative MRI detects delayed perfusion and impact of bronchial artery dilatation on pulmonary circulation in patients with cystic fibrosis.European radiology · 2025Article
- Screening and monitoring across the lifespan of an ageing cystic fibrosis population.ERJ open research · 2025Review
- Real-World Improvements of Lung Clearance Index and Ventilation Distribution Efficiency in Children With Cystic Fibrosis After Elexacaftor/Tezacaftor/Ivacaftor Initiation.Pediatric pulmonology · 2025Article
- The potentiator ivacaftor is essential for pharmacological restoration of F508del-CFTR function and mucociliary clearance in cystic fibrosis.JCI insight · 2025Article
- Identification and Characterization of a Rare Exon 22 Duplication inInternational journal of molecular sciences · 2025Article
- Progress of personalized medicine of cystic fibrosis in the times of efficient CFTR modulators.Molecular and cellular pediatrics · 2025Review
- Evolution of Lung Disease Studied by Computed Tomography in Adults with Cystic Fibrosis Treated with Elexacaftor/Tezacaftor/Ivacaftor.Journal of imaging · 2025Article
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Authors and funding
23 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundWe recently demonstrated that elexacaftor/tezacaftor/ivacaftor (ETI) improves the lung clearance index (LCI) and abnormalities in lung morphology detected by magnetic resonance imaging (MRI) in adolescent and adult patients with cystic fibrosis (CF). However, real-world data on the effect of ETI on these sensitive outcomes of lung structure and function in school-age children with CF have not been reported. The aim of this study was therefore to examine the effect of ETI on the LCI and the lung MRI score in children aged 6-11 years with CF and one or two
methodsThis prospective, observational, multicentre, post-approval study assessed the longitudinal LCI up to 12 months and the lung MRI score before and 3 months after initiation of ETI.
resultsA total of 107 children with CF including 40 heterozygous for
conclusionsETI improves early abnormalities in lung ventilation and morphology in school-age children with CF and at least one
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