ArticleThe European respiratory journal2026
Changes in sputum viscoelastic properties and airway inflammation in primary ciliary dyskinesia are comparable to cystic fibrosis on elexacaftor/tezacaftor/ivacaftor therapy.
Article in The European respiratory journal, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed.
- The dipeptidyl peptidase 1 inhibition landscape in bronchiectasis and its future direction.European respiratory review : an official journal of the European Respiratory Society · 2026Review
- Effects of hypertonic saline and/or carbocisteine on sputum rheology in bronchiectasis: CLEAR-EME.ERJ open research · 2026Article
- Phase III AIRTIVITY study design: verducatib (BI 1291583) in people with bronchiectasis.ERJ open research · 2026Article
- Airway Clearance and Pediatric Pulmonary Rehabilitation in Primary Ciliary Dyskinesia: A Clinical Framework for Children and Adolescents.Journal of clinical medicine · 2026Review
- Xenon-129 magnetic resonance: a paradigm shift, an expensive toy or another tool for the pulmonologist?ERJ open research · 2026Article
- Myeloperoxidase impairs mucociliary transport on human airway epithelium.Disease models & mechanisms · 2026Article
- Cilia-independent gas-liquid transport, a third mechanism mediating airway mucus clearance.The Journal of clinical investigation · 2026Article
- Use of Recombinant Human Deoxyribonuclease I in Primary Ciliary Dyskinesia Bronchiectasis-A Real Life Pilot Study.Medical sciences (Basel, Switzerland) · 2026Article
- Airway mucus in infection.Frontiers in physiology · 2026Review
- SputOMICs identifies common and distinct markers in cystic fibrosis and chronic obstructive pulmonary disease.Scientific reports · 2025Article
- Myeloperoxidase impairs mucociliary transport on human airway epithelium.bioRxiv : the preprint server for biology · 2025Article
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14 authors.
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No grant is acknowledged in the PubMed record.
Abstract
backgroundPrimary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are muco-obstructive lung diseases that are caused by distinct genetically determined defects in mucociliary clearance; however, knowledge on the relative severity of airway mucus dysfunction and chronic inflammation remains limited. The aim of this study was therefore to compare sputum viscoelastic properties, inflammation markers and the proteome between patients with PCD and patients with CF before and under elexacaftor/tezacaftor/ivacaftor (ETI) therapy.
methodsWe compared sputum rheology, inflammation markers and the proteome in 42 clinically stable adolescent and adult patients with PCD, 40 patients with CF with at least one
resultsThe elastic modulus (
conclusionsClinically stable patients with PCD show changes in sputum viscoelastic properties, inflammation markers and the proteome that are less severe than in patients with CF at baseline, but comparable to CF patients on ETI therapy.
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