Changes in sputum viscoelastic properties and airway inflammation in primary ciliary dyskinesia are comparable to cystic fibrosis on elexacaftor/tezacaftor/ivacaftor therapy
Clinically 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.
Authors
- Kerstin Fentker (ORCID: https://orcid.org/0009-0005-0437-1049)
- Ruth Maria Urbantat (ORCID: https://orcid.org/0000-0001-7360-6001)
- Laura Schaupp (ORCID: https://orcid.org/0000-0003-1383-3821)
- Philipp Mertins (ORCID: https://orcid.org/0000-0002-2245-528X)
- Jobst Roehmel (ORCID: https://orcid.org/0000-0002-1535-8852)
- Mohamed Haji
- Axel Loewe (ORCID: https://orcid.org/0000-0002-2487-4744)
- Felix Doellinger (ORCID: https://orcid.org/0000-0003-3661-8569)
- Marcus Alexander Mall (ORCID: https://orcid.org/0000-0002-4057-2199)
- Simon Y. Graeber (ORCID: https://orcid.org/0000-0002-1666-8924)
- Mirjam Stahl (ORCID: https://orcid.org/0000-0001-6941-8315)
- Julia Duerr (ORCID: https://orcid.org/0000-0002-4862-9487)
- Michael Gradzielski (ORCID: https://orcid.org/0000-0002-7262-7115)
- Hannah Nussstein
Institutions
- American Institute of Biological Sciences (US)
- Max Delbrück Center (DE)
- Major League Baseball (US)
- Humboldt-Universität zu Berlin (DE)
- German Center for Lung Research (DE)
- Berlin Institute of Health at Charité - Universitätsmedizin Berlin (DE)
- Technische Universität Berlin (DE)
- Freie Universität Berlin (DE)
- Charité - Universitätsmedizin Berlin (DE)
Publication Details
- Journal
- European Respiratory Journal
- Published
- 2025-09-18
- DOI
- https://doi.org/10.1183/13993003.00616-2025
- Citations
- 8
- Primary Topic
- Cystic Fibrosis Research Advances
- Type
- article
- Field-Weighted Citation Impact
- 12.15
Funders
- European Respiratory Society
- Vertex Pharmaceuticals
- Insmed
- Deutsche Forschungsgemeinschaft
- Bundesministerium für Bildung und Forschung
- Berlin Institute of Health