Natural variability of lung function in primary ciliary dyskinesia: longitudinal analysis from the PROVALF-PCD cohort
Changes in intra-individual FEV<sub>1</sub> >25% between visits in stable PCD patients lie beyond the expected test variability and therefore could be considered physiologically relevant. These findings inform the selection of end-points for pulmonary intervention trials in PCD, as they suggest that FEV<sub>1</sub> is not a sensitive test for monitoring lung health in PCD.
Authors
- June K. Marthin (ORCID: https://orcid.org/0000-0002-4420-3091)
- Lucy Morgan (ORCID: https://orcid.org/0000-0001-6389-1953)
- Heymut Omran (ORCID: https://orcid.org/0000-0003-0282-6765)
- Claudia E. Kuehni (ORCID: https://orcid.org/0000-0001-8957-2002)
- Claire Hogg (ORCID: https://orcid.org/0000-0002-8368-5994)
- Francesca Santamaria (ORCID: https://orcid.org/0000-0002-9375-7429)
- Melissa Borrelli (ORCID: https://orcid.org/0000-0002-7282-6018)
- Bruna Rubbo (ORCID: https://orcid.org/0000-0002-1629-8601)
- Panayiotis Κouis (ORCID: https://orcid.org/0000-0003-0511-5352)
- Philipp Latzin (ORCID: https://orcid.org/0000-0002-5239-1571)
- Mieke Boon (ORCID: https://orcid.org/0000-0003-2492-2854)
- Dilber Ademhan Tural (ORCID: https://orcid.org/0000-0002-0334-6336)
- Nicola Ullmann (ORCID: https://orcid.org/0000-0003-1111-5690)
- Sandra Rovira‐Amigo (ORCID: https://orcid.org/0000-0001-9896-0621)
- Panayiotis K. Yiallouros (ORCID: https://orcid.org/0000-0002-8339-9285)
- Silvia Castillo (ORCID: https://orcid.org/0000-0002-0121-2676)
- Renato Cutrera (ORCID: https://orcid.org/0000-0001-7711-5672)
- Antonio Moreno (ORCID: https://orcid.org/0000-0002-2496-9786)
- Kim G. Nielsen (ORCID: https://orcid.org/0000-0001-5906-9449)
- Jane S. Lucas (ORCID: https://orcid.org/0000-0001-8701-9975)
- Anne Schlegtendal (ORCID: https://orcid.org/0000-0002-6737-3624)
- Bettina Frauchiger (ORCID: https://orcid.org/0000-0002-9519-9328)
- Cordula Koerner‐Rettberg (ORCID: https://orcid.org/0000-0003-0946-1009)
- Petr Pohunek (ORCID: https://orcid.org/0000-0003-4557-2515)
- Natalie Lorent (ORCID: https://orcid.org/0000-0002-2653-5885)
- Guillaume Thouvenin (ORCID: https://orcid.org/0000-0003-0528-5458)
- Carolina Constant (ORCID: https://orcid.org/0000-0002-6596-1596)
- Ela Erdem Eralp (ORCID: https://orcid.org/0000-0001-8829-3431)
- Amanda Harris (ORCID: https://orcid.org/0000-0002-6953-7370)
- Stefanie Dillenhöfer (ORCID: https://orcid.org/0000-0001-6020-8717)
- Nicole Beydon
- Philip Robinson (ORCID: https://orcid.org/0000-0001-6129-9075)
- Aline Tamalet
- Sanem Eryılmaz Polat (ORCID: https://orcid.org/0000-0003-2309-7952)
- Nathalie Feyaerts
- Uğur Özçelik (ORCID: https://orcid.org/0000-0003-1587-5216)
- Enrico Lombardi (ORCID: https://orcid.org/0000-0003-4749-9091)
- Vendula Martinů (ORCID: https://orcid.org/0000-0001-7773-6700)
- Kewei Zhang (ORCID: https://orcid.org/0000-0003-4871-2261)
- Avni Kant
- Woolf T. Walker
- Camille Pearse
Institutions
- University of Bern (CH)
- University of Copenhagen (DK)
- The University of Sydney (AU)
- Concord Hospital (US)
- University of Lisbon (PT)
- Inserm (FR)
- The University of Melbourne (AU)
- Charles University (CZ)
- Royal Brompton & Harefield NHS Foundation Trust (GB)
- Concord Repatriation General Hospital (AU)
- University Hospital of Bern (CH)
- Copenhagen University Hospital (DK)
- Rigshospitalet (DK)
- University Hospital Southampton NHS Foundation Trust (GB)
- University of Cyprus (CY)
- Sorbonne Université (FR)
- Hôpital Armand-Trousseau (FR)
- Hospital Clínico Universitario de Valencia (ES)
- Assistance Publique – Hôpitaux de Paris (FR)
- University Hospital in Motol (CZ)
- University Hospital Münster (DE)
- Marien Hospital Wesel (DE)
- Centre for Biomedical Network Research on Rare Diseases (ES)
- Meyer Children's Hospital (IT)
- Federico II University Hospital (IT)
- Bambino Gesù Children's Hospital (IT)
- Vall d'Hebron Hospital Universitari (ES)
- Centre de Recherche Saint-Antoine (FR)
- Institute of Social and Preventive Medicine (CH)
- MRC Lifecourse Epidemiology Unit (GB)
- Hospital de Santa Maria (PT)
- Katholisches Klinikum Bochum (DE)
- University of Southampton (GB)
- Hacettepe University (TR)
- Marmara University (TR)
- Ruhr University Bochum (DE)
- KU Leuven (BE)
Publication Details
- Journal
- ERJ Open Research
- Published
- 2025-03-28
- DOI
- https://doi.org/10.1183/23120541.01115-2024
- Citations
- 2
- Primary Topic
- Cystic Fibrosis Research Advances
- Type
- article
- Field-Weighted Citation Impact
- 3.04
Funders
- AstraZeneca
- GlaxoSmithKline
- Sanofi
- LifeArc
- Mylan
- Insmed
- National Institute for Health and Care Research
- University of Southampton
- Ministerstvo Zdravotnictví Ceské Republiky
- National Institute for Health Research Southampton Biomedical Research Centre
- Sanofi Pasteur
- Medical Research Council