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Natural variability of lung function in primary ciliary dyskinesia: longitudinal analysis from the PROVALF-PCD cohort.

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Title: Natural variability of lung function in primary ciliary dyskinesia: longitudinal analysis from the PROVALF-PCD cohort.
Authors: Zhang K; Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK.; Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK., Kant A; Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK.; Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK., Boon M; Department of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium.; Department of CHROMETA, Laboratory for Respiratory Diseases and Thoracic Surgery, KU Leuven, Leuven, Belgium., Borrelli M; Department of Translational Medical Sciences, Pediatric Pulmonology, Federico II University, Naples, Italy., Constant C; Department of Pediatrics, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte and Faculty of Medicine, University of Lisbon, Lisbon, Portugal., Castillo Corullon S; FE Pediatría y Neumología Infantil, Unidad de Neumología Infantil y Fibrosis Quística, Hospital Clínico de Valencia, Valencia, Spain., Cutrera R; Respiratory and Cystic Fibrosis Unit, Academic Pediatric Department, Bambino Gesù Children Hospital, IRCCS, Rome, Italy., Dillenhöfer S; Department of Paediatric Pulmonology, University Children's Hospital, Ruhr University Bochum, Katholisches Klinikum Bochum, Bochum, Germany., Eryılmaz Polat S; Hacettepe University Faculty of Medicine, Department of Pediatric Pulmonology, Ankara, Turkey., Eralp E; Marmara Universitesi, Department of Pediatric Pulmonology, Istanbul, Turkey., Feyaerts N; Department of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium.; Department of CHROMETA, Laboratory for Respiratory Diseases and Thoracic Surgery, KU Leuven, Leuven, Belgium., Harris A; Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK.; Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK., Hogg C; Royal Brompton and Harefield NHS Foundation Trust, Department of Paediatrics, Primary Ciliary Dyskinesia Centre, London, UK., Koerner-Rettberg C; Department of Paediatric Pulmonology, University Children's Hospital, Ruhr University Bochum, Katholisches Klinikum Bochum, Bochum, Germany.; Children's Hospital and Research Institute, Marienhospital Wesel, Wesel, Germany., Kouis P; Medical School, University of Cyprus, Nicosia, Cyprus., Lombardi E; Meyer Children's Hospital IRCCS, Florence, Italy., Lorent N; Department of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium.; Department of CHROMETA, Laboratory for Respiratory Diseases and Thoracic Surgery, KU Leuven, Leuven, Belgium., Marthin JK; Danish PCD and chILD Centre, CF Centre Copenhagen, Paediatric Pulmonary Service, ERN Accredited, Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.; Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark., Martinu V; Department of Paediatrics, Charles University, 2nd Faculty of Medicine, University Hospital Motol, Prague, Czech Republic., Moreno-Galdo A; Paediatric Pulmonology Section, Department of Paediatrics, Hospital Universitari Vall dHebron, Barcelona, Spain.; Universitat Autònoma de Barcelona, Instituto de Salud Carlos III, Centre for Biomedical Network Research on Rare Diseases (CIBERER), Barcelona, Spain., Morgan L; Concord Hospital, Concord Clinical School, University of Sydney, Department of Respiratory Medicine, Sydney, Australia., Nielsen K; Danish PCD and chILD Centre, CF Centre Copenhagen, Paediatric Pulmonary Service, ERN Accredited, Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.; Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark., Omran H; Department of General Pediatrics, University Hospital Muenster, Muenster, Germany., Ozcelik U; Hacettepe University Faculty of Medicine, Department of Pediatric Pulmonology, Ankara, Turkey., Pohunek P; Department of Paediatrics, Charles University, 2nd Faculty of Medicine, University Hospital Motol, Prague, Czech Republic., Robinson P; Faculty of Medicine Dentistry and Health Sciences, Paediatrics, University of Melbourne, Melbourne, Australia., Rovira-Amigo S; Paediatric Pulmonology Section, Department of Paediatrics, Hospital Universitari Vall dHebron, Barcelona, Spain.; Universitat Autònoma de Barcelona, Instituto de Salud Carlos III, Centre for Biomedical Network Research on Rare Diseases (CIBERER), Barcelona, Spain., Santamaria F; Department of Translational Medical Sciences, Pediatric Pulmonology, Federico II University, Naples, Italy., Schlegtendal A; Department of Paediatric Pulmonology, University Children's Hospital, Ruhr University Bochum, Katholisches Klinikum Bochum, Bochum, Germany., Tamalet A; Sorbonne Université, Inserm U938, Centre de Recherche Saint-Antoine, Paris, France.; Sorbonne Université, Assistance Publique - Hôpitaux de Paris, Hôpital Trousseau, Service de Pneumologie Pédiatrique, Paris, France., Thouvenin G; Sorbonne Université, Inserm U938, Centre de Recherche Saint-Antoine, Paris, France.; Sorbonne Université, Assistance Publique - Hôpitaux de Paris, Hôpital Trousseau, Service de Pneumologie Pédiatrique, Paris, France., Ademhan Tural D; Hacettepe University Faculty of Medicine, Department of Pediatric Pulmonology, Ankara, Turkey., Ullmann N; Respiratory and Cystic Fibrosis Unit, Academic Pediatric Department, Bambino Gesù Children Hospital, IRCCS, Rome, Italy., Walker WT; Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK.; Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK., Yiallouros P; Medical School, University of Cyprus, Nicosia, Cyprus., Pearse C; MRC Lifecourse Epidemiology Centre, University of Southampton, Southampton, UK., Frauchiger B; Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics, Inselspital, University Hospital, University of Bern, Bern, Switzerland., Kuehni CE; Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics, Inselspital, University Hospital, University of Bern, Bern, Switzerland.; Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland., Beydon N; AP-HP, Sorbonne Université, Unité d'Exploration Fonctionnelle Respiratoire, Hôpital Armand-Trousseau, and Sorbonne Université, INSERM U938, Paris, France., Latzin P; Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics, Inselspital, University Hospital, University of Bern, Bern, Switzerland., Lucas JS; Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK.; Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK.; These authors contributed equally., Rubbo B; Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK.; Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK.; These authors contributed equally.
Source: ERJ open research [ERJ Open Res] 2025 Jun 23; Vol. 11 (3). Date of Electronic Publication: 2025 Jun 23 (Print Publication: 2025).
Publication Type: Journal Article
Journal Info: Publisher: European Respiratory Society Country of Publication: England NLM ID: 101671641 Publication Model: eCollection Cited Medium: Print ISSN: 2312-0541 (Print) Linking ISSN: 23120541 NLM ISO Abbreviation: ERJ Open Res Subsets: PubMed not MEDLINE
Database: MEDLINE Ultimate
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  Data: Natural variability of lung function in primary ciliary dyskinesia: longitudinal analysis from the PROVALF-PCD cohort.
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  Data: <searchLink fieldCode="AU" term="%22Zhang+K%22">Zhang K</searchLink>; Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK.; Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK.<br /><searchLink fieldCode="AU" term="%22Kant+A%22">Kant A</searchLink>; Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK.; Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK.<br /><searchLink fieldCode="AU" term="%22Boon+M%22">Boon M</searchLink>; Department of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium.; Department of CHROMETA, Laboratory for Respiratory Diseases and Thoracic Surgery, KU Leuven, Leuven, Belgium.<br /><searchLink fieldCode="AU" term="%22Borrelli+M%22">Borrelli M</searchLink>; Department of Translational Medical Sciences, Pediatric Pulmonology, Federico II University, Naples, Italy.<br /><searchLink fieldCode="AU" term="%22Constant+C%22">Constant C</searchLink>; Department of Pediatrics, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte and Faculty of Medicine, University of Lisbon, Lisbon, Portugal.<br /><searchLink fieldCode="AU" term="%22Castillo+Corullon+S%22">Castillo Corullon S</searchLink>; FE Pediatría y Neumología Infantil, Unidad de Neumología Infantil y Fibrosis Quística, Hospital Clínico de Valencia, Valencia, Spain.<br /><searchLink fieldCode="AU" term="%22Cutrera+R%22">Cutrera R</searchLink>; Respiratory and Cystic Fibrosis Unit, Academic Pediatric Department, Bambino Gesù Children Hospital, IRCCS, Rome, Italy.<br /><searchLink fieldCode="AU" term="%22Dillenhöfer+S%22">Dillenhöfer S</searchLink>; Department of Paediatric Pulmonology, University Children's Hospital, Ruhr University Bochum, Katholisches Klinikum Bochum, Bochum, Germany.<br /><searchLink fieldCode="AU" term="%22Eryılmaz+Polat+S%22">Eryılmaz Polat S</searchLink>; Hacettepe University Faculty of Medicine, Department of Pediatric Pulmonology, Ankara, Turkey.<br /><searchLink fieldCode="AU" term="%22Eralp+E%22">Eralp E</searchLink>; Marmara Universitesi, Department of Pediatric Pulmonology, Istanbul, Turkey.<br /><searchLink fieldCode="AU" term="%22Feyaerts+N%22">Feyaerts N</searchLink>; Department of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium.; Department of CHROMETA, Laboratory for Respiratory Diseases and Thoracic Surgery, KU Leuven, Leuven, Belgium.<br /><searchLink fieldCode="AU" term="%22Harris+A%22">Harris A</searchLink>; Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK.; Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK.<br /><searchLink fieldCode="AU" term="%22Hogg+C%22">Hogg C</searchLink>; Royal Brompton and Harefield NHS Foundation Trust, Department of Paediatrics, Primary Ciliary Dyskinesia Centre, London, UK.<br /><searchLink fieldCode="AU" term="%22Koerner-Rettberg+C%22">Koerner-Rettberg C</searchLink>; Department of Paediatric Pulmonology, University Children's Hospital, Ruhr University Bochum, Katholisches Klinikum Bochum, Bochum, Germany.; Children's Hospital and Research Institute, Marienhospital Wesel, Wesel, Germany.<br /><searchLink fieldCode="AU" term="%22Kouis+P%22">Kouis P</searchLink>; Medical School, University of Cyprus, Nicosia, Cyprus.<br /><searchLink fieldCode="AU" term="%22Lombardi+E%22">Lombardi E</searchLink>; Meyer Children's Hospital IRCCS, Florence, Italy.<br /><searchLink fieldCode="AU" term="%22Lorent+N%22">Lorent N</searchLink>; Department of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium.; Department of CHROMETA, Laboratory for Respiratory Diseases and Thoracic Surgery, KU Leuven, Leuven, Belgium.<br /><searchLink fieldCode="AU" term="%22Marthin+JK%22">Marthin JK</searchLink>; Danish PCD and chILD Centre, CF Centre Copenhagen, Paediatric Pulmonary Service, ERN Accredited, Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.; Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark.<br /><searchLink fieldCode="AU" term="%22Martinu+V%22">Martinu V</searchLink>; Department of Paediatrics, Charles University, 2nd Faculty of Medicine, University Hospital Motol, Prague, Czech Republic.<br /><searchLink fieldCode="AU" term="%22Moreno-Galdo+A%22">Moreno-Galdo A</searchLink>; Paediatric Pulmonology Section, Department of Paediatrics, Hospital Universitari Vall dHebron, Barcelona, Spain.; Universitat Autònoma de Barcelona, Instituto de Salud Carlos III, Centre for Biomedical Network Research on Rare Diseases (CIBERER), Barcelona, Spain.<br /><searchLink fieldCode="AU" term="%22Morgan+L%22">Morgan L</searchLink>; Concord Hospital, Concord Clinical School, University of Sydney, Department of Respiratory Medicine, Sydney, Australia.<br /><searchLink fieldCode="AU" term="%22Nielsen+K%22">Nielsen K</searchLink>; Danish PCD and chILD Centre, CF Centre Copenhagen, Paediatric Pulmonary Service, ERN Accredited, Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.; Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark.<br /><searchLink fieldCode="AU" term="%22Omran+H%22">Omran H</searchLink>; Department of General Pediatrics, University Hospital Muenster, Muenster, Germany.<br /><searchLink fieldCode="AU" term="%22Ozcelik+U%22">Ozcelik U</searchLink>; Hacettepe University Faculty of Medicine, Department of Pediatric Pulmonology, Ankara, Turkey.<br /><searchLink fieldCode="AU" term="%22Pohunek+P%22">Pohunek P</searchLink>; Department of Paediatrics, Charles University, 2nd Faculty of Medicine, University Hospital Motol, Prague, Czech Republic.<br /><searchLink fieldCode="AU" term="%22Robinson+P%22">Robinson P</searchLink>; Faculty of Medicine Dentistry and Health Sciences, Paediatrics, University of Melbourne, Melbourne, Australia.<br /><searchLink fieldCode="AU" term="%22Rovira-Amigo+S%22">Rovira-Amigo S</searchLink>; Paediatric Pulmonology Section, Department of Paediatrics, Hospital Universitari Vall dHebron, Barcelona, Spain.; Universitat Autònoma de Barcelona, Instituto de Salud Carlos III, Centre for Biomedical Network Research on Rare Diseases (CIBERER), Barcelona, Spain.<br /><searchLink fieldCode="AU" term="%22Santamaria+F%22">Santamaria F</searchLink>; Department of Translational Medical Sciences, Pediatric Pulmonology, Federico II University, Naples, Italy.<br /><searchLink fieldCode="AU" term="%22Schlegtendal+A%22">Schlegtendal A</searchLink>; Department of Paediatric Pulmonology, University Children's Hospital, Ruhr University Bochum, Katholisches Klinikum Bochum, Bochum, Germany.<br /><searchLink fieldCode="AU" term="%22Tamalet+A%22">Tamalet A</searchLink>; Sorbonne Université, Inserm U938, Centre de Recherche Saint-Antoine, Paris, France.; Sorbonne Université, Assistance Publique - Hôpitaux de Paris, Hôpital Trousseau, Service de Pneumologie Pédiatrique, Paris, France.<br /><searchLink fieldCode="AU" term="%22Thouvenin+G%22">Thouvenin G</searchLink>; Sorbonne Université, Inserm U938, Centre de Recherche Saint-Antoine, Paris, France.; Sorbonne Université, Assistance Publique - Hôpitaux de Paris, Hôpital Trousseau, Service de Pneumologie Pédiatrique, Paris, France.<br /><searchLink fieldCode="AU" term="%22Ademhan+Tural+D%22">Ademhan Tural D</searchLink>; Hacettepe University Faculty of Medicine, Department of Pediatric Pulmonology, Ankara, Turkey.<br /><searchLink fieldCode="AU" term="%22Ullmann+N%22">Ullmann N</searchLink>; Respiratory and Cystic Fibrosis Unit, Academic Pediatric Department, Bambino Gesù Children Hospital, IRCCS, Rome, Italy.<br /><searchLink fieldCode="AU" term="%22Walker+WT%22">Walker WT</searchLink>; Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK.; Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK.<br /><searchLink fieldCode="AU" term="%22Yiallouros+P%22">Yiallouros P</searchLink>; Medical School, University of Cyprus, Nicosia, Cyprus.<br /><searchLink fieldCode="AU" term="%22Pearse+C%22">Pearse C</searchLink>; MRC Lifecourse Epidemiology Centre, University of Southampton, Southampton, UK.<br /><searchLink fieldCode="AU" term="%22Frauchiger+B%22">Frauchiger B</searchLink>; Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics, Inselspital, University Hospital, University of Bern, Bern, Switzerland.<br /><searchLink fieldCode="AU" term="%22Kuehni+CE%22">Kuehni CE</searchLink>; Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics, Inselspital, University Hospital, University of Bern, Bern, Switzerland.; Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.<br /><searchLink fieldCode="AU" term="%22Beydon+N%22">Beydon N</searchLink>; AP-HP, Sorbonne Université, Unité d'Exploration Fonctionnelle Respiratoire, Hôpital Armand-Trousseau, and Sorbonne Université, INSERM U938, Paris, France.<br /><searchLink fieldCode="AU" term="%22Latzin+P%22">Latzin P</searchLink>; Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics, Inselspital, University Hospital, University of Bern, Bern, Switzerland.<br /><searchLink fieldCode="AU" term="%22Lucas+JS%22">Lucas JS</searchLink>; Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK.; Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK.; These authors contributed equally.<br /><searchLink fieldCode="AU" term="%22Rubbo+B%22">Rubbo B</searchLink>; Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK.; Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK.; These authors contributed equally.
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