Associations between respiratory pathogens and lung function in primary ciliary dyskinesia: cross-sectional analysis from the PROVALF-PCD cohort.

Autor:	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., 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., 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., Allegorico A; Respiratory and Cystic Fibrosis Unit, Academic Pediatric Department, Bambino Gesù Children Hospital, IRCCS, Rome, Italy., Basilicata S; Department of Translational Medical Sciences, Pediatric Pulmonology, Federico II University, Naples, Italy., Boon M; Department of Pediatrics, University Hospital Leuven, Leuven, Belgium., Borrelli M; Department of Translational Medical Sciences, Pediatric Pulmonology, Federico II University, Naples, Italy., Calogero C; Azienda Ospedaliero Universitaria Ospedale Pediatrico Meyer, Florence, Italy., Carr SB; Royal Brompton and Harefield NHS Foundation Trust, Department of Paediatrics, Primary Ciliary Dyskinesia Centre, London, UK., Carroll M; Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK., Constant C; Department of Pediatrics, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte and Faculty of Medicine, University of Lisbon, Lisbon, Portugal., Castillo Corullón S; FE Pediatría y Neumología Infantil, Unidad de Neumología Infantil y Fibrosis Quística, Hospital Clínico de Valencia, Valencia, Spain., Corvol H; AP-HP, Sorbonne Université, Unité d'Exploration Fonctionnelle Respiratoire, Hôpital Armand-Trousseau, and Sorbonne Université, INSERM U938, Paris, France., 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.; Children's Hospital and Research Institute, Marienhospital Wesel, Wesel, Germany., Emiralioglu N; Department of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey., Eralp E; Department of Pediatric Pulmonology, Marmara Universitesi, Istanbul, Turkey., Eryilmaz Polat S; Department of Pediatrics, Division of Pediatric Pulmonology, Ankara City Hospital, Ankara, Turkey., Gardner L; Royal Brompton and Harefield NHS Foundation Trust, Department of Paediatrics, Primary Ciliary Dyskinesia Centre, London, UK., Gokdemir Y; Department of Pediatric Pulmonology, Marmara Universitesi, Istanbul, Turkey., 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., Karadag B; Department of Pediatric Pulmonology, Marmara Universitesi, Istanbul, Turkey., Kobbernagel H; Danish PCD Centre, Danish Paediatric Pulmonary Service, Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.; Department of Clinical Medicine, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark., 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., 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., Marcou M; Medical School, University of Cyprus, Nicosia, Cyprus., Mathin JK; Danish PCD Centre, Danish Paediatric Pulmonary Service, Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.; Department of Clinical Medicine, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark., Martinu V; Department of Paediatrics, Charles University, 2nd Faculty of Medicine, University Hospital in Motol, Prague, Czech Republic., Moreno-Galdó A; Hospital Vall d'Hebron, Neumología Infantil, Barcelona, Spain., Morgan L; Concord Hospital, Concord Clinical School, University of Sydney, Department of Respiratory Medicine, Sydney, Australia., Nielsen KG; Danish PCD Centre, Danish Paediatric Pulmonary Service, Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.; Department of Clinical Medicine, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark., Omran H; Department of General Pediatrics, University Hospital Muenster, Muenster, Germany., Ozcelik U; Department of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey., Pohunek P; Department of Paediatrics, Charles University, 2nd Faculty of Medicine, University Hospital in Motol, Prague, Czech Republic., Raidt J; Department of General Pediatrics, University Hospital Muenster, Muenster, Germany., Robinson P; Royal Children's Hospital, Respiratory Medicine, Melbourne, Australia.; Faculty of Medicine Dentistry and Health Sciences, Paediatrics, University of Melbourne, Melbourne, Australia., Rovira-Amigo S; Hospital Vall d'Hebron, Neumología Infantil, 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.; Children's Hospital and Research Institute, Marienhospital Wesel, Wesel, Germany., Tamalet A; AP-HP, Sorbonne Université, Unité d'Exploration Fonctionnelle Respiratoire, Hôpital Armand-Trousseau, and Sorbonne Université, INSERM U938, Paris, France., Thouvenin G; AP-HP, Sorbonne Université, Unité d'Exploration Fonctionnelle Respiratoire, Hôpital Armand-Trousseau, and Sorbonne Université, INSERM U938, Paris, France., Ullmann N; Respiratory and Cystic Fibrosis Unit, Academic Pediatric Department, Bambino Gesù Children Hospital, IRCCS, Rome, Italy., Walker W; Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK., Yiallouros P; Medical School, University of Cyprus, Nicosia, Cyprus., Kuehni CE; Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.; Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics, Inselspital, University Hospital, University of Bern, Bern, Switzerland., Latzin P; Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.; Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics, Inselspital, University Hospital, 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., 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.
Jazyk:	angličtina
Zdroj:	ERJ open research [ERJ Open Res] 2024 Sep 30; Vol. 10 (5). Date of Electronic Publication: 2024 Sep 30 (Print Publication: 2024).
DOI:	10.1183/23120541.00253-2024
Abstrakt:	Introduction: Respiratory pathogens are frequently isolated from airway samples in primary ciliary dyskinesia (PCD) patients. Few studies have investigated associations between these pathogens and lung function, with current management based on evidence from cystic fibrosis. We investigated the association between commonly isolated respiratory pathogens and lung function in PCD patients. Methods: Using a cross-sectional design, we prospectively collected clinical and concurrent microbiology data from 408 participants with probable or confirmed PCD, aged ≥5 years, from 12 countries. We used Global Lung Function Initiative 2012 references to calculate forced expiratory volume in 1 s (FEV 1 ) z-scores. For 351 patients (86%) with complete data, we assessed the association of the four most frequently isolated pathogens with lung function by fitting multilevel linear models with country as random intercept, adjusted for age at diagnosis, age at lung function, use of antibiotic prophylaxis and body mass index z-scores. Results: Individuals with Pseudomonas aeruginosa growth in culture had significantly lower FEV 1 z-scores (β= -0.87, 95% CI -1.40- -0.34), adjusted for presence of Haemophilus influenzae , methicillin-sensitive Staphylococcus aureus and Streptococcus pneumoniae , and for covariates. When stratified by age, associations remained strong for adults but not for children. Results were similar when ciliary defects by transmission electron microscopy were included in the models and when restricting analysis to only confirmed PCD cases. Conclusions: We found that P. aeruginosa was associated with worse lung function in individuals with PCD, particularly adults. These findings suggest that it is prudent to aim for P. aeruginosa eradication in the first instance, and to treat exacerbations promptly in colonised patients. Competing Interests: Conflict of interest: M. Boon declares receiving grants or contracts from the Fund Alphonse and Jean Forton, managed by the King Baudouin Foundation, and by the Belgian Cystic Fibrosis Association (number 2020-J1810150-217926); and payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Vertex. Conflict of interest: S.B. Carr declares receiving payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Chiesi Pharmaceuticals, and participation on a Data Safety Monitoring Board or Advisory Board for Vertex Pharmaceuticals. Conflict of interest: S. Dillenhöfer declares receiving support for attending meetings and/or travel from Vertex (Advance Program 2022). Conflict of interest: B. Karadag declares receiving payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from VEM Ilac, Abdi Ibrahim and OMRON, and participating on a Data Safety Monitoring Board or Advisory Board for Abdi Ibrahim. Conflict of interest: C. Koerner-Rettberg declares receiving speaker honoraria from Berlin Chemie, and advisory board participation and being a member of the medical board of the German PCD patient organisation. Conflict of interest: V. Martinu declares having received grants or contracts from Ministry of Health of the Czech Republic (grant number NV19-07-00210) and participation on an advisory board for indication of tobramycin in noncystic fibrosis bronchiectasis for Chiesi, and receiving payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Chiesi Pharmaceuticals. Conflict of interest: A. Moreno-Galdó declares receiving personal payment for lectures from AstraZeneca, Sanofi-Pasteur and Janssen, receiving support for attending meetings or travel from Sanofi-Pasteur and Vivisol, participation on a Data Safety Monitoring Board or Advisory Board for AstraZeneca and Sanofi-Pasteur, and is President of the Spanish Society of Pediatric Pulmonology. Conflict of interest: U. Ozcelik is Head of the Turkish Respiratory Disease and Cystic Fibrosis Society (unpaid). Conflict of interest: P. Pohunek declares receiving payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from GlaxoSmithKline, AstraZeneca and Chiesi, and consulting fees, support for attending meetings and/or travel, and participation on a Data Safety Monitoring Board or Advisory Board from AstraZeneca and GlaxoSmithKline. Conflict of interest: J. Raidt declares receiving the following funds: DFG CRU326 RA3522/1. Conflict of interest: P. Latzin declares receiving grants or contracts from Vertex and OM Pharma, payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Vertex, Vifor and OM Pharma, and participating on a Data Safety Monitoring Board or Advisory Board for the following: Polyphor, Santhera (DMC), Vertex, OM Pharma, Vifor, Allecra and Sanofi Aventis. Conflict of interest: J.S. Lucas declares receiving support for the present manuscript (funds the clinical investigations (spirometry, microbiology)) from NHS England, to the institution; and grants or contracts from COST ACTION to fund the BEATPCD COST Action BM1407, which funded the network that undertook this work, and the institution managed the funding. Conflict of interest: The other authors have nothing to disclose. (Copyright ©The authors 2024.)
Databáze:	MEDLINE
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