ERJ Open Research (Sep 2024)
Associations between respiratory pathogens and lung function in primary ciliary dyskinesia: cross-sectional analysis from the PROVALF-PCD cohort
- Bruna Rubbo,
- Avni Kant,
- Kewei Zhang,
- Annalisa Allegorico,
- Simona Basilicata,
- Mieke Boon,
- Melissa Borrelli,
- Claudia Calogero,
- Siobhán B. Carr,
- Mary Carroll,
- Carolina Constant,
- Silvia Castillo Corullón,
- Harriet Corvol,
- Renato Cutrera,
- Stefanie Dillenhöfer,
- Nagehan Emiralioglu,
- Ela Eralp,
- Sanem Eryilmaz Polat,
- Laura Gardner,
- Yasemin Gokdemir,
- Amanda Harris,
- Claire Hogg,
- Bulent Karadag,
- Helene Kobbernagel,
- Cordula Koerner-Rettberg,
- Panayiotis Kouis,
- Natalie Lorent,
- Markella Marcou,
- June K. Mathin,
- Vendula Martinu,
- Antonio Moreno-Galdó,
- Lucy Morgan,
- Kim G. Nielsen,
- Heymut Omran,
- Ugur Ozcelik,
- Petr Pohunek,
- Johanna Raidt,
- Phil Robinson,
- Sandra Rovira-Amigo,
- Francesca Santamaria,
- Anne Schlegtendal,
- Aline Tamalet,
- Guillaume Thouvenin,
- Nicola Ullmann,
- Woolf Walker,
- Panayiotis Yiallouros,
- Claudia E. Kuehni,
- Philipp Latzin,
- Nicole Beydon,
- Jane S. Lucas
Affiliations
- Bruna Rubbo
- Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK
- Avni Kant
- Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK
- Kewei Zhang
- Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK
- Annalisa Allegorico
- Respiratory and Cystic Fibrosis Unit, Academic Pediatric Department, Bambino Gesù Children Hospital, IRCCS, Rome, Italy
- Simona Basilicata
- Department of Translational Medical Sciences, Pediatric Pulmonology, Federico II University, Naples, Italy
- Mieke Boon
- Department of Pediatrics, University Hospital Leuven, Leuven, Belgium
- Melissa Borrelli
- Department of Translational Medical Sciences, Pediatric Pulmonology, Federico II University, Naples, Italy
- Claudia Calogero
- Azienda Ospedaliero Universitaria Ospedale Pediatrico Meyer, Florence, Italy
- Siobhán B. Carr
- Royal Brompton and Harefield NHS Foundation Trust, Department of Paediatrics, Primary Ciliary Dyskinesia Centre, London, UK
- Mary Carroll
- Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK
- Carolina Constant
- Department of Pediatrics, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte and Faculty of Medicine, University of Lisbon, Lisbon, Portugal
- Silvia Castillo Corullón
- FE Pediatría y Neumología Infantil, Unidad de Neumología Infantil y Fibrosis Quística, Hospital Clínico de Valencia, Valencia, Spain
- Harriet Corvol
- AP-HP, Sorbonne Université, Unité d'Exploration Fonctionnelle Respiratoire, Hôpital Armand-Trousseau, and Sorbonne Université, INSERM U938, Paris, France
- Renato Cutrera
- Respiratory and Cystic Fibrosis Unit, Academic Pediatric Department, Bambino Gesù Children Hospital, IRCCS, Rome, Italy
- Stefanie Dillenhöfer
- Department of Paediatric Pulmonology, University Children's Hospital, Ruhr University Bochum, Katholisches Klinikum Bochum, Bochum, Germany
- Nagehan Emiralioglu
- Department of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey
- Ela Eralp
- Department of Pediatric Pulmonology, Marmara Universitesi, Istanbul, Turkey
- Sanem Eryilmaz Polat
- Department of Pediatrics, Division of Pediatric Pulmonology, Ankara City Hospital, Ankara, Turkey
- Laura Gardner
- Royal Brompton and Harefield NHS Foundation Trust, Department of Paediatrics, Primary Ciliary Dyskinesia Centre, London, UK
- Yasemin Gokdemir
- Department of Pediatric Pulmonology, Marmara Universitesi, Istanbul, Turkey
- Amanda Harris
- Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK
- Claire Hogg
- Royal Brompton and Harefield NHS Foundation Trust, Department of Paediatrics, Primary Ciliary Dyskinesia Centre, London, UK
- Bulent Karadag
- Department of Pediatric Pulmonology, Marmara Universitesi, Istanbul, Turkey
- Helene Kobbernagel
- Danish PCD Centre, Danish Paediatric Pulmonary Service, Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
- Cordula Koerner-Rettberg
- Department of Paediatric Pulmonology, University Children's Hospital, Ruhr University Bochum, Katholisches Klinikum Bochum, Bochum, Germany
- Panayiotis Kouis
- Medical School, University of Cyprus, Nicosia, Cyprus
- Natalie Lorent
- Department of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium
- Markella Marcou
- Medical School, University of Cyprus, Nicosia, Cyprus
- June K. Mathin
- Danish PCD Centre, Danish Paediatric Pulmonary Service, Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
- Vendula Martinu
- Department of Paediatrics, Charles University, 2nd Faculty of Medicine, University Hospital in Motol, Prague, Czech Republic
- Antonio Moreno-Galdó
- Hospital Vall d'Hebron, Neumología Infantil, Barcelona, Spain
- Lucy Morgan
- Concord Hospital, Concord Clinical School, University of Sydney, Department of Respiratory Medicine, Sydney, Australia
- Kim G. Nielsen
- Danish PCD Centre, Danish Paediatric Pulmonary Service, Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
- Heymut Omran
- Department of General Pediatrics, University Hospital Muenster, Muenster, Germany
- Ugur Ozcelik
- Department of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey
- Petr Pohunek
- Department of Paediatrics, Charles University, 2nd Faculty of Medicine, University Hospital in Motol, Prague, Czech Republic
- Johanna Raidt
- Department of General Pediatrics, University Hospital Muenster, Muenster, Germany
- Phil Robinson
- Royal Children's Hospital, Respiratory Medicine, Melbourne, Australia
- Sandra Rovira-Amigo
- Hospital Vall d'Hebron, Neumología Infantil, Barcelona, Spain
- Francesca Santamaria
- Department of Translational Medical Sciences, Pediatric Pulmonology, Federico II University, Naples, Italy
- Anne Schlegtendal
- Department of Paediatric Pulmonology, University Children's Hospital, Ruhr University Bochum, Katholisches Klinikum Bochum, Bochum, Germany
- Aline Tamalet
- AP-HP, Sorbonne Université, Unité d'Exploration Fonctionnelle Respiratoire, Hôpital Armand-Trousseau, and Sorbonne Université, INSERM U938, Paris, France
- Guillaume Thouvenin
- AP-HP, Sorbonne Université, Unité d'Exploration Fonctionnelle Respiratoire, Hôpital Armand-Trousseau, and Sorbonne Université, INSERM U938, Paris, France
- Nicola Ullmann
- Respiratory and Cystic Fibrosis Unit, Academic Pediatric Department, Bambino Gesù Children Hospital, IRCCS, Rome, Italy
- Woolf Walker
- Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK
- Panayiotis Yiallouros
- Medical School, University of Cyprus, Nicosia, Cyprus
- Claudia E. Kuehni
- Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland
- Philipp Latzin
- Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland
- Nicole Beydon
- AP-HP, Sorbonne Université, Unité d'Exploration Fonctionnelle Respiratoire, Hôpital Armand-Trousseau, and Sorbonne Université, INSERM U938, Paris, France
- Jane S. Lucas
- Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK
- DOI
- https://doi.org/10.1183/23120541.00253-2024
- Journal volume & issue
-
Vol. 10,
no. 5
Abstract
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 (FEV1) 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 FEV1 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.