Associations between respiratory pathogens and lung function in primary ciliary dyskinesia: cross-sectional analysis from the PROVALF-PCD cohort
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 assoc...
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European Respiratory Society
2024-09-01
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| Series: | ERJ Open Research |
| Online Access: | http://openres.ersjournals.com/content/10/5/00253-2024.full |
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| author | 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 |
| author_facet | 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 |
| author_sort | Bruna Rubbo |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-64e53d57433e415b84d128a5a43870e1 |
| institution | Kabale University |
| issn | 2312-0541 |
| language | English |
| publishDate | 2024-09-01 |
| publisher | European Respiratory Society |
| record_format | Article |
| series | ERJ Open Research |
| spelling | doaj-art-64e53d57433e415b84d128a5a43870e12024-11-11T16:33:07ZengEuropean Respiratory SocietyERJ Open Research2312-05412024-09-0110510.1183/23120541.00253-202400253-2024Associations between respiratory pathogens and lung function in primary ciliary dyskinesia: cross-sectional analysis from the PROVALF-PCD cohortBruna Rubbo0Avni Kant1Kewei Zhang2Annalisa Allegorico3Simona Basilicata4Mieke Boon5Melissa Borrelli6Claudia Calogero7Siobhán B. Carr8Mary Carroll9Carolina Constant10Silvia Castillo Corullón11Harriet Corvol12Renato Cutrera13Stefanie Dillenhöfer14Nagehan Emiralioglu15Ela Eralp16Sanem Eryilmaz Polat17Laura Gardner18Yasemin Gokdemir19Amanda Harris20Claire Hogg21Bulent Karadag22Helene Kobbernagel23Cordula Koerner-Rettberg24Panayiotis Kouis25Natalie Lorent26Markella Marcou27June K. Mathin28Vendula Martinu29Antonio Moreno-Galdó30Lucy Morgan31Kim G. Nielsen32Heymut Omran33Ugur Ozcelik34Petr Pohunek35Johanna Raidt36Phil Robinson37Sandra Rovira-Amigo38Francesca Santamaria39Anne Schlegtendal40Aline Tamalet41Guillaume Thouvenin42Nicola Ullmann43Woolf Walker44Panayiotis Yiallouros45Claudia E. Kuehni46Philipp Latzin47Nicole Beydon48Jane S. Lucas49 Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK Respiratory and Cystic Fibrosis Unit, Academic Pediatric Department, Bambino Gesù Children Hospital, IRCCS, Rome, Italy Department of Translational Medical Sciences, Pediatric Pulmonology, Federico II University, Naples, Italy Department of Pediatrics, University Hospital Leuven, Leuven, Belgium Department of Translational Medical Sciences, Pediatric Pulmonology, Federico II University, Naples, Italy Azienda Ospedaliero Universitaria Ospedale Pediatrico Meyer, Florence, Italy Royal Brompton and Harefield NHS Foundation Trust, Department of Paediatrics, Primary Ciliary Dyskinesia Centre, London, UK Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK Department of Pediatrics, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte and Faculty of Medicine, University of Lisbon, Lisbon, Portugal FE Pediatría y Neumología Infantil, Unidad de Neumología Infantil y Fibrosis Quística, Hospital Clínico de Valencia, Valencia, Spain AP-HP, Sorbonne Université, Unité d'Exploration Fonctionnelle Respiratoire, Hôpital Armand-Trousseau, and Sorbonne Université, INSERM U938, Paris, France Respiratory and Cystic Fibrosis Unit, Academic Pediatric Department, Bambino Gesù Children Hospital, IRCCS, Rome, Italy Department of Paediatric Pulmonology, University Children's Hospital, Ruhr University Bochum, Katholisches Klinikum Bochum, Bochum, Germany Department of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey Department of Pediatric Pulmonology, Marmara Universitesi, Istanbul, Turkey Department of Pediatrics, Division of Pediatric Pulmonology, Ankara City Hospital, Ankara, Turkey Royal Brompton and Harefield NHS Foundation Trust, Department of Paediatrics, Primary Ciliary Dyskinesia Centre, London, UK Department of Pediatric Pulmonology, Marmara Universitesi, Istanbul, Turkey Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK Royal Brompton and Harefield NHS Foundation Trust, Department of Paediatrics, Primary Ciliary Dyskinesia Centre, London, UK Department of Pediatric Pulmonology, Marmara Universitesi, Istanbul, Turkey Danish PCD Centre, Danish Paediatric Pulmonary Service, Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark Department of Paediatric Pulmonology, University Children's Hospital, Ruhr University Bochum, Katholisches Klinikum Bochum, Bochum, Germany Medical School, University of Cyprus, Nicosia, Cyprus Department of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium Medical School, University of Cyprus, Nicosia, Cyprus Danish PCD Centre, Danish Paediatric Pulmonary Service, Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark Department of Paediatrics, Charles University, 2nd Faculty of Medicine, University Hospital in Motol, Prague, Czech Republic Hospital Vall d'Hebron, Neumología Infantil, Barcelona, Spain Concord Hospital, Concord Clinical School, University of Sydney, Department of Respiratory Medicine, Sydney, Australia Danish PCD Centre, Danish Paediatric Pulmonary Service, Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark Department of General Pediatrics, University Hospital Muenster, Muenster, Germany Department of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey Department of Paediatrics, Charles University, 2nd Faculty of Medicine, University Hospital in Motol, Prague, Czech Republic Department of General Pediatrics, University Hospital Muenster, Muenster, Germany Royal Children's Hospital, Respiratory Medicine, Melbourne, Australia Hospital Vall d'Hebron, Neumología Infantil, Barcelona, Spain Department of Translational Medical Sciences, Pediatric Pulmonology, Federico II University, Naples, Italy Department of Paediatric Pulmonology, University Children's Hospital, Ruhr University Bochum, Katholisches Klinikum Bochum, Bochum, Germany AP-HP, Sorbonne Université, Unité d'Exploration Fonctionnelle Respiratoire, Hôpital Armand-Trousseau, and Sorbonne Université, INSERM U938, Paris, France AP-HP, Sorbonne Université, Unité d'Exploration Fonctionnelle Respiratoire, Hôpital Armand-Trousseau, and Sorbonne Université, INSERM U938, Paris, France Respiratory and Cystic Fibrosis Unit, Academic Pediatric Department, Bambino Gesù Children Hospital, IRCCS, Rome, Italy Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK Medical School, University of Cyprus, Nicosia, Cyprus Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland AP-HP, Sorbonne Université, Unité d'Exploration Fonctionnelle Respiratoire, Hôpital Armand-Trousseau, and Sorbonne Université, INSERM U938, Paris, France Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK 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.http://openres.ersjournals.com/content/10/5/00253-2024.full |
| spellingShingle | 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 Associations between respiratory pathogens and lung function in primary ciliary dyskinesia: cross-sectional analysis from the PROVALF-PCD cohort ERJ Open Research |
| title | Associations between respiratory pathogens and lung function in primary ciliary dyskinesia: cross-sectional analysis from the PROVALF-PCD cohort |
| title_full | Associations between respiratory pathogens and lung function in primary ciliary dyskinesia: cross-sectional analysis from the PROVALF-PCD cohort |
| title_fullStr | Associations between respiratory pathogens and lung function in primary ciliary dyskinesia: cross-sectional analysis from the PROVALF-PCD cohort |
| title_full_unstemmed | Associations between respiratory pathogens and lung function in primary ciliary dyskinesia: cross-sectional analysis from the PROVALF-PCD cohort |
| title_short | Associations between respiratory pathogens and lung function in primary ciliary dyskinesia: cross-sectional analysis from the PROVALF-PCD cohort |
| title_sort | associations between respiratory pathogens and lung function in primary ciliary dyskinesia cross sectional analysis from the provalf pcd cohort |
| url | http://openres.ersjournals.com/content/10/5/00253-2024.full |
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