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Associations between respiratory pathogens and lung function in primary ciliary dyskinesia: cross-sectional analysis from the PROVALF-PCD cohort
B. Rubbo, A. Kant, K. Zhang, A. Allegorico, S. Basilicata, M. Boon, M. Borrelli, C. Calogero, SB. Carr, M. Carroll, C. Constant, S. Castillo Corullón, H. Corvol, R. Cutrera, S. Dillenhöfer, N. Emiralioglu, E. Eralp, S. Eryilmaz Polat, L. Gardner,...
Status neindexováno Jazyk angličtina Země Anglie, Velká Británie
Typ dokumentu časopisecké články
NLK
Directory of Open Access Journals
od 2015
PubMed Central
od 2015
Europe PubMed Central
od 2015
Open Access Digital Library
od 2015-01-01
ROAD: Directory of Open Access Scholarly Resources
od 2015
- Publikační typ
- časopisecké články MeSH
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.
Azienda Ospedaliero Universitaria Ospedale Pediatrico Meyer Florence Italy
Children's Hospital and Research Institute Marienhospital Wesel Wesel Germany
Clinical and Experimental Sciences University of Southampton Faculty of Medicine Southampton UK
Department of General Pediatrics University Hospital Muenster Muenster 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
Department of Pediatrics University Hospital Leuven Leuven Belgium
Department of Respiratory Diseases University Hospitals Leuven Leuven Belgium
Hospital Vall d'Hebron Neumología Infantil Barcelona Spain
Institute of Social and Preventive Medicine University of Bern Bern Switzerland
Medical School University of Cyprus Nicosia Cyprus
Royal Children's Hospital Respiratory Medicine Melbourne Australia
Citace poskytuje Crossref.org
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- $a Rubbo, Bruna $u Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK $u Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK $1 https://orcid.org/0000000216298601
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- $a Associations between respiratory pathogens and lung function in primary ciliary dyskinesia: cross-sectional analysis from the PROVALF-PCD cohort / $c B. Rubbo, A. Kant, K. Zhang, A. Allegorico, S. Basilicata, M. Boon, M. Borrelli, C. Calogero, SB. Carr, M. Carroll, C. Constant, S. Castillo Corullón, H. Corvol, R. Cutrera, S. Dillenhöfer, N. Emiralioglu, E. Eralp, S. Eryilmaz Polat, L. Gardner, Y. Gokdemir, A. Harris, C. Hogg, B. Karadag, H. Kobbernagel, C. Koerner-Rettberg, P. Kouis, N. Lorent, M. Marcou, JK. Mathin, V. Martinu, A. Moreno-Galdó, L. Morgan, KG. Nielsen, H. Omran, U. Ozcelik, P. Pohunek, J. Raidt, P. Robinson, S. Rovira-Amigo, F. Santamaria, A. Schlegtendal, A. Tamalet, G. Thouvenin, N. Ullmann, W. Walker, P. Yiallouros, CE. Kuehni, P. Latzin, N. Beydon, JS. Lucas
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- $a 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.
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