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Lung function in patients with Primary Ciliary Dyskinesia: an iPCD Cohort study

Florian S Halbeisen, Myrofora Goutaki, Ben D Spycher, Israel Amirav, Laura Behan, Mieke Boon, Claire Hogg, Carmen Casaulta, Suzanne Crowley, Eric G Haarman, Bulent Karadag, Cordula Koerner-Rettberg, Michael R Loebinger, Henryk Mazurek, Lucy Morgan, Kim G Nielsen, Heymut Omran, Francesca Santamaria, Nicolaus Schwerk, Guillaume Thouvenin, Panayiotis Yiallouros, Jane S Lucas, Philipp Latzin, Claudia E Kuehni
European Respiratory Journal 2018; DOI: 10.1183/13993003.01040-2018
Florian S Halbeisen
1Institute of Social and Preventive Medicine, University of Bern, Switzerland
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Myrofora Goutaki
1Institute of Social and Preventive Medicine, University of Bern, Switzerland
2Paediatric Respiratory Medicine, Children's University Hospital of Bern, University of Bern, Switzerland
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  • ORCID record for Myrofora Goutaki
Ben D Spycher
1Institute of Social and Preventive Medicine, University of Bern, Switzerland
2Paediatric Respiratory Medicine, Children's University Hospital of Bern, University of Bern, Switzerland
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Israel Amirav
3on behalf of the PCD Israeli Consortium
4Department of Pediatrics, Faculty of Medicine, Bar IIan University, Israel
5Department of Pediatrics, University of Medicine, Edmonton AB, Canada
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Laura Behan
6Primary Ciliary Dyskinesia Centre, NIHR Respiratory Biomedical Research Centre, University of Southampton, UK
7School of Applied Psychology, University College Cork, Ireland
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Mieke Boon
8Department of Paediatrics, University Hospital Gasthuisberg, Leuven, Belgium
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Claire Hogg
9Department of Paediatrics, Primary Ciliary Dyskinesia Centre, Royal Brompton and Harefield Foundation Trust, London, UK
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Carmen Casaulta
2Paediatric Respiratory Medicine, Children's University Hospital of Bern, University of Bern, Switzerland
10on behalf of the Swiss PCD Group
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Suzanne Crowley
11Unit for Paediatric Heart, Lung, Allergic Diseases, Rikshospitalet, Oslo, Norway
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Eric G Haarman
12Department of Pediatric Pulmonology, VU University Medical Center, Amsterdam, The Netherlands
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Bulent Karadag
13Department of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey
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Cordula Koerner-Rettberg
14Department of Paediatric Pneumology, University Children's Hospital of Ruhr University Bochum, Germany
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Michael R Loebinger
15Host Defence Unit, Royal Brompton and Harefield NHS Foundation Trust, London, UK
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Henryk Mazurek
16Department of Pneumonology and Cystic Fibrosis, Institute of Tuberculosis and Lung Disorders, ul.Prof.Rudnika 3b, 34-700 Rabka - Zdrój, Poland
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Lucy Morgan
17Department of Respiratory Medicine, Concord Hospital Clinical School, University of Sydney, Australia
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Kim G Nielsen
18Danish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital, Denmark
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Heymut Omran
19Dept of General Paediatrics and Adolescent Medicine, University Hospital Muenster, Muenster, Germany
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Francesca Santamaria
20Department of Translational Medical Sciences, Federico II University, Napoli, Italy
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Nicolaus Schwerk
21Clinic for paediatric pulmonology, allergiology and neonatology, Hannover Medical School, Germany
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Guillaume Thouvenin
22on behalf of the French Reference Centre for Rare Lung Diseases
23Paediatric Pulmonary Department, Trousseau Hospital APHP, Sorbonne Universities and Pierre et Marie Curie University, Paris, France
24INSERM U938-CRSA, Paris, France
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Panayiotis Yiallouros
25Medical School, University of Cyprus, Nicosia, Cyprus
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Jane S Lucas
6Primary Ciliary Dyskinesia Centre, NIHR Respiratory Biomedical Research Centre, University of Southampton, UK
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Philipp Latzin
2Paediatric Respiratory Medicine, Children's University Hospital of Bern, University of Bern, Switzerland
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Claudia E Kuehni
1Institute of Social and Preventive Medicine, University of Bern, Switzerland
2Paediatric Respiratory Medicine, Children's University Hospital of Bern, University of Bern, Switzerland
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Abstract

Primary ciliary dyskinesia (PCD) has been considered to be relatively mild disease, especially compared to cystic fibrosis (CF), but studies on lung function in PCD patients have been few and small.

This study compared lung function from spirometry of PCD patients to normal reference values and to published data from CF. We calculated z-scores and percentage of predicted values for FEV1 and FVC using the Global Lung Function Initiative 2012 for 991 patients from the international PCD (iPCD) Cohort. We then assessed associations with age, sex, country, diagnostic certainty, organ laterality, body mass index and age at diagnosis in linear regression models. Lung function in PCD patients was reduced compared to reference values in both sexes and all age groups. Children aged 6–9 years had the smallest impairment (FEV1 z-score −0.84 [−1.03 to −0.65], FVC z-score −0.31 [−0.51 to −0.11]). Compared to CF patients, FEV1 was similarly reduced in children (age 6–9 years PCD, 91% [88–93%]; CF, 90% [88–91%]), but less impaired in young adults (age 18–21 years PCD, 79% [76–82%]; CF, 66% [65–68%]). The results suggest that PCD affects lung function from early in life, which emphasizes the importance of early, standardised care for all patients.

Footnotes

Conflict of interest: Dr. Halbeisen has nothing to disclose.

Conflict of interest: Dr. Goutaki has nothing to disclose.

Conflict of interest: Dr. Spycher has nothing to disclose.

Conflict of interest: Dr. Amirav has nothing to disclose.

Conflict of interest: Dr. Behan has nothing to disclose.

Conflict of interest: Dr. Boon has nothing to disclose.

Conflict of interest: Dr. Hogg has nothing to disclose.

Conflict of interest: Dr. Casaulta has nothing to disclose.

Conflict of interest: Dr. Crowley has nothing to disclose.

Conflict of interest: Dr. Haarman has nothing to disclose.

Conflict of interest: Prof. Karadag has nothing to disclose.

Conflict of interest: Dr. Koerner-Rettberg has nothing to disclose.

Conflict of interest: Dr. Loebinger has nothing to disclose.

Conflict of interest: Dr. Mazurek reports grants from Bestcilia, during the conduct of the study;.

Conflict of interest: Dr. Morgan has nothing to disclose.

Conflict of interest: Dr. Nielsen has nothing to disclose.

Conflict of interest: Dr. Omran has nothing to disclose.

Conflict of interest: Dr. Santamaria has nothing to disclose.

Conflict of interest: Dr. Schwerk reports personal fees from paid lectures for the following companies: Novartis, Allergopharma, Infectopharm, grants from FP7-ChILD EU, outside the submitted work; .

Conflict of interest: Dr. Thouvenin has nothing to disclose.

Conflict of interest: Dr. Yiallouros reports grants from European Union's Seventh Framework Programme under EG-GA, during the conduct of the study; .

Conflict of interest: Dr. Lucas has nothing to disclose.

Conflict of interest: Dr. Latzin has nothing to disclose.

Conflict of interest: Prof. Kuehni has nothing to disclose.

This is a PDF-only article. Please click on the PDF link above to read it.

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Lung function in patients with Primary Ciliary Dyskinesia: an iPCD Cohort study
Florian S Halbeisen, Myrofora Goutaki, Ben D Spycher, Israel Amirav, Laura Behan, Mieke Boon, Claire Hogg, Carmen Casaulta, Suzanne Crowley, Eric G Haarman, Bulent Karadag, Cordula Koerner-Rettberg, Michael R Loebinger, Henryk Mazurek, Lucy Morgan, Kim G Nielsen, Heymut Omran, Francesca Santamaria, Nicolaus Schwerk, Guillaume Thouvenin, Panayiotis Yiallouros, Jane S Lucas, Philipp Latzin, Claudia E Kuehni
European Respiratory Journal Jan 2018, 1801040; DOI: 10.1183/13993003.01040-2018

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Lung function in patients with Primary Ciliary Dyskinesia: an iPCD Cohort study
Florian S Halbeisen, Myrofora Goutaki, Ben D Spycher, Israel Amirav, Laura Behan, Mieke Boon, Claire Hogg, Carmen Casaulta, Suzanne Crowley, Eric G Haarman, Bulent Karadag, Cordula Koerner-Rettberg, Michael R Loebinger, Henryk Mazurek, Lucy Morgan, Kim G Nielsen, Heymut Omran, Francesca Santamaria, Nicolaus Schwerk, Guillaume Thouvenin, Panayiotis Yiallouros, Jane S Lucas, Philipp Latzin, Claudia E Kuehni
European Respiratory Journal Jan 2018, 1801040; DOI: 10.1183/13993003.01040-2018
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