Lung function in patients with Primary Ciliary Dyskinesia: an iPCD Cohort study
- Florian S Halbeisen1,
- Myrofora Goutaki1,2,
- Ben D Spycher1,2,
- Israel Amirav3,4,5,
- Laura Behan6,7,
- Mieke Boon8,
- Claire Hogg9,
- Carmen Casaulta2,10,
- Suzanne Crowley11,
- Eric G Haarman12,
- Bulent Karadag13,
- Cordula Koerner-Rettberg14,
- Michael R Loebinger15,
- Henryk Mazurek16,
- Lucy Morgan17,
- Kim G Nielsen18,
- Heymut Omran19,
- Francesca Santamaria20,
- Nicolaus Schwerk21,
- Guillaume Thouvenin22,23,24,
- Panayiotis Yiallouros25,
- Jane S Lucas6,
- Philipp Latzin2 and
- Claudia E Kuehni1,2
- 1Institute of Social and Preventive Medicine, University of Bern, Switzerland
- 2Paediatric Respiratory Medicine, Children's University Hospital of Bern, University of Bern, Switzerland
- 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
- 6Primary Ciliary Dyskinesia Centre, NIHR Respiratory Biomedical Research Centre, University of Southampton, UK
- 7School of Applied Psychology, University College Cork, Ireland
- 8Department of Paediatrics, University Hospital Gasthuisberg, Leuven, Belgium
- 9Department of Paediatrics, Primary Ciliary Dyskinesia Centre, Royal Brompton and Harefield Foundation Trust, London, UK
- 10on behalf of the Swiss PCD Group
- 11Unit for Paediatric Heart, Lung, Allergic Diseases, Rikshospitalet, Oslo, Norway
- 12Department of Pediatric Pulmonology, VU University Medical Center, Amsterdam, The Netherlands
- 13Department of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey
- 14Department of Paediatric Pneumology, University Children's Hospital of Ruhr University Bochum, Germany
- 15Host Defence Unit, Royal Brompton and Harefield NHS Foundation Trust, London, UK
- 16Department of Pneumonology and Cystic Fibrosis, Institute of Tuberculosis and Lung Disorders, ul.Prof.Rudnika 3b, 34-700 Rabka - Zdrój, Poland
- 17Department of Respiratory Medicine, Concord Hospital Clinical School, University of Sydney, Australia
- 18Danish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital, Denmark
- 19Dept of General Paediatrics and Adolescent Medicine, University Hospital Muenster, Muenster, Germany
- 20Department of Translational Medical Sciences, Federico II University, Napoli, Italy
- 21Clinic for paediatric pulmonology, allergiology and neonatology, Hannover Medical School, Germany
- 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
- 25Medical School, University of Cyprus, Nicosia, Cyprus
- Claudia Kuehni, Finkenhubelweg 11, 3012 Bern, Switzerland. E-mail: claudia.kuehni{at}ispm.unibe.ch
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.
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