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Eur Respir J. 2018 Aug 23;52(2). pii: 1801040. doi: 10.1183/13993003.01040-2018. Print 2018 Aug.

Lung function in patients with primary ciliary dyskinesia: an iPCD Cohort study.

Author information

1
Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
2
Paediatric Respiratory Medicine, Children's University Hospital of Bern, University of Bern, Bern, Switzerland.
3
The PCD Israeli Consortium.
4
Dept of Pediatrics, Faculty of Medicine, Bar IIan University, Ramat Gan, Israel.
5
Dept of Pediatrics, University of Medicine, Edmonton, AB, Canada.
6
Primary Ciliary Dyskinesia Centre, NIHR Respiratory Biomedical Research Centre, University of Southampton, Southampton, UK.
7
School of Applied Psychology, University College Cork, Cork, Ireland.
8
Dept of Paediatrics, University Hospital Gasthuisberg, Leuven, Belgium.
9
Dept of Paediatrics, Primary Ciliary Dyskinesia Centre, Royal Brompton and Harefield Foundation Trust, London, UK.
10
The Swiss PCD Group.
11
Unit for Paediatric Heart, Lung, Allergic Diseases, Rikshospitalet, Oslo, Norway.
12
Dept of Pediatric Pulmonology, VU University Medical Center, Amsterdam, The Netherlands.
13
Dept of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey.
14
Dept of Paediatric Pneumology, University Children's Hospital of Ruhr University Bochum, Bochum, Germany.
15
Host Defence Unit, Royal Brompton and Harefield NHS Foundation Trust, London, UK.
16
Dept of Pneumonology and Cystic Fibrosis, Institute of Tuberculosis and Lung Disorders, Rabka-Zdrój, Poland.
17
Dept of Respiratory Medicine, Concord Hospital Clinical School, University of Sydney, Sydney, Australia.
18
Danish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital, Copenhagen, Denmark.
19
Dept of General Paediatrics and Adolescent Medicine, University Hospital Muenster, Muenster, Germany.
20
Dept of Translational Medical Sciences, Federico II University, Napoli, Italy.
21
Clinic for Paediatric Pulmonology, Allergiology and Neonatology, Hannover Medical School, Hannover, Germany.
22
The French Reference Centre for Rare Lung Diseases.
23
Paediatric Pulmonary Dept, Trousseau Hospital APHP, Sorbonne Universities and Pierre et Marie Curie University, Paris, France.
24
INSERM U938-CRSA, Paris, France.
25
Medical School, University of Cyprus, Nicosia, Cyprus.

Abstract

Primary ciliary dyskinesia (PCD) has been considered a 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 patients. We calculated z-scores and % predicted values for forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) using the Global Lung Function Initiative 2012 values for 991 patients from the international PCD 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 emphasises the importance of early standardised care for all patients.

PMID:
30049738
DOI:
10.1183/13993003.01040-2018
[Indexed for MEDLINE]

Conflict of interest statement

Conflict of interest: N. Schwerk reports personal fees for lecturing from Novartis, Allergopharma and Infectopharm, and grants from FP7-ChILD EU, outside the submitted work. Conflict of interest: P. Yiallouros reports grants from European Union's Seventh Framework Programme under EG-GA (number 35404 BESTCILIA), during the conduct of the study. Conflict of interest: P. Latzin reports personal fees from Gilead, Novartis, Polyphor, Roche, Santhera, Schwabe, Vertex, Vifor and Zambon, outside the submitted work. Conflict of interest: H. Mazurek reports grants from Bestcilia, during the conduct of the study.

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