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Lung function from school age to adulthood in primary ciliary dyskinesia
FS. Halbeisen, ESL. Pedersen, M. Goutaki, BD. Spycher, I. Amirav, M. Boon, M. Cohen-Cymberknoh, S. Crowley, N. Emiralioglu, EG. Haarman, B. Karadag, C. Koerner-Rettberg, P. Latzin, MR. Loebinger, JS. Lucas, H. Mazurek, L. Morgan, J. Marthin, P....
Language English Country England, Great Britain
Document type Journal Article, Research Support, Non-U.S. Gov't
NLK
Free Medical Journals
from 1994 to 18 months ago
Open Access Digital Library
from 1988-01-01
- MeSH
- Child MeSH
- Adult MeSH
- Cohort Studies MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Lung MeSH
- Ciliary Motility Disorders * MeSH
- Forced Expiratory Volume MeSH
- Vital Capacity MeSH
- Check Tag
- Child MeSH
- Adult MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
Primary ciliary dyskinesia (PCD) presents with symptoms early in life and the disease course may be progressive, but longitudinal data on lung function are scarce. This multinational cohort study describes lung function trajectories in children, adolescents and young adults with PCD. We analysed data from 486 patients with repeated lung function measurements obtained between the age of 6 and 24 years from the International PCD Cohort and calculated z-scores for forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC) and FEV1/FVC ratio using the Global Lung Function Initiative 2012 references. We described baseline lung function and change of lung function over time and described their associations with possible determinants in mixed-effects linear regression models. Overall, FEV1, FVC and FEV1/FVC z-scores declined over time (average crude annual FEV1 decline was -0.07 z-scores), but not at the same rate for all patients. FEV1 z-scores improved over time in 21% of patients, remained stable in 40% and declined in 39%. Low body mass index was associated with poor baseline lung function and with further decline. Results differed by country and ultrastructural defect, but we found no evidence of differences by sex, calendar year of diagnosis, age at diagnosis, diagnostic certainty or laterality defect. Our study shows that on average lung function in PCD declines throughout the entire period of lung growth, from childhood to young adult age, even among patients treated in specialised centres. It is essential to develop strategies to reverse this tendency and improve prognosis.
Dept of Clinical Medicine University of Copenhagen Copenhagen Denmark
Dept of Paediatrics University Hospital Gasthuisberg Leuven Belgium
Dept of Pediatric Pulmonology Hacettepe University Faculty of Medicine Ankara Turkey
Dept of Pediatric Pulmonology Marmara University School of Medicine Istanbul Turkey
Dept of Pediatrics Faculty of Medicine Tel Aviv University Tel Aviv Israel
Dept of Pediatrics University of Alberta Edmonton AB Canada
Dept of Respiratory Medicine Concord Hospital Clinical School University of Sydney Sydney Australia
Dept of Translational Medical Sciences Federico 2 University Napoli Italy
Institute of Social and Preventive Medicine University of Bern Bern Switzerland
Medical School University of Cyprus Nicosia Cyprus
On behalf of the French Reference Centre for Rare Lung Diseases RespiRare
On behalf of the Swiss PCD Group
Paediatric Dept of Allergy and Lung Diseases Oslo University Hospital Oslo Norway
References provided by Crossref.org
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