Back
Full-text
Journal article

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

  • Halbeisen FS Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
  • Goutaki M Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
  • Spycher BD Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
  • Amirav I The PCD Israeli Consortium.
  • Behan L Primary Ciliary Dyskinesia Centre, NIHR Respiratory Biomedical Research Centre, University of Southampton, Southampton, UK.
  • Boon M Dept of Paediatrics, University Hospital Gasthuisberg, Leuven, Belgium.
  • Hogg C Dept of Paediatrics, Primary Ciliary Dyskinesia Centre, Royal Brompton and Harefield Foundation Trust, London, UK.
  • Casaulta C Paediatric Respiratory Medicine, Children's University Hospital of Bern, University of Bern, Bern, Switzerland.
  • Crowley S Unit for Paediatric Heart, Lung, Allergic Diseases, Rikshospitalet, Oslo, Norway.
  • Haarman EG Dept of Pediatric Pulmonology, VU University Medical Center, Amsterdam, The Netherlands.
  • Karadag B Dept of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey.
  • Koerner-Rettberg C Dept of Paediatric Pneumology, University Children's Hospital of Ruhr University Bochum, Bochum, Germany.
  • Loebinger MR Host Defence Unit, Royal Brompton and Harefield NHS Foundation Trust, London, UK.
  • Mazurek H Dept of Pneumonology and Cystic Fibrosis, Institute of Tuberculosis and Lung Disorders, Rabka-Zdrój, Poland.
  • Morgan L Dept of Respiratory Medicine, Concord Hospital Clinical School, University of Sydney, Sydney, Australia.
  • Nielsen KG Danish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital, Copenhagen, Denmark.
  • Omran H Dept of General Paediatrics and Adolescent Medicine, University Hospital Muenster, Muenster, Germany.
  • Santamaria F Dept of Translational Medical Sciences, Federico II University, Napoli, Italy.
  • Schwerk N Clinic for Paediatric Pulmonology, Allergiology and Neonatology, Hannover Medical School, Hannover, Germany.
  • Thouvenin G The French Reference Centre for Rare Lung Diseases.
  • Yiallouros P Medical School, University of Cyprus, Nicosia, Cyprus.
  • Lucas JS Primary Ciliary Dyskinesia Centre, NIHR Respiratory Biomedical Research Centre, University of Southampton, Southampton, UK.
  • Latzin P Paediatric Respiratory Medicine, Children's University Hospital of Bern, University of Bern, Bern, Switzerland.
  • Kuehni CE Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
Show more…
  • 2018-07-28
Published in:
  • The European respiratory journal. - 2018
Adolescent
Adult
Age Factors
Body Mass Index
Child
Child, Preschool
Ciliary Motility Disorders
Cystic Fibrosis
Female
Forced Expiratory Volume
Humans
Infant
Infant, Newborn
Internationality
Linear Models
Lung
Male
Middle Aged
Reference Values
Retrospective Studies
Sex Factors
Spirometry
Vital Capacity
Young Adult
English 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.
Language
  • English
Open access status
bronze
Identifiers
Persistent URL
https://sonar.rero.ch/global/documents/166936
Statistics
Document views: 30 File downloads:
  • fulltext.pdf: 0