Lung function from school age to adulthood in primary ciliary dyskinesia.


Journal

The European respiratory journal
ISSN: 1399-3003
Titre abrégé: Eur Respir J
Pays: England
ID NLM: 8803460

Informations de publication

Date de publication:
10 2022
Historique:
received: 08 07 2021
accepted: 26 02 2022
pubmed: 19 3 2022
medline: 25 10 2022
entrez: 18 3 2022
Statut: epublish

Résumé

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 (FEV

Identifiants

pubmed: 35301251
pii: 13993003.01918-2021
doi: 10.1183/13993003.01918-2021
pmc: PMC9582507
pii:
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

Copyright ©The authors 2022.

Déclaration de conflit d'intérêts

Conflict of interest: P. Latzin reports grants from Vertex and Vifor, payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Vertex, Vifor and OM Pharma, and participation on a data safety monitoring board or advisory board for Polyphor, Santhera (DMC), Vertex, OM Pharma and Vifor. M.R. Loebinger reports consultancy fees from Insmed, AstraZeneca and Grifols, and payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Insmed and Grifols. The remaining authors disclose no potential conflicts of interest.

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Auteurs

Florian S Halbeisen (FS)

Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
Basel Institute for Clinical Epidemiology and Biostatistics, Dept of Clinical Research, University Hospital Basel, University of Basel, Basel, Switzerland.
These authors contributed equally to the manuscript.

Eva S L Pedersen (ESL)

Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
These authors contributed equally to the manuscript.

Myrofora Goutaki (M)

Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
Division of Paediatric Respiratory Medicine and Allergology, Dept of Paediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

Ben D Spycher (BD)

Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.

Israel Amirav (I)

The PCD Israeli Consortium.
Dept of Pediatrics, Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Dept of Pediatrics, University of Alberta, Edmonton, AB, Canada.

Mieke Boon (M)

Dept of Paediatrics, University Hospital Gasthuisberg, Leuven, Belgium.

Malena Cohen-Cymberknoh (M)

Pediatric Pulmonology Unit and Cystic fibrosis Center, Hadassah Medical Center and Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel.

Suzanne Crowley (S)

Paediatric Dept of Allergy and Lung Diseases, Oslo University Hospital, Oslo, Norway.

Nagehan Emiralioglu (N)

Dept of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Eric G Haarman (EG)

Dept of Pediatric Pulmonology, Emma Children's Hospital, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.

Bulent Karadag (B)

Dept of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey.

Cordula Koerner-Rettberg (C)

Dept of Paediatric Pneumology, University Children's Hospital of Ruhr University Bochum, Bochum, Germany.

Philipp Latzin (P)

Division of Paediatric Respiratory Medicine and Allergology, Dept of Paediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
On behalf of the Swiss PCD Group.

Michael R Loebinger (MR)

Host Defence Unit, Royal Brompton and Harefield NHS Foundation Trust and Imperial College London, London, UK.

Jane S Lucas (JS)

Primary Ciliary Dyskinesia Centre, NIHR Respiratory Biomedical Research Centre, University of Southampton and University Hospital, Southampton, UK.

Henryk Mazurek (H)

Dept of Pneumonology and Cystic Fibrosis, Institute of Tuberculosis and Lung Disorders, Rabka -‌ Zdrój, Poland.

Lucy Morgan (L)

Dept of Respiratory Medicine, Concord Hospital Clinical School, University of Sydney, Sydney, Australia.

June Marthin (J)

Danish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital, Copenhagen, Denmark.

Petr Pohunek (P)

Paediatric Dept, 2nd Faculty of Medicine, Charles University Prague and University Hospital Motol, Prague, Czech Republic.

Francesca Santamaria (F)

Dept of Translational Medical Sciences, Federico II University, Napoli, Italy.

Nicolaus Schwerk (N)

Clinic for Paediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.

Guillaume Thouvenin (G)

Pediatric Pulmonology Dept, APHP Hôpital Trousseau, Sorbonne Université, Centre de Recherche Saint-Antoine (CRSA), Inserm UMR_S938, Paris, France.
On behalf of the French Reference Centre for Rare Lung Diseases RespiRare.

Panayiotis Yiallouros (P)

Medical School, University of Cyprus, Nicosia, Cyprus.

Kim G Nielsen (KG)

Danish PCD Centre Copenhagen, Paediatric Pulmonary Service, Copenhagen University Hospital, Copenhagen, Denmark.
Dept of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark.

Claudia E Kuehni (CE)

Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland claudia.kuehni@ispm.unibe.ch.
Division of Paediatric Respiratory Medicine and Allergology, Dept of Paediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

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Classifications MeSH