-
Autor
Amirav, Israel 1 Boon, Mieke 1 Cohen-Cymberknoh, Malena 1 Crowley, Suzanne 1 Emiralioglu, Nagehan 1 Goutaki, Myrofora 1 Haarman, Eric G 1 Halbeisen, Florian S 1 Karadag, Bulent 1 Koerner-Rettberg, Cordula 1 Kuehni, Claudia E 1 Latzin, Philipp 1 Loebinger, Michael R 1 Lucas, Jane S 1 Marthin, June 1 Mazurek, Henryk 1 Morgan, Lucy 1 Nielsen, Kim G 1 Pedersen, Eva S L 1 Pohunek, Petr 1
-
Pracoviště
Basel Institute for Clinical Epidemio... 1 Clinic for Paediatric Pneumology Alle... 1 Danish PCD Centre Copenhagen Paediatr... 1 Dept of Clinical Medicine University ... 1 Dept of Paediatric Pneumology Univers... 1 Dept of Paediatrics University Hospit... 1 Dept of Pediatric Pulmonology Emma Ch... 1 Dept of Pediatric Pulmonology Hacette... 1 Dept of Pediatric Pulmonology Marmara... 1 Dept of Pediatrics Faculty of Medicin... 1 Dept of Pediatrics University of Albe... 1 Dept of Pneumonology and Cystic Fibro... 1 Dept of Respiratory Medicine Concord ... 1 Dept of Translational Medical Science... 1 Division of Paediatric Respiratory Me... 1 Host Defence Unit Royal Brompton and ... 1 Institute of Social and Preventive Me... 1 Medical School University of Cyprus N... 1 On behalf of the French Reference Cen... 1 On behalf of the Swiss PCD Group 1
- Formát
- Publikační typ
- Check Tag
- Kategorie
- Jazyk
- Země
- Časopis/zdroj
- Dostupnost
- Vlastník
-
Autor
Amirav, Israel 1 Boon, Mieke 1 Cohen-Cymberknoh, Malena 1 Crowley, Suzanne 1 Emiralioglu, Nagehan 1 Goutaki, Myrofora 1 Haarman, Eric G 1 Halbeisen, Florian S 1 Karadag, Bulent 1 Koerner-Rettberg, Cordula 1 Kuehni, Claudia E 1 Latzin, Philipp 1 Loebinger, Michael R 1 Lucas, Jane S 1 Marthin, June 1 Mazurek, Henryk 1 Morgan, Lucy 1 Nielsen, Kim G 1 Pedersen, Eva S L 1 Pohunek, Petr 1
-
Pracoviště
Basel Institute for Clinical Epidemio... 1 Clinic for Paediatric Pneumology Alle... 1 Danish PCD Centre Copenhagen Paediatr... 1 Dept of Clinical Medicine University ... 1 Dept of Paediatric Pneumology Univers... 1 Dept of Paediatrics University Hospit... 1 Dept of Pediatric Pulmonology Emma Ch... 1 Dept of Pediatric Pulmonology Hacette... 1 Dept of Pediatric Pulmonology Marmara... 1 Dept of Pediatrics Faculty of Medicin... 1 Dept of Pediatrics University of Albe... 1 Dept of Pneumonology and Cystic Fibro... 1 Dept of Respiratory Medicine Concord ... 1 Dept of Translational Medical Science... 1 Division of Paediatric Respiratory Me... 1 Host Defence Unit Royal Brompton and ... 1 Institute of Social and Preventive Me... 1 Medical School University of Cyprus N... 1 On behalf of the French Reference Cen... 1 On behalf of the Swiss PCD Group 1
- Formát
- Publikační typ
- Check Tag
- Kategorie
- Jazyk
- Země
- Časopis/zdroj
- Dostupnost
- Vlastník
-
Halbeisen, Florian S
Autor Halbeisen, Florian S 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
- Pedersen, Eva S L
- Goutaki, Myrofora
- Spycher, Ben D
- Amirav, Israel
- Boon, Mieke
- Cohen-Cymberknoh, Malena
- Crowley, Suzanne
- Emiralioglu, Nagehan
- Haarman, Eric G
Open Access Digital Library od 1988-01-01
PubMed
35301251
DOI
10.1183/13993003.01918-2021
Knihovny.cz E-zdroje
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.
- MeSH
- dítě MeSH
- dospělí MeSH
- kohortové studie MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- plíce MeSH
- poruchy ciliární motility * MeSH
- usilovný výdechový objem MeSH
- vitální kapacita MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
Sdílet
Název dokumentu
Po ukončení testovacího provozu bude odkaz přesměrován adresu produkční verze portálu Medvik.