Lung function from school age to adulthood in primary ciliary dyskinesia
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11130%2F22%3A10442008" target="_blank" >RIV/00216208:11130/22:10442008 - isvavai.cz</a>
Alternative codes found
RIV/00064203:_____/22:10442008
Result on the web
<a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=92UwndM1IZ" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=92UwndM1IZ</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1183/13993003.01918-2021" target="_blank" >10.1183/13993003.01918-2021</a>
Alternative languages
Result language
angličtina
Original language name
Lung function from school age to adulthood in primary ciliary dyskinesia
Original language description
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 (iPCD) and calculated z-scores for forced expiratory volume in the first second (FEV(1)), forced vital capacity (FVC), and FEV(1)/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, FEV(1), FVC, and FEV(1)/FVC z-scores declined over time (average crude annual FEV(1) decline was -0.07 z-scores) but not at the same rate for all patients. FEV(1) z-scores improved over time in 21% of patients, remained stable in 40% and declined in 39%. Low BMI 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.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30209 - Paediatrics
Result continuities
Project
<a href="/en/project/NV19-07-00210" target="_blank" >NV19-07-00210: Primary ciliary dyskinesia: Genetic, structural and functional determinants of the disease course and prognosis.</a><br>
Continuities
P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)
Others
Publication year
2022
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
European Respiratory Journal
ISSN
0903-1936
e-ISSN
1399-3003
Volume of the periodical
60
Issue of the periodical within the volume
4
Country of publishing house
CH - SWITZERLAND
Number of pages
14
Pages from-to
2101918
UT code for WoS article
000881848900003
EID of the result in the Scopus database
2-s2.0-85140416482