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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

  • Czech description

Classification

  • Type

    J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database

  • CEP classification

  • 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