Lung function from school age to adulthood in primary ciliary dyskinesia

Kód výsledku v 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>

Nalezeny alternativní kódy

RIV/00064203:_____/22:10442008

Výsledek na webu

<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>

Jazyk výsledku

angličtina

Název v původním jazyce

Lung function from school age to adulthood in primary ciliary dyskinesia

Popis výsledku v původním jazyce

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.

Název v anglickém jazyce

Lung function from school age to adulthood in primary ciliary dyskinesia

Popis výsledku anglicky

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.

Druh

J_imp - Článek v periodiku v databázi Web of Science

CEP obor

—

OECD FORD obor

30209 - Paediatrics

Projekt

<a href="/cs/project/NV19-07-00210" target="_blank" >NV19-07-00210: Primární ciliární dyskineze: Genetické, strukturální a funkční determinanty průběhu a prognózy onemocnění.</a><br>

Návaznosti

P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)

Rok uplatnění

2022

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

European Respiratory Journal

ISSN

0903-1936

e-ISSN

1399-3003

Svazek periodika

60

Číslo periodika v rámci svazku

4

Stát vydavatele periodika

CH - Švýcarská konfederace

Počet stran výsledku

14

Strana od-do

2101918

Kód UT WoS článku

000881848900003

EID výsledku v databázi Scopus

2-s2.0-85140416482