Lung function in patients with primary ciliary dyskinesia: an iPCD Cohort study
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Lung function in patients with primary ciliary dyskinesia: an iPCD Cohort study. / Halbeisen, Florian S.; Goutaki, Myrofora; Spycher, Ben D.; Amirav, Israel; Behan, Laura; Boon, Mieke; Hogg, Claire; Casaulta, Carmen; Crowley, Suzanne; Haarman, Eric G.; Karadag, Bulent; Koerner-rettberg, Cordula; Loebinger, Michael R.; Mazurek, Henryk; Morgan, Lucy; Nielsen, Kim G.; Omran, Heymut; Santamaria, Francesca; Schwerk, Nicolaus; Thouvenin, Guillaume; Yiallouros, Panayiotis; Lucas, Jane s.; Latzin, Philipp; Kuehni, Claudia E.
I: European Respiratory Journal, Bind 52, Nr. 2, 1801040, 2018.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - Lung function in patients with primary ciliary dyskinesia: an iPCD Cohort study
AU - Halbeisen, Florian S.
AU - Goutaki, Myrofora
AU - Spycher, Ben D.
AU - Amirav, Israel
AU - Behan, Laura
AU - Boon, Mieke
AU - Hogg, Claire
AU - Casaulta, Carmen
AU - Crowley, Suzanne
AU - Haarman, Eric G.
AU - Karadag, Bulent
AU - Koerner-rettberg, Cordula
AU - Loebinger, Michael R.
AU - Mazurek, Henryk
AU - Morgan, Lucy
AU - Nielsen, Kim G.
AU - Omran, Heymut
AU - Santamaria, Francesca
AU - Schwerk, Nicolaus
AU - Thouvenin, Guillaume
AU - Yiallouros, Panayiotis
AU - Lucas, Jane s.
AU - Latzin, Philipp
AU - Kuehni, Claudia E.
PY - 2018
Y1 - 2018
N2 - Primary ciliary dyskinesia (PCD) has been considered a relatively mild disease, especially compared to cystic fibrosis (CF), but studies on lung function in PCD patients have been few and small.This study compared lung function from spirometry of PCD patients to normal reference values and to published data from CF patients. We calculated z-scores and % predicted values for forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) using the Global Lung Function Initiative 2012 values for 991 patients from the international PCD Cohort. We then assessed associations with age, sex, country, diagnostic certainty, organ laterality, body mass index and age at diagnosis in linear regression models. Lung function in PCD patients was reduced compared to reference values in both sexes and all age groups. Children aged 6–9 years had the smallest impairment (FEV1 z-score −0.84 (−1.03 to −0.65), FVC z-score −0.31 (−0.51 to −0.11)). Compared to CF patients, FEV1 was similarly reduced in children (age 6–9 years PCD 91% (88–93%); CF 90% (88–91%)), but less impaired in young adults (age 18–21 years PCD 79% (76–82%); CF 66% (65–68%)). The results suggest that PCD affects lung function from early in life, which emphasises the importance of early standardised care for all patients.
AB - Primary ciliary dyskinesia (PCD) has been considered a relatively mild disease, especially compared to cystic fibrosis (CF), but studies on lung function in PCD patients have been few and small.This study compared lung function from spirometry of PCD patients to normal reference values and to published data from CF patients. We calculated z-scores and % predicted values for forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) using the Global Lung Function Initiative 2012 values for 991 patients from the international PCD Cohort. We then assessed associations with age, sex, country, diagnostic certainty, organ laterality, body mass index and age at diagnosis in linear regression models. Lung function in PCD patients was reduced compared to reference values in both sexes and all age groups. Children aged 6–9 years had the smallest impairment (FEV1 z-score −0.84 (−1.03 to −0.65), FVC z-score −0.31 (−0.51 to −0.11)). Compared to CF patients, FEV1 was similarly reduced in children (age 6–9 years PCD 91% (88–93%); CF 90% (88–91%)), but less impaired in young adults (age 18–21 years PCD 79% (76–82%); CF 66% (65–68%)). The results suggest that PCD affects lung function from early in life, which emphasises the importance of early standardised care for all patients.
U2 - 10.1183/13993003.01040-2018
DO - 10.1183/13993003.01040-2018
M3 - Journal article
C2 - 30049738
VL - 52
JO - The European Respiratory Journal
JF - The European Respiratory Journal
SN - 0903-1936
IS - 2
M1 - 1801040
ER -
ID: 222624084