RT Journal Article
SR Electronic
T1 Spirometry in children with primary ciliary dyskinesia
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P4272
VO 44
IS Suppl 58
A1 Vilya Bulgakova
A1 Liliya Selimzyanova
A1 Elena Sereda
A1 Olga Lukina
A1 Elena Vishneva
YR 2014
UL http://erj.ersjournals.com/content/44/Suppl_58/P4272.abstract
AB The aim: to describe lung function changes in children with primary ciliary dyskinesia (PCD) and evaluation of bronchodilatation test in these patients.Methods: we performed spirometry in 44 children with PCD in median age 10 years (range 4-15). In 28 of them was performed a bronchodilatation test with ipratropii bromidum/fenoterol or/and formoterol.Results: lung function was normal in 16 (36%) patients. There were obstructive changes in 19 (43%), mixed abnormalities in 9 (21%). Median of FVC (forced vital capacity) was 88,75% of predicted (range 45-122%); FEV1 (forced expiratory volume in 1 second)-87,5% (38-131%); PEF (peak expiratory flow)-81% (41-126%); MEF25 (maximum expiratory flow after 25% expired FVC)-76,5% (29-123%); MEF50 (MEF after 50% expired FVC)-68,3% (21-118); MEF50 (MEF after 75% expired FVC)-61,9% (15-126). Patients with obstructive and mixed abnormalities of lung function were performed the bronchodilatation tests with ipratropii bromidum/fenoterol (n=19) or/and formoterol (n=14). Bronchodilatator response was positive in 10 patients (52%) after ipratropii bromidum/fenoterol and in 7 patients (50%) after formoterol.Conclusion: lung function in children with PCD has high degree variability. Most frequent we can identify obstructive abnormalities. And there are patients, who have bronchospasm as a component in bronchoobstructive syndrome, what can validate using bronchodilatators in therapy some children with PCD.