RT Journal Article
SR Electronic
T1 Evidence for ciliary dyskinesia in cystic fibrosis
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP PA3374
DO 10.1183/13993003.congress-2021.PA3374
VO 58
IS suppl 65
A1 Bonhiver, Romane
A1 Bricmont, Noemie
A1 Pirotte, Maud
A1 Moermans, Catherine
A1 Wuidart, Marc-Antoine
A1 Pirson, Jessica
A1 Louis, Renaud
A1 Seghaye, Marie-Christine
A1 Kempeneers, Céline
A1 Boboli, Hedwige
YR 2021
UL http://erj.ersjournals.com/content/58/suppl_65/PA3374.abstract
AB Background: Mucociliary clearance (MCC) results in an effective interaction between the mucus layer and the normal coordinated ciliary beating. In cystic fibrosis (CF), MCC is impaired due to dehydrated mucus, but recent data suggest that an abnormal ciliary beating may contribute to poor MCC in CF patients. However, results are controversial: in CF patients, ciliary beat frequency (CBF) might be either decreased, or increased, and ciliary dyskinesia might be increased, compared with healthy subjects.Aims: To compare ciliary function in CF patients and in healthy subjects.Method: Ciliated epithelial samples were obtained by nasal brushing from 4 non-transplanted patients with CF (range age: 4-23). Beating cilia were recorded using digital high-speed videomicroscopy at 37°C within 9 hours after brushing. Ciliary functional analysis (CFA) is assessed by CBF and the percentage of dyskinetic ciliary beat pattern (CBP) and is compared with our laboratory normal values, calculated on 14 healthy subjects (range age: 22-54).Results: There is no difference in CBF between CF patients and healthy subjects. However, ciliary dyskinesia is significantly higher in CF patients. CF patientsHealthy subjects p-valueCBF(Hz)13.4±0.114.8±2.20.10Dyskinetic CBP(%)32.1±8.717.3±7.60.01Data expressed as mean±SDConclusion: This pilot study suggests that, unlike previous studies, CBF is normal in CF patients. However, our results suggest that an abnormal ciliary function might contribute to impaired MCC in CF patients, as shown by the higher dyskinetic CBP compared with healthy subjects. To evaluate if ciliary dyskinesia is primary or secondary to chronic inflammation, CFA will be repeated after air-liquid interface cell culture.FootnotesCite this article as: European Respiratory Journal 2021; 58: Suppl. 65, PA3374.This abstract was presented at the 2021 ERS International Congress, in session “Prediction of exacerbations in patients with COPD”.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).