Evidence for secondary ciliary dyskinesia in patients with cystic fibrosis

[en] Background Mucociliary clearance (MCC) impairment can be due to mucus abnormalities or to a ciliary dysfunction, which can be innate, or secondary to infection and/or inflammation. In cystic fibrosis (CF), it is well documented that MCC is impaired due to mucus abnormalities, but little is known concerning ciliary beating. This study aimed to confirm that ciliary dyskinesia is present in CF, and if this might be innate or secondary to the chronic infection and/or inflammation. Methods Ciliated epithelial samples were obtained by nasal brushing from 51 CF patients, and from 30 healthy subjects. Ciliary beating was evaluated using digital high-speed videomicroscopy at 37 °C, allowing to evaluate ciliary beat frequency (CBF) and the percentage of abnormal beat pattern (CBP); this was repeated after air-liquid interface (ALI) cell culture. Results Ciliary dyskinesia was higher in CF patients than in healthy subjects, with a lower CBF and a higher percentage of abnormal CBP. Ciliary dyskinesia, already present in childhood, normalized after ALI cell culture. A chronic airway colonization did not worsen ciliary dyskinesia. Conclusions We showed that, in CF, a ciliary dyskinesia, present from childhood, might contribute to the impaired MCC. Our results also found that the abnormal ciliary beating was not associated with a chronic infection, and resolved after ALI cell culture, suggesting that ciliary dyskinesia in CF is not innate, and might be secondary to chronic inflammation.

Disciplines :

Pediatrics

Author, co-author :

Bonhiver, Romane ; Université de Liège - ULiège > GIGA

Bricmont, Noémie ; Centre Hospitalier Universitaire de Liège - CHU > > Service de pédiatrie

Pirotte, Maud

Wuidart, Marc-Antoine

Monseur, Justine ; Université de Liège - ULiège > Département des sciences de la santé publique > Biostatistique

Benchimol, Lionel ; Centre Hospitalier Universitaire de Liège - CHU > > Service d'ORL, d'audiophonologie et de chir. cervico-faciale

Poirrier, Anne-Lise ; Université de Liège - ULiège > Département des sciences cliniques

Moermans, Catherine ; Université de Liège - ULiège > GIGA > GIGA I3 - Pneumology

CALMES, Doriane ; Centre Hospitalier Universitaire de Liège - CHU > > Service de pneumologie - allergologie

Schleich, Florence ; Université de Liège - ULiège > GIGA > GIGA I3 - Pneumology

More authors (3 more)

Language :

English

Title :

Evidence for secondary ciliary dyskinesia in patients with cystic fibrosis

Publication date :

October 2024

Journal title :

Journal of Cystic Fibrosis

ISSN :

1569-1993

eISSN :

1873-5010

Publisher :

Elsevier BV

Peer reviewed :

Peer Reviewed verified by ORBi

Additional URL :

https://api.elsevier.com/content/article/PII:S156919932401796X?httpAccept=text/xml

Available on ORBi :

since 20 November 2024

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