A comparative study of airway inflammation and sputum viscoelastic properties in patients with primary ciliary dyskinesia and patients with cystic fibrosis on Elexacaftor/Tezacaftor/Ivacaftor therapy
Hannah Nussstein, Ruth M. Urbantat, Mirjam Völler, Kerstin Fentker, Annalisa Addante, Aditi Kuppe, Jacques A. Dürr, Linus Piehler, et al. (20 authors)
Klinische Pädiatrie · 2024-02
Abstract
Hintergrund While previous studies provided thorough insights on airway inflammation and infection as well as viscoelastic properties of airway mucus in people with CF (pwCF), limited data is available on these aspects in patients with other chronic muco-obstructive lung diseases. The aim of this study was therefore to analyze airway inflammation and viscoelastic properties in people with primary ciliary dyskinesia (pwPCD) and to compare these findings with published data on pwCF before and under triple combination CFTR modulator therapy Elexacaftor/Tezacaftor/Ivacaftor (ETI).
MeSH terms
- Primary ciliary dyskinesia
- Ivacaftor
- Cystic fibrosis
- Medicine
- Sputum
- Airway
- Inflammation
- Bronchiectasis