Effects of Elexacaftor/Tezacaftor/Ivacaftor on Sputum Viscoelastic Properties, Airway Infection and Inflammation in Patients with Cystic Fibrosis
Simon Y. Graeber, Annalisa Addante, Mirjam Völler, Laura Schaupp, Kerstin Fentker, Markus Bardua, Aditi Kuppe, Julia Duerr, et al. (20 authors)
Klinische Pädiatrie · 2023-03
Abstract
Background We recently demonstrated that the triple combination CFTR modulator therapy elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) improves lung ventilation and airway mucus plugging determined by multiple-breath washout and magnetic resonance imaging in CF patients with at least one F508del allele. However, effects of ELX/TEZ/IVA on viscoelastic properties of airway mucus, chronic airway infection and inflammation have not been studied. The aim of this study was, therefore, to determine the effects of ELX/TEZ/IVA on airway mucus rheology, microbiome and inflammation in CF patients with one or two F508del alleles aged 12 years and older.
MeSH terms
- Ivacaftor
- Cystic fibrosis
- Sputum
- Airway
- Medicine
- Mucus
- Inflammation