Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on sputum viscoelastic properties, airway infection and inflammation in patients with cystic fibrosis
Humboldt-Universität zu Berlin · German Center for Lung Research · +8 more institutions
Abstract
Background Recent studies demonstrated that the triple combination cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI) improves lung function and reduces pulmonary exacerbations in cystic fibrosis (CF) patients with at least one F508del allele. However, effects of ETI on downstream consequences of CFTR dysfunction, i.e. abnormal viscoelastic properties of airway mucus, chronic airway infection and inflammation have not been studied. The aim of this study was to determine the longitudinal effects of ETI on airway mucus rheology, microbiome and inflammation in CF patients with one or two F508del alleles aged ≥12 years throughout the first 12 months…
Citation impact
- FWCI
- 42.83
- Percentile
- 100%
- References
- 58
Authors
20- LSLaura SchauppCorresponding
Humboldt-Universität zu Berlin, German Center for Lung Research, Freie Universität Berlin, Charité - Universitätsmedizin Berlin
- AAAnnalisa Addante
Humboldt-Universität zu Berlin, German Center for Lung Research, Freie Universität Berlin, Charité - Universitätsmedizin Berlin
- MVMirjam Völler
Humboldt-Universität zu Berlin, German Center for Lung Research, Freie Universität Berlin, Charité - Universitätsmedizin Berlin
- KFKerstin Fentker
Max Delbrück Center, Freie Universität Berlin
- AKAditi Kuppe
Humboldt-Universität zu Berlin, German Center for Lung Research, Freie Universität Berlin, Charité - Universitätsmedizin Berlin
Topics & keywords
- Medicine
- Cystic fibrosis
- Ivacaftor
- Sputum
- Airway
- Inflammation
- Immunology
- Pathology