Article
Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on sputum viscoelastic properties, airway infection and inflammation in patients with cystic fibrosis.
The European respiratory journal - 1 Aug 2023
Schaupp Laura, Addante Annalisa, Völler Mirjam, Fentker Kerstin, Kuppe Aditi, Bardua Markus, Duerr Julia, Piehler Linus, Röhmel Jobst, Thee Stephanie, Kirchner Marieluise, Ziehm Matthias, Lauster Daniel, Haag Rainer, Gradzielski Michael, Stahl Mirjam, Mertins Philipp, Boutin Sébastien, Graeber Simon Y, Mall Marcus A
Abstract excerpt
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...
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