Abstract

Objective: The triple combination CFTR modulator therapy with elexacaftor/tezacaftor/ivacaftor (ETI) improves CFTR function and abnormalities in lung morphology, including airway mucus plugging as shown by improved ventilation and reduced mucus on MRI in CF patients with at least one F508del allele. However, the effects on sputum viscoelasticity, airway microbiome and inflammation have not been studied. The aim of this study was therefore to examine the effects of ETI on sputum viscoelasticity, airway microbiome and inflammation, and airway proteome in CF patients with one or two F508del alleles aged 12 years and older.

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