Publications by authors named "Linus Piehler"
CFTR modulator therapy with elexacaftor/tezacaftor/ivacaftor (ETI) has been approved for people with CF and at least one F508del allele in Europe. In the US, the ETI label has been expanded to 177 rare mutations responsive in Fischer rat thyroid cells, including , but not . However, knowledge on the effect of ETI on G85E or N1303K CFTR function remains limited.
View Article and Find Full Text PDFArticle Synopsis
- Recent studies showed that the triple combination CFTR modulator therapy, elexacaftor/tezacaftor/ivacaftor (ETI), enhances lung function and reduces flare-ups in cystic fibrosis (CF) patients with at least one CFTR allele, but its impact on mucus properties, infection, and inflammation was previously unexplored.
- This study tracked changes in mucus rheology, microbiome, and inflammation markers in CF patients aged 12 and older over the first year of ETI therapy.
- Results indicated that ETI significantly improved mucus properties, increased microbiome diversity, and reduced inflammation markers, suggesting ETI positively affects airway health, but did not fully normalize conditions to levels seen in healthy individuals.