作者
Laura Schaupp, Annalisa Addante, Mirjam Völler, Kerstin Fentker, Aditi Kuppe, Markus Bardua, Julia Duerr, Linus Piehler, Jobst Röhmel, Stephanie Thee, Marieluise Kirchner, Matthias Ziehm, Daniel Lauster, Rainer Haag, Michael Gradzielski, Mirjam Stahl, Philipp Mertins, Sébastien Boutin, Simon Y Graeber, Marcus A Mall
发表日期
2023/8/1
期刊
European Respiratory Journal
卷号
62
期号
2
出版商
European Respiratory Society
简介
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 of therapy.
Methods
In this prospective observational study, we assessed sputum rheology, the microbiome, inflammation markers and proteome before and 1, 3 and 12 months after initiation of …
学术搜索中的文章
L Schaupp, A Addante, M Völler, K Fentker, A Kuppe… - European Respiratory Journal, 2023