作者
Simon Y Graeber, Sébastien Boutin, Mark O Wielpütz, Cornelia Joachim, Dario L Frey, Sabine Wege, Olaf Sommerburg, Hans-Ulrich Kauczor, Mirjam Stahl, Alexander H Dalpke, Marcus A Mall
发表日期
2021/6
期刊
Annals of the American Thoracic Society
卷号
18
期号
6
页码范围
971-980
出版商
American Thoracic Society
简介
Rationale: Previous studies showed that lumacaftor–ivacaftor therapy results in partial rescue of CFTR (cystic fibrosis [CF] transmembrane conductance regulator) activity and a moderate improvement of spirometry in Phe508del homozygous patients with CF. However, the effects of lumacaftor–ivacaftor on lung clearance index (LCI), lung morphology and perfusion detected by chest magnetic resonance imaging (MRI), and effects on the airway microbiome and inflammation remain unknown.
Objectives: To investigate the effects of lumacaftor–ivacaftor on LCI, lung MRI scores, and airway microbiome and inflammation.
Methods: In this prospective observational study we assessed clinical outcomes including spirometry and body mass index, LCI, lung MRI scores, sputum microbiome, and proinflammatory cytokines in 30 Phe508del homozygous patients with CF 12 years and older before …
学术搜索中的文章
SY Graeber, S Boutin, MO Wielpütz, C Joachim… - Annals of the American Thoracic Society, 2021