作者
Katherine B Hisert, Sonya L Heltshe, Christopher Pope, Peter Jorth, Xia Wu, Rachael M Edwards, Matthew Radey, Frank J Accurso, Daniel J Wolter, Gordon Cooke, Ryan J Adam, Suzanne Carter, Brenda Grogan, Janice L Launspach, Seamas C Donnelly, Charles G Gallagher, James E Bruce, David A Stoltz, Michael J Welsh, Lucas R Hoffman, Edward F McKone, Pradeep K Singh
发表日期
2017/6/15
期刊
American journal of respiratory and critical care medicine
卷号
195
期号
12
页码范围
1617-1628
出版商
American Thoracic Society
简介
Rationale: Previous work indicates that ivacaftor improves cystic fibrosis transmembrane conductance regulator (CFTR) activity and lung function in people with cystic fibrosis and G551D-CFTR mutations but does not reduce density of bacteria or markers of inflammation in the airway. These findings raise the possibility that infection and inflammation may progress independently of CFTR activity once cystic fibrosis lung disease is established.
Objectives: To better understand the relationship between CFTR activity, airway microbiology and inflammation, and lung function in subjects with cystic fibrosis and chronic airway infections.
Methods: We studied 12 subjects with G551D-CFTR mutations and chronic airway infections before and after ivacaftor. We measured lung function, sputum bacterial content, and inflammation, and obtained chest computed tomography scans.
Measurements and Main Results: Ivacaftor …
学术搜索中的文章
KB Hisert, SL Heltshe, C Pope, P Jorth, X Wu… - American journal of respiratory and critical care …, 2017