Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections

• Published in: American journal of respiratory and critical care medicine Vol. 195; no. 12; pp. 1617 - 1628
• Main Authors: Hisert, Katherine B., Heltshe, Sonya L., Pope, Christopher, Jorth, Peter, Wu, Xia, Edwards, Rachael M., Radey, Matthew, Accurso, Frank J., Wolter, Daniel J., Cooke, Gordon, Adam, Ryan J., Carter, Suzanne, Grogan, Brenda, Launspach, Janice L., Donnelly, Seamas C., Gallagher, Charles G., Bruce, James E., Stoltz, David A., Welsh, Michael J., Hoffman, Lucas R., McKone, Edward F., Singh, Pradeep K.
• Format: Journal Article
• Language: English
• Published: United States American Thoracic Society 15.06.2017
• Subjects: Adult; Aminophenols - therapeutic use; Bacteria; Chloride Channel Agonists - therapeutic use; Clinical medicine; Cystic fibrosis; Cystic Fibrosis - diagnostic imaging; Cystic Fibrosis - drug therapy; Cystic Fibrosis - metabolism; Cystic Fibrosis Transmembrane Conductance Regulator - drug effects; Cystic Fibrosis Transmembrane Conductance Regulator - metabolism; Cysts; Female; Humans; Infections; Inflammation; Inflammation - metabolism; Inflammation - prevention & control; Lung - diagnostic imaging; Lung - metabolism; Lung diseases; Male; Mutation; Original; Pathogenesis; Quinolones - therapeutic use; Respiratory Tract Infections - metabolism; Respiratory Tract Infections - prevention & control; Sputum - drug effects; Sputum - metabolism; Tomography; Tomography, X-Ray Computed; Pseudomonas aeruginosa; ivacaftor; inflammation; cystic fibrosis

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 pos...