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American journal of respiratory and critical care medicine, ISSN 1073-449X, 2014, Volume 190, Issue 2, pp. 175 - 184
Rationale: Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator recently approved for patients with CF age 6 and older with... 
PH | Cystic fibrosis | Pseudomonas aeruginosa | Ivacaftor | CFTR modulator | LUNG-FUNCTION | ivacaftor | MUCIN SECRETION | ADRENERGIC SWEAT SECRETION | MUCOCILIARY CLEARANCE | INTESTINAL PH | CHILDREN | RESPIRATORY SYSTEM | cystic fibrosis | PSEUDOMONAS-AERUGINOSA | INFLAMMATION | DISEASE | QUESTIONNAIRE | CRITICAL CARE MEDICINE | Sputum - microbiology | Lung - microbiology | Pseudomonas aeruginosa - isolation & purification | Follow-Up Studies | Forced Expiratory Volume - drug effects | Sweat - drug effects | Humans | Microbiota - drug effects | Respiratory System Agents - therapeutic use | Hospitalization - statistics & numerical data | Male | Aminophenols - therapeutic use | Quinolones - pharmacology | Aminophenols - pharmacology | Mucociliary Clearance - drug effects | Pseudomonas Infections - prevention & control | Young Adult | Hydrogen-Ion Concentration - drug effects | Quinolones - therapeutic use | Adult | Female | Lung - metabolism | Child | Biomarkers - metabolism | Respiratory System Agents - pharmacology | Cystic Fibrosis - metabolism | Cystic Fibrosis - microbiology | Pseudomonas Infections - complications | Pseudomonas Infections - diagnosis | Treatment Outcome | Lung - physiopathology | Genetic Markers | Sputum - metabolism | Cystic Fibrosis - genetics | Intestine, Small - drug effects | Lung - drug effects | Adolescent | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Intestine, Small - metabolism | Mutation | Cystic Fibrosis - drug therapy | Sweat - metabolism | Index Medicus | Abridged Index Medicus | Original
Journal Article
Journal Article
The New England Journal of Medicine, ISSN 0028-4793, 11/2011, Volume 365, Issue 18, pp. 1663 - 1672
Journal Article
Journal Article
American Journal of Respiratory and Critical Care Medicine, ISSN 1073-449X, 11/2010, Volume 182, Issue 10, pp. 1262 - 1272
Journal Article