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The New England Journal of Medicine, ISSN 0028-4793, 11/2011, Volume 365, Issue 18, pp. 1663 - 1672
Journal Article
The New England Journal of Medicine, ISSN 0028-4793, 07/2015, Volume 373, Issue 3, pp. 220 - 231
Journal Article
The New England Journal of Medicine, ISSN 0028-4793, 11/2010, Volume 363, Issue 21, pp. 1991 - 2003
Journal Article
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 | Original
Journal Article
American Journal of Respiratory and Critical Care Medicine, ISSN 1073-449X, 05/2017, Volume 195, Issue 9, pp. 1092 - 1099
Journal Article
The New England Journal of Medicine, ISSN 0028-4793, 10/2018, Volume 379, Issue 17, pp. 1599 - 1611
This companion article to the VX-445 report shows that VX-659, a new CFTR potentiator, when administered with tezacaftor and ivacaftor improved lung function,... 
G551D MUTATION | MEDICINE, GENERAL & INTERNAL | IN-VITRO | CFTR POTENTIATOR | TEZACAFTOR-IVACAFTOR | Pyrazoles - therapeutic use | Forced Expiratory Volume - drug effects | Humans | Pyrrolidines - adverse effects | Chloride Channel Agonists - therapeutic use | Male | Aminophenols - therapeutic use | Chloride Channel Agonists - adverse effects | Aminophenols - adverse effects | Pyrrolidines - pharmacology | Pyrrolidines - therapeutic use | Sweat - chemistry | Young Adult | Quinolones - adverse effects | Quinolones - therapeutic use | Adult | Benzodioxoles - adverse effects | Female | Benzodioxoles - therapeutic use | Pyrazoles - adverse effects | Pyrazoles - pharmacology | Double-Blind Method | Cells, Cultured | Genotype | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Chlorides - analysis | Indoles - adverse effects | Cystic Fibrosis - genetics | Adolescent | Alleles | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Indoles - therapeutic use | Mutation | Cystic Fibrosis - drug therapy | Drug Combinations | Cystic fibrosis | Ivacaftor | Genetic aspects | Dosage and administration | Drug therapy | Epithelial cells | Chloride transport | Clinical trials | Recovery of function | Patients | Proteins | Genotype & phenotype | Conductance | Full text | Cystic fibrosis transmembrane conductance regulator | Protein transport | Sweat | Binding sites | Genotypes
Journal Article
The New England Journal of Medicine, ISSN 0028-4793, 10/2018, Volume 379, Issue 17, pp. 1612 - 1620
This preclinical, phase 2 report shows that VX-445, a CFTR potentiator when administered with tezacaftor and ivacaftor, improved lung function and reduced... 
G551D MUTATION | MEDICINE, GENERAL & INTERNAL | TEZACAFTOR-IVACAFTOR | CFTR | Pyrazoles - therapeutic use | Forced Expiratory Volume - drug effects | Humans | Pyrrolidines - administration & dosage | Chloride Channel Agonists - therapeutic use | Male | Aminophenols - therapeutic use | Chloride Channel Agonists - adverse effects | Aminophenols - adverse effects | Pyrrolidines - pharmacology | Pyrrolidines - therapeutic use | Sweat - chemistry | Young Adult | Quinolones - adverse effects | Chlorides - metabolism | Quinolones - therapeutic use | Adult | Benzodioxoles - adverse effects | Female | Benzodioxoles - therapeutic use | Pyrazoles - pharmacology | Pyridines - therapeutic use | Pyridines - administration & dosage | Double-Blind Method | Genotype | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Chlorides - analysis | Pyrazoles - administration & dosage | Indoles - adverse effects | Cystic Fibrosis - genetics | Adolescent | Alleles | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Indoles - therapeutic use | Pyridines - pharmacology | Mutation | Cystic Fibrosis - drug therapy | Drug Combinations | Cystic fibrosis | Ivacaftor | Dosage and administration | Drug therapy | Statistical analysis | Writers | Epithelial cells | Chloride transport | Recovery of function | Patients | Design | Proteins | Genotype & phenotype | Chloride | Collaboration | Conductance | Full text | Cystic fibrosis transmembrane conductance regulator | Protein transport | Sweat | Pharmaceuticals
Journal Article
Proceedings of the National Academy of Sciences of the United States of America, ISSN 0027-8424, 5/2006, Volume 103, Issue 22, pp. 8487 - 8492
Journal Article