Lumacaftor/Ivacaftor in patients aged 6-11 years with cystic fibrosis and homozygous for F508del-CFTR

American Journal of Respiratory and Critical Care Medicine

Volumn 195, Issue 7, 2017, Pages 912-920

  Milla, Carlos E ^a   Ratjen, Felix ^b   Marigowda, Gautham ^c   Liu, Fang ^c   Waltz, David ^c   Rosenfeld, Margaret ^d,e  

^a STANFORD UNIVERSITY   (United States)

^b HOSPITAL FOR SICK CHILDREN   (Canada)

^c VERTEX PHARMACEUTICALS   (United States)

^d SEATTLE CHILDREN S HOSPITAL   (United States)

^e UNIVERSITY OF WASHINGTON SCHOOL OF MEDICINE   (United States)

Author keywords

Cystic fibrosis transmembrane conductance regulator protein; Ivacaftor; Lumacaftor; Lung clearance index; Sweat test

Indexed keywords

ANTIBIOTIC AGENT; BRONCHODILATING AGENT; CHLORIDE; CORTICOSTEROID; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; IVACAFTOR PLUS LUMACAFTOR; SODIUM CHLORIDE; 1,3 BENZODIOXOLE DERIVATIVE; AMINOPHENOL DERIVATIVE; AMINOPYRIDINE DERIVATIVE; IVACAFTOR; LUMACAFTOR; QUINOLONE DERIVATIVE;

ABDOMINAL PAIN; ARTICLE; BODY MASS; BREATHING DISORDER; CATARACT; CHILD; CLINICAL EVALUATION; CONFIDENCE INTERVAL; COUGHING; CYSTIC FIBROSIS; CYSTIC FIBROSIS QUESTIONNAIRE REVISED RESPIRATORY DOMAIN SCORE; DISEASE EXACERBATION; DRUG EFFICACY; DRUG SAFETY; DRUG TOLERABILITY; DRUG WITHDRAWAL; DYSPNEA; FATIGUE; FEMALE; FEVER; FORCED EXPIRATORY VOLUME; GENE MUTATION; HEADACHE; HOMOZYGOSITY; HUMAN; HYPERTRANSAMINASEMIA; ILEUS; LUNG CLEARANCE; LUNG INFECTION; LUNG VOLUME; MAJOR CLINICAL STUDY; MALE; NAUSEA; NOSE OBSTRUCTION; NUTRITIONAL STATUS; OPEN STUDY; PHARMACODYNAMICS; PHASE 3 CLINICAL TRIAL; PRIORITY JOURNAL; QUALITY OF LIFE; RASH; SCORING SYSTEM; SIDE EFFECT; SPIROMETRY; SPUTUM LEVEL; SWEAT; TREATMENT OUTCOME; UPPER ABDOMINAL PAIN; VOMITING; WHEEZING; CLINICAL TRIAL; COMBINATION DRUG THERAPY; DRUG EFFECTS;

AMINOPHENOLS; AMINOPYRIDINES; BENZODIOXOLES; CHILD; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DRUG THERAPY, COMBINATION; FEMALE; FORCED EXPIRATORY VOLUME; HUMANS; MALE; QUINOLONES; TREATMENT OUTCOME;

EID: 85017338127     PISSN: 1073449X     EISSN: 15354970     Source Type: Journal    
DOI: 10.1164/rccm.201608-1754OC     Document Type: Article

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