title: Lumacaftor/ivacaftor for patients homozygous for Phe508del-CFTR: should we curb our enthusiasm?
authors.affiliation: Manchester Adult Cystic Fibrosis Centre, University Hospital of South Manchester NHS Trust, Manchester, UK.
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Jones
authors.affiliation: Manchester Adult Cystic Fibrosis Centre, University Hospital of South Manchester NHS Trust, Manchester, UK.
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Barry
mesh_terms: D000627:Aminophenols / Q000627:therapeutic use*; D000631:Aminopyridines / Q000627:therapeutic use*; D052117:Benzodioxoles / Q000627:therapeutic use*; D003550:Cystic Fibrosis / Q000188:drug therapy* / Q000235:genetics; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics*; D004338:Drug Combinations; D017353:Gene Deletion; D006720:Homozygote; D006801:Humans; D058990:Molecular Targeted Therapy / Q000379:methods; D015363:Quinolones / Q000627:therapeutic use*
chemical_list: D000627:Aminophenols; D000631:Aminopyridines; D052117:Benzodioxoles; C505032:CFTR protein, human; D004338:Drug Combinations; D015363:Quinolones; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; C545203:ivacaftor; C569105:lumacaftor
keywords: Cystic Fibrosis