title: The effect of ivacaftor in adolescents with cystic fibrosis (G551D mutation): an exercise physiology perspective.
abstract: OBJECTIVE
  The purpose of this report was to evaluate the influence of 12 weeks of ivacaftor treatment on the aerobic function of 2 teenage patients with cystic fibrosis (CF; ΔF508/G551D) using a maximal cardiopulmonary exercise test.
  
  
  CONCLUSIONS
  One patient, with relatively mild disease, demonstrated no clinically meaningful changes in maximal oxygen uptake ((Equation is included in full-text article.)O2max). However, in the second case, with more established lung disease on imaging, (Equation is included in full-text article.)O2max improved by approximately 30%, an improvement out of proportion with early lung function changes. This improvement resulted from increased muscle oxygen delivery and extraction.
  
  
  CONCLUSIONS
  Cardiopulmonary exercise testing can monitor the extent and cause(s) of change following interventions such as ivacaftor, with the potential to identify functional changes independent from spirometry indices.
  
  
  CONCLUSIONS
  Cardiopulmonary exercise testing represents an important and comprehensive clinical assessment tool, and its use as an outcome measure in the functional assessment of patients with CF is encouraged.
authors.affiliation: Children's Health and Exercise Research Centre (Ms Saynor and Drs Barker and Williams) Sport and Health Sciences, University of Exeter, Exeter, Devon, United Kingdom; Royal Devon and Exeter NHS Foundation Trust Hospital (Ms Saynor and Dr Oades), Exeter, Devon, United Kingdom.
authors.forename: Zoe Louise
authors.initials: ZL
authors.lastname: Saynor
authors.forename: Alan Robert
authors.initials: AR
authors.lastname: Barker
authors.forename: Patrick John
authors.initials: PJ
authors.lastname: Oades
authors.forename: Craig Anthony
authors.initials: CA
authors.lastname: Williams
mesh_terms: D000293:Adolescent; D000627:Aminophenols / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D001837:Body Weights and Measures; D003550:Cystic Fibrosis / Q000188:drug therapy* / Q000534:rehabilitation*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000819:agonists*; D005080:Exercise Test; D005260:Female; D006801:Humans; D008297:Male; D015363:Quinolones / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D012129:Respiratory Function Tests
chemical_list: D000627:Aminophenols; D015363:Quinolones; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; C545203:ivacaftor