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dc.contributor.authorSaynor, ZL
dc.contributor.authorBarker, AR
dc.contributor.authorOades, PJ
dc.contributor.authorWilliams, CA
dc.date.accessioned2015-08-27T10:46:51Z
dc.date.issued2014
dc.description.abstractPURPOSE: 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. SUMMARY OF KEY POINTS: 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. STATEMENT OF 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. RECOMMENDATIONS FOR CLINICAL PRACTICE: 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.en_GB
dc.identifier.citationVol. 26, pp. 454 - 461en_GB
dc.identifier.doi10.1097/PEP.0000000000000086
dc.identifier.other00001577-201426040-00019
dc.identifier.urihttp://hdl.handle.net/10871/18138
dc.language.isoenen_GB
dc.publisherLippincott, Williams & Wilkinsen_GB
dc.relation.urlhttp://www.ncbi.nlm.nih.gov/pubmed/25251804en_GB
dc.rights(C) 2014 Wolters Kluwer Health Lippincott Williams & Wilkins and the Section on Pediatrics of the American Physical Therapy Association.en_GB
dc.subjectAdolescenten_GB
dc.subjectAminophenolsen_GB
dc.subjectBody Weights and Measuresen_GB
dc.subjectCystic Fibrosisen_GB
dc.subjectCystic Fibrosis Transmembrane Conductance Regulatoren_GB
dc.subjectExercise Testen_GB
dc.subjectFemaleen_GB
dc.subjectHumansen_GB
dc.subjectMaleen_GB
dc.subjectQuinolonesen_GB
dc.subjectRespiratory Function Testsen_GB
dc.titleThe effect of ivacaftor in adolescents with cystic fibrosis (G551D mutation): an exercise physiology perspective.en_GB
dc.typeCase studyen_GB
dc.identifier.issn0898-5669
exeter.place-of-publicationUnited States
dc.descriptionThis is the author version of an article published in Pediatric Physical Therapy 26(4):454-461, Winter 2014. The final published version is available via: http://dx.doi.org/10.1097/PEP.0000000000000086Pediatricen_GB
dc.identifier.eissn1538-005X
dc.identifier.journalPediatric Physical Therapyen_GB
dc.identifier.pmid25251804


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