The effect of ivacaftor in adolescents with cystic fibrosis (G551D mutation): an exercise physiology perspective

2.50
Hdl Handle:
http://hdl.handle.net/11287/593808
Title:
The effect of ivacaftor in adolescents with cystic fibrosis (G551D mutation): an exercise physiology perspective
Authors:
Saynor, Z.; Barker, A. R.; Oades, Patrick ( 0000-0001-5265-6923 ) ; Williams, Craig A.
Abstract:
PURPOSE: 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; DeltaF508/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.
Citation:
Pediatr Phys Ther. 2014 Winter;26(4):454-61.
Publisher:
Wolters Kluwer
Journal:
Pediatric physical therapy : the official publication of the Section on Pediatrics of the American Physical Therapy Association
Issue Date:
1-Dec-2014
URI:
http://hdl.handle.net/11287/593808
DOI:
10.1097/PEP.0000000000000086
PubMed ID:
25251804
Additional Links:
http://www.ncbi.nlm.nih.gov/pubmed/25251804
Type:
Case Report
Language:
eng
ISSN:
1538-005X
Appears in Collections:
2014 RD&E publications; Paediatrics

Full metadata record

DC FieldValue Language
dc.contributor.authorSaynor, Z.en
dc.contributor.authorBarker, A. R.en
dc.contributor.authorOades, Patricken
dc.contributor.authorWilliams, Craig A.en
dc.date.accessioned2016-01-19T12:35:10Zen
dc.date.available2016-01-19T12:35:10Zen
dc.date.issued2014-12-01en
dc.identifier.citationPediatr Phys Ther. 2014 Winter;26(4):454-61.en
dc.identifier.issn1538-005Xen
dc.identifier.pmid25251804en
dc.identifier.doi10.1097/PEP.0000000000000086en
dc.identifier.urihttp://hdl.handle.net/11287/593808en
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; DeltaF508/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
dc.language.isoengen
dc.publisherWolters Kluweren
dc.relation.urlhttp://www.ncbi.nlm.nih.gov/pubmed/25251804en
dc.titleThe effect of ivacaftor in adolescents with cystic fibrosis (G551D mutation): an exercise physiology perspectiveen
dc.typeCase Reporten
dc.identifier.journalPediatric physical therapy : the official publication of the Section on Pediatrics of the American Physical Therapy Associationen

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