AI Article Synopsis
- The report assesses the impact of 12 weeks of ivacaftor treatment on aerobic function in two teenage cystic fibrosis patients, using maximal cardiopulmonary exercise testing.
- One patient saw no significant change in maximal oxygen uptake, while the other experienced a notable 30% increase in oxygen uptake, attributed to better muscle oxygen delivery and extraction.
- The study suggests using cardiopulmonary exercise testing as a valuable clinical tool to evaluate functional changes in CF patients that may not be reflected in traditional lung function tests.
Article 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; 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.
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| http://dx.doi.org/10.1097/PEP.0000000000000086 | DOI Listing |
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