Correlated Drug Terms (2)

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	Name (Synonyms)	Correlation
drug433	CFTR Modulators Wiki	1.00
drug880	Exercise Wiki	0.71

Correlated MeSH Terms (2)

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	Name (Synonyms)	Correlation
D003550	Cystic Fibrosis NIH	0.58
D005355	Fibrosis NIH	0.45

Correlated HPO Terms (0)

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	Name (Synonyms)	Correlation

Primary Outcomes

Description: Changes in strength will be measured using a five repetition maximum test (5RM)

Measure: Change in Strength

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Description: Changes in cardiorespiratory fitness will be measured using a cardiopulmonary exercise test (CPET)

Measure: Change in Cardiorespiratory Fitness

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Secondary Outcomes

Description: Changes in FEV1 will be measured using Spirometry (z-score based on Global Lung Function Initiative reference DOI: 10.1016/j.arbres.2017.07.019)

Measure: Changes in Forced expiratory volume in 1 second (FEV1)

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Description: Changes in FVC will be measured using Spirometry (z-score based on Global Lung Function Initiative reference DOI: 10.1016/j.arbres.2017.07.019)

Measure: Changes in Forced vital capacity (FVC)

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Description: Changes in FEV1/FVC ratio (FEV1%) will be measured using Spirometry (z-score based on Global Lung Function Initiative reference DOI: 10.1016/j.arbres.2017.07.019)

Measure: Changes in FEV1/FVC ratio (FEV1%)

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Description: Changes in FEF will be measured using Spirometry (z-score based on Global Lung Function Initiative reference DOI: 10.1016/j.arbres.2017.07.019)

Measure: Changes in Forced expiratory flow (FEF)

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Description: Changes in physical activity levels will be measured using PAQ-C for children under 14 years of age and PAQ-A for adolescents over 14 years of age. Items 1 to 9 will be used in the physical activity composite score, and means will be calculated to obtain the final PAQ-C activity summary score. Items 1 to 8 will be used in the physical activity composite score, and means will be calculated to obtain the final PAQ-A activity summary score. A score of 1 indicates low physical activity, whereas a score of 5 indicates high physical activity.

Measure: Changes in Physical Activity Questionnaire (PAQ) for children and adolescents

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Description: Changes in quality of life will be measures with the Cystic Fibrosis-Questionnaire-Revised (CFQ-R). Scores for each health related quality of life domain are calculated; after recoding, each item is summed to generate a domain score and standardized. Scores range from 0 to 100, with higher scores indicating better health.

Measure: Change in quality of life: Cystic Fibrosis-Questionnaire-Revised (CFQ-R)

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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Description: Chloride concentration in sweat (mEq/L) will be measured in the laboratory using an MK II Chloride Analyzer 926S

Measure: Sweat chloride level

Time: Four assessment points throughout the study: baseline and after each 8-week intervention

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