Abstract
IntroductionThis systematic review aims to analyze the validity and reliability of available tools to assess health-related quality of life (HRQoL) and exercise tolerance in children and adolescents with cystic fibrosis (CF). Materials and methodsA review of observational studies studying the validity and reliability of the tools was conducted. The COSMIN (COnsesus-based Standards for the selection of health Measurements INstruments) guide was followed to analyze the quality of these tools. ResultsA total of 18 studies were selected. Of the eight HRQoL tools, five generic and three specific, analyzed in 14 studies, the Cystic Fibrosis Questionnaire-Revised (CFQ-R) presents the best properties. Of the four tools analyzed to assess exercise tolerance, cycle ergometry and the modified shuttle test are the most valid and reliable. ConclusionsThe CFQ-R, cycloergometry and the modified shuttle test have the best properties for the assessment of children with CF, but more studies are needed.
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