LAUSR

INTRODUCTION This 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 METHODS A 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. RESULTS A 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. CONCLUSIONS The CFQ-R, cycloergometry and the modified shuttle test have the best properties for the assessment of children with CF, but more studies are needed.