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.
Keywords: Calidad de vida; Children; Cystic fibrosis; Exercise tolerance; Fibrosis quística; Función pulmonar; Niños; Pulmonary function; Quality of life; Tolerancia al ejercicio.
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