Abstract
Cystic fibrosis (CF) is a life-shortening genetic disease, affecting multiple life domains including physical activity (PA). Although higher PA levels are associated with multiple health benefits, little insight exists on the PA level of people with CF (PwCF) compared to healthy peers. Evidence on the influencing factors (i.e. correlates) of PA in this clinical population is scarce, but essential to fully understand their PA behaviour. Therefore, the present review aims to provide an overview of the PA level of PwCF compared to healthy peers, and the correlates of PA in PwCF. A systematic search of three databases resulted in 46 included studies. Analysis of 16 studies showed that the CF population is equally active compared to healthy peers, but there is a trend towards less high-intensity PA in youths with CF. Furthermore, PA is positively associated with quality of life, lung function, (maximal) exercise capacity, bone mineral density and quadriceps force. Also, PA was lower on weekdays compared to weekend days and lower when experiencing pulmonary exacerbations. More high-quality research is required in PwCF, particularly longitudinal studies that further explore the correlates of PA, with PA investigated as a primary outcome and measured objectively.
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