Background: Exercise training is a cornerstone of care for people with cystic fibrosis (pwCF); it improves exercise capacity and health-related physical fitness, but no meta-analysis has investigated its effects on muscle function in young pwCF. The objective of this meta-analysis was to assess the effects of exercise on peripheral muscle strength in young pwCF. Methods: An electronic search was conducted in four databases (Pubmed, Science Direct, CENTRAL, and PEDRO) from their inception to July 2022. Grey literature databases (OpenGrey, the European Respiratory Society, the American Thoracic Society, and the European Cystic Fibrosis Society) were also consulted. Randomized controlled trials comparing any type of exercise with standard care in young pwCF (5 to 19 years old) were included. Two authors independently selected the relevant studies, extracted the data, assessed the risk of bias (using the Rob2 tool), and rated the quality of the evidence. Results: Ten studies met the inclusion criteria, involving 359 pwCF. Exercise training improved both lower and upper limb muscle strength (SMD 1.67 (95%CI 0.80 to 2.53), I2 = 76%, p < 0.001 and SMD 1.30 (95%CI 0.66 to 1.93), I2 = 62%, p < 0.001, respectively). Improvements were also reported in muscle mass and maximal oxygen consumption. Results regarding physical activity levels were inconclusive. The overall risk of bias for the primary outcome was high. Conclusions: Exercise training may have a positive effect on peripheral muscle strength in young pwCF. The evidence quality is very low and the level of certainty is poor. There is a need for high-quality randomized controlled studies to confirm these results.
Keywords: cystic fibrosis; exercise; pediatrics; physical activity; respiratory disease.