PURPOSE: The aim of this article was to review the evidence for using exercise programs to improve pulmonary function and fitness in children with cystic fibrosis (CF). METHOD: Electronic databases -- Medline, SPORTDiscus, CINAHL, AMED, Embase, and Cochrane Central Register of Controlled Trials were searched with terms 'CF' and keywords related to 'exercise'. These papers were analysed for study quality, participant details, exercise intervention details, and outcomes on pulmonary function and fitness components. RESULTS: Only four randomized controlled trials (RCTs) met the eligibility criteria for review. These detailed exercise interventions cover short- and long-term duration. Modalities consisted of aerobic, strength, and anaerobic interventions. Severity of CF ranged from mild to severe. Significant improvement in pulmonary function was seen from short-term in-hospital aerobic or strength interventions. Significant strength gains were seen from strength training interventions. Aerobic fitness was shown to improve with short-term aerobic training. CONCLUSIONS: There is some evidence to support that both aerobic and strength training can impact positively on pulmonary function, aerobic fitness, and strength. More RCTs in this area would be welcomed. There is potential for future research into designing exercise programs for children with CF using a combination of modalities.

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