Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
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Randomized Controlled Trial
Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation.
Ivacaftor is used to treat patients with CF and a G551D gating mutation; the KONNECTION study assessed the efficacy and safety of ivacaftor in patients with CF and a non-G551D gating mutation. ⋯ Ivacaftor was efficacious in a group of patients with CF who had selected non-G551D gating mutations.
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Comparative Study Observational Study
Physiological response during activity programs using Wii-based video games in patients with cystic fibrosis (CF).
Patients with cystic fibrosis (CF) are characterized by an abnormal ventilation response that limits the exercise capacity. Exercise training increases exercise capacity, decreases dyspnea and improves health-related quality of life in CF. Adherence to pulmonary rehabilitation programs is a key factor to guarantee optimal benefits and a difficult goal in this population. The aim of this study was to determine the physiological response during three Nintendo Wii™ video game activities (VGA) candidates to be used as training modalities in patients with CF. ⋯ Active video game are well tolerated by patients with CF. All the modalities evaluated imposed a constant load but were associated with different physiological responses reflecting the different intensities imposed. Wii-Acti and Wii-Train impose a significantly high metabolic demand comparable to the 6MWT. Further research is needed to evaluate the effects of VGA as a training program to increase exercise capacity for CF patients.
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Despite increased life expectancy among patients with cystic fibrosis (CF), few studies have examined coping among adolescents and young adults with CF. Previous research suggests that stigma associated with chronic disease is related to worse physical and psychological health, but optimism may be protective. This study examined stigma and optimism among patients with CF. ⋯ Stigma is related to worse lung function and psychological health in patients with CF, but higher levels of optimism may act as a protective factor.