Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
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Multicenter Study
Effects of ivacaftor on severely ill patients with cystic fibrosis carrying a G551D mutation.
Recently, ivacaftor, a CFTR-potentiator, has been shown to be effective and safe in patients with cystic fibrosis carrying a G551D mutation and moderately impaired lung function. The objective of this retrospective study was to assess efficacy and safety of ivacaftor in severely ill patients with at least one G551D mutation. ⋯ Ivacaftor was effective in many patients with poor lung function. The response was, however, variable. Although the drug appeared safe for most of these patients, increased bronchial secretions may warrant intensified physiotherapy and intravenous antibiotic treatment when ivacaftor is initiated.
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Observational Study
Xbox Kinect™ represents high intensity exercise for adults with cystic fibrosis.
Exercise is important for patients with cystic fibrosis (CF). Interactive gaming consoles are a new trend in exercise. This study sought to determine the exercise intensity of training using the Xbox Kinect(™). ⋯ Training using the Xbox Kinect™ represents high intensity exercise for adults with CF and may be a suitable alternative to conventional exercise modalities.
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Multicenter Study
Improved air trapping evaluation in chest computed tomography in children with cystic fibrosis using real-time spirometric monitoring and biofeedback.
The quality of chest Computed Tomography (CT) images in children is dependent upon a sufficient breath hold during CT scanning. This study evaluates the influence of spirometric breath hold monitoring with biofeedback software on inspiratory and expiratory chest CT lung density measures, and on trapped air (TA) scoring in children with cystic fibrosis (CF). This is important because TA is an important component of early and progressive CF lung disease. ⋯ In children with comparable CF lung disease, spirometric breath hold monitoring during examination yielded a large difference in lung volume between inhalation and exhalation, and allowed for a significantly greater measured change in lung density and TA score, compared to unmonitored breath hold maneuvers. This has implications to the clinical use of chest CT, especially in children with early CF lung disease.
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We studied the ability of 4 single-breath gas washout (SBW) tests to measure immediate effects of airway clearance in children with CF. ⋯ With the tidal DTG-SBW, an easy and promising test for peripheral gas mixing efficiency, immediate pulmonary function response to airway clearance can be assessed in CF children.