Respiratory care
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Duchenne muscular dystrophy (DMD) is a devastating, progressive neuromuscular disease that results in cardiopulmonary failure and death. In 2018, the DMD Care Considerations guidelines were updated to improve the multidisciplinary approach to care and promote early respiratory management. We sought to evaluate the impact of a multidisciplinary clinic on access to pulmonary care and adherence to respiratory care guidelines. ⋯ Development of a multispecialty clinic expanded access to pulmonary care and evaluation in subjects with DMD. Continued care in this clinic will allow a better understanding of barriers to access and the opportunity to monitor long-term pulmonary health.
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Observational Study
Physical Activity and Sedentary Behavior in Adults With Cystic Fibrosis: Association With Aerobic Capacity, Lung Function, Sleep, Well-Being, and Quality of Life.
Physical activity (PA) and sedentary behavior (SB) have marked impact on key prognostic indicators such as aerobic capacity and lung function in people with cystic fibrosis (CF) and may have associations with sleep, well-being, and health-related quality of life (HRQOL). ⋯ Most participants did not meet PA targets. PA levels correlated to aerobic capacity, FEV1, and self-reported sleep quality, and this should be considered in longitudinal studies and in PA interventions.
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Noninvasive ventilation (NIV) facilitates management of acute respiratory failure without intubation. Many pediatric patients cannot tolerate the discomfort associated with noninvasive support and require sedation with agents that may decrease respiratory drive. Dexmedetomidine does not decrease respiratory drive, and we hypothesized that its use would increase tolerance of noninvasive respiratory support without increasing risk for intubation. ⋯ Dexmedetomidine may allow tolerance of NIV in acute respiratory failure without increasing risk for intubation, especially in preschool age patients and those with DD or ID. A larger study involving multiple centers would help support our conclusions.
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Successful extubation is difficult to predict. Ultrasound measurement of the diaphragm thickening fraction (DTF) might help predict weaning failure after cardiothoracic surgery. ⋯ U1111-1180-1999.