Resp Care
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Case Reports
Lung transplantation for Williams-Campbell syndrome with a probable familial association.
Williams-Campbell syndrome is a rare disorder characterized by deficiency of subsegmental bronchial cartilage and development of airway collapse and bronchiectasis that may subsequently progress to respiratory failure and death. There are only 2 published reports suggesting a familial association, and only one report of lung transplantation being used as a therapeutic modality. Due to postoperative airway complications, transplantation has not been recommended for this disease. We report the first lung transplant with prolonged survival, approaching 10 years, in a patient with Williams-Campbell syndrome, and provide further evidence to support a familial association.
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This study investigated whether descriptors of breathlessness differed after participation in an 8 week pulmonary rehabilitation program and whether changes in sensory quality would be reflected in responsiveness to pulmonary rehabilitation. ⋯ The sensory quality of breathlessness was modified for approximately one third of subjects after pulmonary rehabilitation, with significant improvements in the 6MWD for subjects who reduced their use of descriptors of air hunger.
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Electrical activity of the diaphragm (E(di)) has been proposed as a parameter to evaluate respiratory function. Normative values of electrical activity of the diaphragm in full-term neonates are not known. This is a small case series to establish preliminary values of E(di) in term neonates and to determine how these values vary while awake and asleep and during feeding states. ⋯ These are the first preliminary values for E(di) in neonates. Higher E(di) peak while awake may reflect larger tidal volume to meet increased metabolic requirements when awake and active. Post-prandial lower E(di) peak and higher respiratory rate may indicate compensation for decreased tidal volume from increased intra-abdominal pressure. These data may be useful in identifying respiratory pathology in neonates and monitoring progression toward respiratory health.
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Patients with COPD and patients with bronchiectasis undertake airway clearance therapy and exercise as part of management, but the effect of these activities on gastroesophageal acid exposure is unknown. This study aimed to determine if positive expiratory pressure (PEP) therapy and standardized exercise tasks were associated with increased gastroesophageal reflux. ⋯ Episodes of gastroesophageal reflux may occur during physiotherapy tasks, including airway clearance therapy using mouthpiece PEP, the 6MWT, and a measure of upper limb movement. However, as these activities did not increase the frequency of these events, no modifications to these tasks to minimize the occurrence of gastroesophageal reflux are necessary.
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Expiratory positive airway pressure (EPAP) is a form of noninvasive positive-pressure ventilatory support that, in spite of not unloading respiratory muscles during inspiration, may reduce the inspiratory threshold load and attenuate expiratory dynamic airway compression, contributing to reduced expiratory air-flow limitation in patients with COPD. We sought to determine the effects of EPAP on operational lung volumes during exercise in COPD patients. ⋯ The application of EPAP reduced DH, as shown by lower operational lung volumes after submaximal exercise in COPD patients who previously manifested exercise DH.