Articles: chronic.
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Primary ciliary dyskinesia (PCD) is characterised by chronic suppurative lung disease, rhino-sinusitis, hearing impairment and sub-fertility. We have developed the first multidimensional measure to assess health-related quality of life (HRQoL) in adults with PCD (QOL-PCD). Following a literature review and expert panel meeting, open-ended interviews with patients investigated the impact of PCD on HRQoL in the UK and North America (n=21). ⋯ Cognitive testing confirmed that content was comprehensive and the items were well-understood by respondents. Content validity and cognitive testing supported the items and structure. QOL-PCD has been translated into other languages and is awaiting psychometric testing.
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Semin Respir Crit Care Med · Aug 2015
ReviewThe Role of Noninvasive Ventilation in the Hospital and Outpatient Management of Chronic Obstructive Pulmonary Disease.
Positive pressure noninvasive ventilation (NIV) has become widely accepted in the treatment of both hospitalized and outpatient subjects with chronic obstructive pulmonary disease (COPD). The support has evolved over the past two decades to be part of first-line management in acute exacerbations of COPD and is also instrumental in discontinuing mechanical ventilation in COPD patients with acute respiratory failure. It is also suitable for treatment of COPD with other associated conditions including pneumonia, following lung resectional surgery, with concomitant obstructive sleep apnea and as part of end-of-life care. ⋯ However, an approach with higher pressures with a goal of significant reduction in daytime PaCO2 may be an effective strategy. NIV can also facilitate exercise training in pulmonary rehabilitation. A portable device which can augment tidal volume during ambulation and other activities of daily living may further expand the use of NIV in COPD patients.
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The high frequency of readmissions in patients with COPD remains a significant problem. The impact of a pulmonologist follow-up visit during the month after discharge from hospital because of COPD exacerbation on reducing readmissions was examined. A profile of patients who did not attend the follow-up visits was built. ⋯ Early follow-up visits with pulmonologists seem to reduce the exacerbation-related rehospitalization rates of patients with COPD. We recommend that patients have early postdischarge follow-up visits with pulmonologists.
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Metabolomic evaluation of cystic fibrosis (CF) airway secretions could identify metabolites and metabolic pathways involved in neutrophilic airway inflammation that could serve as biomarkers and therapeutic targets. ⋯ MS metabolomics identified multiple metabolic pathways associated with neutrophilic airway inflammation. These findings provide insight into disease pathophysiology and can serve as the basis for developing disease biomarkers and therapeutic interventions for airways diseases.