Chest
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The cystic fibrosis (CF) protein forms an anion channel in epithelial cells, and the absence or defective function of this channel results in the clinical manifestations of CF. CF is an autosomal recessive disorder, and its many disease-causing mutations divide into five or six classes. ⋯ We report complete normalization of sweat chloride concentration and rapid clinical improvement over 6 weeks of treatment with ivacaftor in a patient with CF with the gating mutation S549N. The findings suggest that ivacaftor should be considered for use in patients with any of the known gating mutations.
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The FEV3/FVC ratio is not discussed in the American Thoracic Society/European Respiratory Society (ATS/ERS) guidelines for lung function interpretation in spite of narrow confidence limits of normal and its association with smoking. We sought to determine whether a reduction in only the FEV3/FVC ratio was associated with physiologic changes compared with subjects with normal FEV1/FVC and FEV3/FVC ratios. ⋯ The FEV3/FVC ratio should be routinely reported on spirometry. An isolated reduction may indicate an early injury pattern of hyperinflation, air trapping, and loss of Dlco.
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The high costs of American health care, the related problem of the uninsured, and the grim fiscal prognosis of Medicare and Medicaid are among the most pressing challenges facing the United States today. A solution to the cost problem that does not reduce access or quality is sought by those at all points on the political spectrum. ⋯ Higher performing primary care, supported by changes in payment, has driven a sustainable business model that preserves the option of independent practice for physicians. We are now testing competencies developed for Medicare Advantage in the Pioneer Accountable Care Organization program, which preserves the broad patient choice that is an important feature of traditional Medicare.
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COPD is an important global health problem. In addition to pulmonary impairment, systemic inflammation, musculoskeletal abnormalities, and cardiovascular comorbidity influence disease burden and mortality risk. Body weight and body composition are important discriminants in classifying disease heterogeneity. ⋯ For maintenance of muscle and bone tissue, appropriately timed, high-quality protein intake and addressing vitamin D deficiency must be considered. Specific nutrients (eg, n-3 polyunsaturated fatty acids and polyphenolic compounds) may have the pharmacologic potential to boost decreased muscle mitochondrial metabolism and enhance impaired physical performance, particularly when the metabolic stimulus of physical activity alone is limited. At this stage, evidence is insufficient to support an intake of high doses of single nutritional supplements to modulate respiratory pathology, but some small studies have identified micronutrient modulation via the diet as a promising intervention.