The Lancet. Respiratory medicine
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Lumacaftor/ivacaftor combination therapy has shown clinical benefits in patients with cystic fibrosis homozygous for the Phe508del CFTR mutation; however, pretreatment lung function is a confounding factor that potentially affects the efficacy and safety of this therapy. We aimed to assess the efficacy and safety of lumacaftor/ivacaftor therapy in these patients, defined by specific categories of lung function. ⋯ Vertex Pharmaceuticals.
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Acute pulmonary exacerbations are associated with progressive lung function decline and increased mortality in cystic fibrosis. The role of pulmonary vascular disease in pulmonary exacerbations is unknown. We aimed to assess the association between pulmonary artery enlargement (defined as pulmonary artery diameter to ascending aorta diameter [PA:A] ratio >1), a marker of pulmonary vascular disease, and exacerbations. ⋯ US National Heart, Lung, and Blood Institute/National Institutes of Health, Cystic Fibrosis Foundation, the University of Alabama at Birmingham, and the Queensland Health Fellowship.
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Randomized Controlled Trial Multicenter Study
Effect of non-invasive oxygenation strategies in immunocompromised patients with severe acute respiratory failure: a post-hoc analysis of a randomised trial.
The use of non-invasive ventilation is controversial in immunocompromised patients with acute respiratory failure, whereas the use of high-flow nasal cannula oxygen therapy is growing as an alternative to standard oxygen. We aimed to compare outcomes of immunocompromised patients with acute respiratory failure treated with standard oxygen with those treated with high-flow nasal cannula oxygen alone or high-flow nasal cannula oxygen associated with non-invasive ventilation. ⋯ French Ministry of Health, the French societies of intensive care (Société de Réanimation de Langue Française, SRLF) and pneumology (Société de Pneumologie de Langue Française, SPLF), La Mutuelle de Poitiers, AADAIRC (Association pour l'Assistance à Domicile Aux Insuffisants Respiratoires Chroniques), and Fisher&Paykel Healthcare.
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Randomized Controlled Trial Comparative Study
Non-invasive ventilation by helmet more effective than face mask in acute respiratory distress syndrome.