Current opinion in pulmonary medicine
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Review
The art and science of continuous positive airway pressure therapy in obstructive sleep apnea.
Despite the high prevalence of obstructive sleep apnea (OSA) syndrome, no ideal therapy has emerged to date. Based on recent randomized trials, continuous positive airway pressure (CPAP) therapy is the treatment of choice. Although CPAP can prevent pharyngeal collapse in virtually all patients who choose to wear it, poor patient adherence with treatment limits its effectiveness. ⋯ Patient adherence with CPAP can be improved with optimization of mask comfort, heated humidification, and intensive support and education. For those who remain poorly compliant, alternative therapies such as autotitrating devices and oral positive airway pressure can be considered. Further research into the basic mechanisms underlying OSA will be required for new therapeutic targets to develop.
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The current standards of care for allergic bronchopulmonary aspergillosis in patients with cystic fibrosis are presented. Recent studies have increased understanding of the inflammatory process that occurs in cystic fibrosis patients with allergic bronchopulmonary aspergillosis. This has resulted in more precise diagnostic criteria that facilitate more timely diagnosis and treatment of ABPA in these patients. In addition to traditional treatment with systemic corticosteroids, promising results have been documented with antifungal therapy.
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Pulmonary infections continue to be a significant source of morbidity and mortality among patients with cystic fibrosis. Although our understanding of the pathogenesis and clinical consequences of pulmonary infections with Pseudomonas aeruginosa has increased greatly in recent years, very little is known about potentially emerging pathogens such as Burkholderia cepacia complex, Stenotrophomonas maltophilia, Alcaligenes xylosoxidans, and methicillin-resistant Staphylococcus aureus. In this review, the authors discuss methods for appropriate identification of these "unusual" organisms and their epidemiologic and clinical features. Multicenter surveillance studies are needed to more clearly establish the pathogenicity of these organisms.
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Recent advances in cellular and molecular biology have furthered the understanding of several genetic diseases, including cystic fibrosis. Mutations that cause cystic fibrosis are now understood in terms of the specific molecular consequences to the cystic fibrosis transmembrane conductance regulator (CFTR) protein expression and function. This knowledge has spawned interest in the development of therapies aimed directly at correcting the defective CFTR itself. ⋯ Opportunities for protein-repair therapy appear to be vast and range from naturally occurring compounds, such as isoflavonoids, to pharmaceuticals already in clinical use, including aminoglycoside antibiotics, butyrate analogues, phosphodiesterase inhibitors, and adenosine nucleotides. Future therapies may resemble designer compounds like benzo[c]quinoliziniums or take the form of small peptide replacements. Given the heterogeneity and progressive nature of cystic fibrosis, however, optimal benefit from protein-repair therapy will most likely require the initiation of combined therapies early in the course of disease to avoid irreparable organ damage.