Current opinion in pulmonary medicine
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Airway inflammation plays a central role in the lung disease of cystic fibrosis (CF). Biomarkers of inflammation may be useful for monitoring disease progression and evaluating response to therapy. Much of our knowledge of the chronic inflammatory process in the CF airway derives from studies of bronchoscopy and bronchoalveolar lavage. A number of noninvasive approaches have been recently developed to more readily assess airway inflammation including sputum induction, collection of exhaled air, analysis of systemic markers of inflammation, and computed tomography imaging. ⋯ It is hoped that biomarkers obtained by noninvasive means will be useful in determining specific pathways of injury (ie, oxidative or proteolytic) in individual persons with CF and in assessing response to antiinflammatory treatments.
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There is growing awareness of the significance of obstructive sleep apnea in the general population and in the medical community and, as a result, there is a growing demand for diagnosis and treatment. Attended, in-laboratory polysomnography is resource intensive and not readily available in some communities. Alternate diagnostic strategies have been proposed including the use of home sleep studies. Although these portable systems have been in use for many years, only in the past few years have a significant number of studies been performed to evaluate these systems in the home setting. The use of actigraphy and peripheral arterial tonometry for diagnostic purposes has also recently been investigated. ⋯ Home polysomnography is a viable option for evaluating patients with moderate or high clinical suspicion for sleep-disordered breathing. However, patients with failed or equivocal home studies and those with negative studies but persistent symptoms should undergo standard polysomnography. Further investigations are needed to compare long-term outcomes in patients evaluated using portable devices versus standard polysomnography.
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It has been an ongoing challenge to translate knowledge pertaining to the molecular basis of cystic fibrosis (CF) into a clear understanding of the development of CF lung disease. Various hypotheses have attempted to explain the apparent breach of innate defenses in CF, although a definitive explanation has been elusive. ⋯ With a better understanding of pathogenic steps leading to CF lung disease, we may now be able to direct the development of therapies that will substantially improve disease outcomes.