Current opinion in pulmonary medicine
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The current paradigm shift in the diagnosis of sleep apnea in adults has further emphasized the urgent need for the development and validation of less inconvenient and laborious approaches than the in-laboratory nocturnal polysomnography for evaluation of children. ⋯ The overall improvements in technologies and in our understanding of pediatric sleep-disordered breathing should enable population-tailored effective home-based diagnostic approaches that reduce the overall burden to the family, while achieving high levels of diagnostic accuracy. Newer algorithms will have to be developed and validated to allow for effective implementation of such approaches.
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MRI has emerged as radiation-free imaging modality for assessment of cystic fibrosis lung disease. Here, we review the current status and new developments of MRI in cystic fibrosis. ⋯ MRI is sensitive to detect hallmarks of cystic fibrosis lung disease such as bronchial wall thickening, bronchiectasis, mucus plugging and abnormal lung perfusion. A morpho-functional MRI score has been established for semiquantitative assessment of these characteristic abnormalities over a broad range of disease severity. Recent studies demonstrated that MRI is sensitive to detect changes in mucus plugging and lung perfusion in response to antibiotic therapy for pulmonary exacerbations. These results suggest that MRI may be suitable for noninvasive monitoring and as a quantitative endpoint in clinical trials for cystic fibrosis. Technical advances including ultra-short echo time and Fourier decomposition imaging are expected to enhance morphological and functional MRI of cystic fibrosis lung disease without the need of contrast medium in the near future.
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With our growing understanding of the pathophysiology of cystic fibrosis, the pace of drug discovery is accelerating. Newer agents and therapies have traditionally been added to available medications, given the urgency in treating the disease. As the cystic fibrosis population ages, the number of associated comorbidities increases, requiring additional therapeutic approaches. Thus, while current management strategies have dramatically extended projected life expectancy, the treatment burden of the disease in adulthood has become onerous, and there is increasing concern over unintended effects and drug-drug interactions of new and existing therapies. ⋯ As the cystic fibrosis formulary grows, a primary emphasis will be for providers to develop personalized treatment plans, with a goal to reduce unnecessary treatment burden and an awareness of potential unanticipated effects of medications.
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About one out of 10 patients with idiopathic pulmonary fibrosis (IPF) develop lung cancer. This review provides an epidemiology and clinical update of the association of these two lethal diseases. In addition, we focus on the emerging overlapping epigenetic mechanisms in both diseases. ⋯ The two novel drugs approved for IPF, pirfenidone and nintedanib, open a new scenario in which treated patients with fibrosis will live longer, and possibly have a lower incidence of lung cancer. However, prospective studies are urgently needed to definitively clarify the role of lung cancer treatment in the management of IPF patients. Furthermore, common epigenetic alterations may represent a promising target for therapeutic approaches in the near future.