Current opinion in pulmonary medicine
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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.
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Interstitial lung diseases (ILDs) encompass diverse clinicopathological disease entities ranging from idiopathic interstitial pneumonia (IIP) to interstitial pneumonia accompanied by collagen vascular diseases and diseases related to smoking, drug reactions, occupational environments, infections, and malignancies. Our focus is on the smoking-related interstitial lung diseases. Specifically, we focus on reports suggesting that chronic smoking is deeply involved in the disease pathogenesis and on reports suggesting that chronic smoking may influence the clinical course in terms of either disease severity or progression. Pulmonary Langerhans' cell granulomatosis (PLCG), desquamative interstitial pneumonia (DIP), respiratory bronchiolitis with interstitial lung diseases (RB-ILD), interstitial pneumonia associated with rheumatoid arthritis, acute respiratory distress syndrome (ARDS), and idiopathic pulmonary fibrosis (IPF) are covered.
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High resolution computed tomography (HRCT) has considerably improved the ability to diagnose and characterize interstitial lung disease (ILD). This paper assesses the role of HRCT in evaluating small airway disease associated with ILD particularly sarcoidosis, hypersensitivity pneumonitis, and interstitial pulmonary fibrosis.
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Systemic lupus erythematosis (SLE) is a heterogenous disease of unknown etiology. It is not uncommon to see pleuropulmonary involvement in isolation or along with the involvement of other organ systems in SLE. Pulmonary manifestations of SLE can involve the pleura, lung parenchyma, airways, pulmonary vasculature, and the respiratory muscles. ⋯ These two conditions have a major impact on the mortality and morbidity of patients with SLE and it is essential to recognize and treat them appropriately. High-resolution computed tomographic scans of the chest and pulmonary function tests help to establish a diagnosis and aid long-term follow-up of these patients. High-dose corticosteroids are the mainstay of treatment for the two conditions, although other agents such as cyclophosphamide, azathioprine, intravenous gamma globulin, and plasmapheresis have been used with varying degrees of success.