Annals of the American Thoracic Society
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Review
Idiopathic Pulmonary Fibrosis Is a Genetic Disease Involving Mucus and the Peripheral Airways.
Idiopathic pulmonary fibrosis (IPF) is localized to the lung, is characterized by a pattern of heterogeneous, subpleural patches of fibrotic, remodeled lung, and is associated with a median survival of 3-5 years after diagnosis. A common gain-of-function MUC5B promoter variant, rs35705950, is the strongest risk factor (genetic and otherwise), accounting for at least 30% of the total risk of developing IPF. ⋯ Thus, MUC5B represents a key molecule to understand the mechanisms that appear to initiate the fibroproliferative process in the bronchoalveolar epithelium. Moreover, focusing on MUC5B may provide a unique opportunity to define the early molecular events that lead to, and potentially prevent, the development of IPF.
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Autopsy studies in fatal asthma have clearly documented the central role of airway plugging with pathologic mucus in the pathophysiology of death from asthma, but the role of mucus plugs in chronic severe asthma has been less well understood. Recently, multidetector computerized tomography imaging of the lungs has emerged as a valuable method to visualize mucus plugs in asthma. ⋯ In addition, an image-based mucus plug scoring system shows that mucus plugs are strongly associated with measures of airflow obstruction and with biomarkers of type 2 cytokine and eosinophilic inflammation. These data provide a rationale for treating airflow obstruction in severe asthma with mucolytics, and they also raise the possibility that treatments that target type 2 inflammation may decrease mucus plugs in asthma.
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Hypoxemia is a common complication during tracheal intubation of critically ill adults and is a frequently used endpoint in airway management research. Identifying patients likely to experience low oxygen saturations during tracheal intubation may be useful for clinical practice and clinical trials. ⋯ Lowest oxygen saturation and severe hypoxemia during tracheal intubation in the intensive care unit can be accurately predicted using routinely available preprocedure clinical data, with saturation at induction and hypoxemic respiratory failure being the strongest predictors. A simple bedside score may identify patients at risk for hypoxemia during intubation to help target preventative interventions and facilitate enrichment in clinical trials.
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Individuals diagnosed with cystic fibrosis (CF) as adults represent a growing subpopulation of CF cases, but there are limited studies describing their characteristics and prognosis. ⋯ CF care providers can use this information to inform individuals who received a diagnosis of CF as adults about their prognosis and to guide the necessity of treatments, specifically with regard to those who are at high risk for a worse prognosis.
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Although chronic obstructive pulmonary disease has been related to heart failure, the relationship between the restrictive spirometry pattern (forced vital capacity [FVC] < 80% predicted with preserved forced expiratory volume in 1 second [FEV1]/FVC ratio) and heart failure is poorly understood. ⋯ Both a restrictive pattern on spirometry and airflow obstruction identify African Americans with impaired lung health at risk for heart failure.