American journal of respiratory and critical care medicine
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Am. J. Respir. Crit. Care Med. · Apr 2019
Elastase Exocytosis by Airway Neutrophils Associates with Early Lung Damage in Cystic Fibrosis Children.
Neutrophils are recruited to the airways of individuals with cystic fibrosis (CF). In adolescents and adults with CF, airway neutrophils actively exocytose the primary granule protease elastase (NE), whose extracellular activity correlates with lung damage. During childhood, free extracellular NE activity is measurable only in a subset of patients, and the exocytic function of airway neutrophils is unknown. ⋯ NE exocytosis by airway neutrophils occurs in all children with CF, and its cellular measure correlates with early lung damage. These findings implicate live airway neutrophils in early CF pathogenesis, which should instruct biomarker development and antiinflammatory therapy in children with CF.
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Am. J. Respir. Crit. Care Med. · Apr 2019
Bone Morphogenetic Protein 9 is a Mechanistic Biomarker of Portopulmonary Hypertension.
BMP9 (bone morphogenetic protein 9) is a circulating endothelial quiescence factor with protective effects in pulmonary arterial hypertension (PAH). Loss-of-function mutations in BMP9, its receptors, and downstream effectors have been reported in heritable PAH. ⋯ BMP9 is a sensitive and specific biomarker of PoPH, predicting transplant-free survival and the presence of PAH in liver disease. In rodent models, acquired deficiency of BMP9 signaling can predispose to or exacerbate PH, providing a possible mechanistic link between PoPH and heritable PAH. These findings describe a novel experimental model of severe PH that provides insight into the synergy between pulmonary vascular injury and diminished BMP9 signaling in the pathogenesis of PAH.
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Am. J. Respir. Crit. Care Med. · Apr 2019
Comparative StudyAge-Specific Associations of Ozone and PM2.5 with Respiratory Emergency Department Visits in the US.
Whereas associations between air pollution and respiratory morbidity for adults 65 years and older are well documented in the United States, the evidence for people under 65 is less extensive. To address this gap, the Centers for Disease Control and Prevention's National Environmental Public Health Tracking Program collected respiratory emergency department (ED) data from 17 states. ⋯ These results address a gap in the evidence used to ensure adequate public health protection under national air pollution policies.