American journal of respiratory and critical care medicine
-
Am. J. Respir. Crit. Care Med. · Jul 2017
Magnetic Resonance Imaging in the Prognostic Evaluation of Patients with Pulmonary Arterial Hypertension.
Prognostication is important when counseling patients and defining treatment strategies in pulmonary arterial hypertension (PAH). ⋯ MRI measurements reflecting right ventricular structure and stiffness of the proximal pulmonary vasculature are independent predictors of outcome in PAH. In combination with clinical data MRI has moderate prognostic accuracy in the evaluation of patients with PAH.
-
Am. J. Respir. Crit. Care Med. · Jul 2017
Observational StudyPhysical Activity in Pediatric Pulmonary Arterial Hypertension Measured by Accelerometry: A Candidate Clinical Endpoint.
The development of evidence-based treatment guidelines for pediatric pulmonary arterial hypertension (PAH) is hampered by lack of pediatric clinical trials. Trial design is hampered by lack of a feasible clinical endpoint in this population. ⋯ PA is markedly decreased in children with PAH. Accelerometer output correlated with clinical disease severity markers and may predict outcome. We showed an exciting potential of PA as a meaningful endpoint for clinical trials in pediatric PAH, although its clinical utility and prognostic value need to be further validated.
-
Am. J. Respir. Crit. Care Med. · Jul 2017
Microbes Associate with Host Innate Immune Response in Idiopathic Pulmonary Fibrosis.
Differences in the lung microbial community influence idiopathic pulmonary fibrosis (IPF) progression. Whether the lung microbiome influences IPF host defense remains unknown. ⋯ These findings suggest that host-microbiome interactions influence PFS and fibroblast responsiveness.
-
Am. J. Respir. Crit. Care Med. · Jul 2017
Reduced Frizzled Receptor 4 Expression Prevents WNT/β-catenin-driven Alveolar Lung Repair in COPD.
Chronic obstructive pulmonary disease (COPD), in particular emphysema, is characterized by loss of parenchymal alveolar tissue and impaired tissue repair. Wingless and INT-1 (WNT)/β-catenin signaling is reduced in COPD; however, the mechanisms thereof, specifically the role of the frizzled (FZD) family of WNT receptors, remain unexplored. ⋯ Reduced FZD4 expression in COPD contributes to impaired alveolar repair capacity.
-
Am. J. Respir. Crit. Care Med. · Jul 2017
Anti-histone Properties of C1 Esterase Inhibitor Protect Against Lung Injury.
Acute respiratory distress syndrome is characterized by alveolar epithelial cell injury, edema formation, and intraalveolar contact phase activation. ⋯ Collectively, C1INH administration provides a new therapeutic option for disorders associated with histone release.