American journal of respiratory and critical care medicine
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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.
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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.