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Synthesis and remodeling of the lung matrix is necessary for primary and compensatory lung growth. Because cyclic negative force is applied to developing lung tissue during the respiratory cycle, we hypothesized that stretch is a critical regulator of lung matrix remodeling. By using quantitative image analysis of whole-lung and whole-lobe elastin in situ(More)
Lung stretch is critical for normal lung development and for compensatory lung growth after pneumonectomy (PNX), but the mechanisms by which strain induces matrix remodeling are unclear. Our prior work demonstrated an association of chymotrypsin-like elastase 1 (Cela1) with lung elastin remodeling, and that strain triggered a near-instantaneous(More)
Thy-1 is a cell surface glycoprotein present on normal lung fibroblasts but absent from the fibroblastic foci of idiopathic pulmonary fibrosis. Thy-1 correlates inversely with fibrogenic phenotypic characteristics and functions as a "fibrosis suppressor." Promoter region hypermethylation can silence Thy-1 expression in fibroblastic foci, suggesting that(More)
OBJECTIVE Septic shock heterogeneity has important implications for clinical trial implementation and patient management. We previously addressed this heterogeneity by identifying three putative subclasses of children with septic shock based exclusively on a 100-gene expression signature. Here we attempted to prospectively validate the existence of these(More)
Wntless (Wls), a gene highly conserved across the animal kingdom, encodes for a transmembrane protein that mediates Wnt ligand secretion. Wls is expressed in developing lung, wherein Wnt signaling is necessary for pulmonary morphogenesis. We hypothesize that Wls plays a critical role in modulating Wnt signaling during lung development and therefore affects(More)
OBJECTIVE True pediatric emergencies are rare. Because resident work hours are restricted and national attention turns toward patient safety, teaching methods to improve physician performance and patient care are vital. We hypothesize that a critical-care simulation course will improve resident confidence and performance in critical-care situations. (More)
Thy-1 is a glycosylphosphytidylinositol-linked cell-surface glycoprotein present on a subset of lung fibroblasts, which plays an important role in postnatal alveolarization. In the present study, we define the role of Thy-1 in pulmonary lipofibroblast differentiation and in the regulation of lipid homeostasis via peroxisome proliferator-activated receptor-γ(More)
OBJECTIVE To report the successful reversal of intractable hypoxemia after exogenous surfactant (calfactant) administration, facilitating neurological recovery in a toddler cold-water drowning victim with significant circulatory arrest time. METHODS Case report and review of literature. RESULTS A 2(1/2)-year-old girl cold-water drowning victim with(More)
Acute lung injury (ALI) secondary to sepsis is one of the leading causes of death in sepsis. As such, many pharmacologic and nonpharmacologic strategies have been employed to attenuate its course. Very few of these strategies have proven beneficial. In this paper, we discuss the epidemiology and pathophysiology of ALI, commonly employed pharmacologic and(More)
Postnatal lung development requires coordination of three processes (surface area expansion, microvascular growth, and matrix remodeling). Because normal elastin structure is important for lung morphogenesis, because physiological remodeling of lung elastin has never been defined, and because elastin remodeling is angiogenic, we sought to test the(More)