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• W2079350150 abstract "We examined inflammatory mediators in patients with pulmonary hypertension related to congenital systemic-to-pulmonary shunts and the change in these markers during treatment with bosentan.Inflammatory mechanisms probably play a pathogenic role in idiopathic pulmonary arterial hypertension. Their involvement in pulmonary hypertension related to congenital systemic-to-pulmonary shunts is largely unknown.Plasma levels of several inflammatory mediators were determined by enzyme immunoassays in 14 children and adolescents with pulmonary hypertension related to congenital systemic-to-pulmonary shunts before and after 12 months treatment with bosentan, and compared with levels in 54 healthy controls.The patients were characterized by increased plasma levels of von Willebrand factor ( approximately 2.5-fold), C-reactive protein ( approximately 3.5-fold), and soluble CD40 ligand ( approximately 2.5-fold) as compared with controls, representing markers of endothelial cell activation, systemic inflammation, and platelet-mediated inflammation, respectively. Patients also had significantly elevated plasma levels of osteoprotegerin ( approximately 1.6-fold). Within the study group, N-terminal pro-brain natriuretic peptide levels correlated significantly with the concentrations of C-reactive protein (r= 0.61, P < .027) and von Willebrand factor (r= 0.74, P= .004). Except for a decline in monocyte chemoattractant protein-1 and receptor activator of nuclear factor-kappaB ligand, bosentan therapy did not attenuate the systemic inflammation.Children and adolescents with pulmonary hypertension related to congenital systemic-to-pulmonary shunts are characterized by enhanced systemic inflammation involving increased endothelial cell activation and platelet-mediated inflammation. These inflammatory responses seem essentially to be unmodified by bosentan, potentially representing new targets for therapy in this disorder." @default.
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• W2079350150 date "2009-05-01" @default.
• W2079350150 modified "2023-10-18" @default.
• W2079350150 title "Patients with Pulmonary Hypertension Related to Congenital Systemic-to-Pulmonary Shunts are Characterized by Inflammation Involving Endothelial Cell Activation and Platelet-mediated Inflammation" @default.
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• W2079350150 doi "https://doi.org/10.1111/j.1747-0803.2009.00297.x" @default.
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