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Location of Care Delivery for Pulmonary Arterial Hypertension in the United States.
Am J Respir Crit Care Med
January 2025
University of Utah, Division of Cardiovascular Medicine, Department of Medicine, Salt Lake City, Utah, United States.
Rationale: Guidelines recommend patients with pulmonary arterial hypertension (PAH) be referred to pulmonary hypertension (PH) centers, but little is known about where care is actually delivered in the United States (US).
Objectives: To use prescription patterns to estimate the proportion of PAH care delivered at US PH centers and explore factors associated with location of care.
Methods: This retrospective study analyzed claims from the Komodo database in adults who received ≥1 PAH prescription between March 2021 and February 2022.
Sotatercept in pulmonary hypertension and beyond.
Eur J Clin Invest
January 2025
Department of Surgical, Medical and Molecular Pathology and Critical Area, Laboratory of Biochemistry, University of Pisa, Pisa, Italy.
Sotatercept binds free activins by mimicking the extracellular domain of the activin receptor type IIA (ACTRIIA). Additional ligands are BMP/TGF-beta, GDF8, GDF11 and BMP10. The binding with activins leads to the inhibition of the signalling pathway and the deactivation of the bone morphogenic protein (BMP) receptor type 2.
View Article and Find Full Text PDFThe Role of Intravenous Selexipag in Managing PAH and Bridging Gaps in Oral Treatment: A Narrative Review.
Ther Clin Risk Manag
January 2025
Departments of Medicine and Cardiology, Westchester Medical Center and New York Medical College, Valhalla, NY, USA.
Pulmonary arterial hypertension (PAH) is a rare and potentially fatal condition characterized by progressive increases in blood pressure in the arteries of the lungs. Oral selexipag, approved by the Food and Drug Administration (FDA) in 2015 for the treatment of PAH, targets prostacyclin receptors on pulmonary arterial vascular smooth muscle and endothelial cells to improve blood flow through the lungs and reduce pulmonary vascular resistance. Oral selexipag is effective, but may be discontinued due to factors like side effects, emergency conditions, or inability to take oral medication, potentially leading to severe adverse events, such as rebound pulmonary hypertension and right heart failure.
View Article and Find Full Text PDFSimulation of clinical trials of oral treprostinil in pulmonary arterial hypertension using a virtual population.
NPJ Syst Biol Appl
January 2025
United Therapeutics Corporation, Silver Spring, MD, USA.
Challenges in drug development for rare diseases such as pulmonary arterial hypertension can be addressed through the use of mathematical modeling. In this study, a quantitative systems pharmacology model of pulmonary arterial hypertension pathophysiology and pharmacology was used to predict changes in pulmonary vascular resistance and six-minute walk distance in the context of oral treprostinil clinical studies. We generated a virtual population that spanned the range of clinical observations and then calibrated virtual patient-specific weights to match clinical trials.
View Article and Find Full Text PDFSafety and Efficacy of Selexipag for Pediatric Pulmonary Arterial Hypertension in Japanese Patients - An Open-Label Phase 2 Study.
Circ J
January 2025
Department of Echo-imaging Center, Aizawa Hospital.
Background: Selexipag, an oral prostacyclin (PGI) receptor agonist, is approved for adult patients with pulmonary arterial hypertension (PAH). This study evaluated the efficacy and safety of selexipag for Japanese pediatric patients with PAH.
Methods And Results: The study enrolled 6 patients who received selexipag twice daily at an individualized dose based on body weight; maintenance doses were determined for each patient by 12 weeks after starting administration.
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