作者: Aaron W. Trammell , Anna R. Hemnes
DOI: 10.1007/978-1-4939-2636-7_3
关键词: Bioinformatics 、 Thrombosis 、 Pathophysiology of hypertension 、 Disease 、 BMPR2 、 Medicine 、 Rare disease 、 Vascular disease 、 Inflammation 、 Pathogenesis
摘要: Pulmonary arterial hypertension (PAH) is a rare disease of the pulmonary vasculature characterized by progressive vascular obliteration, right heart failure, and ultimately death. Basic science has focused for past several decades on identification underlying molecular causes this disease, multiple potential derangements have been identified some led to development drugs treat PAH. Unfortunately, however, there no cure PAH research continues identify common mechanisms develop new, more effective therapies. This chapter highlights much our understanding basic pathobiology including genetic underpinnings, vasoactive substances, alterations in cell proliferation apoptosis, inflammation, thrombosis, endocrine factors.