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中文Heritable Pulmonary Arterial Hypertension (FPAH)
Alias:
Hereditary Pulmonary Arterial Hypertension
Familial Pulmonary Arterial Hypertension
Familial Primary Pulmonary Hypertension
Fpah
Hpah
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遗传性肺动脉高压，也称为遗传性肺动脉高压，与肺静脉闭塞性疾病1、常染色体显性遗传和动静脉畸形有关。与遗传性肺动脉高压有关的重要基因是BMPR2（骨形态发生蛋白受体2），其相关通路/超级通路包括TGF-β家族成员的信号传导和Wnt / Hedgehog / Notch。在该疾病的背景下提到了药物奥昔美唑和苯肾上腺素。附属组织包括心脏和内皮，相关表型为shRNA丰度增加（Z-score> 2）和人类巨细胞病毒（HCMV）AD169株复制增加。
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