摘要
肺动脉高压（pulmonary arterial hypertension，PAH）是儿科常见的一种难治性疾病．以肺血管床的进行性狭窄为主要特征，分原发性（IPAH）和继发性PAH。无论是IPAH还是继发性PAH。它们的共同病理生理特点是致病因素造成直接肺血管床的损伤。肺血管收缩和重塑、原位血栓形成和血管内皮损伤是可能的病因，血管内皮细胞功能紊乱导致血管收缩和舒张障碍．上皮细胞一氧化氮（NO）及前列环素（PGI2）的合成下降及内皮素（ET）表达的增加引起肺血管收缩反应增强和血管结构重塑．
出处
《江西医药》
CAS
2007年第9期851-853,共3页
Jiangxi Medical Journal
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