The pathobiology of pulmonary hypertension - Endothelium
The pathobiology of pulmonary hypertension - Endothelium
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DOI：
10.1016/s0272-5231(05)70280-x
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发表时间：
2001-09-01
影响因子：
5.7
通讯作者：
Voelkel, NE
中科院分区：
文献类型：
--
作者：
Tuder, RM;Cool, CD;Voelkel, NE
轻至中度的肺动脉高压(PH)的组织学特征是中膜平滑肌细胞肥大。重度PH(原发和继发)的特点是在肺动脉内存在内皮细胞簇。我们回顾了重度肺高压时肺内皮细胞功能障碍的概念演变。我们最新的发现表明，突变是PPH中内皮细胞增殖的基础，这表明内皮祖细胞可能被激活生长并最终扰乱肺动脉血流。
Mild to moderate pulmonary hypertension (PH) is characterized histologically by medial smooth muscle cell hypertrophy. Sever PH (primary and secondary) is characterized by the presence of intraluminal clusters of endothelial cells in pulmonary arteries. We review the evolving concepts of pulmonary endothelial cell dysfunction in severe PH. Our most recent findings indicate that mutations underlie the endothelial cell proliferation seen in PPH, suggesting that endothelial progenitor-like cells may be activated to grow and eventually disrupt pulmonary artery flow.