Endothelin-1 in primary pulmonary hypertension and the Eisenmenger syndrome.
Endothelin-1 in primary pulmonary hypertension and the Eisenmenger syndrome.
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DOI：
10.1016/0002-9149(93)90452-i
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发表时间：
1993-02
期刊：
影响因子：
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通讯作者：
P. Cacoub;R. Dorent;G. Maistre;P. Nataf;A. Carayon;C. Piette;P. Godeau;C. Cabrol;I. Gandjbakhch
中科院分区：
文献类型：
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作者：
P. Cacoub;R. Dorent;G. Maistre;P. Nataf;A. Carayon;C. Piette;P. Godeau;C. Cabrol;I. Gandjbakhch
原发性肺动脉高压(PPH)是一种罕见的疾病，其病因和发病机制尚不清楚。PPH的组织学特征是内皮细胞损伤和肺动脉平滑肌细胞的增殖。血管收缩在PPH的病理生理学中的作用被早期自发逆转的可能性和PPH1患者中雷诺现象的发生率高于预期所支持。1血管收缩的启动机制以及梗阻的持续或进展尚不清楚。内皮素-1(ETL1)是一种新近从血管内皮细胞中分离出来的多肽，2具有强大的血管收缩活性，并诱导血管平滑肌细胞增殖。3、4这些观察表明，ETL1可能在PPH小动脉张力增加或中层肥厚或两者兼而有之的过程中发挥重要作用。在这项研究中，我们测量了PPH患者的静脉血浆ETL1浓度，并将其与先天性心脏病继发肺动脉高压患者和正常受试者的浓度进行了比较。
Primary pulmonary hypertension (PPH) is an uncommon condition, the etiology and pathogenesis of which are unknown. PPH is histologically characterized by endothelial injury and the proliferation of pulmonary arterial smooth muscle cells. A role for vasoconstriction in the pathophysiology of PPH is supported by the possibility of spontaneous reversal at early stages and by the greater than expected incidence of Raynaud's phenomenon in patients with PPH.1The mechanism of the initiation of vasoconstriction, and the perpetuation or progression of the obstruction are unclear. Endothelin-1 (ETL1), a newly isolated peptide from vascular endothelial cells,2has potent vasoconstricting activity and induces vascular smooth muscle cell proliferation.3,4These observations suggest that ETL1 may have an important role in the increased vascular tone or medial hypertrophy, or both, of small arteries observed in PPH. In this study, we measured venous plasma ETL1 concentrations in patients with PPH, and compared them with those found in patients with pulmonary hypertension secondary to congenital heart defects and in normal subjects.