TRANEXAMIC ACID THERAPY IN HEREDITARY ANGIONEUROTIC EDEMA
TRANEXAMIC ACID THERAPY IN HEREDITARY ANGIONEUROTIC EDEMA
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DOI：
10.1056/nejm197208312870907
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发表时间：
1972-01-01
影响因子：
158.5
通讯作者：
AUSTEN, KF
中科院分区：
文献类型：
--
作者：
SHEFFER, AL;ROSEN, FS;AUSTEN, KF
遗传性血管神经性水肿是由于血清α-2球蛋白生物合成中的先天性错误引起的，该球蛋白抑制补体第一组分的酶活性，因此被称为C1抑制剂（C1 β INH）。1由于这种缺陷，补体第一成分（C1）的作用不受抑制在受影响的患者的血浆中，随后消耗补体的第二和第四组分（C4和C2）。2，3似乎C1的间歇性激活与血管性水肿的发作在病理学上相关。因此，抑制血管性水肿发作的潜在策略可能是. .... .
HEREDITARY angioneurotic edema results from an inborn error in the biosynthesis of a serum alpha-2 globulin that inhibits the enzymatic activity of the first component of complement and is thus designated the C1 inhibitor (C1̄ INH).1As a consequence of this defect, there is unopposed action of the first component of complement (C1) in the plasma of affected patients and consequent consumption of the second and fourth components of complement (C4 and C2).2,3It appears that the episodic activation of C1 is pathogenetically related to attacks of angioedema. Thus, a potential strategy for the suppression of attacks of angioedema might . . .