Article
Hereditary angioedema: Pathophysiology (HAE type I, HAE type II, and HAE nC1-INH).
Allergy and asthma proceedings - 1 Nov 2020
Wedner H James
Abstract excerpt
The pathophysiology of hereditary angioedema (HAE) in virtually all cases is the result of the uncontrolled production of the vasoactive peptide bradykinin. C1 inhibitor (C1-INH) is a serine protease inhibitor, which, under normal circumstances, is the regulator of critical enzymes that are active in the cascades that result in bradykinin generation. In the classic forms of HAE, C1-INH is not produced in...
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