Hereditary 英 [həˈredɪtri] 美 [həˈredɪteri]
释义 adj. 遗传的；世袭的；世代相传的
例句 The susceptibility may be familial and hereditary.
释义 n. [医] 血管性水肿；神经性水肿
例句 Anaphylaxis and angioedema, which can occur as early as the first time the product is taken.
作者： Yahiya Y. Syed
Hereditary angioedema (HAE) due to C1 esterase inhibitor (C1-INH) deficiency (type 1 HAE) or dysfunction (type 2 HAE) is a rare, autosomal dominant disease caused by mutations in the SERPING1 gene, which encodes C1-INH. Plasma kallikrein (pKal) cleaves high-molecular- weight kininogen (HMWK) to generate bradykinin, which is a potent vasodilator that increases vascular permeability and leads to tissue swelling and pain associated with HAE attacks. Endogenous C1-INH regulates pKal activity; thus, C1-INH deficiency or dysfunction leads to an uncontrolled increase in pKal activity and bradykinin levels, resulting in HAE attacks.HAE attacks are managed by on-demand treatment with plasma-derived or recombinant C1-INH concentrates, a kallikrein inhibitor (ecallantide) or bradykinin-receptor antagonist (icatibant); some patients may also benefit from long-term prophylaxis.