hereditary angioedema


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hereditary angioedema

n.
A rare genetic disorder marked by episodes of painful swelling of the subcutaneous tissues, especially of the hands, feet, face, and genitals, and other body structures, including the abdominal organs. Swelling of the breathing passages can lead to asphyxiation if untreated.
References in periodicals archive ?
[ClickPress, Tue Jul 02 2019] This report on the global Hereditary Angioedema Market analyzes the current and future prospects of the market.
The "Market Spotlight: Hereditary Angioedema (HAE)" report has been added to ResearchAndMarkets.com's offering.
They were aged between 7 and 70 years (mean: 34.5 y; median: 32 y), and 36/51 (70.6%) had a family history of suspected or confirmed hereditary angioedema. Symptoms were reported in 46/51 (90.2%), and 5/51 (9.8%) remained asymptomatic.
The drug BCX7353, developed by BioCryst Pharmaceuticals, is taken orally and works by inhibiting plasma kallikrein, an enzyme overexpressed in hereditary angioedema, a rare genetic disease that causes severe tissue swelling.
The product is claimed to be the only monoclonal antibody that provides targeted inhibition of plasma kallikrein, an enzyme which is chronically uncontrolled in people with hereditary angioedema, to help prevent attacks.
Emel Aygören-Pürsün, M.D., from University Hospital Frankfurt in Germany, and colleagues randomized 77 patients with type I or II hereditary angioedema with a history of at least two angioedema attacks per month to BCX7353 (at four doses: 62.5, 125, 250, and 350 mg) or placebo.
The histologic findings are prominent minor salivary glands and a mixed inflammatory cell infiltrate.5 The differential diagnosis includes hereditary angioedema, early dermatochalasis, acquired cutis laxa and variants of granulomatous cheilitis.
The subject of the contract is continuous deliveries of medicinal products used for hereditary angioedema for 4 years according to the current needs of the sponsor.
Evaluation of the case reveals several failings in the patient's treatment; although the emergency physician was made aware of the nature of the edema, hereditary angioedema was not recognized by the treating physician.
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