hereditary angioedema


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angioedema

 [an″je-o-ĕ-de´mah]
a localized edematous reaction of the deep dermis or subcutaneous or submucosal tissues appearing as giant wheals; urticaria is the same physiologic reaction occurring in the superficial portions of the dermis.
hereditary angioedema an autosomal dominant disorder of the complement system manifested as recurrent episodes of edema of the skin, upper respiratory tract, and gastrointestinal tract. It may be mediated by such factors as minor trauma, sudden changes in environmental temperature, and sudden emotional stress. adj., adj angioede´matous.

he·red·i·tar·y an·gi·o·e·de·ma

an inherited, autosomal-dominant disease characterized by episodic appearance of brawny nonpitting edema, most often affecting the limbs, but capable of involving other parts of the body, including mucosal surfaces such as those of the intestine (causing abdominal pain) or respiratory tract (causing asphyxia, which can require intubation to avoid fatal outcome). Associated with deficiency of inhibitor of first component of complement pathway (C1). Epinephrine is used in emergency treatment, long-term treatment with a variety of agents is effective.

hereditary angioedema

n.
A hereditary disorder characterized by recurrent episodes of subcutaneous and submucosal swelling, especially affecting the limbs, face, upper respiratory tract, and gastrointestinal tract.

hereditary angioedema

an inherited autosomal-dominant disorder characterized by the episodic appearance of nonpitting edema involving any part of the body, including mucosal surfaces. The attacks last 48 to 72 hours and can be life-threatening if edema obstructs the airway.

Hereditary angioedema

A complement deficiency characterized by lymphatic vessel blockages that cause temporary swelling (edema) of areas of the skin, mucous membranes, and, sometimes, internal organs.

he·red·i·tar·y an·gi·o·e·de·ma

(hĕr-edi-tar-ē anjē-ō-ĕ-dēmă)
Inherited disease characterized by episodic appearance of brawny nonpitting edema, most often affecting the limbs, but capable of involving other body parts, including mucosal surfaces of the intestine or respiratory tract.

angioedema

a condition characterized by the sudden and temporary appearance of large areas of painless swelling in the subcutaneous tissue or submucosa, with or without pruritus. Caused by immunological reactions, usually immediate type hypersensitivities. Sometimes referred to as angioneurotic edema.

hereditary angioedema
in humans, the periodic occurrence of angioedema caused by a deficiency of the complement regulatory protein.
References in periodicals archive ?
Increased activity of coagulation factor 12 (Hageman Factor) causes hereditary angioedema type 3.
Treatment of acute edema attacks in hereditary angioedema with a bradykinin receptor-2 antagonist (Icatibant).
Symptoms, course, and complications of abdominal attacks in hereditary angioedema due to C1 inhibitor deficiency.
Hermann G, Schneider L, Krieg T et al: Efficacy of danazol treatment with the new variant of hereditary angioedema (HAE III).
Other studies have suggested that the mortality rate of hereditary angioedema is 15%-33%.
Four members of a previously-reported family with hereditary angioedema were studied.
A review of the Hereditary Angioedema (HAE) products under development by companies and universities/research institutes based on information derived from company and industry-specific sources.
According to the company, RUCONEST (conestat alfa) is a recombinant human C1 esterase inhibitor approved for the treatment of acute Hereditary Angioedema ("HAE") attacks in patients in Europe, the US, Israel and South Korea.
The report reviews key players involved in the therapeutics development for Hereditary Angioedema (HAE) (C1 Esterase Inhibitor [C1-INH] Deficiency) and enlists all their major and minor projects
This is a bright new future for patients with hereditary angioedema, " Dr.
today announced the launch of RUCONEST 50 IU/kg in the United States for the treatment of acute angioedema attacks in adult and adolescent patients with hereditary angioedema (HAE).

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