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 ?
Open-Label Use of Nanofiltered C1 Esterase Inhibitor (Human) (C1 INH-nf) for Treatment of Acute Attacks of Hereditary Angioedema (HAE) in Pediatric Subjects
Food and Drug Administration for approval of DX-88 for the treatment of hereditary angioedema (HAE).
Lev's lead product candidate, Cinryze (C1 inhibitor), is being developed as a replacement therapy for both the acute and prophylactic treatment of hereditary angioedema (HAE), also known as C1 inhibitor deficiency.
CSL Behring therapies are used around the world to treat coagulation disorders including hemophilia and von Willebrand disease, primary immune deficiencies, hereditary angioedema and inherited respiratory disease, and neurological disorders in certain markets.
ViroPharma provides treatments for patients living with rare diseases including hereditary angioedema, adrenal insufficiency, prolonged, acute, convulsive seizures in children, and Clostridium difficile infection.
Dyax developed KALBITOR on its own and, since February 2010, the Company has been selling it in the United States for the treatment of acute attacks of hereditary angioedema (HAE) in patients 16 years of age and older.
Hereditary angioedema is a very rare disease, with only 1,000 cases registered in Spain, but its patients still need a treatment, he added.
Findings demonstrated that the clinical trial, which assessed the safety and efficacy of the investigational drug Ruconest 50 U/kg for the treatment of acute attacks of angioedema in patients with hereditary angioedema (HAE), met the primary endpoint of time to beginning of symptom relief.
Type 1 hereditary angioedema results from a mutation in C1 esterase inhibitor gene leading to decreased secretion of the protein, thus leading to reduced plasma levels.
As its name suggests, hereditary angioedema is an inherited disorder.
Urine-histamine levels in patients with hereditary angioedema (HAE).

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