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

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.
References in periodicals archive ?
- There are only seven drugs in active clinical development for hereditary angioedema, with five drugs in Phase I and two in Phase III.
An ABC of the warning signs of hereditary angioedema. Int Arch Allergy Immunol 2017; 174: 1-6, doi: 10.1159/000479839.
Witzke, "Fatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency," The Journal of Allergy and Clinical Immunology, vol.
Contraception in hereditary angioedema. Fertil Steril 2008;90(5):2015.e21-2.
Outcomes after ecallantide treatment of laryngeal hereditary angioedema attacks.
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.
Shire announced that the European Medicines Agency's, or EMA, Committee for Medicinal Products for Human Use, or CHMP, adopted a positive opinion recommending the granting of marketing authorization of lanadelumab injection for routine prevention of recurrent attacks of hereditary angioedema, or HAE, in patients aged 12 years and older.
M2 PHARMA-November 27, 2017-Pharming Group NV submits RUCONEST BLA with US FDA for hereditary angioedema
M2 EQUITYBITES-October 2, 2017-Pharming reports safe data from RUCONEST clinical trial in children with hereditary angioedema

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