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hereditary angioedema
hereditary angioedeman. 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.EncyclopediaSeeangioedemahereditary angioedema
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·maan 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 angioedeman. A hereditary disorder characterized by recurrent episodes of subcutaneous and submucosal swelling, especially affecting the limbs, face, upper respiratory tract, and gastrointestinal tract.Hereditary angioedemaA complement deficiency characterized by lymphatic vessel blockages that cause temporary swelling (edema) of areas of the skin, mucous membranes, and, sometimes, internal organs.Mentioned in: Complement Deficiencieshe·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. See HAE See HAE |