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Angioedema: Hereditary Angioedema
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Angioedema is a problem that relates to the specialty of otolaryngology because its symptoms manifest most often in the head and neck, often in dramatic form. The term angioedema is used to describe several closely related diseases that manifest by recurrent acute edema of the skin and mucosa. The most serious manifestation of the disease is laryngeal edema which can be fatal. Hereditary angioedema is rare, but knowledge of its mechanism allows diagnosis and initiation of potentially lifesaving therapy. Angiotensin converting enzyme inhibitors account for an increasing proportion of angioedema cases as the popularity of the drug class grows.
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Hereditary Angioedema (HAE) is a rare and serious genetic condition occurring in about 1/10,000 to 1/50,000 individuals. The disease is characterized by episodes of edema (swelling) in body parts, most notably the hands, feet, face, and airway passages. In addition, patients often have bouts of excruciating abdominal pain, nausea, and vomiting that is caused by swelling in the intestinal wall.
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Angioedema, which may be hereditary or non-hereditary, is characterized by an acute-onset non-pitting edema of the skin, mucosa and sc tissues. Hereditary angioedema is a rare autosomal dominant disorder which is characterized by recurrent attacks of angio-edema resulting from a deficiency of C1 esterase inhibitor enzyme (C1-INH).6 The complement system consists of about 20 proteins, most of which circulate as inactive precursors. Activation of the system via one of two pathways - classical or alternative, results in opsonization, lysis, chemotaxis, histamine release and immune complex clearance. The enzymes are identified by the numbers C1–C9. The first component of the classical complement pathway is C1, which is composed of three subunits C1q, C1r and C1s.58 C1 binds to immunoglobulins which have bound antigen, and this triggers the sequence of events which activate other components of the cascade. Control proteins, including C1 esterase inhibitor enzyme (C1-INH), normally limit the amount of complement activation, but in the presence of hereditary or acquired C1-INH deficiency the complement pathway is activated, leading to the generation of biologically active substances such as bradykinin.
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Angioedema is a swelling similar to urticaria (hives), but the swelling is beneath the skin rather than on the surface. There seems to be a hereditary tendency toward the development of both angioedema and hives (see hereditary angioedema). Angioedema is associated with the release of histamine and other chemicals into the bloodstream, which is part of the allergic response.
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Angioedema is a hereditary condition that is ... tied to allergies and histamine release into the bloodstream. Common angioedema allergens include medications, food, pollen, insect bites, stress, pet dander and exposure to environmental elements.
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It is possible that the main title of the report Angioedema, Hereditary is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
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Hereditary Angioedema