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Hereditary Angioedema

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Key Points Î Most cases of hereditary angioedema (HAE) result from a deficiency of the serine protease inhibitor, C1 inhibitor (C1INH). (LB) Î HAE is caused by mutations in the C1INH gene, resulting in a C1INH functional deficiency. (LB) Î HAE is an autosomal dominant disease. Most patients with HAE have a positive family history of angioedema. (A) Î Two forms of HAE, which are indistinguishable clinically, can be diagnosed by laboratory findings: type I HAE presents with low C1INH antigenic and functional levels, whereas type II HAE presents with normal C1INH antigenic levels but decreased C1INH functional levels. (A) Î Diagnosis of type I or type II HAE requires evidence of low C1INH antigenic or functional levels, as well as decreased C4 levels and generally normal C1q levels. (A) Î Onset of swelling in HAE most often begins during childhood and frequently worsens around puberty. (C) Î HAE is characterized by relatively prolonged attacks of angioedema involving the extremities, genitourinary tract, abdomen, face, oropharynx or larynx. (C) Î The primary mediator of swelling in HAE is bradykinin. (A) Î Disease severity in HAE is highly variable, characterized by episodic rather than continuous swelling. (D) Î A precipitating cause for most episodes of HAE attacks is unknown, but stress and trauma have been clearly recognized as precipitants. (D) Î Attacks of swelling in HAE generally involve the extremities, abdomen, genitourinary tract, face, oropharynx, or larynx and follow a stereotypical pattern in which the swelling worsens over 24 hours, peaks, then slowly resolves over the following 48-72 hours. (A) Î Attacks of HAE may be preceded by a prodrome. (C) Î HAE attacks are associated with significant potential morbidity and potential mortality. (A) Î Optimal management of HAE depends on early identification of patients. (D)

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