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Angioedema, Hereditary

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A genetic form of angioedema.Angioedema, hereditary: A genetic form of angioedema.(Angioedema is also referred to as Quinke's disease.) Persons with it are bornlacking an inhibitor protein (called C1 esterase inhibitor) that normally preventsactivation of a cascade of proteins leading to the swelling of angioedema. Patients candevelop recurrent attacks of swollen tissues, pain in the abdomen, and swelling of thevoice box (larynx) which can compromise breathing. The diagnosis is suspected with ahistory of recurrent angioedema. It is confirmed by finding abnormally low levels of C1esterase inhibitor in the blood. Treatment options include antihistamines and malesteroids (androgens) that can also prevent the recurrent attacks. Also called hereditaryangioneurotic edema.


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