Hereditary And Acquired Angioedema

(Acquired C1 inhibitor deficiency)

Hereditary and acquired angioedema (acquired C1 inhibitor deficiency) occur due to a deficiency or dysfunction of the C1 inhibitor, a protein that regulates the classical Komplementaktivierungsweg. The diagnosis is made by determining the Komplementspiegels. For treatment of acute attacks C1 inhibitor can be used. Prophylaxis with attenuated androgens to increase the C1 inhibitor.

C1 inhibitor deficiency or dysfunction resulting in increased bradykinin, because the C1 inhibitor inhibits the factor X11A and kallikrein activated (required for production of bradykinin in kinin system process).

Hereditary and acquired angioedema (acquired C1 inhibitor deficiency) occur due to a deficiency or dysfunction of the C1 inhibitor, a protein that regulates the classical Komplementaktivierungsweg. The diagnosis is made by determining the Komplementspiegels. For treatment of acute attacks C1 inhibitor can be used. Prophylaxis with attenuated androgens to increase the C1 inhibitor. C1 inhibitor deficiency or dysfunction resulting in increased bradykinin, because the C1 inhibitor inhibits the factor X11A and kallikrein activated (required for production of bradykinin in kinin system process). Hereditary angioedema have Hereditary angioedema 2 forms: type 1 (85%): characterized by C1 inhibitor deficiency type 2 (15%): characterized by C1 inhibitor dysfunction Inheritance carried autosomal dominant. Clinical shows it is usually during childhood or adolescence. Acquired C1 inhibitor deficiency C1 inhibitor deficiency can be acquired when the complement is consumed in neoplastic diseases (for. Example, B-cell lymphoma) or immune complex diseases. C1 inhibitor autoantibodies are produced in monoclonal gammopathy. In autoimmune diseases (eg., Systemic lupus erythematosus (SLE), dermatomyositis) of the C1 inhibitor autoantibodies is rarely produced. Clinical he is usually at older ages when patients have an associated disorder. Trigger In all forms of hereditary and acquired angioedema, attacks can be triggered by minor injuries (eg dental work, tongue piercing.), Viral disease, cold, pregnancy or the use of certain foods; Angioedema may be aggravated by emotional stress. Symptoms and discomfort symptoms and complaints are similar to those of other forms of bradykinin-mediated angioedema, with asymmetrical and slightly painful swelling, often on the face, lips and / or tongue. Also on the wrist or back of the foot or on the genitals Schwellunge may occur. The gastrointestinal tract is frequently involved with manifestations suggestive of bowel obstruction, nausea, vomiting, colic and discomfort. Pruritus, urticaria and bronchospasm have no symptoms, but a laryngeal edema may be present, which stridor (and sometimes death) caused. Swelling disappear within about 1 to 3 days after onset. In hereditary angioedema symptoms disappear after the administration of complement components. Angioedema with permission of the publisher. From Joe E., Soter N. In Current Dermatologic Diagnosis and Treatment, edited by I. Freedberg, I.M. Freedberg and M. R. Sanchez. Philadelphia, Current Medicine, 2001. var model = {thumbnailUrl: ‘/-/media/manual/professional/images/angioedema_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/ – / media / manual / professional / images / angioedema_high_de.jpg lang = en & thn = 0 ‘, title:’? angioedema ‘description:’ u003Ca id = “v37895576 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “”para “” u003e u003cp u003eAkute swelling of the lips in a patient with hereditary angioedema u003c / p u003e u003c / div u003e. ‘credits’ with permission of the publisher. From Joe E.

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