Hereditary edema of the quinta

It flows with periodic exacerbations,manifested painful swelling of the skin, mucous upper respiratory tract and gastrointestinal tract. In 26% of patients, the laryngeal edema leads to death. Exacerbation can be triggered by a small injury, emotional overexertion, infection, sudden changes in temperature. The disease is inherited by an autosomal dominant type. It is due to the deficiency of the C1-esterase inhibitor. Activation of complement results in excessive accumulation of C-kinin and other mediators, which cause edema.

Hereditary edema Quincke should be suspected,if there is a history of frequent swelling, especially after trauma. In 80% of cases, the disease is of a family nature. The most accurate method of diagnosis is to determine the level of C1-esterase inhibitor, which is almost all patients significantly reduced.

Treatment and prevention: Fresh or fresh-frozen native plasma as source C1 - Inactivator should be administered in a dose of at least 250-300ml one-stage or amikaproic acid is intravenously dripped for 100-200 ml. 5% solution, then 100 ml. drip every 4 hours or 4 grams orally until complete relief of the sharpness. Instead of aminocaproic acid tranexamic acid 1-1.5 grams per day can be used 2-3 times a day. As a preventive treatment, patients are prescribed danazol (danol, danaval) in the initial daily dose of 600 ml. Upon achievement of clinical remission, the patient takes 200 mg. per day constantly. An analogue of danazol is stanozolol 1-2 mg each. per day according to a similar scheme. If there are contraindications to taking danazol and stanozolol, the appointment of aminocaproic acid 4-12 g per day orally or tranexamic acid 1-1.5 g per day under the control of the blood coagulation system is recommended.

Submit date: 2015-07-14; views: 30;

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