Clinical and epidemiological profile of patients with hereditary angioedema treated in a referral outpatient clinic in Vitória, Espírito Santo - Brazil
نویسندگان
چکیده
Results Data from 51 patients, 29 (57%) females and 22 (43%) males, from 5 to 88 years old (mean: 32 years) was evaluated. Patients belonged to 7 families, 20 of them from the same family. The mean age of onset was 10 years and of diagnosis 26 years. Fifty (98%) patients were symptomatic, and 28 (55%) had experienced laryngeal edema. Deaths by laryngeal edema had occurred in 6 families. Crisis triggering factors were identified in 44 (86%) patients. Forty-five (88%) patients presented HAE due to quantitative deficit of C1-INH. Maintenance treatment was required for 32 (63%) patients, of whom 26 (81%) used Danazol, 5 (16%) Tranexamic acid, and 1 (3%) both. Thirteen (28%) patients needed icatibant to treat 23 crises.
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