[Registry of Members of the Association of Patients with Hereditary Angioedema of Perú].

Abstract

Objective: To report the registry of the HAE Peruvian patient's association.

Methods: We used the questionnaire of the Latin American HAE committee. Consent was requested from the patient's association to report the data.

Results: We report data of 63 patients, 51 Female, 12 Male, range age between 6 to 74 years. Nine under 18 years old, 5/9 between 6 to 13 years. Forty-five HAE C1-INH type I, 12 HAE-FXII, 5 HAE UNK, 1 AAE. Symptoms onset average age in 56/62 HAE patients was 16.8. In a group of 50/62 adult HAE patients, the average diagnostic delay approximately was 19.3 years. Laboratory tests: we can perform C4 complement C1-inhibitor antigenic and functional tests. Treatments: The patients have access to tranexamic acid (TA) and attenuated androgens. We do not have registered specific long-term prophylaxis treatments. We used moderate/high doses of TA, in most patients up to 6 gr i.v./in 24 hours, we start with the treatment immediately the HAE acute crisis is beginning, it helps to the HAE attacks are less symptomatic, resolves in a few days and decrease the frequency.

Conclusions: We present 63 members of the Association of Patients with Hereditary Angioedema of Perú. We have improved blood tests for HAE diagnosis. Moderate and high doses of Tranexamic Acid are used for prophylaxis and acute crisis respectively, with acceptable response. No deaths have been reported due to HAE crisis in the patient's association.