A Registry of members of the Association of Patients with Hereditary Angioedema of Perú4o

Abstract

Introduction:
Hereditary Angioedema (HAE) is a rare disease characterized by episodes of swelling, HAE crisis could cause death by suffocation, also affect the quality of life in these patients, In Perú there are 33 million inhabitants, according to the worldwide prevalence (1:50,000) it is estimated that there could be approximately 700 patients with HAE C1-Inhibitor.
Our main objective is to report the registry of the HAE Peruvian patient’s association.
Material and 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 test 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.