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Hereditary angioedema: a prospective study of a Brazilian single‐center cohort
- Source :
- International Journal of Dermatology. 59:341-344
- Publication Year :
- 2019
- Publisher :
- Wiley, 2019.
-
Abstract
- BACKGROUND Hereditary angioedema (HAE) is rare and still underdiagnosed in some countries. We aimed to describe HAE diagnosis and sociodemographic and clinical features in patients with HAE due to C1 inhibitor deficiency (HAE-C1-INH) followed up at a tertiary-level center in Rio de Janeiro, Brazil. METHODS A descriptive, cross-sectional study with prospective data collection of 138 Brazilian patients with HAE was performed. From the total, 107 patients with HAE-C1-INH were selected. Data were assessed based on a specific questionnaire. RESULTS One hundred and five patients had HAE type I (76.1%), and two had HAE type II (1.4%). Seventy-two were female (67.3%), and 35 were male (32.7%). Mean age was 38.0 ± 15.0 years (range: 12-73 years). A long delay (17.7 ± 12.6 years) until diagnosis was observed. About 86.9% had a familial history. Cutaneous edema (95.8%), abdominal pain (88.5%), and laryngeal edema (65.6%) were the most frequent symptoms. Triggering factors (95.8%) and prodromal symptoms (47.9%) were referred. Attacks were severe in 55.1% and moderate in 24.3%. Eleven (10.3%) were asymptomatic. HAE attacks were more frequent and severe (P = 0.021) in females. CONCLUSIONS We observed a considerable delay in diagnosis, even with familial history. The severity of HAE attacks, especially in females, highlights the need for an awareness of disease by gynecologists and obstetricians. Screening of familial members, including asymptomatic individuals, is critical for earlier diagnosis. Regional evaluation of patient profiles can be helpful to draw more attention about HAE and to improve quality of life.
- Subjects :
- Adult
Male
Abdominal pain
Pediatrics
medicine.medical_specialty
Adolescent
Dermatology
Disease
Single Center
Asymptomatic
Young Adult
030207 dermatology & venereal diseases
03 medical and health sciences
0302 clinical medicine
Quality of life
medicine
Humans
Prospective Studies
Child
Prospective cohort study
Aged
business.industry
Angioedemas, Hereditary
Middle Aged
medicine.disease
Cross-Sectional Studies
030220 oncology & carcinogenesis
Hereditary angioedema
Cohort
Female
medicine.symptom
business
Brazil
Subjects
Details
- ISSN :
- 13654632 and 00119059
- Volume :
- 59
- Database :
- OpenAIRE
- Journal :
- International Journal of Dermatology
- Accession number :
- edsair.doi.dedup.....dcb0db2730636ca37980a6af1ed087cf
- Full Text :
- https://doi.org/10.1111/ijd.14676