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International journal of Immunopathology, allergology, infectology.

Demographic characteristics of patients with genetically confirmed hereditary angioedema in the Republic of Belarus

Guryanova I.E., Skapavets K.Ya., Liubushkin A.V., Polyakova E.A., Ausianik I.V., Aleshkevich S.N., Salivonchik A.P., Vertelko V.R., Zharankova Yu.S., Belevtsev M.V.

Belarusian Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Belarus
Belarusian National Public Organization HAE Patients Care, Grodno, Belarus Republican Research Centre for Radiation Medicine and Human Ecology, Gomel, Belarus

Introduction. Hereditary angioedema (HAE) is a rare, difficult to diagnose, and life-threatening genetic disorder with an expected incidence of about 1:30 000.
Purpose. To study the demographic characteristics in a cohort of patients with genetically confirmed hereditary angioedema in the Republic of Belarus.
Materials and methods. The study included 92 Belarusian patients from 34 unrelated families with genetically confirmed HAE, the ratio of male and female patients was 1:1.3. Clinical and demographic data was taken from patient questionnaires and/or medical records.
Results. The median age of tpatients was 32 years, and the median age at diagnosis was 28 years. The onset of the disease in 72% of patients occurred before the age of 18 years (median 13 years), the median delay from the onset of the first symptoms and the diagnosis of HAE was 12.5 years. Half of male patients develop first attacks before the age 9, and 90% by the age of 20. Of female patients, 50 and 90% have first attacks before the age of 15 and 23, respectively.

Keywords

Hereditary angioedema, C1-inhibitor, SERPING1

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DOI

10.14427/jipai.2022.2.12

Reference

Guryanova I.E., Skapavets K.Ya., Liubushkin A.V., Polyakova E.A., Ausianik I.V., Aleshkevich S.N., Salivonchik A.P., Vertelko V.R., Zharankova Yu.S., Belevtsev M.V. Immunopathology, allergology, infectology 2022; 2:12-18. DOI: 10.14427/jipai.2022.2.12