Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children14910.24287/1726-1708-2016-15-1-54-60ИММУНОЛОГИЯPrinciples of diagnosis and treatment of hereditary angioedema (Review of literature)Принципы диагностики и лечения наследственного ангионевротического отека (обзор литературы)KuzmenkoNatalya B.КузьменкоНаталья Борисовна
Russian Federation
plunge@list.ruDibirovaSuna A.ДибироваСуна Абдурагимовна
Russian Federation
suna06@mail.ruVarlamovaTatyana V.ВарламоваТатьяна Владимировна
Russian Federation
varltatwell@mail.ruRaikinaElena V.РайкинаЕлена Владиславовна
Russian Federation
e_raikina@inbox.ruViktorovaEkaterina A.ВиктороваЕкатерина Андреевна
Russian Federation
vikat.10@mail.ruShcherbinaAnna Yu.ЩербинаАнна Юрьевна
Russian Federation
shcher26@hotmail.comFederal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry RogachevФедеральный научно-клинический центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева Минздрава России19032016151546019092018Copyright ©; 2016, «D. Rogachev NMRCPHOI»Copyright ©; 2016, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2016«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/149

Hereditary angioedema (HAE) is a rare, often life threatening primary immunodeficiency, caused by disorders in the complement system and manifesting by recurrent swelling attacks. The most incident cause of HAE is genetically determined decrease in the content of C1 inhibitor or in its function. Mutations leading to HAE are usually located in the SERPING1 gene and in the gene encoding coagulation factor XII: in some cases it is impossible to identify the genetic defect. Surgical procedures, traumas, stress, and other factors can provoke the development of swelling attacks. A characteristic sign of swelling attacks is the absence of therapeutic effect of antihistamines and corticosteroids. Antifibrinolytics, fresh frozen plasma, and attenuated androgens are now used in Russia for therapy and prevention of HAE recurrence. However, the efficacy of this therapy for the treatment of severe swelling attacks is extremely low and it is fraught with a risk of side effects. Drugs targeted to initial (C1 inhibitor concentrate, physiologically replacing its deficit) and final (icatibant - bradykinin pathway inhibitor) stages of angioedema pathogenesis are used for the treatment of swelling attacks in the world practice. By today only one C1 inhibitor concentrate - Berinert® (“CSL Behring GmbH”, Germany) is registered in Russia. It can be used for the treatment of acute swelling attacks and for their prevention. Use of specific drugs for the treatment of acute swelling attacks and for their early prevention in HAE patients is extremely important for a favorable prognosis of the disease.

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