Redkoe zabolevanie - nasledstvennyy angiootek: kak postavit' diagnoz?
- Autores: Bodnya O.S1, Drobik K.I1
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Afiliações:
- Edição: Nº 1S (2015)
- Páginas: 15-21
- Seção: Articles
- URL: https://journals.eco-vector.com/2073-4034/article/view/288438
- ID: 288438
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Resumo
Hereditary angioedema (HAE) is one of the orphan diseases, presented in their - register defect of the complement system (D 84.1). HAE is a potentially life-threatening, genetic disease associated with deficiency or reduction in the function of C1 inhibitor (C1INH), characterized by recurrent swelling of the deep layers of the dermis of different localization. Currently, the issues of underdiagnosis of the disease, associated with primary immunodeficiency, are very urgent. For the first time, pediatricians, general practitioners, surgeons, otolaryngologists are faced with clinical manifestations of this disease. The article presents the principles of treatment of the HAE. The organizational and legal aspects of care for patients with this disease are discussed.
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