Short-term prophylaxis of hereditary angioedema
T.V. Latysheva, E.A. Latysheva, I.A. Manto

“National Research Center — Institute of Immunology” Federal Medical-Biological Agency of Russia, Moscow

Hereditary angioedema (HAE) due to the deficiency of the C1-inhibitor is an orphan disease characterized by derma deep layers’ and membrane mucosa’s edema development. Edema can be debilitating, it can significantly reduce patient’s quality of life and often lead to disability and even to death. Edema can occur both for no apparent reason, and under the influence of various trigger factors. Injury is one of the most common causes of edema, while there is a special group of triggers, which most often provoke life-threatening edema: various medical interventions such as diagnostic, dental and surgical. That is why patients with HAE are often denied the necessary examination or treatment due to the fear of edema, or, vise versa, medical interventions are provided without proper preparation, which leads to resuscitation, including tracheostomy. In addition, patients themselves are afraid of the edema development and reject the necessary medical care. Low awareness of medical staff about this rare disease worsens the situation. For today, there are some recommendations which have been developed to reduce the risk of edema. The introduction of these recommendations into practice allows not only to perform the required intervention without complications, but also to improve the quality of patient’s life.

Key words: hereditary angioedema, HAE, short-term prophylaxis, C1-inhibitor, Berinert, danazol, angioedema, bradykinin.
For citation: Latysheva T.V., Latysheva E.A., Manto I.A. Short-term prophylaxis of hereditary angioedema // RMJ. 2018. 
№ 8(I). P. 53–58.