DOI:
https://doi.org/10.71337/inlibrary.uz.science-research.139585Keywords:
hereditary angioedema C1 inhibitor bradykinin complement kallikrein–kinin system pharmacological therapy.Abstract
Hereditary angioedema (HAE) is a rare autosomal dominant disorder in which episodes of subcutaneous and submucosal swelling are driven not by histamine release but by dysregulation of the complement and kallikrein–kinin systems. This paper briefly summarizes the biochemical basis of HAE, including deficiency or dysfunction of C1 inhibitor, uncontrolled complement activation and excessive bradykinin generation as the key mediator increasing vascular permeability. The main pharmacological approaches are outlined, such as C1 inhibitor replacement, bradykinin B2 receptor blockade, kallikrein inhibition and modern long-term prophylactic strategies. The material is linked to fundamental biochemical concepts presented in the textbook by Severin and Nikolaev.References
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