Hypersensitivity reactions amongst Hungarian Patients with Hereditary Angioedema due to C1-Inhibitor Deficiency.

ABSTRACT:

Background

In hereditary angioedema (HAE) due to C1-inhibitor deficiency (C1-INH-HAE), bradykinin-mediated submucosal and/or subcutaneous angioedema dominates the clinical picture. The deficiency of C1-inhibitor can lead to the over-activation of the complement system. Complement plays an important role in all types of hypersensitivity reactions. On the other hand, during the degranulation of mast cells, heparin is also released amongst other substances. Heparin can activate the plasma kinin-kallikrein system, leading to bradykinin generation. These observations suggest a possible connection between C1-INH-HAE and mast cell-mediated hypersensitivity reactions.

Objective

To assess the occurrence of hypersensitivity reactions in the Hungarian C1-INH-HAE population.

Methods<

SUBMITTER: Horvath HR 

PROVIDER: S-EPMC10618765 | biostudies-literature | 2023 Nov

REPOSITORIES: biostudies-literature