Impaired control of the contact system in hereditary angioedema with normal C1-inhibitor.
Maria BovaChiara SuffrittiValeria BafunnoStefania LoffredoGiorgia CordiscoStefano Del GiaccoTiziana Maria Angela De PasqualeDavide FirinuMaurizio MargaglioneVincenzo MontinaroAngelica PetraroliAnna RadiceLuisa BrussinoAndrea ZanichelliAlessandra ZoliMarco CicardiPublished in: Allergy (2020)
Our results suggest that pathogenesis of FXII-, ANGPT1-, and U-HAE moves through an unbalanced control of kallikrein activity, with bradykinin as most likely mediator. VEGFs and Ang1 participate in the pathophysiology of U-HAE increasing the basal vascular permeability.