Activation of factor XII and cleavage of high molecular weight kininogen during acute attacks in hereditary and acquired Cl-inhibitor deficiencies
Hereditary and acquired Cl-inhibitor (C1-INH) deficiencies (hereditary angioedema, HAE
and acquired angioedema, AAE) are characterized by episodic increases in
vasopermeability due to kinin release. Despite continuously defective Cl-INH levels,
symptoms only recur occasionally suggesting an episodic generation of pathogenetic factors
induced by activation of the protease systems regulated by C1-INH. We evaluated activation
of factor XII by measuring plasma levels of FXIIa with a commercial ELISA and cleavage of …
and acquired angioedema, AAE) are characterized by episodic increases in
vasopermeability due to kinin release. Despite continuously defective Cl-INH levels,
symptoms only recur occasionally suggesting an episodic generation of pathogenetic factors
induced by activation of the protease systems regulated by C1-INH. We evaluated activation
of factor XII by measuring plasma levels of FXIIa with a commercial ELISA and cleavage of …