FXIII: mechanisms of action in the treatment of hemophilia A.

摘要: SummaryBackground Hemophilia is characterized by abnormal thrombin generation and impaired clot stability. FXIII promotes stability may be a useful adjunct treatment for hemophilia. Objectives This study examined the stabilizing effects safety of supra-physiological explored mechanisms via which exerts its in hemophilia A. Methods The on formation were using thromboelastometry assay blood samples collected from six patients with severe A. The effect was also assessed murine model. action A measuring generation, rates activation permeability, pore size fibrin fiber diameter. Results This demonstrates that concentrations stabilize clots improving resistance to t-Pa-induced fibrinolysis even at low FVIII (FVIII< 0.1 IU mL−1, P < 0.05, anova). Addition stoichiometrically up-regulates activation, correcting structure, reducing permeability facilitating generation; significantly shortens ttPeak lagtime (P < 0.05) FVIII-deficient plasma, providing novel explanation positive structure. model indicates tolerated does not alter time formation. Conclusion The physical structure are seen FVIII, indicating could variety clinical scenarios.