OBJECTIVE Platelet-bound von Willebrand factor (VWF) was recently demonstrated to be a better substrate for ADAMTS13, suggesting that 1 conformational change exposes both the glycoprotein Ibα binding site in the A1 domain and the ADAMTS13 cleavage site in the A2 domain. Because ristocetin induces VWF to bind glycoprotein Ibα in the absence of shear stress, we evaluated whether it could also enh...

Up until recently, von Willebrand Factor (VWF) structure-function relationships have only been studied through in vitro approaches. A powerful technique known as hydrodynamic gene transfer, which allows transient expression of a transgene by mouse hepatocytes, has led to an important shift in VWF research. Indeed this approach has now enabled us to transiently express a number of VWF mutants in...

PURPOSE Prophylaxis with von Willebrand factor (VWF)/factor VIII (FVIII) concentrates is a potential approach for patients with severe von Willebrand disease (VWD). As far as we are aware, to date there have been no pharmacoeconomic analyses in order to assess the economic impact of treatments for severe VWD. The analysis presented here estimates the cost-benefit ratio of VWF with a low FVIII c...

Von Willebrand disease (vWD) is a frequent inherited disorder of hemostasis that affects both sexes. Two abnormalities are characteristic of the disease, which is caused by a deficiency or a defect in the multimeric glycoprotein called von Willebrand factor: low platelet adhesion to injured blood vessels and defective intrinsic coagulation owing to low plasma levels of factor VIII. There are 2 ...

Von Willebrand disease (VWD) is the most common inherited bleeding disorder, with an estimated prevalence ranging from 0.6% to 1.3% [1]. Therapies approved for VWD in the United States include desmopressin (eg, intravenous DDAVP®, intranasal Stimate®) and von Willebrand factor/factor VIII (VWF/FVIII) concentrates (Humate-P®, CSL Behring, Kankakee, IL; Alphanate® SD/HT, Grifols Biologicals, Los ...

6.5 Range of Application, Dosage, Mode of Administration 6.5.1 General Information 6.5.2 Indications for Replacement Therapy Using Factor Concentrates 6.5.3 Dosage, Mode of Administration 6.5.3.1 Replacement in Children with Hemophilia A, B or von Willebrand Syndrome 6.5.3.2 Replacement in Adults with Hemophilia A, B or von Willebrand Syndrome 6.5.3.3 Indications and Recommended Doses for Treat...

Von Willebrand disease (vWD) is a frequent inherited disorder of hemostasis that affects both sexes. Two abnormalities are characteristic of the disease, which is caused by a deficiency or a defect in the multimeric glycoprotein called von Willebrand factor: low platelet adhesion to injured blood vessels and defective intrinsic coagulation owing to low plasma levels of factor VIII. There are 2 ...

6.5 Range of Application, Dosage, Mode of Administration 6.5.1 General Information 6.5.2 Indications for Replacement Therapy Using Factor Concentrates 6.5.3 Dosage, Mode of Administration 6.5.3.1 Replacement in Children with Hemophilia A, B or von Willebrand Syndrome 6.5.3.2 Replacement in Adults with Hemophilia A, B or von Willebrand Syndrome 6.5.3.3 Indications and Recommended Doses for Treat...