نتایج جستجو برای: von willebrand disease

تعداد نتایج: 1579959  

2018
Nikolett Wohner Vincent Muczynski Amel Mohamadi Paulette Legendre Valérie Proulle Gabriel Aymé Olivier D. Christophe Peter J. Lenting Cécile V. Denis Caterina Casari

Previously, we found that LDL-receptor related protein-1 on macrophages mediated shear stress-dependent clearance of von Willebrand factor. In control experiments, however, we observed that von Willebrand factor also binds to macrophages independently of this receptor under static conditions, suggesting the existence of additional clearance-receptors. In search for such receptors, we focused on...

2013
Fatih Şap Tülay Kavaklı Kaan Kavaklı Ceyhun Dizdarer

OBJECTIVE von Willebrand disease (vWD) is the most common hereditary bleeding disorder. The purpose of this investigation was to determine the prevalence of vWD among adolescents in İzmir and to assess the sensitivity and specificity of PFA-100 as a screening method in detecting this disease. MATERIAL AND METHODS Our study was conducted on adolescents in the city of İzmir between October 2006...

Journal: :Blood 1987
A Asakura J Harrison E Gomperts C Abildgaard

Type IIA von Willebrand's Disease (vWD) is the most common type II variant, and all reported cases (56 individuals in 26 families) have had autosomal dominant inheritance. An eight-year-old female with an increased bleeding tendency since infancy was found to have laboratory values typical of type IIA vWD, but her parents and siblings were asymptomatic. With the exception of uniformly decreased...

2013
Cindy A Leissinger Diane Nugent

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 ...

Journal: :Haemophilia : the official journal of the World Federation of Hemophilia 2013
G Castaman A Coppola E Zanon E Boeri M Musso S Siragusa A B Federici G Mancuso G Barillari C Biasoli G Feola M Franchini S Moratelli G Gamba P Schinco L Valdrè A Dragani G Mazzucconi A Tagliaferri M Morfini

Von Willebrand disease (VWD) is an inherited bleeding disorder caused by the quantitative or qualitative deficiency of von Willebrand factor (VWF). Replacement therapy with plasma-derived VWF/factor VIII (FVIII) concentrates is required in patients unresponsive to desmopressin. To assess the efficacy, safety and ease of use of a new, volume-reduced (VR) formulation of VWF/FVIII concentrate Haem...

Journal: :Circulation research 1982
V Fuster D N Fass M P Kaye M Josa A R Zinsmeister E J Bowie

In a long-term prospective study, five normal control pigs and five pigs with homozygous von Willebrand's disease received a nonfatty diet from age 3 months to 4 years; then the aortas were analyzed. The fibrous arteriosclerotic plaques in the distal abdominal aortic region involved an average of 28% of the surface area in control pigs and only 7% of the surface area in pigs with von Willebrand...

Journal: :Mayo Clinic proceedings 2006
Rajiv K Pruthi

von Willebrand disease (vWD) is the most commonly diagnosed congenital bleeding disorder. The laboratory diagnosis of type 2 variants and type 3 vWD is reasonably well defined, and characterization of the von Willebrand factor (vWF) gene has facilitated definition of their molecular basis. However, for type 1 vWD, the laboratory diagnosis poses a diagnostic dilemma, and knowledge of its molecul...

Journal: :Value in health : the journal of the International Society for Pharmacoeconomics and Outcomes Research 2014
A Cipolla D Cultrera C Teruzzi M Mantuano

Von Willebrand Disease (VWD) is the most common inherited bleeding disorder, caused by a deficiency or abnormality of the Von Willebrand Factor (VWF). The mainstays of treatment are therapy with Desmopressin (DDAVP) and replacement therapy with Factor VIII-containing VWF product (FVIII/VWF). Therapy with FVIII/VWF can be administered as Long-term prophylaxis (LTP) in the more severe forms of th...

Journal: :Blood 2001
P M Mannucci

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 ...

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