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The hierarchical hemostasis response involves a self-inhibitory feature of von Willebrand Factor (VWF) that has not been fully characterized. The residues flanking the A1 domain of VWF are important in this self-inhibition by forming an autoinhibitory module (AIM) that masks A1.
This article was published in the following journal.
Name: Journal of thrombosis and haemostasis : JTH
von Willebrand Disease (VWD) is an inherited bleeding disorder caused by quantitative (type 1, 3) or qualitative (type 2) von Willebrand factor (VWF) defect. VWD diagnosis and classification require n...
: Type III von Willebrand's disease (vWD) is an inherited bleeding disorder, which is frequently associated with menorrhagia in women. Treatment options include antifibrinolytics, desmopressin, von Wi...
Diagnosis of von Willebrand disease (VWD) is difficult due to the heterogenic phenotype of patients and to the complex tests that are required for an adequate investigation. The collagen binding assay...
Noninvasive methods have been established to detect clinically significant portal hypertension in liver cirrhosis with variable limitations. The von Willebrand factor (vEF) has been found to increase ...
Evaluation of von Willebrand factor (VWF) multimeric distribution is useful for subclassification of von Willebrand disease (VWD). Multimer analysis has historically been a manual, labor-intensive lab...
OBJECTIVES: I. Evaluate the effect of a new von Willebrand factor concentrate on bleeding time, in vivo recovery, and circulating half-life of the infused factor in patients with von Will...
The Low Von Willebrand in Ireland Cohort (LoVIC) study focuses on the bleeding phenotype and biological mechanisms underlying low Von Willebrand Factor (VWF) levels.
The purpose of this phase 3 study is to investigate the efficacy and safety, including immunogenicity and thrombogenicity of prophylactic treatment with recombinant von Willebrand factor (...
During treatments with extracorporeal circuits such as extracorporeal membrane oxygenation (ECMO) degradation of high molecular weight (HMW) of von Willebrand factor (vWF) multimers occur ...
The objectives of this study are to evaluate the immediate tolerability and safety of rVWF:rFVIII in subjects with Type 3 Von Willebrand Disease after administration of various dosages of ...
A high-molecular-weight plasma protein, produced by endothelial cells and megakaryocytes, that is part of the factor VIII/von Willebrand factor complex. The von Willebrand factor has receptors for collagen, platelets, and ristocetin activity as well as the immunologically distinct antigenic determinants. It functions in adhesion of platelets to collagen and hemostatic plug formation. The prolonged bleeding time in VON WILLEBRAND DISEASES is due to the deficiency of this factor.
A subtype of von Willebrand disease that results from a partial deficiency of VON WILLEBRAND FACTOR.
A subtype of von Willebrand disease that results from qualitative deficiencies of VON WILLEBRAND FACTOR. The subtype is divided into several variants with each variant having a distinctive pattern of PLATELET-interaction.
A subtype of von Willebrand disease that results from a total or near total deficiency of VON WILLEBRAND FACTOR.
Group of hemorrhagic disorders in which the VON WILLEBRAND FACTOR is either quantitatively or qualitatively abnormal. They are usually inherited as an autosomal dominant trait though rare kindreds are autosomal recessive. Symptoms vary depending on severity and disease type but may include prolonged bleeding time, deficiency of factor VIII, and impaired platelet adhesion.