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Diagnosis of von Willebrand Disease
Haemophilia, 1999The haemorrhagic diathesis in von Willebrand disease (vWD) is caused by a quantitative deficiency or a qualitative defect in the von Willebrand factor (vWF) in plasma and/or platelets causing insufficient primary haemostasis. Since vWF binds and protects factor VIII (FVIII) towards random proteolysis, coagulation may also be impaired in patients with a
Ingerslev, J, Gursel, T
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Hematology/Oncology Clinics of North America, 1990
The study of a wide variety of patients with vWD disorders has been extensive and intense over the last decade. In many instances the peculiarities of the expressions of the disorder have been critical in defining the underlying structure and function of vWF itself.
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The study of a wide variety of patients with vWD disorders has been extensive and intense over the last decade. In many instances the peculiarities of the expressions of the disorder have been critical in defining the underlying structure and function of vWF itself.
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von Willebrand factor and von Willebrand disease.
[Rinsho ketsueki] The Japanese journal of clinical hematology, 2016von Willebrand factor (VWF) has two major roles in hemostasis, as a form of molecular glue which functions in platelet plug formation and as a protective transporter for coagulation factor VIII (FVIII). VWF shows a multimeric chain structure composed of 270 kDa subunits containing binding domains for FVIII, platelet and collagens. Biosynthesis, storage,
Jiharu Hamako, Taei Matsui
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Postgraduate Medicine, 1980
Von Willebrand's disease is an autosomally transmitted disorder of hemostasis caused by a deficiency of or defect in the von Willebrand factor in the blood, a protein required for adherence of platelets to an injured vessel wall. The disease's principal manifestations are spontaneous bleeding from mucous membranes, excessive bleeding from wounds, and ...
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Von Willebrand's disease is an autosomally transmitted disorder of hemostasis caused by a deficiency of or defect in the von Willebrand factor in the blood, a protein required for adherence of platelets to an injured vessel wall. The disease's principal manifestations are spontaneous bleeding from mucous membranes, excessive bleeding from wounds, and ...
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Treatment of von Willebrand's disease
Journal of Internal Medicine, 1997von Willebrand's disease is the most frequent of inherited bleeding disorders (1:100 affected individuals in the general population). The aim of therapy is to correct the dual defects of haemostasis, i.e. abnormal coagulation expressed by low levels of factor VIII and abnormal platelet adhesion expressed by a prolonged bleeding time. There are two main
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Antibodies to von Willebrand factor in von Willebrand disease
1995The occurrence of an alloantibody directed against von Willebrand factor in a multitransfused patient with severe (type III) von Willebrand disease was first reported in 2 consecutive studies by Sarji et al. (1974) and Stratton et al. (1975). After this, 14 additional cases of alloantibodies were described and reviewed by Mannucci and Mari (1984).
Pier Mannuccio Mannucci +1 more
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2018
Von Willebrand disease (vWD) is the most common bleeding disorder in humans. It is the result of an abnormality in the amount, structure, or function of von Willebrand factor (vWF), a glycoprotein important in maintaining normal hemostasis.. In children with vWD, the most frequent presentation is easy bruising and epistaxis.
Nina A. Guzzetta, Laura A. Downey
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Von Willebrand disease (vWD) is the most common bleeding disorder in humans. It is the result of an abnormality in the amount, structure, or function of von Willebrand factor (vWF), a glycoprotein important in maintaining normal hemostasis.. In children with vWD, the most frequent presentation is easy bruising and epistaxis.
Nina A. Guzzetta, Laura A. Downey
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2020
von Willebrand disease (VWD), the most common inherited bleeding disorder, is caused by quantitative or qualitative deficiencies in the von Willebrand factor (VWF) protein. VWF is crucial for platelet adhesion and aggregation at sites of vessel injury and also for stabilizing and transporting factor VIII (FVIII) to these sites.
Lakshmi Srivaths, Trinh T Nguyen
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von Willebrand disease (VWD), the most common inherited bleeding disorder, is caused by quantitative or qualitative deficiencies in the von Willebrand factor (VWF) protein. VWF is crucial for platelet adhesion and aggregation at sites of vessel injury and also for stabilizing and transporting factor VIII (FVIII) to these sites.
Lakshmi Srivaths, Trinh T Nguyen
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2013
Abstract The interactions between clinical and laboratory medicine moving to and from bedside, bench, and back to bedside are no more apparent than in our unraveling of von Willebrand Disease. Discovered nearly a century ago, we are still expanding both our clinical and laboratory knowledge of this disorder.
Margaret E. Rick, Barbara A. Konkle
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Abstract The interactions between clinical and laboratory medicine moving to and from bedside, bench, and back to bedside are no more apparent than in our unraveling of von Willebrand Disease. Discovered nearly a century ago, we are still expanding both our clinical and laboratory knowledge of this disorder.
Margaret E. Rick, Barbara A. Konkle
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