When 1 plus 1 equals 3 in VWD.

نویسنده

  • Anne C Goodeve
چکیده

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The molecular basis of von Willebrand disease: the under investigated, the unexpected and the overlooked.

Von Willebrand disease (VWD) is a common autosomally inherited bleeding disorder resulting from quantitative and qualitative defects of plasma von Willebrand factor (VWF). VWD can be divided into three types: type 1 includes partial quantitative deficiencies of VWF, type 3 includes virtually complete quantitative deficiencies while type 2 encompasses four subtypes of qualitative deficiency in w...

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Postpartum Hemorrhage in Women with Von Willebrand Disease – A Retrospective Observational Study

INTRODUCTION von Willebrand disease (VWD) is a hereditary bleeding disorder, caused by a deficiency in the levels and/or function of von Willebrand factor (VWF). Women with VWD appear to be at increased risk of experiencing postpartum hemorrhage (PPH), though the levels of VWF increase during pregnancy. There is limited knowledge of how PPH is associated with the subtype of VWD, plasma levels o...

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A Laboratory Phenotype/Genotype Correlation of 1167 French Patients From 670 Families With von Willebrand Disease

von Willebrand disease (VWD) is a genetic bleeding disease due to a defect of von Willebrand factor (VWF), a glycoprotein crucial for platelet adhesion to the subendothelium after vascular injury. VWD include quantitative defects of VWF, either partial (type 1 with VWF levels <50 IU/dL) or virtually total (type 3 with undetectable VWF levels) and also qualitative defects of VWF (type 2 variants...

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The discriminant power of bleeding history for the diagnosis of type 1 von Willebrand disease: an international, multicenter study.

OBJECTIVE The aim of this study was the validation of the criteria defining a significant mucocutaneous-bleeding history in type 1 von Willebrand disease (VWD). SUBJECTS AND METHODS To avoid selection bias, 42 obligatory carriers (OC) of type 1 VWD were identified from a panel of 42 families with type 1 VWD enrolled by 10 expert centers. OC were identified by the presence of an offspring and ...

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Diagnosis of inherited von Willebrand disease: a clinical perspective.

von Willebrand disease (VWD) is the most frequent inherited disorder of hemostasis and is due to quantitative (types 1 and 3) or qualitative (type 2) defects of von Willebrand factor (VWF). Due to the large heterogeneity of VWF defects and to the external variables influencing VWF levels in the circulation, VWD diagnosis can be difficult, especially in relatively mild forms. Three criteria shou...

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عنوان ژورنال:
  • Blood

دوره 114 5  شماره 

صفحات  -

تاریخ انتشار 2009