BACKGROUND Pregnancy in von Willebrand's disease may carry a significant risk of bleeding. Information on changes in factor VIII and von Willebrand factor and pregnancy outcome in relation to von Willebrand factor gene mutations are very scanty. DESIGN AND METHODS We examined biological response to desmopressin, changes in factor VIII and von Willebrand factor and pregnancy outcome in a cohor...

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...

von Willebrand factor-cleaving protease (ADAMTS13) cleaves von Willebrand factor (VWF) and regulates its physiologic function. To investigate the relation between ADAMTS13 activity and VWF, we compared ADAMTS13 activity with the VWF-related parameters VWF antigen (VWF:Ag), VWF collagen-binding activity (VWF:CBA), VWF-propeptide, proVWF, and VWF multimeric composition in 10 healthy volunteers an...

We have studied the binding of von Willebrand factor to extracellular matrices of endothelial cells and to the vessel wall of human umbilical arteries in relation to its function in supporting platelet adhesion. CLB-RAg 201, an MAb against von Willebrand factor, completely inhibits the binding of von Willebrand factor to collagen type I and type III. CLB-RAg 201 does not inhibit the binding of ...

background von hippel-lindau (vhl) disease is a hereditary, autosomal dominant syndrome which is manifested by a range of different benign and malignant tumors. this disease can present with different clinical presentations such as; retinal angioma (ra), hemangioblastoma (hb) of the central nervous system (cns), pheochromocytoma (pheo), and epididymal cystadenoma. tumors are usually accompanied...

Symphony, das in [1] beschriebene Framework zur ansichtsorientierten Rekonstruktion von Softwarearchitekturen, basiert auf drei wesentlichen Konzepten: dem Quell-Blickwinkel, dem Ziel-Blickwinkel und der Zuordnung von Elementen des einen zu Elementen des anderen Blickwinkels. Ausgangspunkt für den Quell-Blickwinkel und seine Ansichten sind Implementationsfakten, die durch geeignete Relationen ü...

We present a new analysis of the relative rate of growth or shrinkage of grains in a two-dimensional network, based on the classical von Neumann-Mullins (VN-M) analysis. We find that an analysis of the stability of the grain shape during shrinkage or growth shows that any change in the regular 2D grain leads to changes in the shape. We also re-examine a recent analysis that claims to have inval...

we report an unusual case of a young male with cerebellar hemangioblastoma treated previously for medullary carcinoma of thyroid, whose pet/ct scans revealed a constellation of findings that suggested the rare von hippel lindau syndrome. the diagnosis was clinched by confirming the findings on whole body contrast enhanced computed tomography (cect) and contrast enhanced magnetic resonance imagi...

Maharam algebras are complete Boolean algebras carrying a positive continuous submeasure. They were introduced and studied by Maharam in [24] in relation to Von Neumann’s problem on the characterization of measure algebras. The question whether every Maharam algebra is a measure algebra has been the main open problem in this area for around 60 years. It was finally resolved by Talagrand [31] wh...