Von willbrand disease

Pulmonary endothelial functions are critical to maintain the low pressure of the pulmonary circulation and effective diffusion capacity of the lung. To investigate pulmonary endothelial cell biology in healthy or diseased lungs, we developed methods to harvest and culture pure populations of primary pulmonary arterial endothelial cells and microvascular endothelial cells from human lung explanted at time of transplantation or from donor lungs not used in transplantation. The purity and characteristics of cultured endothelial cells is ascertained by morphologic criteria using phase contrast and electron microscopy; phenotypic expression profile for endothelial specific proteins such as endothelial nitric oxide synthase, platelet/endothelial cell adhesion molecule, and von Willbrand factor; and endothelial function assays such as Dil-acetylated low-density lipoprotein uptake and tube formation...

The objective of the study was to determine the frequency, types, presenting symptoms and management options in patients with bleeding disorders in the local obstetric and gynaecological practice. This was a descriptive study of oneyear duration conducted at the Pakistan Atomic Energy Commission General Hospital, Islamabad. Types of bleeding disorders in decreasing order of frequency were HELLP syndrome in 27.7% (n=5), gestational thrombocytopenia in 22% (n=4), Von Willbrand disease in 16.66% (n=3), Glanzmann's thrombesthenia in 11...

ADAMTS13 is a metalloproteinase that specifically cleaves unusually large von Willbrand factor multimers under high-shear stress. Deficiency of ADAMTS13 activity induces a life-threatening generalized disease, thrombotic thrombocytopenic purpura. We established a simple and efficient method to purify plasma ADAMTS13 (pADAMTS13) from cryosupernatant using an anti-ADAMTS13 monoclonal antibody (A10) that recognizes a conformational epitope within the disintegrin-like domain. Using the purified pADAMTS13, the amino acid residues involved in cleavage by thrombin, plasmin and leucocyte elastase were determined, and the carbohydrate moieties of this enzyme was analysed by lectin blots...

Thrombotic diseases or fatalities have been reported to occasionally occur under conditions of hypergravity although the mechanism is still unclear. To investigate the effect of hypergravity on platelets that are the primary players in thrombus formation, platelet rich plasma (PRP) or washed platelets were exposed to hypergravity at 8 G for 15 minutes. No platelet aggregation was induced by 8 G alone, whereas ristocetin or collagen-induced platelet aggregation was significantly increased. The number of platelets adherent to immobilized fibrinogen and the area of platelets spreading on von Willbrand factor (VWF) matrix were increased simultaneously...

von Willebrand disease is one of the inherited bleeding disorders, and caused by the deficiency or the abnormality of von Willebrand factor. A 28-year-old woman with von Willebrand disease (type II) was scheduled for cesarean section under general anesthesia. Platelet count was within normal limits. However, the prolongation of both APTT and bleeding time was confirmed. The level of von Willbrand factor was within normal ranges, but its activity was about 55%. The platelet aggregation was below 1.3%. We managed to administer factor VIII concentrate to her intravenously before and after operation...

PURPOSE: This study aimed to evaluate the carotid artery intima-media thickness (CA-IMT) in adult patients with epilepsy and its contribution to oxidative stress and vascular risk biomarkers. METHODS: This study included 225 adult epileptic and 60 control subjects. For all, CA-IMT, fasting lipid profile (TC, TG, HDL-c and LDL-c), total homocysteine (tHcy), von Willbrand factor (vWF), fibrinogen, oxidized LDL (Ox-LDL), malondialdehyde (MDA), thiobarbituric acid reactive substances (TBARs), uric acid, total antioxidant capacity (TAC) and glutathione peroxidase (GSH...

Four members (from four generations) of a family with a mild bleeding disorder and intermittent thrombocytopenia had decreased plasma levels of properties related to factor VIII/von Willebrand factor (FVIII/VWF), an absence of high-molecular-weight forms of FVIII/VWF in the plasma (but normal multimeric structure in the platelets), and increased ristocetin-induced platelet aggregation, as in Type IIB von Willebrand's disease. However, unlike the abnormality in FVIII/VWF in Type IIB disease, the basic defect in this family was in their platelets, which absorbed FVIII/VWF high-molecular-weight multimers at lower concentrations of ristocetin than did normal platelets...

Patterns of VIIIR:AG in the plasma and its fractions, cryoprecipitate and cryosupernatant, from various types of von Willebrand's disease (vWd) were observed by SDS 1.5% polyacrylamide gel electrophoresis - cross immunoelectrophoresis (SDS PAGE - CIE). VIIIR:AG in normal cryoprecipitate showed several precipitin peaks which correspond to molecular weights ranging from 8 X 10(5) to 1 X 10(7) daltons and are similar to those in normal plasma. Normal cryosupernatant VIIIR:AG gave smaller molecular weights from 8 X 10(5) to 2 X 10(6) daltons...

Clinical and laboratory data, including polymorphic marker traits for linkage analysis, were collected from two large multigenerational families segregating for von Willebrand disease. A new approach to the identification of gene carriers in these families, combining pedigree segregation analysis with multivariate discriminant analysis, is applied. Whereas individually the clinical symptoms and the factor VIII related activities could not distinguish between hypotheses, it was possible to find a discriminant function-showing consistency of the data with a dominant gene hypothesis, but not with a recessive gene or an environmental hypothesis...

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Twenty nine cases of oral hemangiomas composed of capillary hemangioma, cavernous hemangioma and pyogenic granuloma were examined with lectin histochemistry using ulex europaeus agglutinin I, ricinus communis agglutinin I, wheat germ agglutinin, concanavalin A, dolichos biflorus agglutinin, soybean agglutinin and peanut agglutinin, and immunohistochemistry using anti von Willebrand factor antibody in order to understand the nature of each endothelial cell. The following results were obtained; 1. UEA-I that specifically bound alpha-L-fucose was observed mainly in the endothelial cells of capillary hemangioma and hemangioma of granulation tissue type...

Isolated, normal, neonatal pig livers released VIII:C into perfusates of porcine von Willebrand blood lacking VIIIR:AG and VIII:RWF. Neonatal von Willebrand livers released VIII:C only when partially purified VIIIR:AG was added to the von Willbrand blood. Adult rat livers, perfused with frozen-thawed erythrocytes suspended in Tyrode's solution containing 6% bovine serum albumin and hence no VIIIR:AG released VIII:C only when serum, adsorbed serum or cryoprecipitate from rat plasma was added to the perfusate...

A study of over 700 pigs affected with von Willebrand's disease suggests that the classic disease is transmitted as an autosomal recessive trait. Less clear is the genetic basis of inheritance of variant types of this disease produced by selective mating.