نتایج جستجو برای: von willebrand disease
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INTRODUCTION Acquired von Willebrand disease is initiated by autoantibodies and hyperviscosity syndrome caused by a massive polyclonal hypergammaglobulinemia. Acquired von Willebrand disease associated with autoimmune disease in addition to pulmonary hypertension during emergency room presentation is a rare condition. To the best of our knowledge, this is the second case reported in the literat...
Abstract Background Von Willebrand disease (VWD) is an autosomal recessive congenital bleeding disorder with deficiency or dysfunction of von Willebrand factor (VWF). The gene encoding for the VWF is located on chromosome 12, which is 178 Kb with 52 exons. Various mutations of this gene is responsible for the clinical features of VWD, but some single nucleotide polymorphisms make the molecu...
Precipitating antibodies to factor VlIl/von Willebrand factor can develop in patients with severe homozygouslike von Willebrand’s disease following multiple transfusions with blood derivatives. This study of 4 patients treated with cryoprecipitate for 1 3 different bleeding episodes demonstrates that the occurrence of such antibodies interferes with the management of the disease. The control of...
AIMS To determine whether circulating von Willebrand factor concentrations are prospectively related to risk of coronary heart disease in the general population. METHODS AND RESULTS We measured baseline von Willebrand factor values in the stored serum samples of 625 men with major coronary events and in 1266 controls 'nested' in a prospective study of 5661 men aged 40-59 years, recruited from...
We have analyzed the multimeric structure of factor VIII/von Willebrand factor in plasma by sodium dodecyl sulfate electrophoresis using gels of varying porosity and a discontinuous buffer system. Factor VIII/von Willebrand factor bands were identified by reaction with 125I-labeled affinity-purified antibody and subsequent autoradiography. In 1% agarose gels, normal plasma displayed a series of...
The clinical expression of type 1 von Willebrand disease may be modified by co-inheritance of other mild bleeding diatheses. We previously showed that mutations in the platelet P2Y12 ADP receptor gene (P2RY12) could contribute to the bleeding phenotype in patients with type 1 von Willebrand disease. Here we investigated whether variations in platelet G protein-coupled receptor genes other than ...
Molecular diagnosis of patients with von Willebrand disease is pending in most populations due to the complexity and high cost of conventional molecular analyses. The need for molecular and clinical characterization of von Willebrand disease in Spain prompted the creation of a multicenter project (PCM-EVW-ES) that resulted in the largest prospective cohort study of patients with all types of vo...
When normal volunteers or patients with type I von Willebrand disease (VWD) are given desmopressin (DDAVP). a set of larger-than-normal (supranormal) von Willebrand factor (VWF) multimers. similar to those present in VWFcontaining cells such as platelets megakaryocytes and endothelial cells, appear transiently in postinfusion plasma. In two kindreds with mild lifelong bleeding symptoms transmit...
Hydroxyethyl starch is commonly used as a plasma volume expander in the surgical patient. Although it is generally considered a safe plasma substitution, some reports of an acquired von Willebrand's disease-like syndrome have been documented. To examine this further, von Willebrand factor: ristocetin cofactor activity (RCoF) was measured in two groups of patients perioperatively, following hydr...
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