von Willebrand disease (VWD) was first described nearly a century ago in 1924 by Erik Adolf von Willebrand. Diagnostic testing at the time was very limited and it was not until the mid to late 1900s that more tests became available to assist with the diagnosis and classification of VWD. Two of these tests are based on ristocetin, one being ristocetin-induced platelet aggregation (RIPA) and the ...

Type I von Willebrand disease (vWD) is very common in caucasians. Its genetic basis is possibly heterogenous, lying both within and out of the vWF gene locus. We sought to investigate vWF levels in the Thai population, to compare with those of western countries. The vWF antigen and activity were measured using ELISA and Collagen Binding Assay (CBA), respectively, in 311 healthy Thai volunteers....

Signal crossing is a significant problem within von Neumann cellular automata (29-states); only three solutions are known. This paper gives a thorough examination of these three solutions, including discussion of performance and the effective means of construction, including the mechanism of autoinitialisation. Application of these solutions within self-replicators is then examined for Nobili a...

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Type I von Willebrand disease (vWD) is characterized by equally low plasma concentrations of von Willebrand factor antigen (vWF:Ag) and ristocetin cofactor (RiCof) and by the presence of all vWF multimers in sodium dodecyl sulfate (SDS)-agarose gel electrophoresis. For 17 patients (13 kindreds) diagnosed with these criteria, we have studied the platelet contents of vWF:Ag and RiCof and the chan...

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...

Von Willebrand disease (VWD) may be caused by an impaired von Willebrand factor (VWF) synthesis, its increased clearance or abnormal function, or combinations of these factors. It may be difficult to recognize the different contributions of these anomalies. Here we demonstrate that VWD diagnostics gains from measuring platelet VWF, which can reveal a defective VWF synthesis. Measuring platelet ...

Aortic endothelial cells from normal pigs and pigs with von Willebrand disease have been established in long-term cultures. Both cultures appeared similar in terms of general growth characteristics, morphologic features and ultrastructure. Immunofluorescent staining of these cultures with chicken (or rabbit) antiporcine ristocetin-Willebrand factor sera (or IgG) resulted in extensive perinuclea...

Type 2A von Willebrand disease (vWD), the most common qualitative form of vWD, is characterized by a relative decrease in circulating intermediate and high molecular weight (HMW) multimers. We studied the biosynthesis of recombinant von Willebrand factor (vWF) containing each of two type 2A vWD mutations previously reported by us, Arg834Gln and Va1902Glu. The structure of recombinant Arg834Gln ...