Evaluation of an automated von Willebrand factor glycoprotein IbM activity assay compared with 3 alternative von Willebrand factor activity assays.

Background: To overcome deficiencies of the traditional von Willebrand factor (VWF) ristocetin cofactor activity assay (VWF:RCo), several automated assays for VWF platelet-binding activity have been developed. Information on the performance of these assays and their diagnostic utility remains limited.

Objectives: To validate the VWF:glycoprotein IbM assay INNOVANCE VWF Ac and compare it with an automated VWF:RCo assay as well as with an automated assay and a manual VWF:Ab assay and to generate reference ranges and analyze reproducibility of the VWF:glycoprotein IbM assay.

Receptor and Molecular Mechanism of AGGF1 Signaling in Endothelial Cell Functions and Angiogenesis.

Objective: Angiogenic factor AGGF1 (angiogenic factor with G-patch and FHA [Forkhead-associated] domain 1) promotes angiogenesis as potently as VEGFA (vascular endothelial growth factor A) and regulates endothelial cell (EC) proliferation, migration, specification of multipotent hemangioblasts and venous ECs, hematopoiesis, and vascular development and causes vascular disease Klippel-Trenaunay syndrome when mutated. However, the receptor for AGGF1 and the underlying molecular mechanisms remain to be defined.

Approach And Results: Using functional blocking studies with neutralizing antibodies, we identified [alpha]5[beta]1 as the receptor for AGGF1 on ECs.

Willingness-to-pay for a hypothetical Ebola vaccine in Indonesia: A cross-sectional study in Aceh.

Some Ebola vaccines have been developed and tested in phase III clinical trials. However, assessment of whether public have willingness to purchase or not, especially in unaffected areas, is lacking. The aim of this study was to determine willingness to pay (WTP) for a hypothetical Ebola vaccine in Indonesia.

Effect of 6% hydroxyethyl starch 130/0.4 on kidney and haemostatic function in cardiac surgical patients: a randomised controlled trial.

Whether third-generation hydroxyethyl starch solutions provoke kidney injury or haemostatic abnormalities in patients having cardiac surgery remains unclear. We tested the hypotheses that intra-operative administration of a third-generation starch does not worsen postoperative kidney function or haemostasis in cardiac surgical patients compared with human albumin 5%. This triple-blind, non-inferiority, clinical trial randomly allocated patients aged 40-85 who underwent elective aortic valve replacement, with or without coronary artery bypass grafting, to plasma volume replacement with 6% starch 130/0.

The relation between ABO blood types and clinical and platelet function parameters in patients who underwent percutaneous coronary intervention.

Background: ABO blood groups have been associated with venous thromboembolism and arterial thrombosis. Although platelets play key roles in thrombogenesis, the relation between ABO groups and platelets is not well known and was investigated in this study.

Patients And Methods: ABO blood type information was retrospectively obtained for 206 patients who underwent percutaneous coronary intervention (PCI) and received dual antiplatelet therapy with aspirin and clopidogrel.

Multi-parameter assessment of platelet inhibition and its stability during aspirin and clopidogrel therapy.

Introduction: Poor response to antiplatelet drugs is associated with adverse outcomes. We assessed platelet inhibition and its stability and tested correlation and agreement between platelet function assays.

Methods: Peripheral blood from 58 patients on both aspirin and clopidogrel who underwent percutaneous coronary intervention (PCI) was collected at hospital discharge (visit-1) and at 30-90 days (visit-2).

P2RY1 and P2RY12 polymorphisms and on-aspirin platelet reactivity in patients with coronary artery disease.

Introduction: Association of P2RY1 and P2RY12 polymorphisms with on-aspirin platelet reactivity was investigated.

Materials And Methods: Platelet reactivity was assessed by the light transmission aggregometry and TxB(2) assay in 423 patients with coronary artery disease (CAD) on aspirin. High residual platelet reactivity (RPR) was defined by ≥20% and ≥70% maximal aggregation stimulated with 0.

Has the European union achieved a single pharmaceutical market?

This paper explores price differences in the European Union (EU) pharmaceutical market, the EU's fifth largest industry. With the aim of enhancing quality of life along with industry competitiveness and R&D capability, many EU directives have been adopted to achieve a single EU-wide pharmaceutical market. Using annual 1994-2003 data on prices of molecules that treat cardiovascular disease, we examine whether drug price dispersion has indeed decreased across five EU countries.

Effects of the nano-tubular anodic TiO2 buffer layer on bioactive hydroxyapatite coating.

We studied the effect of nano-tubular anodic TiO2 buffer layers on hydroxyapatite (HA) coating. The pulsed laser deposition (PLD) method was used to deposit HA on a well arranged nano-tubular anodic TiO2 (NT-ATO) buffer layer prepared by an electrochemical anodization technique. The surface morphology and chemical composition of HA coatings were characterized by using scanning electron microscopy (SEM), energy dispersive X-ray spectroscopy (EDS), X-ray diffraction (XRD), and contact angle measurement.

Novel roles of GATA1 in regulation of angiogenic factor AGGF1 and endothelial cell function.

AGGF1 is an angiogenic factor, and its deregulation is associated with a vascular malformation consistent with Klippel-Trenaunay syndrome (KTS). This study defines the molecular mechanism for transcriptional regulation of AGGF1 expression. Transcription of AGGF1 starts at two nearby sites, -367 and -364 bp upstream of the translation start site.

Pharmaceutical price differences in the EU: investigation from cardiovascular disease drugs.

Despite the market integration through the Single Market Program and European Monetary Union, and coordination efforts by the European Commission, the pharmaceutical industry in the EU has remained as separate markets in each member nation rather than integrated owing to several distinguished characteristics of the industry and member countries. As a result, there are large price differences across EU member nations. Although there are increasing harmonization efforts by the European Commission, price differences among the five major markets remain high.

Identification of association of common AGGF1 variants with susceptibility for Klippel-Trenaunay syndrome using the structure association program.

Klippel-Trenaunay syndrome (KTS) is a severe congenital disorder characterized by capillary malformations, venous malformations or varicose veins, and hypertrophy of the affected tissues. The angiogenic factor gene AGGF1 was previously identified as a candidate susceptibility gene for KTS, but further genetic studies are needed to firmly establish the genetic relationship between AGGF1 and KTS. We analyzed HapMap data and identified two tagSNPs, rs13155212 and rs7704267 that capture information for all common variants in AGGF1.

Construction of somatic cell hybrid lines: fusion of mouse thymidine kinase-deficient 3T3 fibroblasts and human lymphoblastoid cells.

Somatic cell hybrids are generated by fusion of two different parental cells. This technology has been used extensively in the production of monoclonal antibodies and has made significant contributions to the field of human genetics through its applications in gene expression, gene mapping, and positional cloning of human disease genes. In our laboratory, we have employed this technique in the positional cloning of several genes for human diseases associated with cytogenetic abnormalities (chromosomal disorders), including translocations.

Fluorescence in situ hybridization in cardiovascular disease.

Many human diseases are associated with cytogenetic abnormalities or chromosomal disorders including translocations, deletions, duplications, inversions, and other complicated chromosomal changes. Fluorescence in situ hybridization (FISH), a technique involving hybridization of labeled probes to chromosomes and detection of hybridization via fluorochromes, has become a popular method for identification and characterization of cytogenetic abnormalities. For FISH analysis, metaphase chromosomes are prepared by mitotic arrest and hypotonic shock, and denatured.

Biomedicine and diseases: the Klippel-Trenaunay syndrome, vascular anomalies and vascular morphogenesis.

Vascular morphogenesis is a vital process for embryonic development, normal physiologic conditions (e.g. wound healing) and pathological processes (e.

Identification and molecular characterization of a de novo supernumerary ring chromosome 18 in a patient with Klippel-Trenaunay syndrome.

Klippel-Trenaunay syndrome (KTS) is a congenital vascular disorder comprised of capillary, venous and lymphatic malformations associated with overgrowth of the affected tissues. In this study, we report the identification of a de novo supernumerary ring chromosome in a patient with mild mental retardation, long tapering fingers, elongated, thin feet and Klippel-Trenaunay syndrome (KTS). The ring marker chromosome was found to be mosaic, present in 24% of cells, and was later shown to be derived from chromosome 18, r(18).

Identification of an angiogenic factor that when mutated causes susceptibility to Klippel-Trenaunay syndrome.

Angiogenic factors are critical to the initiation of angiogenesis and maintenance of the vascular network. Here we use human genetics as an approach to identify an angiogenic factor, VG5Q, and further define two genetic defects of VG5Q in patients with the vascular disease Klippel-Trenaunay syndrome (KTS). One mutation is chromosomal translocation t(5;11), which increases VG5Q transcription.

Identification and molecular characterization of de novo translocation t(8;14)(q22.3;q13) associated with a vascular and tissue overgrowth syndrome.

Klippel-Trenaunay syndrome (KTS) is a disorder primarily characterized by capillary-venous vascular malformations associated with altered limb bulk and/or length. We report the identification of a balanced translocation involving chromosomes 8q22.3 and 14q13 in a patient with a vascular and tissue overgrowth syndrome consistent with KTS.

Molecular pathology of haemophilia A in Turkish patients: identification of 36 independent mutations.

Haemophilia A is an X-linked recessive bleeding disorder caused by heterogeneous mutations in the factor VIII gene. In an attempt to reveal the molecular pathology of Turkish haemophilia A patients, the coding sequence of the gene, excluding a large portion of exon 14, was amplified from genomic DNA and subjected to denaturing gradient gel electrophoresis prior to DNA sequencing. Fifty-nine haemophilia A patients were included in the study with severe, moderate and mild phenotypes observed in 24, 15 and 16 patients, respectively.

Rapid Identification of Family-Specific Mutations in the Factor VIII Gene by One-Step DGGE.

A one-step denaturing gradient gel electrophoresis (DGGE) strategy for the rapid detection of mutations in the factor VIII gene of haemophilia A patients is described. All coding (except the middle part of exon 14) and flanking intronic regions of the gene corresponding to approximately 6.6 kb were amplified in 27 fragments using four PCR programs.

Analysis of the two microsatellite repeat polymorphisms of the factor VIII gene in the Turkish population.

DNA-based diagnosis of haemophilia A has previously been carried out by linkage analysis using two highly informative markers, Hind III RFLP and St14 VNTR, for affected Turkish families. In the present study the number and frequency of the microsatellite alleles at introns 13 and 22 in the factor VIII (FVIII) gene were analysed in order to increase the rate of informative females and accuracy of linkage analysis. Six alleles were observed at both loci.

Fluid Flow in Porous Media Studied by a Nuclear Magnetic Resonance Technique.

A nuclear magnetic resonance technique was used to determine the hydrocarbon content of a porous material during its displacement by an oil-miscible but hydrogen-free liquid (CCl(4)). After displacement of heptane by an amount of carbon tetrachloride equal to 1.5 times the pore volume, about 10 percent of the heptane remained, presumably in "dead-end" pores.