Abstract:
:Von Willebrand disease (VWD) is classified into three primary subtypes; type 1 includes partial quantitative deficiency, type 2 (A, B, M and N) includes qualitative defects and type 3 includes virtually complete deficiency of von Willebrand factor (VWF). Type 2A includes variants with decreased platelet adhesion caused by selective deficiency of high molecular weight VWF multimers. Type 2B includes variants with increased affinity for platelet glycoprotein Ib. Type 2M includes variants with markedly defective platelet adhesion despite normal sized VWF multimers. Finally, type 2N includes variants with markedly decreased affinity for factor VIII (FVIII). Studies have begun to address the association between particular genetic variants of VWF and the disease phenotype. For example, the recessive type 3 disease appears to be due to a variety of mutations that result in a null phenotype, while type 2N disease results from a group of recessive missense mutations that interfere with FVIII binding. Recently, the molecular pathogenesis of type 1 VWD has begun to be characterised revealing a diverse spectrum of genetic changes. In two large studies, patients with type 1 VWD were found to be heterozygous for a variety of missense, splice site and transcriptional mutations. In some patients, more than one variant sequence was present, and in a significant number of individuals no changes were evident in the VWF gene. These results suggest that the molecular correlates for type 1 VWD are complex and, in addition to a wide array of changes at the VWF locus, are likely to involve mutations in other genes.
journal_name
Thromb Resjournal_title
Thrombosis researchauthors
Lillicrap Ddoi
10.1016/j.thromres.2007.03.014subject
Has Abstractpub_date
2007-01-01 00:00:00pages
S11-6eissn
0049-3848issn
1879-2472pii
S0049-3848(07)00117-Xjournal_volume
120 Suppl 1pub_type
杂志文章,评审abstract:INTRODUCTION:Management of pregnant women at risk for venous thromboembolism (VTE) remains complex. Guidelines do not definitively fix optimal strategies due to limited trial data. Our objective was to build an easy-to-use tool allowing individualised, risk-adapted prophylaxis. MATERIALS AND METHODS:A Delphi exercise ...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/j.thromres.2010.01.012
更新日期:2010-08-01 00:00:00
abstract:INTRODUCTION:Growth differentiation factor-15 (GDF-15) has been identified as a predictor in cardiovascular diseases and acute pulmonary embolism. However, the association of GDF-15 and deep venous thrombosis (DVT) remains unclear. This study aimed to investigate levels of GDF-15 in patients with DVT, and determine its...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/j.thromres.2020.01.022
更新日期:2020-03-01 00:00:00
abstract:INTRODUCTION:Sepsis is frequently complicated by disseminated intravascular coagulation (DIC), which promotes multiple organ dysfunctions and significantly increase the mortality of patients with sepsis. How bacteria cause DIC is not fully understood. Outer membrane vesicles (OMVs) are membrane-enclosed microvesicles r...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/j.thromres.2019.03.019
更新日期:2019-06-01 00:00:00
abstract::The standard modality of administration of rFVIIa to patients with FVIII and FIX inhibitors is the intermittent infusion every 2 to 6 hours. No untoward local or systemic effects have been reported; laboratory data of activation of coagulation were reported in the presence of coexistent problems (sepsis, septic shock)...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/s0049-3848(00)00220-6
更新日期:2000-07-01 00:00:00
abstract::Preanalytical and biological variability both have strong influences on test results of coagulation. Beyond age and gender, increasing emphasis is now being given to a variety of demographic and lifestyle variables, including ethnicity, smoking, diet, and exercise, which should be taken into account when interpreting ...
journal_title:Thrombosis research
pub_type: 杂志文章,评审
doi:10.1016/j.thromres.2008.04.010
更新日期:2008-01-01 00:00:00
abstract::Surviving an embolism exposes patients to potential long-term complications, such as altered quality of life, persistent dyspnea, impaired exercise capacity or pulmonary hypertension. The common objective factor in most of these situations is the presence of residual pulmonary vascular obstruction (RPVO). Planar venti...
journal_title:Thrombosis research
pub_type: 杂志文章,评审
doi:10.1016/j.thromres.2019.09.038
更新日期:2019-12-01 00:00:00
abstract:INTRODUCTION:Prediction of venous thromboembolism (VTE) occurrence in cancer patients using individual risk factors may contribute to preventing the burden of disease associated with VTE. Congestive heart failure in patients with cancer may increase the risk of VTE and worsen the prognosis. AIM:We sought to investigat...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/S0049-3848(16)30134-7
更新日期:2016-04-01 00:00:00
abstract:BACKGROUND:Administrative data can be used to identify venous thromboembolism (VTE) and major bleeding (MB) events. However, the validity of this data in emergency room discharge records in Canada is unknown. METHODS:We conducted a single-institution retrospective chart re-abstraction study in London, Canada. We ident...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/j.thromres.2015.10.035
更新日期:2015-12-01 00:00:00
abstract::A new approach to the investigation of accumulation of acridine derivatives and release by platelets is proposed. At high concentrations of acridine orange (AO) inside dense bodies, a two component fluorescence spectrum with maxima at 530 and 640 nm has been recorded. Computer deconvolution of spectra provided additio...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/0049-3848(87)90102-2
更新日期:1987-09-15 00:00:00
abstract:INTRODUCTION:Conventional Vacutainer® Sodium Citrate blood collection tubes contain a relatively high concentration of contaminating magnesium ions, which may result in shortening of the prothrombin time (PT) and the International Normalized Ratio (INR). Recently the manufacturer of Vacutainer® Sodium Citrate tubes int...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/j.thromres.2014.04.016
更新日期:2014-07-01 00:00:00
abstract::Resistance to Activated Protein C (APC) was evaluated using 3 different methods: two of them were based on the prolongation of the Activated Partial Thromboplastin Time (APTT) using 2 different APTT reagents in the presence of APC, whereas the third method was based on the prolongation of prothrombin time when APC is ...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/0049-3848(94)90206-2
更新日期:1994-10-01 00:00:00
abstract::To investigate the effects of new drugs on the prevention of venous thrombosis, the Wessler stasis model is extensively employed usually in the rabbit. In this model, a hypercoagulable state is achieved by the administration of either heterologous serum (often human serum) or an activated coagulation factor followed b...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/0049-3848(96)00023-0
更新日期:1996-02-15 00:00:00
abstract::Plasma from patients with thrombotic thrombocytopenic purpura (TTP) caused the aggregation of washed human platelets, which was inhibited by preincubation with normal plasma. Using salt fractionation, ion exchange chromatography, and preparative agarose gel electrophoresis, we purified a protein from normal plasma whi...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/0049-3848(84)90155-5
更新日期:1984-01-01 00:00:00
abstract::Concentration-response relationships of human platelet aggregation rates were analyzed for a variety of agonists and inhibitors. Their approximation by the Hill equation showed that the values of the Hill coefficient (h) were agonist-dependent and increased as follows: hADP = hL-EPINEPHRINE = hPAF = hPGH2 = hU46619 le...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/0049-3848(92)90308-w
更新日期:1992-06-01 00:00:00
abstract::Plasma membranes of pig platelets were isolated and subjected to solubilization with digitonin. Binding of [3H]ketanserin to the solubilized membrane fraction, optimal at pH 7.5, was time and protein dependent and was greatly reduced by increasing ionic strength of the incubation buffer. Saturation analysis and inhibi...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/0049-3848(85)90253-1
更新日期:1985-09-15 00:00:00
abstract::The present investigation has used an aggregation-disaggregation-reaggregation model to determine if exposure to potent aggregating agents causing a refractory state in deaggregated platelets results in down-regulation and clearance of GPIb/IX, the receptor for von willebrand factor. Thrombin, the thrombin receptor ac...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/s0049-3848(96)00185-5
更新日期:1996-11-15 00:00:00
abstract::SK, t-PA or APSAC were incubated in human plasma (adjusted to 300,000 platelets/mm3), in vitro, for up to 90 minutes using concentrations which were equivalent to those achieved in the treatment of AMI patients. Aggregation was measured in response to ADP and collagen. SK inhibited platelet aggregation after a 60 minu...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/0049-3848(90)90105-l
更新日期:1990-11-15 00:00:00
abstract::Since the first description by Dahlbäck et al (1) of a hereditary defect in the plasma of three unrelated thrombophilic patients characterized by a poor anticoagulant response to activated protein C (APC), many reports have shown a high prevalence of the APC resistance in both the general and thrombophilic populations...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/s0049-3848(97)00022-4
更新日期:1997-02-15 00:00:00
abstract::Optimal conditions for a method to simultaneously measure aggregation in 96 samples using a microplate reader were developed. The temperature of the assay was set at 25 degrees C, the optimal platelet concentration range was determined to be from 1-3 x 10(8) per mL, the assay volume was determined to be best at 100 mi...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/0049-3848(95)93881-y
更新日期:1995-03-01 00:00:00
abstract:BACKGROUND:There is a substantial local release of tissue-type plasminogen activator (t-PA) in the splanchnic vascular bed, and this release is increased at high sympathetic tone. Coronary t-PA release is also significant, and this release increases during cardiac nerve stimulation and during reperfusion after 10 min o...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/j.thromres.2009.10.004
更新日期:2010-03-01 00:00:00
abstract::In this study we examined in 100 patients testing positive for Helicobacter pylori infection whether successful eradication therapy with pantoprazole, clarithromycin, and metronidazole alters fibrinogen and other acute phase response markers. Of 100 patients, only 11 showed a fibrinogen level above 300 mg/dL. Successf...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/s0049-3848(99)00230-3
更新日期:2000-03-15 00:00:00
abstract:INTRODUCTION:Plasminogen activator inhibitor (PAI-1) may have an independent prognostic value in breast cancer (BC). PAI-1 4G/5G polymorphism may have significance for antigen expression. Thus, we analyzed the possible associations between PAI-1 4G/5G polymorphism, plasma PAI-1 levels, and clinicopathological features ...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/j.thromres.2009.02.014
更新日期:2009-09-01 00:00:00
abstract:BACKGROUND:In patients who are receiving warfarin therapy and require cataract surgery, it may be possible to continue warfarin in the perioperative period but the safety of this management strategy has not been systematically evaluated. METHODS:We performed a systematic review of the literature to assess the safety (...
journal_title:Thrombosis research
pub_type: 杂志文章,meta分析,评审
doi:10.1016/j.thromres.2009.01.007
更新日期:2009-07-01 00:00:00
abstract::Lupus anticoagulants (LA) are IgG or IgM antibodies which prolong phospholipid-dependent coagulation tests. For the detection and quantitation of such antibodies, we have developed an ELISA with cephalin as the coating antigen. The sensitivity of this assay was compared to the activated partial thromboplastin time (AP...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/0049-3848(90)90323-5
更新日期:1990-01-15 00:00:00
abstract:INTRODUCTION:Tissue factor plays a key role in the extrinsic coagulation pathway and is induced by inflammatory cytokines. Atrial myocarditis has been detected recently in some patients with lone atrial fibrillation. Virchow's triad of low blood flow, hypercoagulability, and endothelial dysfunction, enhances thrombus f...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/s0049-3848(03)00405-5
更新日期:2003-01-01 00:00:00
abstract::Many patients with diabetes mellitus show increased platelet aggregation and prostaglandin synthesis in response to physiological agents such as ADP and collagen when their platelets are tested in platelet-rich plasma or washed platelet suspensions. However, the relationship between increased platelet aggregation in v...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/0049-3848(87)90265-9
更新日期:1987-06-01 00:00:00
abstract:INTRODUCTION:Fluid resuscitation after trauma often results in dilutional coagulopathy that may hinder control of bleeding and, once initial hemostasis has been secured, heighten risk of perioperative bleeding when further surgery is required. Since multiple coagulation factor deficiencies typically accompany fluid res...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/j.thromres.2009.10.011
更新日期:2010-03-01 00:00:00
abstract::An electron transport system regulates the initiation of thrombus formation through the activation of critical receptors involved in hemostasis and thrombosis. Protein disulfide isomerase along with other thiol isomerases, important for intracellular protein synthesis, are responsible for this extracellular activity d...
journal_title:Thrombosis research
pub_type: 杂志文章,评审
doi:10.1016/j.thromres.2012.08.272
更新日期:2012-10-01 00:00:00
abstract:INTRODUCTION:Anedoctal reports suggest that some thrombotic primary antiphospholipid antibody syndrome (PAPS) patients on oral anticoagulation require higher than average doses to achieve given targets international normalized ratios (INR). MATERIALS AND METHODS:To test the hypothesis of relative warfarin resistance i...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/j.thromres.2011.01.014
更新日期:2011-06-01 00:00:00
abstract::Porcine and human umbilical vein and adult blood vessels were studied for the presence and synthesis of factor VIII related antigen (VIIIR:Ag) and fibronectin (Fn) by immunofluorescence histology and immunoautoradiography. Investigation of human tissue confirmed the widespread distribution of VIIIR:Ag on the endotheli...
journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/0049-3848(83)90042-7
更新日期:1983-02-01 00:00:00