Abstract：
:Hemophilia B is a sex-linked recessive bleeding disorder characterized by the presence of either a decreased amount of normal factor IX (FIX) or the presence of a dysfunctional FIX. We have identified a unique mutation in a family with mild hemophilia B. DNA analysis of family members revealed a single base transition in the 8th exon of the FIX gene predicting an amino acid change of Asn 346-->Asp in the catalytic domain. The FIX variant, named FIX Denver, was purified from proband plasma. Kinetic studies of factor X (FX) interactions with normal FIXa or FIXa Denver and phospholipid (PL) showed little difference in kcat but a significant difference when factor VIIIa (FVIIIa) was included in the reaction. Using kinetic assays to infer the Kd of FIXa for FVIIIa, normal FIXa had a Kd of 0.095 nM while that of FIXa Denver was 9.85 nM. The major defect caused by this point mutation is a marked decrease in the affinity of FIXa Denver for factor VIIIa.
journal_name
Thromb Haemostjournal_title
Thrombosis and haemostasisauthors
Lefkowitz JB,Nuss R,Haver T,Jacobson L,Thompson AR,Manco-Johnson Mkeywords:
subject
Has Abstractpub_date
2001-09-01 00:00:00pages
862-70issue
3eissn
0340-6245issn
2567-689Xpii
01090862journal_volume
86pub_type
杂志文章abstract：:A new genetic test has been developed for detection of the mutation known as factor V Leiden. The test employs heteroduplex technology and comprises a single PCR reaction followed immediately by PCR product analysis. It therefore represents the minimum practical route from blood/tissue sample to genetic result. A coho...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1997-01-01 00:00:00
abstract：:Plaque rupture underlies most myocardial infarctions. Plaques vulnerable to rupture have thin fibrous caps, an excess of macrophages over vascular smooth muscle cells, large lipid cores, and depletion of collagen and other matrix proteins form the cap and lipid core. Production of matrix metalloproteinases from macrop...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：2009-06-01 00:00:00
abstract：:Lupus anticoagulants (LA) are immunoglobulins directed to either prothrombin or Beta-2-glycoprotein 1(beta1GPI) bound to phospholipids. Most patients with LA have both beta2GPI- and prothrombin-dependent antibodies. Several recent reports have shown that LA is more strongly associated with thrombosis than anticardioli...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,多中心研究
doi：
更新日期：1999-06-01 00:00:00
abstract：:The object was to assess the variability in displayed International Normalised Ratio (INR) between monitors of the same manufacture using whole blood samples from the same subjects. Two brands of monitor, CoaguChek Mini and the TAS PT-NC were tested. 14 instruments of each brand were tested on the same day at the same...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：2002-12-01 00:00:00
abstract：:The calcium electrode is a convenient, inexpensive, and non-traumatic method for measuring changes in the extracellular calcium which accompanies a platelet release reaction. With this instrument, the temporal pattern of release by platelets in a buffered-saline medium following thrombin stimulation was observed as fo...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1977-06-30 00:00:00
abstract：:Platelet counts were evaluated in 714 patients with advanced non-small cell lung cancer (N-SCLC), small cell carcinoma of the lung (SCCL), and colon cancer entered to a clinical trial. Patients had not received prior chemotherapy. Platelet counts were not different in patients who had received radiation therapy prior ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1990-12-28 00:00:00
abstract：:Thrombopoietin is produced at a constant rate by the liver and kidney and is removed from the circulation upon binding and subsequent uptake via the Tpo receptor, c-Mpl, expressed by platelets and mega-karyocytes. Apart from uptake, this study shows that platelets can also function as a storage pool for Tpo. Upon stim...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：2000-06-01 00:00:00
abstract：:Patients with diabetes display increased platelet activation. Recent data show a markedly increased risk for cardiovascular events already in pre-diabetic individuals with impaired glucose tolerance (IGT). We investigated whether IGT is associated with platelet activation. Blood samples were collected from young lean ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH04-02-0118
更新日期：2004-07-01 00:00:00
abstract：:There is no patient-reported medication adherence measure that has been validated in Singapore. This study aimed to validate the 8-item Morisky Medication Adherence Scale (MMAS) in patients taking warfarin in Singapore. A cross-sectional survey was conducted in a convenience sample of 151 patients taking warfarin at a...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH12-05-0368
更新日期：2012-10-01 00:00:00
abstract：:Factor Xa (fXa)-inhibitors are as effective and safer than vitamin-K-antagonists (VKA) in the treatment of venous thromboembolism (VTE). We previously classified the severity of clinical presentation and course of all major bleeding events from the EINSTEIN, AMPLIFY and HOKUSAI-VTE trials separately. The current aim w...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH16-12-0946
更新日期：2017-10-01 00:00:00
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journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH04-12-0809
更新日期：2005-05-01 00:00:00
abstract：:Myocardial infarction results from a platelet-rich occlusive coronary thrombus. Platelet membrane glycoprotein IIb/IIIa plays an important role in platelet adhesion and aggregation. Two polymorphisms of the gene encoding the IIIa subunit. PLA1 and PLA2, have been identified. We investigated the frequency of these poly...
journal_title：Thrombosis and haemostasis
pub_type： 临床试验,杂志文章
doi：
更新日期：1998-08-01 00:00:00
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journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1997-09-01 00:00:00
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journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,评审
doi：10.1160/TH09-05-0323
更新日期：2009-12-01 00:00:00
abstract：:One hundred patients were included in a randomized open trial to assess the systemic factor Xa (FXa) and thrombin inhibitory effect as well as the safety profile of low molecular weight heparin (LMWH) given subcutaneously in conjunction with streptokinase (SK) in patients with acute myocardial infarction (MI). The tre...
journal_title：Thrombosis and haemostasis
pub_type： 临床试验,杂志文章,随机对照试验
doi：
更新日期：1997-01-01 00:00:00
abstract：:MicroRNAs are small non-coding RNAs that negatively regulate posttranscriptional gene expression. Several microRNAs have been described to regulate the process of angiogenesis. Previously, we have shown that bone morphogenetic protein 4 (BMP4) increased the pro-angiogenic activity of endothelial cells. In this project...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH16-08-0643
更新日期：2017-04-03 00:00:00
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journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1979-08-31 00:00:00
abstract：:Moderate or severe paravalvular regurgitation after transcatheter aortic valve implantation (TAVI) is frequently associated with a loss of high-molecular-weight multimers of von Willebrand factor (VWF) and a reduced VWF collagen-binding capacity. It is unclear whether this phenomenon can also be observed in patients w...
journal_title：Thrombosis and haemostasis
pub_type： 临床试验,杂志文章
doi：10.1160/17-07-0506
更新日期：2018-01-01 00:00:00
abstract：:Tissue factor (thromboplastin or Factor III), a glycoprotein cofactor, is required for Factor VII to express its catalytic activity, thereby initiating the extrinsic as well as intrinsic pathway of blood coagulation. Human brain tissue factor was purified 2,500-fold to 98% homogeneity from 2% Triton X-100 extraction o...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1988-06-16 00:00:00
abstract：:The most accepted therapy for DVT consists of anticoagulation with unfractionated heparin or low molecular weight heparin, followed by variable duration oral anticoagulation but thrombolytic therapy has been proposed in addition to standard anticoagulation. This paper reviews the literature on post thrombotic syndrome...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,meta分析
doi：
更新日期：2001-07-01 00:00:00
abstract：:Venous thromboembolism (VTE) is a central concern in the intensive care unit (ICU). However, little is known about both current practices for VTE prevention in the ICU and the risk for VTE in persons with severe sepsis and septic shock. XPRESS was a randomized, double-blind, placebo-controlled trial of prophylactic he...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,随机对照试验
doi：
更新日期：2009-01-01 00:00:00
abstract：:Venous thromboembolism, which encompasses deep-vein thrombosis and acute pulmonary embolism (PE), represents a major contributor to global disease burden worldwide. For patients who present with cardiogenic shock or persistent hypotension (acute high-risk PE), there is consensus that immediate reperfusion treatment ap...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,评审
doi：10.1160/TH14-11-0998
更新日期：2015-06-01 00:00:00
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journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,评审
doi：
更新日期：1999-08-01 00:00:00
abstract：:Examination of the entire von Willebrand factor (VWF) gene for mutations, particularly in types 1 and 3 von Willebrand disease (VWD) is becoming more widely practised. The sequence of the entire VWF gene will soon be compiled as a single sequence. For these reasons, a clearly defined nomenclature to use for numbering ...
journal_title：Thrombosis and haemostasis
pub_type： 指南,杂志文章
doi：
更新日期：2001-05-01 00:00:00
abstract：:Thrombolytic therapy has become the mainstay of treatment for acute transmural myocardial infarction. Present fibrinolytic regimens have a number of shortcomings, including the failure to induce early and sustained reperfusion in as many as 40-50% of the patients, and to prevent reocclusion in another 10-20% of the pa...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,评审
doi：
更新日期：1999-09-01 00:00:00
abstract：:It was found that the effect of heparin on the amidase activity of urokinase (E C 3.4.21.31), plasmin (E C 3.4.21.7) and trypsin (E C 3.4.21.4) depended on the substrate used. No effect of heparin on the amidase activity of urokinase and trypsin was observed when Pyro Glu-Gly-Arg-p-nitroanilide (S-2444) and alpha-N-ac...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1983-06-28 00:00:00
abstract：:A robust two-way analysis of variance technique was applied to determine simultaneously the effects of method and thromboplastin on prothrombin time. A new approach to outlier detection for two-way analysis of variance was used. Focusing on the underlying error structure improved the uniformity of the grading procedur...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1985-10-30 00:00:00
abstract：:Patients with inflammatory bowel disease (IBD) are at higher risk of venous thromboembolism and coronary artery disease despite having a lower burden of traditional risk factors. Platelets from IBD patients release more soluble CD40 ligand (CD40L), and this has been implicated in IBD platelet hyper-activation. We here...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH16-02-0167
更新日期：2016-08-30 00:00:00
abstract：:A four-decade clinical experience and recent evidence from randomised controlled studies definitively recognised primary prophylaxis, i.e. the regular infusion of factor concentrates started after the first haemarthrosis and/or before the age of two years, as the first-choice treatment in children with severe haemophi...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,评审
doi：
更新日期：2009-04-01 00:00:00
abstract：:Heparin cofactor II (HC II) is a heparin-dependent inhibitor of thrombin, distinct from antithrombin III (AT III). This study was designed to evaluate its metabolism in healthy subjects. Purified HC II was labelled with 125I by the lactoperoxidase-glucose oxidase technique. The biological activity of the HC II was unc...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1985-10-30 00:00:00
