Abstract：
:In adult patients with sickle cell disease two distinct subphenotypes have previously been defined: patients with the viscosity-vaso-occlusion subphenotype (VVO) suffer mainly from vaso-occlusive pain crises and have a relatively high hemoglobin concentration. Patients classified as the hemolysis-endothelial dysfunction subphenotype (HED) suffer from stroke and pulmonary hypertension and have an elevated concentration of lactate dehydrogenase. However, this classification is not possible in children due to low rates of complications. We used laboratory markers to classify children into the two subphenotypes, and measured vWF and vWF propeptide as markers of endothelial dysfunction. We included 106 children with sickle cell disease (mean age 8.7years), 74 (70%) with HbSS/HbSβ° genotype and 32 (30%) with HbSC/HbSβ(+) genotype. vWF and vWF propeptide were significantly elevated in patients with sickle cell disease; this was more pronounced in patients with the HbSS/HbSβ° genotype. Patients with the HED subphenotype had higher levels of vWF propeptide, and a trend towards higher levels of vWF compared to those with the VVO subphenotype. We demonstrated that even young children in a stable clinical condition show signs of persistent endothelial dysfunction. A prospective study should demonstrate whether elevated levels of vWF and its propeptide are associated with an increased risk of complications specific for the HED subphenotype.
journal_name
Thromb Resjournal_title
Thrombosis researchauthors
van der Land V,Peters M,Biemond BJ,Heijboer H,Harteveld CL,Fijnvandraat Kdoi
10.1016/j.thromres.2013.10.006subject
Has Abstractpub_date
2013-01-01 00:00:00pages
712-7issue
6eissn
0049-3848issn
1879-2472pii
S0049-3848(13)00462-3journal_volume
132pub_type
杂志文章abstract：INTRODUCTION:Pre-eclampsia is an important cause of maternal morbidity and mortality. Its etiology is still unknown. Clinical symptoms correlate with activation of coagulation and inherited thrombophilia has been associated with pre-eclampsia. ABO blood group has been associated with thrombotic disorders and pre-eclamp...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2008.11.012
更新日期：2009-06-01 00:00:00
abstract：:Pharmacovigilance is an essential element of any drug treatment and considering the history of adverse events due to products used to treat inherited bleeding disorders, it should be an integral component of modern haemophilia treatment. Because inherited bleeding disorders and adverse events are rare, a multicentre, ...
journal_title：Thrombosis research
pub_type： 杂志文章,多中心研究
doi：10.1016/S0049-3848(10)70150-X
更新日期：2011-01-01 00:00:00
abstract：BACKGROUND:Immune thrombocytopenia (ITP) is an immune-mediated bleeding disorder in children. Activated T cells have been shown to play important roles in ITP. The aims of this study were to evaluate whether these T cell activation markers could be used as indicators to differentiate ITP patients from controls, and to ...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2019.05.010
更新日期：2019-08-01 00:00:00
abstract：:We report an inbred family with two cases of homozygous protein C deficiency and review 11 other such cases. Both patients presented in the second half of their first year of life with recurrent rapidly disappearing ecchymotic skin lesions, disseminated intravascular coagulation, and venous thrombosis. Successful trea...
journal_title：Thrombosis research
pub_type： 杂志文章,评审
doi：10.1016/0049-3848(89)90202-8
更新日期：1989-03-01 00:00:00
abstract：:Venous thromboembolism (VTE), comprising deep vein thrombosis (DVT) and pulmonary embolism (PE), presents a large clinical burden. Prompt, effective and sustained anticoagulation is vital because of the risk of recurrent events, including life-threatening PE, and complications such as post-thrombotic syndrome and chro...
journal_title：Thrombosis research
pub_type： 杂志文章,评审
doi：10.1016/j.thromres.2014.05.013
更新日期：2014-08-01 00:00:00
abstract：:Fibrin is found in most solid tumours, and there is speculation regarding its role in tumour invasion and metastasis. An assay to quantitate fibrin levels in tissues would be a useful preliminary tool in assessing the above. Such an assay to quantitatively detect levels of fibrin in various types of canine tumour was ...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/s0049-3848(99)00054-7
更新日期：1999-10-01 00:00:00
abstract：:Highly purified bovine von Willebrand protein (vWP) has been studied with two complementary techniques. Light scattering measurements on different samples give weight average molecular weights in the range from 7 to 13 million, radius of gyration between 75 and 140 nm and diffusion coefficient near 4.15 X 10(-8) cm2/s...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(85)90289-0
更新日期：1985-11-15 00:00:00
abstract：:Hemostasis, the system of generation and degradation of thrombi, consists of coagulation and fibrinolysis. Whereas global assays to study coagulation have existed for many years, there has been no simple, rapid, and economic routine test for the plasmatic fibrinolysis parameters plasminogen activator inhibitor-1, alph...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/s0049-3848(99)00165-6
更新日期：2000-02-15 00:00:00
abstract：INTRODUCTION:Pulmonary embolism (PE) is a common cause of morbidity and mortality. In this study, we investigated patterns of morbidity and mortality from PE in Australia. MATERIALS & METHODS:Australian government databases were used to extract data on age and sex specific rates of mortality between 1997 and 2007, and...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2013.04.032
更新日期：2013-07-01 00:00:00
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 cause...
journal_title：Thrombosis research
pub_type： 杂志文章,评审
doi：10.1016/j.thromres.2007.03.014
更新日期：2007-01-01 00:00:00
abstract：INTRODUCTION:Global clotting assays may reflect an individual's net hemostatic balance and could contribute to prothrombotic and hemorrhagic risk assessment. In this research, a global assay that measures both coagulation and fibrinolytic capacities was developed and investigated. MATERIALS AND METHODS:In the Clot For...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2004.12.009
更新日期：2005-01-01 00:00:00
abstract：:Factor Xa-catalyzed prothrombin activation occurs on cellular surfaces, including those of vascular endothelial cells. In a reconstituted model, endothelial cells and platelets acted synergistically to maximally activate prothrombin in the presence of Factor Xa. Synergism was observed at platelet concentrations less t...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(87)90168-x
更新日期：1987-01-15 00:00:00
abstract：:We characterized a platelet specific alloantigen (Yukb). In immunoblotting, anti-Yukb antibody was found to react with both 110 kDa and 96 kDa bands under nonreducing condition. Immunoblotting followed by separation by two-dimensional electrophoresis (isoelectric focusing/SDS-PAGE) showed that the 96 kDa band had a pI...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(92)90136-x
更新日期：1992-07-15 00:00:00
abstract：BACKGROUND:Although risk of hospital-associated venous thromboembolism (HA-VTE) differs between critically and non-critically ill children, studies to date have not led to distinct, pragmatic risk scores. OBJECTIVE:To determine risk factors for HA-VTE in critically ill children not undergoing cardiothoracic surgery, i...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2015.04.036
更新日期：2015-10-01 00:00:00
abstract：:Normal pregnancy is associated with an increasing state of activation of the haemostatic system. This activation state is excessive in women with placenta-mediated pregnancy complications (PMPCs), including preeclampsia (PE). Platelet activation plays a crucial pathophysiological role in PE. The very early activation ...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/S0049-3848(19)30359-7
更新日期：2019-09-01 00:00:00
abstract：:The effects of sodium salicylate (SS) on ADP-induced platelet aggregation and on a metabolism of calcium in platelets were studied, using gel-filtrated platelets (GFP). SS inhibited dose-responsively ADP-induced aggregation in the presence of fibrinogen and Ca2+. It was found that extracellular 45Ca2+ was rapidly take...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(85)90232-4
更新日期：1985-08-01 00:00:00
abstract：INTRODUCTION:To test the hypothesis that the platelet microparticle (PMP) and endothelial microparticle (EMP) may contribute to the hypercoagulability associated with microvascular thrombosis in patients with nontraumatc osteonecrosis of the femoral head (ONFH). MATERIALS AND METHODS:The study comprised 46 patients wh...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2008.04.001
更新日期：2008-01-01 00:00:00
abstract：:Heat treatment is employed to diminish the transmission of hepatitis when blood products are administered. It is possible that such a procedure could reduce the biological activity of the proteins and induce changes in structure and aggregation state. We have therefore made in vitro and pharmacokinetic studies of heat...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(87)90435-x
更新日期：1987-12-15 00:00:00
abstract：:Addition of two commercial luciferin-luciferase reagents caused marked inhibition of the aggregatory response of washed human platelets to thrombin, ADP, vasopressin and platelet-activating factor (PAF). Analysis of the effects of the individual components of one of these reagents revealed that Mg2+, and to a lesser e...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(85)90053-2
更新日期：1985-04-15 00:00:00
abstract：INTRODUCTION:Systemic embolism is the most serious complication of cardioversion of atrial fibrillation (AF) and the immediate post-cardioversion period is associated with increased risk for thrombus formation. For this reason, treatment with vitamin K antagonist (VKA) is recommended for patients with AF. No informatio...
journal_title：Thrombosis research
pub_type： 杂志文章,多中心研究
doi：10.1016/j.thromres.2011.08.031
更新日期：2012-05-01 00:00:00
abstract：BACKGROUND:The plasminogen activator inhibitor type 1 (PAI-1) 4G/5G polymorphism may have significance for PAI-1 expression. High levels of PAI-1 in breast cancer patients are associated with a poor prognosis. In this study, we analyzed the influence of the PAI-1 4G/5G polymorphism on tissue PAI-1 levels and its associ...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2005.03.025
更新日期：2006-01-01 00:00:00
abstract：:Forty women undergoing surgery under general anaesthesia for hyperplasia mammae were randomized to treatment with low dose heparin (5000 IU twice daily) or not. Preoperatively, and repeated on the 3. postoperative day, assays of euglobulin clot lysis time (ELT) after venous stasis, tissue plasminogen activator (t-PA) ...
journal_title：Thrombosis research
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1016/0049-3848(87)90318-5
更新日期：1987-03-01 00:00:00
abstract：:Adenosine is an endogenous antiaggregating substance that influences the platelet responses through specific A-type receptors that activate adenylate cyclase increasing the levels of 3',5'-cyclic adenosine monophosphate (cAMP). In this study, we investigated whether adenosine can also influence the levels of 3',5'-cyc...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/s0049-3848(01)00412-1
更新日期：2002-01-01 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：OBJECTIVES:To measure the variations of different parameters in the hemostatic system and to analyze their roles in the development of hemostatic disorder in patients with orthotopic liver transplantation (OLT) procedures routinely performed without venovenous bypass. METHODS:The blood coagulation and fibrinolysis par...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2007.10.002
更新日期：2008-01-01 00:00:00
abstract：:The effects of ticlopidine treatment (250 mg b.i.d. for 21 months) on fibrinogen and other rheological variables, as compared to placebo, were studied in 44 patients with intermittent claudication due to peripheral arterial occlusive disease. Blood samples were collected every 3 months during this double-blind, random...
journal_title：Thrombosis research
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1016/0049-3848(88)90134-x
更新日期：1988-12-15 00:00:00
abstract：INTRODUCTION:Tissue Factor Pathway Inhibitor-2 (TFPI-2) is a Kunitz-type serine proteinase inhibitor whose expression is up-regulated by VEGF in microvascular and umbilical vein endothelial cells (EC). Despite this, TFPI-2 has been suggested as anti-angiogenic molecule, due to its ability to inhibit the migration/proli...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/S0049-3848(16)30177-3
更新日期：2016-04-01 00:00:00
abstract：:Patients with active cancer have a heightened risk of venous thromboembolism (VTE). This risk is further increased by the initiation of chemotherapy. Although previous studies have suggested that the use of parenteral thromboprophylaxis in all ambulatory cancer patients receiving chemotherapy significantly decreases t...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2018.01.018
更新日期：2018-04-01 00:00:00
abstract：:Among central Europeans polymorphisms of GPIIIa, GPIb, GPIIb, and GPIa named human platelet antigen-1 (HPA-1), -2, -3, and -5 are the clinically most relevant systems in which alloimmunization occurs. These genetically determined polymorphisms of glycoproteins may render platelets sensible for plaque formation and thu...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/s0049-3848(98)00042-5
更新日期：1998-05-01 00:00:00
abstract：:The concentrations of tissue plasminogen activator (t-PA) and urokinase (UK) were measured in the plasma of patients with benign or malignant ovarian and uterine tumors. Plasma levels of t-PA were higher in patients with malignant ovarian and uterine tumors than in patients with benign tumors. Plasma UK levels were, h...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(90)90207-s
更新日期：1990-05-15 00:00:00
