Abstract:
:Haemophilia A and B are X-linked disorders which are due to a reduced activity of coagulation factor VIII or IX, respectively. Female carriers have a wide range of plasma concentration of factor VIII or factor IX, and may in rare cases have an affected phenotype. In order to investigate if this variation is related to X chromosome inactivation, we determined the X inactivation pattern in 31 haemophilia A and 15 haemophilia B carriers, using a PCR in the androgen receptor locus in blood DNA. Seven of the haemophilia A carriers and none of the haemophilia B carriers had a skewed pattern (> or =80:20). One of the skewed haemophilia A carriers had a low plasma concentration of factor VIII (0.15 U/ml), but the remaining 6 carriers did not differ in factor VIII concentration from that of carriers with a random X inactivation pattern. One carrier with a high factor VIII concentration (2.0 U/ml) did not have a skewed pattern. Similarly, for the haemophilia B carriers, there was no tendency to a more skewed X inactivation pattern in the carriers with low or high factor IX concentrations. In addition, we analysed a female with haemophilia B who was heterozygous for the mutation R180W in the factor IX gene. She had a random X chromosome inactivation pattern. We conclude that the wide range in plasma concentration of factor VIII and factor IX in haemophilia A and B carriers cannot in general be explained by the X chromosome inactivation pattern in peripheral blood cells.
journal_name
Thromb Haemostjournal_title
Thrombosis and haemostasisauthors
Orstavik KH,Scheibel E,Ingerslev J,Schwartz Mkeywords:
subject
Has Abstractpub_date
2000-03-01 00:00:00pages
433-7issue
3eissn
0340-6245issn
2567-689Xpii
00030433journal_volume
83pub_type
杂志文章abstract::An experimental model incorporating cultured endothelial cells (EC) was used to study the "factor VIII bypassing" activity of prothrombin complex concentrates (PCC), a property exploited in the treatment of hemophiliacs with alloantibodies to factor VIII. Two PCC preparations were ineffective as stimuli of tissue fact...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1991-11-01 00:00:00
abstract::A previous study of neurosurgical patients demonstrated an imbalance between thrombin and plasmin action following surgery. The present study was designed to determine the effect of intermittent pneumatic calf compression on postoperative enzyme activity. Fibrinopeptide A (FPA) and B beta 1-42 levels, reflecting throm...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1986-10-21 00:00:00
abstract::The antithrombotic effect of MCI-9042, (+-)-2-(dimethyl-amino)-1-((o-(m- methoxyphenethyl)phenoxyl]methyl]ethyl hydrogen succinate hydrochloride was investigated in three different experimental thrombosis models in animals. Simultaneous injection of serotonin and collagen into the tail vein in mice induced acute pulmo...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1991-10-01 00:00:00
abstract::To study the reversible complex formation between the plasma protein histidine-rich glycoprotein (HRG) and plasminogen, crossed immunoelectrophoresis of HRG was modified. In the modification, purified plasminogen was introduced into the gel of the first dimension electrophoresis. Two molecular forms of plasminogen, Gl...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1988-12-22 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::Coagulation factor XIII, a transglutaminase which stabilises blood clots by covalently cross-linking fibrin, is essential for normal haemostasis. FXIII deficiency results in a life-long bleeding disorder with added complications in wound healing and tissue repair. Sequence changes in the human FXIIIA gene, largely mis...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1998-06-01 00:00:00
abstract::A native fucosylated glycosaminoglycan from sea cucumber Holothuria fuscopunctata (nHG), mainly branched with Fuc3S4S, exhibited potent anticoagulant activity by intrinsic tenase iXase (FIXa-FVIIIa complex) and antithrombin-dependent factor IIa (FIIa) inhibition, but also had the effects of FXII activation and platele...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1055/s-0040-1708480
更新日期:2020-04-01 00:00:00
abstract::It is known that the fibrinolysis inhibitors in the human placenta are proteins which, in vitro, block urokinase plasminogen activation and are for this reason called urokinase inhibitors. In the present study it was found, for the first time, that a slow-molecular urokinase inhibitor also occurs in the human placenta...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1980-02-29 00:00:00
abstract::Tissue factor pathway inhibitor (TFPI) plays a crucial role in haemostasis by regulating TF-induced initiation of coagulation. Since it is expressed by endothelial and trophoblastic cells, TFPI is of particular importance at the placental level and might be involved in the occurrence of gestational vascular complicati...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1160/TH10-06-0399
更新日期:2011-01-01 00:00:00
abstract::Catheter-directed thrombolysis has gained increasing acceptance for the treatment of patients who present with vascular occlusion; however, intravenous injection may be preferable in selected patients. Recombinant prourokinase (r-proUK) is a recently-developed fibrin-selective thrombolytic agent with specificity for c...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1999-02-01 00:00:00
abstract::Probands from 15 unrelated families with hereditary protein S deficiency type I, that is having a plasma total protein S concentration fifty percent of normal, were screened for abnormalities in their protein S genes by Southern analysis. Two probands were found to have a deviating DNA pattern with the restriction enz...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1989-11-24 00:00:00
abstract::The effects of argatroban, a direct thrombin inhibitor, on the International Normalized Ratio (INR), activated partial thromboplastin time (aPTT) and functional factor X during warfarin co-administration were established to provide means to interpret INRs during argatroban/warfarin co-therapy. Twenty-four subjects rec...
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:2001-03-01 00:00:00
abstract::Given conflicting data on temporal variability in pharmacodynamic platelet responses to clopidogrel, we investigated platelet reactivity on clopidogrel and aspirin for up to six months after elective percutaneous coronary intervention (PCI) with drug-eluting stents. Platelet reactivity was determined in 102 patients b...
journal_title:Thrombosis and haemostasis
pub_type: 临床试验,杂志文章
doi:10.1160/TH15-03-0257
更新日期:2015-11-01 00:00:00
abstract::The aim of the present study was to evaluate the relative performance of five screening methods for APC resistance caused by the factor V:Q506 mutation: the original method Coatest APC Resistance Chromogenix, a modified method using the same reagents but a predilution 1+4 of the plasma in a factor V deficient plasma f...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1997-11-01 00:00:00
abstract::Heparin-induced thrombocytopenia (HIT) is an immune-mediated adverse drug reaction that occurs following exposure to unfractionated heparin (UFH) or low-molecular-weight heparin (LMWH). HIT with thrombosis (HITT) can cause devastating venous thromboembolism or arterial clots, prolonged hospitalization, and increased c...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:2008-12-01 00:00:00
abstract::The biologically active components from Viperidae venoms specifically affect cell-matrix interactions, and have been utilized for developing anti-adhesive therapy as anti-thrombotic and anti-angiogenic agents. Utilizing platelet aggregometry coupled with flow cytometry, we found that a metalloproteinase isolated from ...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1160/TH03-07-0426
更新日期:2004-02-01 00:00:00
abstract::We tested the response of the fibrinolytic activity and factor VIII-antigen levels to infusion of DDAVP in healthy volunteers and we studied the influence of propranolol and aspirin on this response. After DDAVP, 0.4 microgram/kg in 10 min i.v., the fibrinolytic activity of redissolved euglobulins rose from 179 to 452...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1984-02-28 00:00:00
abstract::Recent investigations have revealed the crucial role of von Willebrand factor (vWF) in platelet thrombus formation under flow conditions. The plasma concentrations of vWF were measured together with various hemodynamic and hemostatic parameters in 51 cases of acute myocardial infarction. In 10 randomly selected cases,...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:2000-08-01 00:00:00
abstract::Macrophage migration inhibitory factor (MIF) is a pleiotropic cytokine with chemokine-like functions. MIF is a critical mediator of the host immune and inflammatory response. Dysregulated MIF expression has been demonstrated to contribute to various acute and chronic inflammatory conditions as well as cancer developme...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章,评审
doi:10.1160/TH12-11-0831
更新日期:2013-03-01 00:00:00
abstract::Activated protein C (APC) is a serine proteinase that regulates blood coagulation. In plasma it is inhibited mainly by the protein C inhibitor (PCI). The plasma concentrations of APC-PCI complex is increased in hypercoagulative states such as deep venous thrombosis. Formation of the APC-PCI complex induces a drastic c...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:2001-08-01 00:00:00
abstract::3-phosphoinositide-dependent protein kinase 1 (PDK1), a member of the protein A,G and C (AGC) family of proteins, is a Ser/Thr protein kinase that can phosphorylate and activate other protein kinases from the AGC family, including Akt at Thr308, all of which play important roles in mediating cellular responses. The fu...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1160/TH13-06-0484
更新日期:2014-03-03 00:00:00
abstract::Increased red blood cell distribution width (RDW), which is a marker of anisocytosis, is associated with mortality and cardiovascular events in the general population and in patients with heart failure or coronary heart disease. We investigated whether RDW in acute cerebral infarction is predictive of functional outco...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1160/TH12-03-0165
更新日期:2012-08-01 00:00:00
abstract::Desmopressin acetate administration markedly stimulates release of tissue plasminogen activator (t-PA) from vascular endothelial cells. The mechanism for this effect is unknown. Because infusion of epinephrine has been shown to increase t-PA levels, we examined the role of endogenous catecholamine mediation of t-PA re...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1988-04-08 00:00:00
abstract::Human endothelial cells isolated from umbilical cords were solubilized in Triton X-100 and examined by crossed immunoelectrophoresis using rabbit antiserum against endothelial cells. Endogenous labelling of the endothelial cell proteins with 14C-mannose followed by crossed immunoelectrophoresis and autoradiography rev...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1990-04-12 00:00:00
abstract::Hereditary defects of the von Willebrand factor (VWF) gene cause von Willebrand's disease (VWD) which shows great variability dependent on the nature and location of the mutation. We here describe the characteristics of a substitution of methionine for threonine 1156 in the D3 domain of the VWF, i.e. the domain involv...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:2002-09-01 00:00:00
abstract::In wound repair, fibrin has a multiplicity of activities, some of which are intrinsic to the protein itself and some attributable to other blood constituents associated with the fibrin clot. Fibrin sealants, which have been approved for hemostasis in the US and Europe, are occasionally used wounds to promote healing. ...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章,评审
doi:10.1160/TH03-08-0526
更新日期:2003-12-01 00:00:00
abstract:BACKGROUND: A strong association between on-thienopyridine platelet reactivity (PR) and the risk of both thrombotic and bleeding events in acute coronary syndrome (ACS) patients undergoing percutaneous coronary intervention (PCI) has been demonstrated. However, no study has analyzed the relationship between on-ticagrel...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1055/a-1326-5110
更新日期:2020-12-01 00:00:00
abstract::Elevated plasma fibrinogen is a risk factor for cardiovascular disease (CVD), but associations between fibrinogen single nucleotide polymorphisms (SNPs) and disease risk are inconsistent. We investigated whether common (> or = 5% minor allele frequency) variation in the fibrinogen genes (FGA, FGB, FGG) is associated w...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章,多中心研究
doi:10.1160/TH07-08-0523
更新日期:2008-02-01 00:00:00
abstract::Many studies showed that the occurrence of cardiovascular and cerebrovascular events exhibits a seasonal and monthly variation. Evidences of a seasonal and monthly variation in the incidence of venous thromboembolism (VTE) are more conflicting. We conducted a systematic review and a meta-analysis of the literature to ...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章,meta分析,评审
doi:10.1160/TH11-02-0116
更新日期:2011-09-01 00:00:00
abstract::Antithrombotic potency of recombinant hirudins rHV2, rHV2-Lys47 and rHV2-Arg47 was studied in a model of experimental thrombosis induced by tissue factor in the rat. Venous thrombosis was induced by i.v. injection of 25 mg/kg tissue factor followed by stasis of the inferior vena cava. In this model natural recombinant...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1990-04-12 00:00:00