Abstract:
BACKGROUND:Prospective studies on the incidence of thrombosis in asymptomatic individuals with hereditary protein C- or protein S deficiency have not been performed so far. OBJECTIVE:We have carried out a prospective cohort study in 44 asymptomatic protein C- and protein S deficient subjects and in 49 asymptomatic non-deficient relatives (age at study entry > 14 years) of symptomatic deficient patients. METHODS:20 asymptomatic protein C deficient (median age 20 years) and 24 asymptomatic protein S deficient patients (median age 21.5 years) were prospectively followed and compared with 20 asymptomatic non-deficient relatives (median age 25 years) of protein C- and 29 (median age 27 years) of protein S deficient patients. The total observation period was 118.8 patient years for protein C deficient and 92.8 for protein S deficient patients. Patients were not on anticoagulants except for short duration in case of high risk situations. RESULTS:Eight thromboembolic events (1 pulmonary embolism, 1 deep vein thrombosis + pulmonary embolism, 3 deep vein thrombosis, 1 caval vein thrombosis and 2 superficial vein thrombosis) occurred in 6 deficient patients. The incidence of thromboembolism was 2.5% per patient year for protein C deficient and 3.5% per patient year for protein S deficient patients. 4 events occurred spontaneously, in 2 patients thromboembolic events were triggered by high risk situations (caesarean section, minor trauma). In the controls no thromboembolic events occurred. The probability for thromboembolism was significantly higher in protein C and protein S deficient patients compared to the control group (Wilcoxon test, p = 0.002, log rank test, p = 0.001). One major and 5 minor uneventful surgeries were carried out in the deficient patients using heparin prophylaxis. 1/8 pregnancies was complicated by superficial vein thrombosis during the second trimester despite prophylactic heparin administration. The same pregnancy was complicated by pulmonary embolism 5 weeks after delivery after discontinuation of heparin. CONCLUSIONS:Asymptomatic deficient relatives of symptomatic patients with protein C or protein S deficiency are at an increased risk of thrombosis compared to nondeficient individuals. Prophylactic treatment seems to be highly effective in high risk situations.
journal_name
Thromb Haemostjournal_title
Thrombosis and haemostasisauthors
Pabinger I,Kyrle PA,Heistinger M,Eichinger S,Wittmann E,Lechner Ksubject
Has Abstractpub_date
1994-04-01 00:00:00pages
441-5issue
4eissn
0340-6245issn
2567-689Xjournal_volume
71pub_type
杂志文章abstract::The inhibitory capacity of antithrombin III (AT III) was measured by a quantitative method independent of the velocity of inhibition. When AT III was in excess of thrombin in plasma or in purified system the capacity of inhibitor decreased quantitatively in proportion to the amount of thrombin neutralized. Heparin pre...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1977-08-31 00:00:00
abstract::We measured and compared the levels of plasma soluble (s) P-selectin, sCD40L, platelet-derived microparticles (PDMP), monocyte-derived microparticles (MDMP), and anti-oxidized LDL antibody, to obtain a better understanding of their potential contribution to vascular complications in acute coronary syndrome (ACS). The ...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1160/TH03-04-0247
更新日期:2004-01-01 00:00:00
abstract::The effects of mental stress (a colour word conflict test, CWT) and adrenaline infusions (0.1 and 0.4 nmol kg-1 min-1) on t-PA activity, t-PA antigen, PAI-1 activity and PAI-1 antigen were studied in 18 healthy male volunteers. Furthermore, the effects of metoprolol (200 mg/day during 1 week) or placebo (double-blind ...
journal_title:Thrombosis and haemostasis
pub_type: 临床试验,杂志文章
doi:
更新日期:1990-06-28 00:00:00
abstract::Platelet activation plays a pivotal role in physiological hemostasis and pathological thrombosis causing heart attack and stroke. Previous studies conclude that simultaneous activation of Gi and G12/13 signaling pathways is sufficient to cause platelet aggregation. However, using Gq knockout mice and Gq-specific inhib...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1055/s-0040-1714745
更新日期:2020-11-01 00:00:00
abstract::The fibrinolytic capacity of 121 patients with a history of venous thrombosis and/or pulmonary embolism was studied by venous occlusion technique, at earliest 3 months after the last thromboembolic episode. After discontinuation of oral anticoagulation treatment the clinical course of the patients was followed and new...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1984-10-31 00:00:00
abstract::We previously found that two peptides (N- and Q-peptide) selected by phage display for binding to an anti-vWF antibody, were able to inhibit vWF-binding to collagen (1). The sequence of those peptides could be aligned with the sequence in vWF at position 1129-1136 just outside the A3-domain. As the peptides represent ...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:2000-10-01 00:00:00
abstract::In this study, the clot accumulation properties of liposome-coupled plasminogen were compared to those of free (non-liposomal) plasminogen in an in vitro, closed-loop, flow-system. After introduction of a clot into the closed system, double-radiolabelled plasminogen-liposomes were administered and the accumulation of ...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1998-01-01 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::We studied the role of various markers of lipid metabolism, hemostasis and inflammation in a two year follow-up of 3,000 patients with angina pectoris, during which time 106 patients experienced myocardial infarction or sudden coronary death. Low levels of high density lipoprotein (HDL) cholesterol and of apolipoprote...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章,多中心研究
doi:
更新日期:2000-12-01 00:00:00
abstract::Despite great efforts in stroke research, disability and recurrence rates in ischaemic stroke remain unacceptably high. To address this issue, one potential target for novel therapeutics is the glycoprotein von Willebrand factor (vWF), which increases in thrombogenicity especially under high shear rates as it bridges ...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章,评审
doi:10.1055/s-0038-1648251
更新日期:2018-06-01 00:00:00
abstract::Most of the knowledge acquired on platelet function and biochemistry has been obtained from platelets prepared from blood anticoagulated with sodium citrate. Using washed platelets from human blood (PNB) to which no anticoagulants were added, we report on responses not observed with platelets prepared from citrate-ant...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1986-10-21 00:00:00
abstract::The multimeric plasma glycoprotein von Willebrand factor (VWF) is best known for recruiting platelets to sites of injury during primary hemostasis. Generally, mutations in the VWF gene lead to loss of hemostatic activity and thus the bleeding disorder von Willebrand Disease. By employing cone and platelet aggregometry...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1055/a-1344-4405
更新日期:2020-12-31 00:00:00
abstract::A recent report indicated that an arrow poison used by the native Indians of Rondonia, Brazil, to kill small animals was associated with profuse bleeding. The arrow poison was prepared from the bark of a tree, known locally as Tike-Uba. We have obtained bark and sap specimens from this tree and have characterized a po...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1990-02-19 00:00:00
abstract::A preliminary clinical trial was conducted to determine the feasibility of achieving and regulating therapeutic anticoagulation with heparin given by continuous subcutaneous infusion. Five patients with deep venous thrombosis confirmed by impedance plethysmography and/or venography were studied. All patients received ...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1982-02-26 00:00:00
abstract::Activation and signaling of circulating blood and vessel wall cells, as well as their direct adhesive interactions, are critical for the regulation of vascular homeostasis related to vasomotor responses, inflammation, hemostasis, and wound repair. Under conditions of stress, inflammation, or infection the targeting an...
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::In consecutive patients with suspected venous thromboembolism the interobserver variability of the SimpliRED D-dimer test was evaluated by two observers who independently scored one plate, the between assay variation was performed simultaneously by a third independent observer, who assessed a second plate. The biologi...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1998-01-01 00:00:00
abstract::The effect of shear rate and fibrinogen concentration on adenosine diphosphate-induced aggregation of suspensions of washed human platelets in Poiseuille flow at 23 degrees C was studied using a previously described double infusion technique and resistive particle counter size analysis. Using suspensions of multiple-c...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1994-01-01 00:00:00
abstract::Plasminogen activator inhibitor 1 (PAI-1) is the main inhibitor of tissue-type and urokinase-type plasminogen activators (t/uPA) and plays an important role in fibrinolysis. Inhibition of PAI-1 activity prevents thrombosis and accelerates fibrinolysis, indicating that PAI-1 inhibitors may be used as effective antithro...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1160/TH16-11-0891
更新日期:2017-06-28 00:00:00
abstract::We studied exercise-induced changes in coagulation and fibrinolytic factors and activation products in different age categories. Thirty-eight sedentary males, divided in three age categories (cats I-III; 20-30, 35-45 and 50-60 y) were subjected to a standardized exercise test. Pre-exercise levels (cats I-III resp) of ...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1995-12-01 00:00:00
abstract::Global coagulation assays display variable effects at different concentrations of rivaroxaban. The aim of this study is to quantify the ex vivo effects of low-dose rivaroxaban on thrombophilia screening assays and coagulation factor activities based on the administration time, and to show how to mask possible inter...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1160/TH12-04-0228
更新日期:2013-01-01 00:00:00
abstract::Endothelial cell protein C receptor (EPCR) enhances the generation of activated protein C (APC) by the thrombin-thrombomodulin complex. A soluble form of EPCR (sEPCR), which is generated by metalloprotease activity, is present in plasma. The distribution of sEPCR levels in healthy populations is bimodal. Previously, w...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1160/TH03-10-0657
更新日期:2004-05-01 00:00:00
abstract::In conclusion, we believe that the baboon offers many advantages as an experimental animal to study vascular disease, thrombus formation and dissolution, the effects of mediating variables, and the relative efficacy of therapeutic interventions. Each specific application for testing therapeutic agents may require test...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章,评审
doi:
更新日期:1987-10-28 00:00:00
abstract::In 70-80% of cases, pulmonary embolism is the consequence of lower extremity deep vein thrombosis. It has been demonstrated that the most common coagulation defect predisposing to venous thrombosis, resistance to activated protein C (APC), is not associated with an increased risk for pulmonary embolism, but the eviden...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1997-03-01 00:00:00
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journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1996-12-01 00:00:00
abstract::Background Polycystic ovarian syndrome (PCOS) affects up to 18% of reproductive-aged women with increased risks of cardiovascular disease and venous thromboembolic disease, related to metabolic and hormonal features, obesity and an apparent hypofibrinolytic state, possibly exacerbated by current PCOS treatments. Objec...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1160/TH17-04-0248
更新日期:2017-11-01 00:00:00
abstract::Patterns of VIIIR:AG in the plasma and its fractions, cryoprecipitate and cryosupernatant, from various types of von Willebrand's disease (vWd) were observed by SDS 1.5% polyacrylamide gel electrophoresis - cross immunoelectrophoresis (SDS PAGE - CIE). VIIIR:AG in normal cryoprecipitate showed several precipitin peaks...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:1981-06-30 00:00:00
abstract::Catabolism of coagulation factor VIII (FVIII) is mediated by low-density lipoprotein receptor-related protein (LRP). The ligand-binding sites of LRP are formed by complement-type repeats (CR), and CR clusters II and IV bind most of LRP ligands. FVIII contains two major LRP-binding sites located in the A2 and A3 domain...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:
更新日期:2007-12-01 00:00:00
abstract::Hormone replacement therapy has been associated with venous thromboembolism. Controversy exists regarding the appropriate management of hormone replacement in the perioperative setting and in other situations--such as acute illness--predisposing to acute venous thromboembolism. We performed a case-control study to det...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1160/TH04-03-0165
更新日期:2004-08-01 00:00:00
abstract::Antiphospholipid antibodies (aPL) have been reported to activate platelets. This is considered to be one of the pathogenic properties of aPL. Even though aPL heterogeneity is quite well established, little is known, if the ability to activate platelets is common to all aPL or depends on antigen specificity. To further...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章
doi:10.1055/s-0039-1685453
更新日期:2019-07-01 00:00:00
abstract::Serine proteases are proteolytic enzymes with an active serine residue in their catalytic site. Kallikreins are a subgroup of the serine protease family and are known to have diverse physiological functions. The human tissue kallikrein gene family has now been fully characterized and includes 15 members, clustered in ...
journal_title:Thrombosis and haemostasis
pub_type: 杂志文章,评审
doi:
更新日期:2003-07-01 00:00:00