Abstract：
:Homocystinuria due to cystathionine-beta-synthase deficiency (CBS-def-HOCY) initially often presents with vascular disorders, e.g. thromboembolic events. The measurement of vascular endothelial markers in plasma could help to assess endothelial damage. We determined von Willebrand factor (measured as Ristocetincofactor, RiCoF) and thrombomodulin (TM), two endothelial cell markers to our knowledge not measured systematically before in homocystinuria patients in a longitudinal study of two homocystinuric patients: Patient1 with thromboembolic disease and his asymptomatic sister, patient2. Before start of therapy in patient 1, TM and RiCoF levels both were increased. In patient 2 a moderately elevated RiCoF and a normal level of TM were found. Vitamin therapy with 15 mg folate and 600 mg pyridoxine per day led to almost complete normalization of amino acids in urine and plasma, and complete normalization of RiCoF and TM levels in both patients. Thus, TM and RiCoF elevations demonstrate that CBS-def-HOCY leads to endothelial cell damage, which resolved under vitamin therapy in the patients studied.
journal_name
Thromb Resjournal_title
Thrombosis researchauthors
Schienle HW,Seitz R,Nawroth P,Rohner I,Lerch L,Krumpholz B,Krauss G,Fowler B,Baumgartner R,Willenbockel Udoi
10.1016/0049-3848(95)90867-fsubject
Has Abstract,Author List Incompletepub_date
1995-01-01 00:00:00pages
79-86issue
1eissn
0049-3848issn
1879-2472pii
004938489590867Fjournal_volume
77pub_type
杂志文章abstract：:Megakaryocytes (MKs) are widely known as the progenitor cells of platelets. These large, polyploid cells are a derivative of the hematopoietic stem cell (HSC), and reside in the bone marrow, lining blood vessel walls where they release their platelet progeny into circulation. Although little is known about how MKs dif...
journal_title：Thrombosis research
pub_type： 杂志文章,评审
doi：10.1016/j.thromres.2019.05.008
更新日期：2019-07-01 00:00:00
abstract：:Cardiovascular diseases are one of the leading causes of morbidity and mortality in industrialized countries, and although many processes play a role in the development of vascular disease, thrombosis is the primary event that precipitates stroke and acute coronary syndromes. The blood platelets are of significant imp...
journal_title：Thrombosis research
pub_type： 杂志文章,评审
doi：10.1016/j.thromres.2011.09.026
更新日期：2012-02-01 00:00:00
abstract：INTRODUCTION:Patients with multiple myeloma (MM) have an increased risk of thromboembolic events (TEE). Due to the complex nature of the prothrombotic state in MM, no single laboratory test has been designed to identify patients with the highest risk of developing TEE. Hence, this study is intended to assess the feasib...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2014.01.011
更新日期：2014-04-01 00:00:00
abstract：:Prothrombin time is a standard screening test to monitor patients treated with oral anticoagulants. We hypothesize that INR measurements will not vary a lot within a given day in a given patient. The present study was thus conducted to compare the change of the international normalized ratio (INR) of prothrombin time ...
journal_title：Thrombosis research
pub_type： 临床试验,杂志文章
doi：10.1016/s0049-3848(02)00339-0
更新日期：2002-09-01 00:00:00
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：:The time course and degree of collagen induced platelet shape change, aggregation and ATP-secretion in citrated human platelet rich plasma was monitored with a lumi-aggregometer. Onset of ATP-secretion was measurable within seconds after collagen challenge and always distinctly before onset of shape change. In absence...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(86)90089-7
更新日期：1986-08-15 00:00:00
abstract：BACKGROUND:Direct oral anticoagulants (DOACs) are high risk medications with short half-lives making adherence vitally important. Global measures for adherence have been described; however, there is a lack of patient-level data on adherence. METHODS:This prospective, single-center study in an interdisciplinary interna...
journal_title：Thrombosis research
pub_type： 信件
doi：10.1016/j.thromres.2019.04.021
更新日期：2019-07-01 00:00:00
abstract：:We studied a Japanese family with type I antithrombin (AT) deficiency and identified a novel in-frame deletion mutation (-ATG at nucleotide position of 2771-2773) in the AT gene, which predicted loss of a methionine (Met) at amino acid number of 103. In addition, we found a single base replacement of G to A at nucleot...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2004.12.004
更新日期：2005-01-01 00:00:00
abstract：INTRODUCTION:Adenosine diphosphate (ADP) as physiological activator of human platelets mediates its effects via three purinergic receptors: P2Y1, P2Y12 and P2X1. The inhibition of P2Y12 is used pharmacologically to suppress aggregation underlining the physiological significance of this receptor. Since the regulation of...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2018.05.029
更新日期：2018-08-01 00:00:00
abstract：:Fucoidan [sulfated poly (L-fucopyranose)] was compared with 6-aminohexanoic acid (6-AH) or CNBr-cleaved fibrinogen (CNBr-Fbg) alone or in combination in enhancing the activation of glutamic plasminogen (Glu-Plg) or lysine plasminogen (Lys-Plg) by two-chain tissue plasminogen activator (t-PA) or LMwt-urokinase or by st...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(95)00111-4
更新日期：1995-08-01 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：BACKGROUND:Serious bleeding is a frequent and feared treatment complication in patients treated with oral anticoagulants (OACs). Levels of von Willebrand factor (VWF) antigen have been linked to the risk of bleeding complications, mortality, and cardiovascular events. OBJECTIVES:In this longitudinal cohort study of ev...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2015.08.016
更新日期：2015-11-01 00:00:00
abstract：INTRODUCTION:Type 2 diabetes mellitus (T2DM) patients have a variable response profile to the P2Y(12) receptor antagonist clopidogrel. P2Y(12) receptor signalling promotes platelet procoagulant activity. The aim of this study was to determine if T2DM patients with suboptimal clopidogrel response have greater platelet p...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2008.10.001
更新日期：2009-07-01 00:00:00
abstract：:S-antigen (also named arrestin or 48K protein) is a protein abundant in photoreceptor cells of vertebrates and invertebrates. The presently known function of this protein in retina is to arrest the enzymatic cascade of phototransduction in retinal rods, through its binding to photoactivated and phosphorylated rhodopsi...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：
更新日期：1991-12-01 00:00:00
abstract：:The effects of extracellular sodium on platelet aggregation and calcium mobilization in platelets stimulated with either rattlesnake (Crotalus atrox) lectin (RSL) or alpha-thrombin were compared. The absence of extracellular sodium had no effect on platelet aggregation or calcium mobilization in response to all levels...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(91)90008-k
更新日期：1991-11-01 00:00:00
abstract：:The thrombin inhibitory role of antithrombin III (ATIII) and heparin cofactor II (HCII) was studied in vitro using intact and injured rabbit aortae. When intact vessels were loaded with thrombin and then exposed to either heat defibrinogenated human plasma (HDHP) or ATIII the same degree of thrombin inhibition was ach...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(91)90014-n
更新日期：1991-06-01 00:00:00
abstract：:A significantly higher platelet malondialdehyde-like material (MDA--LM) content after physical and N-ethylmaleimide (NEM) stimulation is found in chronic ischemic heart disease (CHD) patients when compared to the control group. In subjects under aspirin treatment "in vivo" (1 gr/day) no difference is found between CHD...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(82)90305-x
更新日期：1982-09-01 00:00:00
abstract：:Initiation of clotting cascade by tissue factor (TF): Factor VII (FVII) complex is essential for hemostasis however pathologic expression of TF leads to thrombosis. In contrast, the contact pathway factor XII, and to the smaller extent FXI are dispensable for normal hemostasis but still contribute to pathologic thromb...
journal_title：Thrombosis research
pub_type：
doi：10.1016/j.thromres.2014.03.001
更新日期：2014-05-01 00:00:00
abstract：:The inability of the heparin-antithrombin complex to inhibit fibrin-bound thrombin limits the utility of heparin for treatment of arterial thrombosis. In contrast with heparin, melagatran, a direct thrombin inhibitor (DTI), is equally effective at inhibiting fluid-phase thrombin and thrombin bound to fibrin. This refl...
journal_title：Thrombosis research
pub_type： 杂志文章,评审
doi：10.1016/s0049-3848(03)00251-2
更新日期：2003-07-15 00:00:00
abstract：:In a prospective clinical trial the risk of infection after application of virus inactivated antithrombin III concentrate ANTITHROMBIN III IMMUNO (AT III) was investigated in patients undergoing cardiovascular surgery. The study was conducted according to the recommendations of the International Committee on Thrombosi...
journal_title：Thrombosis research
pub_type： 临床试验,杂志文章
doi：10.1016/0049-3848(92)90107-l
更新日期：1992-03-15 00:00:00
abstract：INTRODUCTION:Whether clinical prediction rules for pulmonary embolism are accepted and used among general internal medicine residents remains uncertain. We therefore evaluated the frequency of use and acceptability of the Revised Geneva Score (RGS) and the Pulmonary Embolism Severity Index (PESI), and explored which fa...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2017.09.028
更新日期：2017-12-01 00:00:00
abstract：:An in vivo rabbit model of venous thrombosis which includes physiological blood flow was used to compare the efficacy of the potent and specific factor Xa inhibitor recombinant tick anticoagulant peptide (rTAP) with standard heparin in the prevention of venous thrombus formation. In anesthetized rabbits, an autologous...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(93)90200-8
更新日期：1993-08-15 00:00:00
abstract：:The effect of suloctidil (600 mg/day) on platelet survival time (PST) and plasma and urine betathromboglobulin (BTG) was studied in a double-blind, placebo-controlled six-week crossover trial in 13 patients with shortened PST (less than 110 hrs, exponential model). Mean PST after suloctidil (110.6 hrs) was significant...
journal_title：Thrombosis research
pub_type： 临床试验,杂志文章
doi：10.1016/0049-3848(84)90231-7
更新日期：1984-08-15 00:00:00
abstract：:A B S T R A C T Thrombotic events are a major cause of morbidity and mortality in cancer. While the association of venous thromboembolic events with cancer is well documented, in recent years arterial events (i.e. acute myocardial infarction and ischemic strokes) have also emerged as relatively common complications am...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/S0049-3848(20)30398-4
更新日期：2020-07-01 00:00:00
abstract：:Recombinant forms of human antithrombin (AT) were expressed in COS-1 cells, and their interaction with human thrombin characterized by comparing the reactivity of two engineered mutant forms of AT with the wild-type recombinant. Both mutant forms contained single amino acid substitutions of Asp (G392D) or Pro (G392P) ...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(94)90240-2
更新日期：1994-08-01 00:00:00
abstract：:Danazol, a synthetic androgen reported to increase factor VIII and IX activity levels, was given to 6 hemophiliacs. With danazol therapy (600 mg/da) the APTTs shortened by 30-45% of pre-treatment times. However, the activity levels of the deficient factors did not increase significantly nor consistently with the APTT ...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(85)90126-4
更新日期：1985-07-01 00:00:00
abstract：:A semi-synthetic 1-0-alkyl-2-acetyl-sn-glycero-3-phosphocholine (alkylacetyl-GPC) was shown to be highly species selective in its capacity to cause platelet aggregation and serotonin release. No effects were elicited on the rat or mouse platelets while platelets from human, dog, cat, rabbit, guinea pig and horse were ...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(82)90234-1
更新日期：1982-02-15 00:00:00
abstract：INTRODUCTION:A paradox seems to exist: exercising leads to clotting activation in conventional clotting tests, but exercising persons have a low risk of thrombosis. In this study we tried to evaluate the effect of exercise performance status on in vitro plasma thrombin generation, which represents an overall function t...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2013.04.009
更新日期：2013-06-01 00:00:00
abstract：INTRODUCTION:The first aim of this study is to evaluate the biological effect of doubling the maintenance dose of clopidogrel in pre-defined clopidogrel "low responders", compared to the biological effect of the standard dose in "responders". The second aim is to test the influence of the CYP 2C19*2 allele on clopidogr...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2007.06.012
更新日期：2008-01-01 00:00:00
abstract：:The immunoglobulin superfamily member EMMPRIN (CD147) plays an important role in a number of organ systems, including the cardiovascular system. Here we review the contemporary understanding of EMMPRIN and EMMPRIN-associated sequelae in the course of atherosclerosis. A significant body of data documents the pivotal ro...
journal_title：Thrombosis research
pub_type： 杂志文章,评审
doi：10.1016/j.thromres.2013.04.017
更新日期：2013-06-01 00:00:00
