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 treatment has been achieved by frequent infusions of plasma or prothrombin complex then maintained with Warfarin. Homozygous recessive protein C deficiency usually presents in the neonatal period with purpura fulminans. Two cases have been described elsewhere which presented in the second decade of life with milder symptoms. The present cases appear to be intermediate in time of presentation and severity of symptoms. We also review the distinction that is now evident between recessive and dominant protein C deficiency.
journal_name
Thromb Resjournal_title
Thrombosis researchauthors
Tuddenham EG,Takase T,Thomas AE,Awidi AS,Madanat FF,Abu Hajir MM,Kernoff PB,Hoffbrand AVdoi
10.1016/0049-3848(89)90202-8subject
Has Abstractpub_date
1989-03-01 00:00:00pages
475-84issue
5eissn
0049-3848issn
1879-2472journal_volume
53pub_type
杂志文章,评审abstract：:The effect of activated protein C (APC) on agonist-induced platelet activation and on thrombin generation after intrinsic (IA) and extrinsic (EA) activation of the coagulation system was studied by flow cytometry and by measuring levels of prothrombin fragment F1+2. In platelet activation studies blood drawn from heal...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/s0049-3848(97)00119-9
更新日期：1997-07-15 00:00:00
abstract：:Two anti-cardiolipin antibody (ACA)-producing cell lines were established, using Epstein-Barr virus transformation followed by a repeated cluster-picking from the same individual with antiphospholipid syndrome who had a history of 8 consecutive fetal losses and deep venous thrombosis. Characterization of the two ACAs ...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(94)90037-x
更新日期：1994-04-01 00:00:00
abstract：INTRODUCTION:D-dimer, a fibrin degradation product, is related to risk of cardiovascular disease and venous thromboembolism. Genetic determinants of D-dimer are not well characterized; notably, few data have been reported for African American (AA), Asian, and Hispanic populations. MATERIALS AND METHODS:We conducted a ...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2014.05.018
更新日期：2014-08-01 00:00:00
abstract：INTRODUCTION:Colorectal cancer (CRC) is often complicated by thromboembolic episodes. It has been recognized that blood coagulation proteins play a role in cancer progression. An important inhibitory mechanism is provided by the protein C (PC) system consisting of PC, protein S (PS) and thrombomodulin (TM). Recently, n...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2009.09.011
更新日期：2010-03-01 00:00:00
abstract：:Platelet activation plays an important role in the development of sepsis. During sepsis, platelet activation leads to endothelial cell injury and promotes neutrophil extracellular trap and microthrombus formation, exacerbating septic coagulation and inflammatory reactions. The resultant induction or aggravation of dis...
journal_title：Thrombosis research
pub_type： 杂志文章,评审
doi：10.1016/j.thromres.2018.04.007
更新日期：2018-06-01 00:00:00
abstract：IMPORTANCE:Unprovoked venous thromboembolism (VTE) can be the first manifestation of cancer. It is unclear if extensive screening for occult cancer including a comprehensive computed tomography (CT) scan of the abdomen/pelvis is cost-effective in this patient population. OBJECTIVE:To assess the health care related cos...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2017.01.007
更新日期：2017-03-01 00:00:00
abstract：:Patients with Familial Hyperlipoproteinaemia Type II showed evidence of increased platelet aggregability when compared to normal controls. This abnormality was not influenced by oral therapy with probucol whether or not the serum cholesterol levels were lowered during the study period. Probucol assays indicated adequa...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(82)90018-4
更新日期：1982-04-15 00:00:00
abstract：:RS-5186, sodium 6-[2-[1-(1H)-imidazolyl]methyl-4,5-dihydrobenzo[b]thiophene]- carboxylate, inhibited platelet thromboxane A2 (TXA2) synthetase with IC50 values of 6 nM and 13 nM for human and rabbit microsomes, respectively. It had a selectivity for TXA2 synthetase 10(5)-fold greater than that for cyclooxygenase, PGI2...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(88)90116-8
更新日期：1988-09-01 00:00:00
abstract：INTRODUCTION:The activated partial thromboplastin time (APTT) is commonly used to monitor unfractionated heparin (UFH) but may not accurately measure the amount of heparin present. The anti-Xa assay is less susceptible to confounding factors and may be a better assay for this purpose. MATERIALS AND METHODS:The validit...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2017.07.006
更新日期：2017-09-01 00:00:00
abstract：BACKGROUND:Anticoagulated patients are often seen unnecessarily in the emergency department (ED) for epistaxis, leading to increased healthcare costs. Patients are often unaware of preventative and management techniques for handling epistaxis in the home. METHODS:In 2016, the Michigan Anticoagulation Quality Improveme...
journal_title：Thrombosis research
pub_type： 信件
doi：10.1016/j.thromres.2021.01.007
更新日期：2021-01-16 00:00:00
abstract：:Plasma from patients with thrombotic thrombocytopenic purpura (TTP) caused the aggregation of washed human platelets, which was inhibited by preincubation with normal plasma. Using salt fractionation, ion exchange chromatography, and preparative agarose gel electrophoresis, we purified a protein from normal plasma whi...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(84)90155-5
更新日期：1984-01-01 00:00:00
abstract：BACKGROUND:Multiple sclerosis (MS) often causes progressive loss of mobility, leading to limb paralysis. Venous and lymphatic stasis is a risk condition for venous thromboembolism (VTE). There is, however, no data on the frequency of VTE complicating the progression of MS. The aim of this study was to assess the freque...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2009.06.023
更新日期：2010-04-01 00:00:00
abstract：:Anticoagulants used for platelet function studies in vitro may affect platelet responsiveness. In the present study we compared the influence of three different anticoagulants, sodium citrate, low molecular mass heparin, and recombinant hirudin, on platelet aggregation in whole blood in vitro, using impedance aggregom...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/s0049-3848(97)00114-x
更新日期：1997-07-01 00:00:00
abstract：:Four murine monoclonal antibodies against rat platelets were produced by fusion of spleen cells from mice intravenously immunized with whole rat platelets. All four antibodies immunoprecipitated two major platelet membrane proteins with apparent molecular weights of 130,000 and 82,000 (nonreduced) and of 120,000 and 9...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(90)90118-v
更新日期：1990-09-15 00:00:00
abstract：:The turnover of tissue plasminogen activator (tPA) was studied in rabbits using a double-label technique which allowed the comparison of various tPA derivatives with a standard tPA in individual animals. Purified recombinant tPA (Alteplase) was labelled with 125I and used as a standard for each experiment. Various tPA...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(88)90278-2
更新日期：1988-07-01 00:00:00
abstract：INTRODUCTION:Endothelium-derived nitric oxide (NO) is synthesized from l-arginine by endothelial nitric oxide synthase (eNOS) encoded by the eNOS3 gene on chromosome 7. The effects of the eNOS polymorphisms with the risk of coronary artery disease are conflicting. In this study, we investigated the association of the e...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2006.06.005
更新日期：2007-01-01 00:00:00
abstract：:Although significant advances in hemophilia treatment have improved patient outcomes and quality of life, one of the greatest complications in severe hemophilia A is the development of anti-Factor VIII (FVIII) antibodies that inhibit FVIII activity in almost 30% of previously untreated patients (PUPs). Inhibitors make...
journal_title：Thrombosis research
pub_type： 杂志文章,评审
doi：10.1016/j.thromres.2018.05.027
更新日期：2018-08-01 00:00:00
abstract：:PAF (1 ug/kg) injected intravenously (i.v.) into anesthetized rabbits resulted in marked loss of circulating platelets and leukocytes. Administration of FR-900452 1-methyl-3-(1-(5-methylthiomethyl-6-oxo-3-(2-oxo-3-cyclopenten-1-y lidene)- 2-piperazinyl) ethyl)-2-indolinone, a specific PAF inhibitor, at a dose of 10 mg...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(86)90344-0
更新日期：1986-06-01 00:00:00
abstract：:Heparin-induced thrombocytopenia (HIT) is a transient hypercoagulability state initiated, paradoxically, by the anticoagulant, heparin. It is characterized by antibody-induced activation of platelets, leading to thrombin generation. Many patients with HIT develop thrombosis; even when heparin is stopped because of "is...
journal_title：Thrombosis research
pub_type： 杂志文章,评审
doi：10.1016/s0049-3848(03)00336-0
更新日期：2003-05-01 00:00:00
abstract：:Beta-thromboglobulin (beta-TG) is a platelet-specific protein present in the alpha-granules and secreted into the surrounding medium on cell activation. The sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE) of platelet releasate after inhibition of metalloproteinases with ethyleneglycol-bis-(beta-a...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/s0049-3848(02)00135-4
更新日期：2002-05-15 00:00:00
abstract：INTRODUCTION:Previous studies have suggested the used of off-label recombinant factor VII (rFVIIa) increases the risk of thromboembolic events, but the effect of the dose of rFVIIa is not well described in the literature. MATERIALS AND METHODS:All adult patients that received off-label rFVIIa from 2005-2012 were inclu...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2014.02.026
更新日期：2014-05-01 00:00:00
abstract：INTRODUCTION:We examined a 6-month-old girl with inherited fibrinogen abnormality and no history of bleeding or thrombosis. Routine coagulation screening tests showed a markedly low level of plasma fibrinogen determined by functional measurement and also a low level by antigenic measurement (functional/antigenic ratio=...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2014.06.002
更新日期：2014-08-01 00:00:00
abstract：INTRODUCTION:To evaluate the pathogenetic role of tissue factor (TF), tissue factor pathway inhibitor (TFPI), and neutrophil elastase in acute respiratory distress syndrome (ARDS), as well as to test the hypothesis that TFPI levels modified by neutrophil activation are not sufficient to prevent TF-dependent intravascul...
journal_title：Thrombosis research
pub_type： 临床试验,杂志文章
doi：10.1016/s0049-3848(03)00151-8
更新日期：2003-01-25 00:00:00
abstract：AIM:Postprandial platelet activation was related to postprandial insulin rather than glucose levels in a previous meal insulin study in type 2 diabetes mellitus (T2DM). We therefore compared postprandial platelet activation in type 1 (T1DM) patients without insulin secretion and T2DM patients with high postprandial ins...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2016.03.009
更新日期：2016-05-01 00:00:00
abstract：INTRODUCTION:Although accumulating evidence within the past 5 years strongly supports the importance of platelet P2X(1) receptors in hemostasis and thrombosis, P2X(1) receptors of platelet and/or its progenitor cell, megakaryocyte, have not been fully characterized. The aim of this study was to electrophysiologically a...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/j.thromres.2006.03.007
更新日期：2007-01-01 00:00:00
abstract：:Severe bleeding took place in a patient when abdominal aneurysm was removed by operation. Bleeding continued after infusion of heparin, antithrombin III (ATIII), fresh platelets and fresh blood. Infusion of tranexamic acid resulted in an immediate cessation of bleeding and improvement of his general condition. Sometim...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(90)90167-b
更新日期：1990-04-15 00:00:00
abstract：:We investigated the alteration of heparan sulfate proteoglycans induced by thrombin in cultured vascular endothelial cells. Heparan sulfate proteoglycans, which were metabolically labeled with [3H] glucosamine and [35S] sulfate, were isolated by DEAE-Sephacel ion-exchange chromatography and characterized by molecular ...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/s0049-3848(97)00257-0
更新日期：1997-11-01 00:00:00
abstract：:The aim of this study was to compare the efficacy and safety of prolonged (35 days) thromboprophylaxis with a standard length (7 days) regimen of a low molecular weight heparin in patients undergoing total hip arthroplasty. The study was multicentre, randomised, double-blind, and prospective with two groups. Following...
journal_title：Thrombosis research
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1016/s0049-3848(98)00018-8
更新日期：1998-03-15 00:00:00
abstract：:A new type of assay for heparins has been devised, in which the test material competes with 125I-labelled heparin for binding to protamine-Sepharose. The assay is very sensitive and will measure heparin concentrations down to 10 ng ml-1. It responds to both the degree of sulphation and the molecular weight of acidic p...
journal_title：Thrombosis research
pub_type： 杂志文章
doi：10.1016/0049-3848(82)90056-1
更新日期：1982-08-15 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
