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Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis

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https://www.readbyqxmd.com/read/28537987/congenital-dysfibrinogenemia-in-a-japanese-family-with-fibrinogen-naples-b%C3%AE-ala68thr-manifesting-as-superior-sagittal-sinus-thrombosis
#1
Satoru Yoshida, Tetsuya Kibe, Risa Matsubara, Shin-Ichiro Koizumi, Kenji Nara, Koji Amano, Nobuo Okumura
: Congenital dysfibrinogenemia refers to the presence of a dysfunctional fibrinogen molecule, typically because of mutations in the fibrinogen gene. About 20% of fibrinogen gene mutations are responsible for thrombosis. Here, we described the case of a 17-year-old Japanese boy, who had a sudden stroke because of superior sagittal sinus thrombosis associated with dysfibrinogenemia. Genetic testing confirmed the presence of homozygous fibrinogen Naples (BβAla68Thr) mutation, which was previously reported as a causative mutation for thrombotic dysfibrinogenemia only in an Italian family...
May 22, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28505011/in-vitro-assessment-of-the-effect-of-dabigatran-on-thrombosis-of-adult-and-neonatal-plasma-comparisons-using-thromboelastography-and-microscopic-visualization-of-fibrin-clot-structure
#2
Fadi F Nossair, Howard H W Chan, Jorell Gantioqui, Helen M Atkinson, Leslie R Berry, Anthony K C Chan
: Thromboelastography (TEG) is a global assay used for evaluating features of clot formation in vitro. Dabigatran is a reversible direct inhibitor of thrombin that has not been studied in neonates using a sophisticated global assay, such as TEG. Neonatal hemostasis differs from adult hemostasis in both quantitative and qualitative characteristics. Our aim was to compare the TEG clotting profile of neonatal and adult platelet-poor plasma when exposed to different concentrations of dabigatran. We used commercially collected adult pooled plasma and neonatal cord blood collected from placentas of healthy full term newborns...
May 12, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28486277/evaluation-of-recombinant-factor-viii-fc-eloctate-activity-by-thromboelastometry-in-a-multicenter-phase-3-clinical-trial-and-correlation-with-bleeding-phenotype
#3
Frank Driessler, Maricel G Miguelino, Glenn F Pierce, Robert T Peters, Jurg M Sommer
: The aim of this study was to compare the hemostatic efficacy of recombinant factor VIII Fc (rFVIIIFc) (Eloctate) and Advate by ex-vivo rotation thromboelastometry (ROTEM) of whole blood and to explore potential ROTEM parameters that may be more predictive of a patient's bleeding tendency than plasma FVIII activity. Thirteen clinical sites were selected to perform ROTEM on freshly collected blood samples from 44 patients in the phase 3 study for rFVIIIFc, including 16 patients undergoing sequential pharmacokinetic assessment of Advate and rFVIIIFc...
May 8, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28448319/tranexamic-acid-reduces-blood-loss-and-transfusion-requirements-in-primary-simultaneous-bilateral-total-knee-arthroplasty-a-meta-analysis-of-randomized-controlled-trials
#4
Yuangang Wu, Timin Yang, Yi Zeng, Haibo Si, Canfeng Li, Bin Shen
: The aim of this meta-analysis is to assess the effectiveness and safety of intravenous application tranexamic acid (TXA) in primary simultaneous bilateral total knee arthroplasty (TKA). We searched electronic databases including PubMed, Embase, the Web of Science, the Cochrane Library and the Google Scholar, for published studies involving the intravenous application TXA in primary simultaneous bilateral TKA. All randomized controlled trials were included. The focus of the meta-analysis was on the outcomes of total blood loss, drainage volume, transfusion requirements and deep venous thrombosis (DVT) and/or pulmonary embolism...
April 26, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28379876/recombinant-von-willebrand-factor-for-severe-gastrointestinal-bleeding-unresponsive-to-other-treatments-in-a-patient-with-type-2a-von-willebrand-disease-a-case-report
#5
Racquel Brown
: A recombinant von Willebrand factor (rVWF) was recently approved in the United States for on-demand treatment and control of bleeding episodes in adults with von Willebrand disease (VWD). In contrast to plasma-derived VWF products available in the United States, rVWF does not contain factor VIII (FVIII). To date, there is no published experience of rVWF in clinical practice. We report the acute and prophylactic use of rVWF in a patient with VWD type 2A and severe gastrointestinal bleeding. Dosing with plasma-derived VWF/FVIII concentrates was constrained by FVIII accumulation; the bleeding was unresponsive, and multiple red blood cell transfusions were required...
April 4, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28379875/comparison-of-two-point-of-care-international-normalized-ratio-devices-and-laboratory-method
#6
Sara R Vazquez, Ryan P Fleming, Stacy A Johnson
: A number of factors contribute to interlaboratory variation of international normalized ratio (INR) results. This variability can lead to differences in clinical decision-making. This prospective cohort study evaluated the accuracy, precision, and clinical decision-making variability of two point-of-care (POC) devices (CoaguChek XS and the Coag-Sense) to a clinical laboratory method of INR measurement as the 'reference' and by using the laboratory's accuracy standard (±25% of the reference INR). Study subjects included adults taking warfarin undergoing a POC INR with each device and a laboratory INR on the same day and evaluated differences in clinical decision-making using a warfarin nomogram...
April 4, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28362649/rapid-and-well-tolerated-action-of-idarucizumab-for-antagonizing-dabigatran-in-a-patient-needing-urgent-thrombolysis-a-case-report
#7
Roberto Facchinetti, Giulia DeGuidi, Federica Pitoni, Giorgio Ricci, Giuseppe Lippi
Dabigatran is a direct oral anticoagulant drug exhibiting clinical benefits over vitamin K antagonists. A procedure for reversing the anticoagulant effect of direct oral anticoagulants may be needed in emergency clinical settings, and is traditionally accomplished by using plasma products or hemostatic physical interventions. Idarucizumab, a specific antidote for dabigatran, has recently become available. This compound can be rapidly administered by intravenous injection and is effective in reversing anticoagulation in few minutes...
March 30, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28362648/abnormal-angiogenesis-in-blood-outgrowth-endothelial-cells-derived-from-von-willebrand-disease-patients
#8
Soundarya N Selvam, Lara J Casey, Mackenzie L Bowman, Lindsey G Hawke, Avery J Longmore, Jeffrey Mewburn, Mark L Ormiston, Stephen L Archer, Donald H Maurice, Paula James
Bleeding associated with angiodysplasia is a common, often intractable complication in patients with von Willebrand disease (VWD). von Willebrand factor (VWF), the protein deficient or defective in VWD, is a negative regulator of angiogenesis, which may explain the pathologic blood vessel growth in VWD. This study explores the normal range of angiogenesis in blood outgrowth endothelial cells (BOECs) derived from healthy donors and compares this to angiogenesis in BOECs from VWD patients of all types and subtypes...
March 30, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28319471/early-secondary-prevention-after-initially-ineffective-revascularization-treatments-for-acute-ischemic-stroke-due-to-tandem-occlusion
#9
Stefano Forlivesi, Elia Pancheri, Giuseppe Moretto, Paolo Bovi, Manuel Cappellari
The most effective treatment of patients with stroke due to tandem occlusion is still unclear. We report the case of a man with stroke due to tandem internal carotid artery/middle cerebral artery occlusion, who underwent initially ineffective intravenous thrombolysis (IVT) and endovascular treatment. Early anticoagulation with apixaban was started after 48 h of IVT, given a newly diagnosed nonvalvular atrial fibrillation. Spontaneous partial recanalization of the cervical internal carotid artery was noted, and carotid endarterectomy was performed 72 h after IVT and 8 h after the last dose of apixaban...
March 17, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28306627/novel-microspheres-reduce-the-formation-of-deep-venous-thrombosis-and-repair-the-vascular-wall-in-a-rat-model
#10
Bingyang Dai, Lan Li, Qiangqiang Li, Xiaoxiao Song, Dongyang Chen, Jin Dai, Yao Yao, Wenjin Yan, Huajian Teng, Fang Yang, Zhihong Xu, Qing Jiang
L-Arginine (L-arg), widely known as a substrate for endogenous nitric oxide synthesis, can improve endothelial function associated with the vasculature, inhibit platelet aggregation, and alter the activity of vascular smooth muscle cells. P-selectin is a membrane component of the platelet alpha-granule and the endothelial cell-specific Wiebel-Palade body that plays a central role in mediating interactions between platelets and both leukocytes and the endothelium. The experiment was designed to evaluate the effect of novel microspheres with L-arg targeting P-selectin on the formation of deep vein thrombosis and repair of vascular wall in a rat model...
March 16, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28306626/pregnancy-and-delivery-in-a-woman-with-severe-haemophilia-a
#11
Jelena N Bodrozic, Predrag S Miljic, Danijela R Lekovic, Milos A Petronijevic, Darko A Antic, Mirjana M Mitrovic, Svetlana M Petronijevic-Vrzic, Irena S Djunic
Severe form of haemophilia in women is an extremely rare condition. Owing to the rarity of the disease there are no precise recommendations concerning the optimal management of pregnancy and delivery in these patients. We are reporting the clinical course and management of a 30-year-old woman with a severe form of haemophilia A (factor VIII <1 IU/dl) during her first pregnancy and delivery. Antepartum, she was treated on demand by FVIII concentrate and she delivered at 37 weeks of gestation by cesarean section...
March 16, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28252515/factor-viii-mutation-spectrum-in-haemophilia-a-patients-in-the-population-of-henan-china
#12
Nan Bai, Xiaofan Zhu, Zhenhua Zhao, Ying Bai, Qinghua Wu, Ning Liu, Duo Chen, Xiangdong Kong
Defects in the coagulation factor VIII gene cause haemophilia A, which is the most common X-linked recessive bleeding disorder. In total, 45 affected families were investigated to elucidate the factor VIII gene mutation spectrum. The families were subjected to clinical, biochemical, and molecular analyses. Inverse-shifting PCR was first applied to severe haemophilia A patients to identify inversions in introns 22 and 1. Then, next-generation sequencing was performed to detect mutations in inversion-negative patients with severe haemophilia A and moderate-mild haemophilia A patients...
March 1, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28177945/concerns-about-andexanet
#13
Claudia Stöllberger, Birke Schneider
No abstract text is available yet for this article.
June 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28151806/goals-defining-therapy-for-primary-immune-thrombocytopenia-in-adults
#14
Lorenzo Cirasino, Stefano Semeraro
: Discordances existing between the two most influential guidelines on the treatment of immune thrombocytopenia, the 2010 International Consensus Report and the 2011 America Society of Hematology guideline, continue to be reflected by the heterogeneity of clinical practice of the physicians who treat this disease. Aimed at overcoming these discordances, we hypothesized that they could be ascribed to nonshared treatment goals. We classify the indications for and goals of the various possible treatments available for adults with primary immune thrombocytopenia according to the line of treatment and the phase of disease...
June 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27676646/evidence-of-both-von-willebrand-factor-deposition-and-factor-v-deposition-onto-al-amyloid-as-the-cause-of-a-severe-bleeding-diathesis
#15
Jonathan S Harrison, Shellaine R Frazier, Diane D McConnell, Senan John Yasar, Nataliya Melnyk, Gratian Salaru
: Acquired coagulopathies are common; uncommonly, adsorption of coagulation factors from the circulation into the tissues by pathologic amyloid exceeds the body's ability to produce factor and results in acquired factor deficiency. When amyloidosis does cause a coagulopathy, it is most often acquired factor X deficiency, but there are rare reports of amyloidosis being associated with other acquired factor deficiencies. We investigated a case of a severe bleeding diathesis, the cause of which was combined acquired factor V deficiency and concomitant acquired von Willebrand syndrome...
June 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27676644/novel-enzyme-immunoassay-system-for-simultaneous-detection-of-six-subclasses-of-antiphospholipid-antibodies-for-differential-diagnosis-of-antiphospholipid-syndrome
#16
Junzo Nojima, Yukari Motoki, Kazusa Hara, Toshiyuki Sakata, Kiyoshi Ichihara
: Antiphospholipid syndrome, which often complicates systemic lupus erythematosus (SLE), features high occurrence of arterial and/or venous thrombosis and recurrent fetal loss. However, which antibody subclass contributes to which clinical event remains uncertain. We newly developed an up-to-date enzyme immunoassay system using the AcuStar automated analyzer (Instrumentation Laboratory, Bedford, Massachusetts, USA) for parallel detection of six subclasses of antiphospholipid antibodies (aPLs): anticardiolipin antibodies (aCL) of IgG, IgM, and IgA and anti-β2-glycoprotein I antibodies (aβ2GPI) of IgG, IgM, and IgA...
June 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27655299/genotyping-analysis-of-the-factor-v-nara-mutation-hong-kong-mutation-and-16-single-nucleotide-polymorphisms-including-the-r2-haplotype-and-the-involvement-of-factor-v-activity-in-patients-with-recurrent-miscarriage
#17
Mari Izuhara, Keiko Shinozawa, Tamao Kitaori, Kinue Katano, Yasuhiko Ozaki, Katsuyuki Fukutake, Mayumi Sugiura-Ogasawara
: Recurrent miscarriage can arise from a large diversity of causes and the factors responsible have not been fully clarified. The coagulation factor V R506Q (Leiden) mutation is a well known risk factor for recurrent miscarriage, although it has not been found in Japanese populations. We examined whether the factor V Nara and Hong Kong mutations, the factor V gene (F5) 16 single-nucleotide polymorphisms (SNPs), including the factor V R2 haplotype, and plasma factor V activity (FV:C) were risk factors for recurrent miscarriage...
June 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27636904/activated-protein-c-resistance-in-patients-following-venous-thromboembolism-receiving-rivaroxaban-versus-vitamin-k-antagonists-assessment-using-russell-viper-venom-time-based-assay
#18
Tadeusz Goralczyk, Katarzyna B Wojtowicz, Anetta Undas
: Activated protein C resistance (APC-R) is assessed as part of thrombophilia screening, preferably in patients not taking oral anticoagulants. Rivaroxaban is known to alter some APC-R assays. To our knowledge, there have been no reports on the effect of rivaroxaban on the Russell viper venom time (RVVT)-based APC-R assay in real-life patients. In 168 consecutive outpatients suspected of having venous thromboembolism because of thrombophilia, APC-R was determined using the RVVT-based ProC Ac R assay (Siemens, Marburg, Germany)...
June 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27607599/temporal-changes-in-clot-lysis-and-clot-stability-following-tranexamic-acid-in-cardiac-surgery
#19
Mariann Tang, Per Wierup, Catherine J Rea, Jørgen Ingerslev, Vibeke E Hjortdal, Benny Sørensen
: Cardiac surgery induces a multifactorial coagulopathy. Regular use of tranexamic acid (TXA) is becoming standard of care. Clinical challenges include selecting optimal dosing regimen and balancing the benefit versus risk of additional dosing with antifibrinolytics. The objective was to evaluate the effect of TXA by assessing kinetic properties of plasma clot formation, clot stability, and clot fibrinolysis. The study was a prospective case follow-up of 28 patients undergoing cardiac surgery (mean age 63.9 years, 29% women)...
June 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27607598/practice-patterns-in-the-diagnosis-of-inherited-platelet-disorders-within-a-single-institution
#20
Juliana Perez Botero, Rajiv K Pruthi, Julie A Majerus, Lea M Coon, Cindy B Uhl, Dong Chen, Mrinal M Patnaik
: The diagnosis of inherited platelet disorders (IPDs) is challenging with variable diagnostic practices existing between institutions. To determine patterns and utility of diagnostic testing practices for IPDs within a single institution, a retrospective cohort study was performed. Records of 50 patients (50% women), median age 32 years (1 day to 81 years) were analyzed. In total, 28 (53%) had a positive International Society of Thrombosis and Hemostasis Bleeding Assessment Tool score. Test-ordering patterns were highly variable...
June 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
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