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

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https://www.readbyqxmd.com/read/28731873/health-related-quality-of-life-in-iranian-adult-men-with-severe-hemophilia
#1
Mohammad Faranoush, Ehsan Shahverdi, Raheb Qorbani, Mostafa Moghaddam
: Health-related quality of life (HRQOL) assessment should be part of the regular clinical assessment of persons with hemophilia. This study assessed quality of life of severe hemophilia patients (Type A) in Tehran comprehensive hemophilia care center. This cross-sectional study was done in 2016 and 84 men aged over 20 years with severe hemophilia (Type A) were assessed. All patients have been treated over 10 years at the comprehensive hemophilia care center, Iran. The HRQOL assessment includes the A36 Hemophilia-QOL questionnaire, sociodemographical and clinical characteristics...
July 20, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28731872/bleeding-risk-assessment-in-hemophilia-a-carriers-from-dakar-senegal
#2
Moussa Seck, Blaise F Faye, Abibatou Sall, Diariétou Sy, Sokhna A Touré, Nata Dieng, Youssou B Guéye, Macoura Gadji, Awa O Touré, Cathérine Costa, Dominique Lasne, Chantal Rothschild, Saliou Diop
: Hemophilia A carriers have an abnormal X chromosome with a molecular abnormality of FVIII gene. These carriers, long considered to be free of bleeding risk, could have the same symptoms as mild hemophiliacs. This study aim to assess bleeding risk of hemophilia A carriers monitored at the Clinical Hematology of Dakar. This is a prospective study of a period of 6 months including 22 hemophilia A carriers aged between 8 and 48 years. Hemophilia carriers were recruited using the genealogical tree of hemophiliacs followed in the service...
July 20, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28704210/factor-xiii-deficiency-in-south-of-tunisia
#3
Ines Maaloul, Moez Medhaffer, Nacim Louhichi, Imen Krichen, Sofien Alibi, Sana Kmiha, Hajer Aloulou, Faiza Fakhfakh, Moez Elloumi, Choumous Kallel, Mongia Hachicha
: Factor XIII deficiency is a rare autosomal recessive disorder of hemostasis characterized by a plasmatic factor XIII level less than 1% in homozygote and bleeding as of the youth. The aim of the study is to describe the clinical features and the outcome of the patients and to determine molecular characteristics. A retrospective study, was conducted on seven patients with factor XIII deficiency in the department of hematology and pediatrics, Hedi Chaker Hospital, Sfax, Tunisia during the period of 14 years (2001-2014)...
July 12, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28692431/differing-mechanisms-of-thrombin-generation-in-live-haematological-and-solid-cancer-cells-determined-by-calibrated-automated-thrombography
#4
Mufuliat Adeola Adesanya, Anthony Maraveyas, Leigh Madden
: Calibrated automated thrombography (CAT) is emerging as a reliable tool for real-time estimation of thrombin generation potential. There is a clinical need for knowledge about the pathways underlying the thrombotic phenotype of different malignancies. Cells from solid (e.g. pancreatic cancer; n = 7) and malignant haematological cell lines (e.g. multiple myeloma; n = 5) were evaluated for thrombin generation, using CAT, with the addition of control plasma (NormTrol; Helena Biosciences, Gateshead, UK)) or plasma deficient in coagulation factors VII and XII...
July 7, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28678027/meta-analysis-on-incidence-of-inhibitors-in-patients-with-haemophilia-a-treated-with-recombinant-factor-viii-products
#5
Matteo Rota, Paolo A Cortesi, Katharina N Steinitz-Trost, Armin J Reininger, Alessandro Gringeri, Lorenzo G Mantovani
: Recent cohort studies showed differences in inhibitor incidence in previously untreated patients (PUPs) with haemophilia A treated with recombinant factor VIII (rFVIII) concentrates. We carried out a systematic literature search and meta-analysis for all randomized clinical trials and observational studies published from 1 January 1988 to 31 August 2015, to assess the incidence of inhibitor development and the relationship with rFVIII product used in PUPs and minimally treated patients (MTPs, ≤5 previous exposure days), with severe haemophilia...
July 3, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28665815/bleeding-manifestations-in-heterozygotes-with-prothrombin-deficiency-or-abnormalities-vs-unaffected-family-members-as-observed-during-a-long-follow-up-study
#6
Antonio Girolami, Claudia Santarossa, Elisabetta Cosi, Silvia Ferrari, Anna Maria Lombardi, Bruno Girolami
: To investigate the prevalence of bleeding in heterozygotes for prothrombin deficiencies. Homozygotes or compound heterozygotes with Factor II (FII) levels of less than 10% of normal are always severely symptomatic.On the contrary little is known about the heterozygous population who have FII levels around 40-50% of normal.Forty-four patients heterozygous for this defect, in comparison with age and sex matched 44 unaffected family members, have been followed during a mean observational period of 22.5 years (range 4-35 years)...
June 29, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28661913/soluble-endothelial-cell-molecules-and-circulating-endothelial-cells-in-patients-with-venous-thromboembolism
#7
Cláudia Torres, Rui Matos, Sara Morais, Manuel Campos, Margarida Lima
: To evaluate the plasma levels of soluble endothelial cell molecules in patients with venous thromboembolism (VTE) out of the acute phase as compared with healthy individuals. We also investigated the possible associations of the soluble endothelial cell molecules among them, as well as with other clinical and laboratory data, including the numbers of circulating endothelial cells (CEC), circulating endothelial progenitor cells (CEP), and CEC expressing activation-related [cluster of differentiation (CD)54 and CD62E] and procoagulant (CD142) markers...
June 28, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28654426/use-of-three-procoagulants-in-improving-bleeding-outcomes-in-the-warfarin-patient-with-intracranial-hemorrhage
#8
Emma P DeLoughery, Thomas G DeLoughery
: When patients on anticoagulation present with intracranial bleeding, stopping the bleeding is paramount. Despite the availability of multiple options to reverse anticoagulation, no study has directly compared the effectiveness of the procoagulants recombinant activated factor VII (rFVIIa), the rFVIIa and 3-factor prothrombin complex concentrate (PCC) combination, and 4-factor PCC on improving patient outcomes compared with a control. This study examined the medical records of 197 warfarin patients with intracranial hemorrhage, an initial international normalized ratio (INR) greater than 1...
June 24, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28654425/association-between-gene-polymorphisms-and-clinical-features-in-idiopathic-thrombocytopenic-purpura-patients
#9
Hadi Rezaeeyan, Kaveh Jaseb, Arash Alghasi, Ali Amin Asnafi, Najmaldin Saki
: Immune thrombocytopenic purpura (ITP) is an autoimmune disease in which increased platelet destruction and thrombocytopenia are diagnostic features. In fact, the exact pathogenesis of this disease is still unknown, but genetic changes can be a potential factor in the development of ITP. In this study, the relationship between polymorphisms with platelet destruction has been studied, which leads to decreased platelet count. Relevant literature was identified by a PubMed search (2000-2016) of English language papers using the terms 'ITP', 'polymorphism,' and 'immune system'...
June 24, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28604569/use-of-four-factor-prothrombin-complex-concentrate-in-the-reversal-of-warfarin-induced-and-nonvitamin-k-antagonist-related-coagulopathy
#10
Hannah Young, Jeremy L Holzmacher, Richard Amdur, Stephen Gondek, Babak Sarani, Mary E Schroeder
: To evaluate the efficacy of international normalized ratio (INR) reversal using four-factor prothrombin complex concentrate (4F-PCC) in nonmedication-induced coagulopathy. We performed a single-site, retrospective cohort study of patients receiving off-label use of 4F-PCC. Cohorts included liver dysfunction if they had acute liver decompensation or cirrhosis without other causative factors of liver failure such as sepsis, coagulopathy of acute sepsis (CAS) if they had documentation of sepsis and no underlying liver disorder, known factor deficiencies, or medication-induced coagulopathy, or warfarin if they were taking warfarin...
June 9, 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
#11
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...
June 2, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28562430/transplantation-of-bone-marrow-derived-mesenchymal-stem-cells-into-a-murine-model-of-immune-thrombocytopenia
#12
Yanling Tao, Dongxiao Song, Fanyong Zhang, Saisai Ren, Hao Zhang, Lirong Sun
: Several reports have demonstrated T regulatory cells may play an important role in the pathophysiology of immune thrombocytopenia (ITP). As the immunomodulator, bone-marrow-derived mesenchymal stem cells (MSCs) (BM-MSCs) regulate T regulatory cells and show therapeutic effects on autoimmune diseases. However, it is not clear how BM-MSCs affect ITP. In this study, we explored the specific effects of BM-MSCs on ITP in mice. Using a murine model of ITP, mice were randomly divided into three groups: normal control group, ITP control group and ITP and BM-MSCs group...
May 29, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28548975/factor-vii-activating-protease-sex-related-association-with-coronary-artery-calcification
#13
Ramshanker Ramanathan, Jørgen B Gram, Niels Peter R Sand, Bjarne L Nørgaard, Axel C P Diederichsen, Frank Vitzthum, Herbert Schwarz, Johannes J Sidelmann
: Factor VII-activating protease (FSAP) may regulate development of cardiovascular disease (CVD). We evaluated sex differences in FSAP measures and examined the association between FSAP and coronary artery calcification (CAC) in a middle-aged population. Participants were randomly selected citizens aged 50 or 60 without CVD, diabetes mellitus, Marburg I polymorphism, or hormone replacement therapy (HRT). FSAP protein concentration (total FSAP), FSAP urokinase-activating capacity (FSAP GP), and FSAP GP/total FSAP (specific FSAP activity) were measured...
May 25, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
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
#14
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
#15
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/28448319/tranexamic-acid-reduces-blood-loss-and-transfusion-requirements-in-primary-simultaneous-bilateral-total-knee-arthroplasty-a-meta-analysis-of-randomized-controlled-trials
#16
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/28704253/preeclampsia-in-a-factor-xi-deficient-woman-discussion-about-one-case
#17
Frédéric Bauduer, Catherine Poumier-Chabanier
No abstract text is available yet for this article.
July 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
#18
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...
July 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28151804/pregnancy-loss-in-women-with-von-willebrand-disease-a-single-center-pilot-study
#19
Leslie Skeith, Natalia Rydz, Maeve O'Beirne, Dawn Goodyear, Haocheng Li, Man-Chiu Poon
: The risk of pregnancy loss in von Willebrand disease (VWD) has been inconsistently reported. Von Willebrand factor (VWF) is a known regulator of angiogenesis, so has the potential to affect placental function. We sought to determine the risk of pregnancy loss and placenta-mediated pregnancy complications in women with VWD, compared with women without VWD. Women with VWD followed in the Southern Alberta Rare Blood and Bleeding Disorders Clinic were invited to participate in a questionnaire (February-June 2014)...
July 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28079537/successful-total-hip-replacement-with-sequential-administration-of-bypassing-agents-in-an-adolescent-boy-with-hemophilia-a-and-high-inhibitor-titers
#20
Zühre Kaya, Özlem Orhan, Sacit Turanlı, İdil Yenicesu, Ülker Koçak, Türkiz Gürsel
: There are only a few reports of total hip replacement in patients with hemophilia A and inhibitors. We performed total hip replacement in an 18-year-old adolescent boy who had high inhibitor titers since infancy. Recombinant factor VIIa (NovoSeven) was used as a bypass agent during the surgery. There was no excessive introperative bleeding; however, postsurgical bleeding occurred and was controlled by sequential administration of recombinant factor VIIa and activated prothrombin complex concentrate (FEIBA)...
July 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
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