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

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https://read.qxmd.com/read/30762591/case-report-of-two-siblings-with-type-2a-von-willebrand-disease-involving-a-novel-mutation-within-the-calcium-binding-site-of-the-a2-domain-of-von-willebrand-factor
#1
Henry P Igid, Kyaw Z Thein, Michael Castine, Donald P Quick
: Calcium-binding at the A2 domain protects von Willebrand factor (VWF) from cleavage by a disintegrin and metalloproteinase with thrombospondin motifs (ADAMTS13) and is coordinated by five important residues (p.Asp1596, p.Arg1597, p.Ala1600, p.Asn1602, and p.Asp1498). Only variants of p.Arg1597 resulting in type 2A von Willebrand disease have been reported. We report a novel VWF variant, a heterozygous single nucleotide change, c.4493A>G, occurring at the p.Asp1498 residue of the calcium-binding site of the A2 domain in two sisters with type 2A von Willebrand disease...
February 11, 2019: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30762590/coagulation-factor-xiii-a-and-activated-xiii-a-decrease-in-some-deep-vein-thrombosis-patients-following-catheter-directed-thrombolysis
#2
Woosuk S Hur, Hannah Warner, Lindsay Machan, Christian J Kastrup
: To assess the effect of tissue plasminogen activator administered during catheter-directed thrombolysis (CDT) on coagulation factor XIII (FXIII). Thrombolytic therapy carries significant risks, such as life-threatening bleeds. The mechanisms responsible for major bleeds and intracerebral hemorrhages during thrombolysis are not fully understood. Activated FXIII (FXIII-A) lies at the intersection of coagulation and fibrinolysis. Using purified proteins and blood collected from nine deep vein thrombosis patients undergoing CDT, the stability of FXIII-A and FXIII were measured immediately before, immediately after and 1-day post thrombolysis...
February 11, 2019: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30720490/successful-discontinuation-of-eltrombopag-in-one-child-with-refractory-primary-immune-thrombocytopenia-and-literature-review
#3
Xiaoli Wang, Xiaoyan Liu, Li Wang, Jian-Yong Wang, Aimin Li
: The treatment of refractory primary immune thrombocytopenia (RITP) remains challenging because of the lack of well tolerated and effective drugs. Eltrombopag is approved for pediatric patients, who were aged at least 1 year, having chronic primary immune thrombocytopenia in 2015. Eltrombopag can quickly promote platelets and be conveniently used, thereby providing a new treatment option for patients with immune thrombocytopenia. Some patients may sustain their platelet response when treatment is withdrawn, but the mechanism of this phenomenon is unknown...
January 31, 2019: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30720489/effects-of-a-protective-agent-on-freeze-dried-platelet-rich-plasma
#4
Linying Shi, Rongjuan Li, Shuzhen Wei, Mou Zhou, Lei Li, Fang Lin, Yanhui Li, Zixuan Guo, Wei Zhang, Mingliang Chen, Guiqiu Shan
: Freeze-drying is an effective means of storing platelets. In this study, we investigated the effects of a protective agent on freeze-dried platelet-rich plasma (FD-PRP) after a 12-week preservation period. Platelet structure was measured by transmission electron microscopy (TEM), and the expression levels of procaspase activating compound (PAC)-1 and CD62P were measured by flow cytometry. The levels of transforming growth factor-beta (TGF-β), platelet-derived growth factor (PDGF) and vascular endothelial growth factor (VEGF) were determined by ELISA...
January 31, 2019: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30720488/a-case-of-a-severe-factor-xi-deficiency-in-a-chinese-woman-with-heavy-menorrhagia
#5
Yingyu Wang, Qingyu Wang, Yonggen Zhang, Ping Ma, Hongxiang Ding
: The current study was to elucidate the molecular defect in a 32-year-old Chinese woman with heavy menorrhagia and delayed wound healing. The F11 gene was amplified by PCR and screened for mutations. Then identified mutations were analyzed by in-silico programs and molecular modeling analysis. This woman was found to have severely low levels of factor XI (FXI) (FXI:C: 2.0%; FXI:Ag: 5.4%) by surgical screening. Further DNA sequencing of F11 reveled a novel mutation (p.Ser295Ile) in the Ap4 domain and an already known mutation (p...
January 31, 2019: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30676336/atypical-hemolytic-uremic-syndrome-recurrent-phenotypic-expression-of-a-patient-with-mcp-gene-mutation-combined-with-risk-haplotypes
#6
Sandra Casal Marini, Marília Gomes, Raquel Guilherme, José P Carda, Catarina Silva Pinto, Teresa Fidalgo, Maria Letícia Ribeiro
: We bring the case of a 38-year-old man who was presented to the emergency department with nausea, fever, and choluria, 4 days after the ingestion of raw oysters. Analytical study revealed thrombocytopenia and acute kidney injury that were associated to a possible thrombotic microangiopathy. Therapeutic plasma exchange was started and resolution of the manifestations was obtained. To identify the cause of the thrombotic microangiopathy a molecular study was performed and a pathogenic variant in the MCP gene, c...
January 22, 2019: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30640729/pseudo-thrombocytopenia-after-autologous-stem-cell-transplantation
#7
Alessandra Di Francesco, Annamaria Pasanisi, Ioannis Tsamesidis, Luigi Podda, Claudio Fozza
No abstract text is available yet for this article.
January 11, 2019: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30632992/recurrent-superficial-venous-thrombophlebitis-because-of-mutations-in-the-protein-c-and-fibrinogen-genes-in-a-young-argentinian-female
#8
Hugo A Guglielmone, Luis Bastos, Gustavo D Jarchum, María A Alvarez-Bollea
: Hypodysfibrinogenemia and protein C deficiency are coagulopathies and in this report, we describe a young patient with both defects confirmed by molecular genetic tests. The patient was a 24-year-old woman referred for recurrent thrombophlebitis and finally deep venous thrombosis. Routine coagulation studies revealed mild decrease of protein C (0.49 IU, reference values 0.7-1.40 IU) and hypodysfibrinogenemia (0.88 g/l and 1.83 g/l for activity and antigen, respectively, reference values 2.0-4.0 g/l)...
January 9, 2019: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30585836/inhibitor-development-in-patients-with-congenital-factor-vii-deficiency-a-study-on-50-iranian-patients
#9
Mahmood Shams, Akbar Dorgalaleh, Nader Safarian, Amir Hossein Emami, Farhad Zaker, Shadi Tabibian, Mohammad Reza Managhchi, Mohammad Faranoush, Tahere Tabatabaei, Sedighe Satari, Mohammad Reza Rezvani
: Congenital factor VII (FVII) deficiency is a rare bleeding disorder with an estimated prevalence of 1 per 500 000 in the general population. On-demand replacement therapy is the main therapeutic choice in patients with congenital FVII deficiency. Inhibitor formation against exogenous FVII is very rare and can cause challenges in the management of the disorder. The present study was conducted to assess the prevalence of FVII inhibitor in 50 patients with congenital FVII deficiency under on-demand or prophylaxis treatment by recombinant activated FVII...
December 21, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30585835/what-concentration-of-tranexamic-acid-is-needed-to-inhibit-fibrinolysis-a-systematic-review-of-pharmacodynamics-studies
#10
Roberto Picetti, Haleema Shakur-Still, Robert L Medcalf, Joseph F Standing, Ian Roberts
: Intravenous tranexamic acid (TXA) reduces death because of bleeding in patients with trauma and postpartum haemorrhage. However, in some settings intravenous injection is not feasible. To find different routes of administration, we first need to determine the minimal concentration of TXA in the blood that is required to inhibit fibrinolysis.We conducted a systematic review of in-vitro and in-vivo pharmacodynamics studies. We searched MEDLINE, EMBASE, OviSP, and ISI Web of Science from database inception to November 2017 for all in-vitro (including simulated clotting models) or in-vivo studies reporting the relationship between the TXA concentration in blood or plasma and any reliable measure of fibrinolysis...
December 21, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30507712/a-history-of-prophylaxis-in-haemophilia
#11
Manuel Moreno Moreno, Rubén Cuesta-Barriuso
Prophylaxis entails long-term continuous intravenous administration of concentrates of the deficient factor with a view to preventing spontaneous bleeds and the development of hemophilic arthropathy. Initiation of prophylaxis at an early age and continuous uninterrupted factor administration in patients with hemophilia have been hailed as essential by such organizations.The most widely used prophylaxis regimens include the Swedish (Malmö), the Dutch and the Canadian protocols. Different international groups have hailed prophylaxis as the most effective treatment in patients with hemophilia...
November 29, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30507711/relationship-between-sex-hormone-binding-globulin-and-blood-coagulation-in-women-on-postmenopausal-hormone-treatment
#12
Anette L Eilertsen, Anders E A Dahm, Else Høibraaten, Cathrine M Lofthus, Marie-Christine Mowinckel, Per M Sandset
: Postmenopausal hormone therapy increases the risk of venous thrombosis. Sex hormone binding globulin (SHBG) is a suggested marker of 'total estrogenicity'. The study objective was to evaluate the impact of hormone therapy on SHBG and the association with coagulation variables. The study populations comprised 202 healthy postmenopausal women randomized to treatment with low-dose or conventional-dose hormone therapy, tibolone or raloxifene (RET-study) and 140 women with a history of venous thrombosis randomized to conventional-dose hormone therapy or placebo (EVTET-study)...
November 29, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30507710/low-molecular-weight-heparin-followed-by-rivaroxaban-or-not-for-the-prevention-of-deep-venous-thromboembolism-after-total-knee-arthroplasty
#13
Jian Zhou, Rui Fang, Qian Yan, Chenxi Li, Yanlin Zhou, Ayub Abdulle Nur, Tang Liu, Wanchun Wang
: To observe the effect of LMWH followed by rivaroxaban for the prevention of DVT after TKA. Between June 2015 and November 2017 as well as in accordance with the inclusion criteria, 118 patients undergoing TKA were randomly allocated to two groups. Patients in group A were applied LMWH, whereas patients in group B received LMWH followed by rivaroxaban postoperatively. The two groups were foIlowed up and compared for the incidence of DVT by color Doppler ultrasonography scan. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), albumin (ALB), Hb and D-Dimer were also compared...
November 29, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30507709/genotype-analysis-and-identification-of-novel-mutations-in-a-multicentre-cohort-of-patients-with-hereditary-factor-x-deficiency
#14
Michael Mitchell, Michael Gattens, Kaan Kavakli, Ri Liesner, Jeanette Payne, Miranda Norton, Steven Austin
: The objective was to examine the genotypic and phenotypic characteristics of individuals with hereditary factor X deficiency (FXD), a rare autosomal recessive bleeding disorder caused by mutations in the F10 gene located on chromosome 13q34-ter. To date, 149 F10 mutations have been identified as contributory to FXD. Three open-label phase 3 trials enrolled individuals with mild, moderate, or severe FXD. Individuals received plasma-derived factor X concentrate as routine prophylaxis, to treat bleeds, and/or during or after surgery...
November 29, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30672814/may-restricted-specificity-of-commercially-available-plasmin-inhibitor-activity-assays-affect-correct-diagnosis-of-antiplasmin-deficiency
#15
Loris Pozzi, Patrizia Della Valle, Armando D'Angelo
No abstract text is available yet for this article.
January 2019: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30439769/protein-c-deficiency-a-novel-mutation-ala291thr-with-systemic-lupus-erythematosus-leads-to-the-deep-vein-thrombosis
#16
Kankan Su, Haiyue Zhang, Weiwei Fang, Feng Zhang, Lihong Yang, Yanhui Jin, Mingshan Wang
: The current study aims to explore the phenotype and genotype of a mutation Ala291Thr, which responsible for type I protein C (PC) deficiency in a Chinese woman. The PROC antigen was tested with chromogenic substrate method. PROC gene were amplified by PCR with direct sequencing. Bioinformatics and model analysis were used to study the harm of the mutation. PC activity (PC: A) levels of three members were reduced to 39, 57 and 56%, respectively, PC: antigen was decreased parallelly same as PC: A. Sequencing analysis showed proband with a novel heterozygous c...
December 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30439768/prevalence-of-factor-viii-inhibitors-among-afghan-patients-with-hemophilia-a-a-first-report
#17
Sayed H Mousavi, Seyed A Mesbah-Namin, Nematullah Rezaie, Mohammad Jazebi, Sirous Zeinali
: Prevalence of inhibitors in Afghan hemophilia patients has not been reported previously. Our aim was to determine the prevalence of factor VIII inhibitors among hemophilia A patients from the Kabul province of Afghanistan to identify and characterize the pattern of inhibitor formation. Clinical information and blood samples were collected from three hemophilia centers in Kabul, Afghanistan. Plasma samples were obtained from 62 patients with severe (80.5%) and 15 patients with moderate hemophilia A (19.5%) in this cross-sectional study design...
December 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30439767/prediction-of-bleeding-by-thromboelastography-in-icu-patients-with-haematological-malignancy-and-severe-sepsis
#18
Lene Russell, Nicolai Haase, Anders Perner
: ICU patients with haematological malignancy have an increased risk of bleeding. Recently, global haemostatic methods such as thromboelastography (TEG) have gained impact in evaluating coagulation. The aim of this study was to observe whether TEG could predict bleeding in haematological ICU patients with severe sepsis. Post-hoc single-centre analysis of patients with haematological malignancy included in the Scandinavian Starch for Severe Sepsis/Septic Shock (6S) trial. Clinical characteristics, TEG measurements and details regarding bleeding complications were retrieved from the 6S database...
December 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30439766/post-transcriptional-post-translational-and-pharmacological-regulation-of-tissue-factor-pathway-inhibitor
#19
Saravanan Subramaniam, Sandip M Kanse, Hema Kothari, Christoph Reinhardt, Craig Fletcher
: Tissue factor (TF) pathway inhibitor (TFPI) is an endogenous natural anticoagulant that readily inhibits the extrinsic coagulation initiation complex (TF-FVIIa-Xa) and prothrombinase (FXa, FVa and calcium ions). Alternatively, spliced TFPI isoforms (α, β and δ) are expressed by vascular and extravascular cells and regulate thrombosis and haemostasis, as well as cell signalling functions of TF complexes via protease-activated receptors (PARs). Proteolysis of TFPI plays an important role in regulating physiological roles of the TF pathway in host defense and possibly haemostasis...
December 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/30325339/molecular-genetic-diagnosis-of-tunisian-glanzmann-thrombasthenia-patients-reveals-a-common-nonsense-mutation-in-the-itga2b-gene-that-seems-to-be-specific-for-the-studied-population
#20
Chaker Aloui, Tahar Chakroun, Viviana Granados, Saloua Jemni-Yacoub, Jocelyne Fagan, Abderrahim Khelif, Najoua Kahloul, Sabeur Hammami, Latifa Chkioua, Céline Barlier, Fabrice Cognasse, Sandrine Laradi, Olivier Garraud
: Glanzmann thrombasthenia is an inherited severe bleeding disease. Mutations associated with Glanzmann thrombasthenia are highly heterogeneous and occur across the two genes coding for the platelet αIIbβ3 integrin. This study was aimed at identifying Glanzmann thrombasthenia-associated novel mutations in Tunisian patients. Seven unrelated Glanzmann thrombasthenia patients issued from high consanguineous families (86%; 6/7 of the patients) were studied. Glanzmann thrombasthenia diagnoses were based on patients' bleeding histories and platelet aggregation tests...
December 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
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