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

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https://www.readbyqxmd.com/read/30431449/safe-childbirth-for-a-type-1-antithrombin-deficient-woman-with-novel-mutation-in-the-serpinc1-gene-undergoing-antithrombin-concentrate-therapy
#1
Yuichi Ikeda, Jun Yamanouchi, Takaaki Hato, Masaki Yasukawa, Katsuto Takenaka
: Inherited antithrombin (AT) deficiency is an autosomal dominant thrombotic disorder. We encountered a case of inherited type I AT deficiency and identified the causative mutation; a novel c.7430A>G missense mutation in the SERPINC1 gene in which tyrosine was substituted for cysteine at the 292nd amino acid. A recombinant AT protein with the 7430A>G mutation was not detected in cell lysates or culture supernatants. And then, our patient without personal or family history of thrombosis was pregnant woman with asymptomatic AT deficiency...
November 13, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/30431448/probable-drug-drug-interaction-between-dabigatran-and-quinidine-resulting-in-thrombin-time-rebound-despite-multiple-idarucizumab-doses
#2
Stephy George, Margarita Taburyanskaya, Vicky Lewis
: Dabigatran is a direct thrombin inhibitor that was approved as an alternative to warfarin because it offers the benefit of predictable pharmacokinetic properties, favorable safety profile and ease of administration. Despite the improved safety profile, dabigatran use can lead to bleeding events. The bleeding risk associated with dabigatran is higher in the setting of renal impairment or drug-drug interactions resulting in supratherapeutic serum concentrations. Unfortunately, clinically significant interactions are not always identified by providers, especially in the case of infrequent drug-drug combinations...
November 13, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/30431447/predicting-mortality-in-patients-with-disseminated-intravascular-coagulation-after-cardiopulmonary-bypass-surgery-by-utilizing-two-scoring-systems
#3
Linda J Demma, David Faraoni, Anne M Winkler, Toshiaki Iba, Jerrold H Levy
: We evaluated clinical and laboratory biomarkers of disseminated intravascular coagulation (DIC) following cardiac surgery in the cardiothoracic surgical ICU (CTICU) to predict mortality. We retrospectively analyzed CTICU patients with suspected DIC identified from the hospital laboratory database, and calculated International Society on Thrombosis and Haemostasis (ISTH) and the Japanese Association for Acute Medicine (JAAM) DIC scores to predict DIC-related mortality. The predictive accuracy of the JAAM and ISTH DIC scoring system were then assessed by logistic regression analysis and receiver operative characteristics analysis, and compared to other potential predictors of mortality (e...
November 13, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/30439769/protein-c-deficiency-a-novel-mutation-ala291thr-with-systemic-lupus-erythematosus-leads-to-the-deep-vein-thrombosis
#4
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://www.readbyqxmd.com/read/30439768/prevalence-of-factor-viii-inhibitors-among-afghan-patients-with-hemophilia-a-a-first-report
#5
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://www.readbyqxmd.com/read/30439767/prediction-of-bleeding-by-thromboelastography-in-icu-patients-with-haematological-malignancy-and-severe-sepsis
#6
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://www.readbyqxmd.com/read/30439766/post-transcriptional-post-translational-and-pharmacological-regulation-of-tissue-factor-pathway-inhibitor
#7
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://www.readbyqxmd.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
#8
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
https://www.readbyqxmd.com/read/30300149/fatal-pulmonary-embolism-and-pulmonary-hemorrhage-in-lupus-anticoagulant-hypoprothrombinemia-syndrome-a-case-report-and-review-of-literature
#9
Xiuxu Chen, Drew Nedved, Fred V Plapp, Mark T Cunningham
: Lupus anticoagulant hypoprothrombinemia syndrome (LAHS) is a rare disorder characterized by development of lupus anticoagulant and antiprothrombin antibodies. The most common clinical manifestation is bleeding. Clinical management can be challenging due to the subtle balance between the bleeding and thrombotic tendencies. We report a novel case of LAHS in which the patient experienced the sequence of hemorrhage-thrombosis-hemorrhage before eventually dying of fatal pulmonary embolism and pulmonary hemorrhage...
December 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/30300148/low-dose-short-course-alteplase-treatment-of-submassive-pulmonary-embolism-a-case-series-from-the-national-institutes-of-health-clinical-center
#10
Jay N Lozier, Jason M Elinoff, Anthony F Suffredini, Douglas R Rosing, Stanislav Sidenko, Richard M Sherry, Adam R Metwalli, Vandana Sachdev, Robert L Danner, Richard Chang
: Guidelines-recommend thrombolytic therapy for pulmonary embolism in patients with severe hemodynamic compromise and low risk of bleeding. Thrombolytics in submassive pulmonary embolism have an unfavorable risk/benefit ratio and remain controversial. Based on our experience with extensive, lower extremity thrombi, nine patients with symptomatic, submassive pulmonary embolisms (five medical, four surgical) were treated with low-dose alteplase (<10 mg/day, infused over 6 h per treatment). Alteplase was delivered by pulse spray and/or directed or undirected central venous catheters depending on clot size and location...
December 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/30234546/sex-gender-and-venous-thromboembolism-do-we-care-enough
#11
Elena M Faioni, Maddalena L Zighetti, Nadia P Vozzo
: The role of sex and gender in determining clinical presentation, diagnostic approach and outcomes of venous thromboembolism is not fully and systematically addressed, except for hormone-related events in women. A lack of knowledge is also apparent regarding drug prescription patterns, physician bias, enrolment in clinical studies and analysis of sex-related confounders in preclinical and clinical studies. As was shown for cardiovascular disease, ignoring sex and gender in medicine can have important impact on outcomes, including mortality...
December 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/30334816/novel-isomeric-metabolite-profiles-correlate-with-warfarin-metabolism-phenotype-during-maintenance-dosing-in-a-pilot-study-of-29-patients
#12
Dakota L Pouncey, Jessica H Hartman, Page C Moore, David J Dillinger, Kimberly W Dickerson, Daniel R Sappington, Eugene S Smith, Gunnar Boysen, Grover P Miller
: For this pilot study, we leveraged metabolite patterns for warfarin patients to more accurately assess clinically relevant differences in drug metabolism. We tested our hypothesis that plasma metabolite levels correlate with the influence of clinical factors on R-warfarin and S-warfarin metabolism (warfarin metabolic phenotype). We recruited 29 patients receiving a maintenance dose and testing within targeted therapeutic range. We determined their CYP2C9 and vitamin K epoxide reductase genotype and profiled 14 isomeric forms of warfarin and its metabolites...
November 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/30325892/thrombosis-in-pediatric-malignancy-a-review-and-future-perspectives-with-focus-on-management
#13
Mohammadreza Bordbar, Mehran Karimi, Nader Shakibazad
: Venous thromboembolism (VTE) result in significant morbidity and mortality in children with cancer. The cause of VTE in children with cancer is multifactorial and includes genetic predisposition (thrombophilia), disease-related factors, and treatment-related factors including use of central venous catheter (CVC), surgery, and chemotherapy. This review aims to examine current knowledge regarding the incidence, risk factors, clinical manifestation, evaluation, prevention, and management of thromboembolic events in children with cancer...
November 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/30234545/thrombogenesis-and-thrombotic-disorders-based-on-two-path-unifying-theory-of-hemostasis-philosophical-physiological-and-phenotypical-interpretation
#14
Jae C Chang
: Hemostasis, endowed to human to protect lives, is a process of logical blood clotting system to prevent blood loss in vascular injury. However, the notion that deadly thrombosis occurs as a result of normal hemostasis in intravascular injury could encounter with conceptual skepticism because the term 'thrombosis' automatically conjures up as serious disease. According to 'two-path unifying theory', normal hemostasis is initiated only by vascular injury through activated unusually large von Willebrand factor (ULVWF) path and/or activated tissue factor (TF) path...
November 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/30234544/reliability-of-the-portable-coagulometer-qlabs-to-accurately-measure-the-activated-thromboplastin-time-and-international-normalized-ratio-a-prospective-study-in-critically-ill-patients
#15
Manuel Casado-Méndez, Victoria Arellano-Orden, Manuel Muñoz-Gómez, Francisco J Rodríguez-Martorell, Aurelio Cayuela, Álvaro Pastor de Las Heras, Ana Díaz Martín, Ignacio Pérez Torres, Santiago R Leal-Noval
: The current prospective study was aimed at investigating whether a portable coagulometer (qLabs) can be used to reliably monitor activated thromboplastin time (aPTT) and international normalized ratio (INR) in critically ill patients, as compared with standard central laboratory measurement. Both precision and accuracy of INR and aPTT measured by qLabs were assessed in this observational study by finger prick group (N = 30 patients) and blood droplet group from central venous catheter drawn (N = 60)...
November 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/30234543/thromboelastography-in-the-management-of-snakebite-induced-coagulopathy-a-case-series-and-literature-review
#16
Patrick Leffers, Jason Ferreira, Dawn Sollee, Jay Schauben
: North American crotaline envenomations are a significant source of morbidity annually. Envenomation is marked by a constellation of effects that can include severe tissue damage, systemic effects such as anaphylaxis, and coagulopathy with bleeding. Traditionally, static plasma-derived tests such as protime and PTT are used to assess coagulation status. However, other tests are available that provide a whole blood assessment of all portions of coagulation including initiation, propagation, and clot maintenance...
November 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/30113321/optimizing-outcome-measurement-with-murine-ferric-chloride-induced-thrombosis
#17
Bryn Kastetter, Amanda B Matrai, Brian C Cooley
: The murine FeCl3 model is a widely used model for studying arterial thrombosis, yet provides limited information from each mouse, often only a single time point for the onset of occlusion (defined as the time to occlusion; TTO). To optimize data from the murine ferric chloride model of thrombosis. FeCl3 injury was induced in the carotid arteries of wild-type and Factor IX (FIX) knockout mice, with infusion of recombinant FIX (rFIX) to normalize FIX deficiency at various times around FeCl3 injury. The TTO was recorded as a percentage of baseline flow as occlusion continued to zero flow, with identification of reflow events...
November 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/30063477/hemostasis-based-on-a-novel-two-path-unifying-theory-and-classification-of-hemostatic-disorders
#18
Jae C Chang
: Hemostasis is the most important protective mechanism for human survival following harmful vascular damage caused by internal disease or external injury. Physiological mechanism of hemostasis is partially understood. Hemostasis can be initiated by either intravascular injury or external bodily injury involving two different levels of damage [i.e., limited to the endothelium or combined with extravascular tissue (EVT)]. In intravascular injury, traumatic damage limited to local endothelium typically is of no consequence, but disease-induced endothelial damage associated with systemic endothelial injury seen in sepsis and other critical illnesses could cause generalized 'endotheliopathy'...
November 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/30045050/management-of-dabigatran-after-overdosage-two-case-reports-and-suggestions-for-monitoring
#19
Paul Billoir, Christophe Girault, Virginie Barbay, Deborah Boyer, Steven Grangé, Marielle Fresel, Marie Hélène Chrétien, Véronique Le Cam Duchez
: Bleeding is the main complication of anticoagulant treatments as dabigatran etexilate. In patients with atrial fibrillation, dabigatran, at certain doses, has been associated with similar rates of stroke and embolism, and a lower rate of major hemorrhage compared to warfarin. Before the recent possibility of reversing the anticoagulant effect of dabigatran with idarucizumab, prothrombin complex concentrate (PCC) was the main available treatment in cases of severe bleeding or emergency surgery . We describe two different cases with very high overdosage in which PCC or idarucizumab was used to reverse the effect of dabigatran etexilate...
November 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/30036281/a-novel-type-2n-vwf-gene-mutation-a-case-report
#20
Matthew S Evans, M Elaine Eyster
: Men and boys who present with bleeding associated with low factor VIII levels and normal von Willebrand studies are assumed to have hemophilia A until proven otherwise. However, routinely available coagulation assays cannot easily distinguish mild hemophilia A from the 2N variant of von Willebrand disease. We present a case that highlights the difficulties of recognizing this diagnosis, the role of genetic testing, and the identification of a 2N variant that has not been previously described.
November 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
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