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https://www.readbyqxmd.com/read/28318109/agreement-between-factor-xiii-activity-and-antigen-assays-in-measurement-of-factor-xiii-a-french-multicenter-study-of-147-human-plasma-samples
#1
C Caron, R Meley, V Le Cam Duchez, M F Aillaud, C Lavenu-Bombled, F Dutrillaux, C Flaujac, A Ryman, C Ternisien, D Lasne, H Galinat, C Pouplard
INTRODUCTION: Factor XIII (FXIII) deficiency is a rare hemorrhagic disorder whose early diagnosis is crucial for appropriate treatment and prophylactic supplementation in cases of severe deficiency. International guidelines recommend a quantitative FXIII activity assay as first-line screening test. FXIII antigen measurement may be performed to establish the subtype of FXIII deficiency (FXIIID) when activity is decreased. METHODS: The aim of this multicenter study was to evaluate the analytical and diagnostic levels of performance of a new latex immunoassay, K-Assay(®) FXIII reagent from Stago, for first-line measurement of FXIII antigen...
March 20, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28222949/rare-bleeding-disorders-old-diseases-in-the-era-of-novel-options-for-therapy
#2
Tami Livnat, Assaf Arie Barg, Sarina Levy-Mendelovich, Gili Kenet
Rare diseases are defined as life-threatening or chronically debilitating diseases with a prevalence of less than one per 2000 according to the European Union or one per 1250 according to the USA. Congenital rare bleeding disorders RBD are reported in most populations, with incidence varying from 1 in 5000 (Hemophilia A), 1:30,000 (Hemophilia B) to much rarer (1:500,000 for FVII deficiency, 1-3 million for Prothrombin or FXIII deficiency). Acquired Hemophilia A is also a rare bleeding disorder with estimated frequency of 1 in million...
February 14, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28166607/recombinant-fibrinogen-reveals-the-differential-roles-of-%C3%AE-and-%C3%AE-chain-cross-linking-and-molecular-heterogeneity-in-fibrin-clot-strain-stiffening
#3
I K Piechocka, N A Kurniawan, J Grimbergen, J Koopman, G H Koenderink
BACKGROUND: Fibrin plays a crucial role in haemostasis and wound healing by forming strain-stiffening fibrous networks that reinforce blood clots. The molecular origin of fibrin's strain-stiffening behavior remains poorly understood, primarily because plasma fibrinogen is a complex mixture of heterogeneous molecular variants and is often contaminated by plasma factors that affect clot properties. OBJECTIVES AND METHODS: To facilitate mechanistic dissection of fibrin nonlinear elasticity, we produced a homogeneous recombinant fibrinogen corresponding to the main variant in human plasma, termed rFib610...
February 6, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28159767/ticagrelor-reversal-in-vitro-assessment-of-four-haemostatic-agents
#4
Leyla Calmette, Anne-Céline Martin, Bernard Le Bonniec, Diane Zlotnik, Isabelle Gouin-Thibault, Christilla Bachelot-Loza, Pascale Gaussem, Anne Godier
AIM: Management of ticagrelor-induced bleeding is challenging as platelet transfusion is ineffective. An effective strategy is needed. This study aimed to investigate in vitro the efficacy of four haemostatic drugs (HDs), namely recombinant activated factor VII (rFVIIa), fibrinogen concentrate (Fib), tranexamic acid (TXA) and factor XIII concentrate (FXIII) to improve the haemostatic capacity in the presence of ticagrelor. METHODS: Blood was spiked with ticagrelor then supplemented by either HD or control...
February 3, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28091415/advances-of-coagulation-factor-xiii
#5
REVIEW
Da-Yu Shi, Shu-Jie Wang
OBJECTIVE: To provide a comprehensive literature review on roles of coagulation factor XIII (FXIII) in coagulation, wound healing, neoplasm, bone metabolism, and pregnancy. DATA SOURCES: All articles in PubMed with key words "Coagulation factor XIII", "wound", "leukemia", "tumor", "bone," and "pregnancy" with published date from 2001 to 2016 were included in the study. Frequently cited publications before 2000 were also included. STUDY SELECTION: We reviewed the role of FXIII in biologic processes as documented in clinical, animal, and in vitro studies...
January 20, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28086795/atherosclerotic-and-thrombotic-genetic-and-environmental-determinants-in-egyptian-coronary-artery-disease-patients-a-pilot-study
#6
Manal S Fawzy, Eman A Toraih, Nagwa M Aly, Abeer Fakhr-Eldeen, Dahlia I Badran, Mohammad H Hussein
BACKGROUND: Coronary artery disease (CAD) is the leading cause of morbidity and mortality worldwide. Multiple genetic variants in combination with various environmental risk factors have been implicated. This study aimed to investigate the association of twelve thrombotic and atherosclerotic gene variants in combination with other environmental risk factors with CAD risk in a preliminary sample of Egyptian CAD patients. METHODS: Twenty three consecutive CAD patients undergoing diagnostic coronary angiography and 34 unrelated controls, have been enrolled in the study...
January 13, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28028391/vascular-genetic-variants-and-ischemic-stroke-susceptibility-in-albanians-from-the-republic-of-macedonia
#7
Bajram Kamberi, Farije Kamberi, Mirko Spiroski
BACKGROUND: Acute first-ever ischemic stroke (FIS) is a heterogeneous, polygenic disorder. The contribution of vascular genetic variants as inherited causes of ischemic stroke has remained controversial. AIM: To examine the association of genetic variants in vascular factors with the occurrence of FIS. MATERIAL AND METHODS: The current research was performed in a group of 39 patients with FIS (study group) and 102 healthy volunteers (control group)...
December 15, 2016: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/27975098/endotoxaemia-augmented-murine-venous-thrombosis-is-dependent-on-tlr-4-and-icam-1-and-potentiated-by-neutropenia
#8
Andrea T Obi, Elizabeth Andraska, Yogendra Kanthi, Chase W Kessinger, Megan Elfline, Cathy Luke, Teruna J Siahaan, Farouc A Jaffer, Thomas W Wakefield, Peter K Henke
Venous thromboembolism is a major cause of death during and immediately post-sepsis. Venous thrombosis (VT) is mediated by cell adhesion molecules and leukocytes, including neutrophil extracellular traps (NETs). Sepsis, or experimentally, endotoxaemia, shares similar characteristics and is modulated via toll like receptor 4 (TLR4). This study was undertaken to determine if endotoxaemia potentiates early stasis thrombogenesis, and secondarily to determine the role of VT TLR4, ICAM-1 and neutrophils (PMNs). Wild-type (WT), ICAM-1(-/-) and TLR4(-/-) mice underwent treatment with saline or LPS (10 mg/kg i...
December 15, 2016: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/27942785/-factor%C3%A2-xiii-pharmacodynamic-and-pharmacokinetic-characteristics
#9
REVIEW
E H Adam, S Kreuer, K Zacharowski, C F Weber, R Wildenauer
Factor XIII (FXIII) plays an important role in the field of blood coagulation. In the last decade, both congenital and acquired deficiencies have been investigated in clinical studies. FXIII is a versatile enzyme that leads to a covalent cross-linking of fibrin fibrils at the end of the clotting cascade and supports platelet adhesion to the damaged sub-endothelium with the result of a mechanically stable clot.Symptoms of FXIII deficiencies vary within a broad spectrum from superficial skin bleeding episodes to severe, sometimes life threatening hemorrhage, requiring prophylactic or therapeutic replacement therapy...
January 2017: Der Anaesthesist
https://www.readbyqxmd.com/read/27929200/obesity-insulin-resistance-rather-than-liver-fat-increases-coagulation-factor-activities-and-expression-in-humans
#10
Susanna Lallukka, Panu K Luukkonen, You Zhou, Elina M Petäjä, Marja Leivonen, Anne Juuti, Antti Hakkarainen, Marju Orho-Melander, Nina Lundbom, Vesa M Olkkonen, Riitta Lassila, Hannele Yki-Järvinen
Increased liver fat may be caused by insulin resistance and adipose tissue inflammation or by the common I148M variant in PNPLA3 at rs738409, which lacks both of these features. We hypothesised that obesity/insulin resistance rather than liver fat increases circulating coagulation factor activities. We measured plasma prothrombin time (PT, Owren method), activated partial thromboplastin time (APTT), activities of several coagulation factors, VWF:RCo and fibrinogen, and D-dimer concentration in 92 subjects divided into groups based on insulin sensitivity [insulin-resistant ('IR') versus insulin-sensitive ('IS')] and PNPLA3 genotype (PNPLA3(148MM/MI) vs PNPLA3(148II))...
December 8, 2016: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/27917497/acquired-factor-xiii-inhibitor-associated-with-mantle-cell-lymphoma
#11
Christian P Nixon, Elizabeth H Prsic, Christine A Guertin, Ryan L Stevenson, Joseph D Sweeney
BACKGROUND: Acquired Factor (F)XIII deficiency is a very rare bleeding diathesis with a potentially fatal outcome, previously described in the context of autoimmune disorders and leukemias. There is minimal information on autoantibody characterization and the role of antifibrinolytic therapy in patient management. CASE REPORT: A 79-year-old woman with a 3-month history of bruising and heavy menorrhagia presented with ongoing vaginal bleeding, symptomatic anemia, and a right thigh hematoma...
March 2017: Transfusion
https://www.readbyqxmd.com/read/27913544/treatment-of-rare-factor-deficiencies-in-2016
#12
Flora Peyvandi, Marzia Menegatti
Rare bleeding disorders (RBDs) are a heterogeneous group of coagulation disorders characterized by fibrinogen, prothrombin, factors V, VII, X, XI, or XIII (FV, FVII, FX, FXI, or FXIII, respectively), and the combined factor V + VIII and vitamin K-dependent proteins deficiencies, representing roughly 5% of all bleeding disorders. They are usually transmitted as autosomal, recessive disorders, and the prevalence of the severe forms could range from 1 case in 500 000 for FVII up to 1 in 2-3 million for FXIII in the general population...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27902939/factor-xiii-deficiency-enhances-thrombin-generation-due-to-impaired-fibrin-polymerization-an-effect-corrected-by-factor-xiii-replacement
#13
Hanna H Pitkänen, Annukka Jouppila, Marja Lemponen, Minna Ilmakunnas, Jouni Ahonen, Riitta Lassila
INTRODUCTION: Factor XIII (FXIII) cross-links fibrin, completing blood coagulation. Congenital FXIII deficiency is managed with plasma-derived FXIII (pdFXIII) or recombinant FXIII (rFXIII) concentrates. AIM: As the mechanisms protecting patients with low FXIII levels (<5IU/dL) from spontaneous bleeds remain unknown we assessed the interplay between thrombin generation (TG), fibrin formation and clot kinetics before and after FXIII administration in three patients with FXIII deficiency...
January 2017: Thrombosis Research
https://www.readbyqxmd.com/read/27891624/quarantine-versus-pathogen-reduced-plasma-coagulation-factor-content-and-rotational-thromboelastometry-coagulation
#14
Oliver M Theusinger, David Goslings, Jan-Dirk Studt, Brigitte Brand-Staufer, Burkhardt Seifert, Donat R Spahn, Beat M Frey
BACKGROUND: Different types of fresh-frozen plasma (FFP) exist, and the concentrations of plasma proteins vary between individuals and blood groups. Furthermore, processing may also influence the content. Quarantine-stored plasma (qFFP) and plasma that was pathogen-reduced using blood-safety (Intercept) technology (piFFP) were analyzed regarding procoagulant and anticoagulant hemostasis proteins, including endogenous thrombin (thrombin-generation) potential (ETP). MATERIALS AND METHODS: Thirty-five samples of each type of FFP were analyzed using only male Blood Group O donors...
March 2017: Transfusion
https://www.readbyqxmd.com/read/27879471/molecular-basis-of-congenital-factor-xiii-deficiency-in-iran
#15
Akbar Dorgalaleh, Vahideh Assadollahi, Shadi Tabibian, Morteza Shamsizadeh
Factor XIII deficiency (FXIIID) is an extremely rare autosomal recessive disorder that has the highest incidence in Iran. The FXIIID is primarily due to mutations in the FXIII-A gene, most of which are unique. In the current study, we report all identified mutations among Iranian patients. Among 483 patients, 366 (75.8%) were molecularly analyzed; 11 different mutations were observed. Of 11, 8 (72.7%) are missense, whereas the remaining 3 (27.3%) are deletion/insertion. Among these patients, 347 (94.9%) had the unique mutation of c...
January 1, 2016: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/27855295/establishment-of-a-bleeding-score-as-a-diagnostic-tool-for-patients-with-rare-bleeding-disorders
#16
Roberta Palla, Simona M Siboni, Marzia Menegatti, Khaled M Musallam, Flora Peyvandi
INTRODUCTION: Bleeding manifestations among patients with rare bleeding disorders (RBDs) vary significantly between disorders and patients, even when affected with the same disorder. In response to the challenge represented by the clinical assessment of the presence and severity of bleeding symptoms, a number of bleeding score systems (BSSs) or bleeding assessment tools (BATs) were developed. The majority of these were specifically developed for patients with more common bleeding disorders than RBDs...
December 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27821352/regulation-of-plasma-factor-xiii-levels-in-healthy-individuals-a-major-impact-by-subunit-b-intron-k-c-1952-144-c-g-polymorphism
#17
Zoltán A Mezei, Éva Katona, Judit Kállai, Zsuzsanna Bereczky, Éva Molnár, Bettina Kovács, Éva Ajzner, Zsuzsa Bagoly, Tünde Miklós, László Muszbek
BACKGROUND: The regulation of plasma factor XIII (FXIII) levels in healthy individuals has been only partially explored. The identification of major non-genetic and genetic regulatory factors might provide important information on the contribution of FXIII to the risk of cardio/cerebrovascular diseases. OBJECTIVES: To determine the effect of age, smoking, BMI, fibrinogen concentration on plasma FXIII activity, complex FXIII antigen (FXIII-A2B2) and total FXIII-B subunit (tFXIII-B) level, to correlate FXIII-B level with the other two FXIII parameters and to assess the variation of FXIII levels in carriers of major FXIII subunit polymorphisms...
December 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27792840/maternal-genetic-polymorphisms-and-unexplained-recurrent-miscarriage-a-systematic-review-and-meta-analysis
#18
REVIEW
X Shi, X Xie, Y Jia, S Li
The roles of genetic polymorphisms in the pathogenesis of recurrent miscarriage (RM) have been intensively studied. However, the results of these studies were inconsistent, especially when conducted in different populations. Therefore, we performed the current study to systematically review the broad spectrum of genetic polymorphisms that were suspected to be involved in RM, and discussed potential genetic biomarkers of RM. Eligible articles were identified in PubMed, Medline, Embase and CNKI. Odd ratios (ORs) and 95% confidence intervals (CIs) were used to describe the strength of association, and a probability value (p value) of 0...
February 2017: Clinical Genetics
https://www.readbyqxmd.com/read/27781024/safety-of-factor-xiii-concentrate-analysis-of-more-than-20-years-of-pharmacovigilance-data
#19
Cristina Solomon, Wolfgang Korte, Dietmar Fries, Inna Pendrak, Christine Joch, Albrecht Gröner, Ingvild Birschmann
BACKGROUND: Plasma-derived factor XIII (FXIII) concentrate is an effective treatment for FXIII deficiency. We describe adverse drug reactions (ADRs) reported during pharmacovigilance monitoring of Fibrogammin®/Corifact® and review published safety data. METHODS: Postmarketing safety reports recorded by CSL Behring from June 1993 to September 2013 were analyzed. Clinical studies published during the same period were also reviewed. RESULTS: Commercial data indicated that 1,653,450,333 IU FXIII concentrate were distributed over the review period, equivalent to 1,181,036 doses for a 70 kg patient...
September 2016: Transfusion Medicine and Hemotherapy
https://www.readbyqxmd.com/read/27777753/coagulative-safety-of-epidural-catheters-after-major-upper-gastrointestinal-surgery-advanced-and-routine-coagulation-analysis-in-38-patients
#20
Owain Thomas, Hampus Rein, Karin Strandberg, Ulf Schött
BACKGROUND: The risk of spinal haematoma in patients receiving epidural catheters is estimated using routine coagulation tests, but guidelines are inconsistent in their recommendations on what to do when results indicate slight hypocoagulation. Postoperative patients are prone to thrombosis, and thromboelastometry has previously shown hypercoagulation in this setting. We aimed to better understand perioperative haemostasis by comparing results from routine and advanced tests, hypothesizing that patients undergoing major upper gastrointestinal surgery would be deficient in vitamin K-dependent coagulation factors because of malnutrition, or hypocoagulative because of accumulation of low molecular weight heparin (LMWH)...
2016: Perioperative Medicine
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