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https://www.readbyqxmd.com/read/28804836/assessment-of-factor-xiii
#1
László Muszbek, Éva Katona, Adrienne Kerényi
Blood coagulation factor XIII (FXIII) is essential for maintaining hemostasis. The absence of FXIII results in severe bleeding diathesis, which without prophylaxis frequently leads to fatal bleeding. As the usual hemostasis screening tests remain normal, the diagnosis of FXIII deficiency needs specific tests. Here, we describe FXIII activity determination by the ammonia release assay, which is the first-line test in the diagnostic algorithm for FXIII deficiency. The method for another activity test, the undeservedly rarely used fibrin cross-linking assay, is also presented...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28761875/inhibition-of-fibrinolysis-by-coagulation-factor-xiii
#2
REVIEW
Dingeman C Rijken, Shirley Uitte de Willige
The inhibitory effect of coagulation factor XIII (FXIII) on fibrinolysis has been studied for at least 50 years. Our insight into the underlying mechanisms has improved considerably, aided in particular by the discovery that activated FXIII cross-links α2-antiplasmin (α2AP) to fibrin. In this review, the most important effects of different cross-linking reactions on fibrinolysis are summarized. A distinction is made between fibrin-fibrin cross-links studied in purified systems and fibrin-α2AP cross-links studied in plasma or whole blood systems...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28734018/influence-of-cryoprecipitate-factor-xiii-and-fibrinogen-concentrate-on-hyperfibrinolysis
#3
Melissa M Cushing, Meghann M Fitzgerald, Rebecca M Harris, Lars M Asmis, Thorsten Haas
BACKGROUND: Hyperfibrinolysis is a potentially life-threatening condition associated with poor clot integrity and excessive bleeding. Although antifibrinolytics are an effective treatment, more liberal use of these drugs may lead to a prothrombotic risk, and an earlier and potentially safer treatment option would be desirable. Hyperfibrinolysis has been shown to be attenuated by in vitro supplementation of purified human Factor (F)XIII concentrate. Cryoprecipitate represents an alternative source of FXIII and the only approved source of concentrated FXIII in some countries...
July 21, 2017: Transfusion
https://www.readbyqxmd.com/read/28728890/clinical-influence-of-preoperative-factor-xiii-activity-in-patients-undergoing-pancreatoduodenectomy
#4
Nobuyuki Watanabe, Yukihiro Yokoyama, Tomoki Ebata, Gen Sugawara, Tsuyoshi Igami, Takashi Mizuno, Junpei Yamaguchi, Masato Nagino
BACKGROUND: The influence of decreased factor XIII (FXIII) activity on perioperative bleeding has been reported in some surgical procedures. The purposes of this study were to investigate the perioperative dynamics of FXIII in patients undergoing pancreatoduodenectomy and to clarify the effects of low preoperative FXIII activity on intraoperative bleeding and postoperative complications. METHODS: Total of 43 patients who underwent a pancreatoduodenectomy were enrolled...
July 17, 2017: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
https://www.readbyqxmd.com/read/28707410/treatment-of-an-acquired-factor-xiii-inhibitor-in-an-adolescent-with-systemic-lupus-erythematosus-and-renal-failure
#5
Cara A Rabik, Meredith A Atkinson, Sangeeta Sule, John J Strouse
BACKGROUND: Factor (F)XIII deficiency is a rare inherited bleeding disorder, but can also be acquired due to the development of inhibitors. CASE REPORT: A 17-year-old female with systemic lupus erythematosus and end-stage kidney disease secondary to Class IV lupus nephritis developed spontaneous subcutaneous and muscular hematomas and delayed major bleeding after invasive procedures. She had abnormal kaolin thromboelastography (kTEG; decreased maximal amplitude, representative of clot strength) initially attributed to thrombocytopenia and uremic platelet dysfunction, but her FXIII activity was undetectable, and a high-titer antibody against FXIII was identified...
July 13, 2017: Transfusion
https://www.readbyqxmd.com/read/28688221/minimal-factor-xiii-activity-level-to-prevent-major-spontaneous-bleeds
#6
M Menegatti, R Palla, M Boscarino, P Bucciarelli, L Muszbek, E Katona, M Makris, F Peyvandi
BACKGROUND: Congenital factor XIII (FXIII) deficiency is a rare bleeding disorder associated with significant bleeding manifestations. The European Network of Rare Bleeding Disorders (EN-RBD) study, performed from 2007 to 2010, showed a strong association between bleeding severity and FXIII activity in plasma of patients with FXIII deficiency. Among these patients variable levels of FXIII activity, from undetectable to 30%, were associated with a wide range of bleeding severity. Objectives and patients: The present cross-sectional study, in the frame of the PRO-RBDD project, a prospective cohort study, analyzed data of 64 patients with FXIII deficiency and different types of clinical and laboratory severity...
July 8, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28687225/screening-cleavage-of-factor-xiii-v34x-activation-peptides-by-thrombin-mutants-a-strategy-for-controlling-fibrin-architecture
#7
Madhavi A Jadhav, Whitney N Goldsberry, Sara E Zink, Kelsey N Lamb, Katelyn E Simmons, Carmela M Riposo, Boris A Anokhin, Muriel C Maurer
In blood coagulation, thrombin converts fibrinogen into fibrin monomers that polymerize into a clot network. Thrombin also activates Factor XIII by cleaving the R37-G38 peptide bond of the Activation Peptide (AP) segment. The resultant transglutaminase introduces covalent crosslinks into the fibrin clot. A strategy to modify clot architecture would be to design FXIII AP sequences that are easier or more difficult to be thrombin-cleaved thus controlling initiation of crosslinking. To aid in this design process, FXIII V34X (28-41) Activation Peptides were kinetically ranked for cleavage by wild-type thrombin and several anticoagulant mutants...
July 4, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28683377/association-of-the-f13a1-val34leu-polymorphism-and-recurrent-pregnancy-loss-a-meta-analysis
#8
Jae Hyun Jung, Jae-Hoon Kim, Gwan Gyu Song, Sung Jae Choi
OBJECTIVE: Factor XIII (FXIII) plays role in stabilizing the linkage between fibrins during blood clotting and has been implicated in recurrent pregnancy loss (RPL). The relationship between the Val34Leu polymorphism in F13A1, which encodes the enzymatic subunit of FXIII, and RPL is unclear. The aim of this meta-analysis was to evaluate the association betweenF13A1 Val34Leu and the risk of RPL. STUDY DESIGN: We performed a meta-analysis of 11 studies involving 1092 cases and 678 controls using published literature from PubMed and Embase...
June 23, 2017: European Journal of Obstetrics, Gynecology, and Reproductive Biology
https://www.readbyqxmd.com/read/28651441/the-effect-of-ex-vivo-factor-xiii-supplementation-on-clot-formation-in-blood-samples-from-cardiac-and-scoliosis-surgery-patients
#9
Caroline Shams Hakimi, Malin S Carling, Emma C Hansson, Helena Brisby, Camilla Hesse, Vladimir Radulovic, Anders Jeppsson
Excessive perioperative bleeding remains a substantial problem. Factor XIII (FXIII) contributes to clot stability, and it has therefore been suggested that supplementation with FXIII concentrate may improve perioperative hemostasis. We evaluated the effects of increasing doses of FXIII, alone or in combination with fibrinogen or platelet concentrate, in blood samples from 2 considerably different groups of surgical patients: cardiac and scoliosis surgery patients. Whole-blood samples were collected immediately after operation from cardiac and scoliosis surgery patients...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28632435/relative-effects-of-plasma-fibrinogen-concentrate-and-factor-xiii-on-rotem-coagulation-profiles-in-an-in-vitro-model-of-massive-transfusion-in-trauma
#10
David E Schmidt, Märit Halmin, Agneta Wikman, Anders Östlund, Anna Ågren
Massive traumatic haemorrhage is aggravated through the development of trauma-induced coagulopathy, which is managed by plasma transfusion and/or fibrinogen concentrate administration. It is yet unclear whether these treatments are equally potent in ensuring adequate haemostasis, and whether additional factor XIII (FXIII) administration provides further benefits. In this study, we compared ROTEM whole blood coagulation profiles after experimental massive transfusion with different transfusion regimens in an in vitro model of dilution- and transfusion-related coagulopathy...
June 20, 2017: Scandinavian Journal of Clinical and Laboratory Investigation
https://www.readbyqxmd.com/read/28596376/cre-lox-studies-identify-resident-macrophages-as-the-major-source-of-circulating-coagulation-factor-xiii-a
#11
COMPARATIVE STUDY
Cora M L Beckers, Kingsley R Simpson, Kathryn J Griffin, Jane M Brown, Lih T Cheah, Kerrie A Smith, Jean Vacher, Paul A Cordell, Mark T Kearney, Peter J Grant, Richard J Pease
OBJECTIVE: To establish the cellular source of plasma factor (F)XIII-A. APPROACH AND RESULTS: A novel mouse floxed for the F13a1 gene, FXIII-A(flox/flox) (Flox), was crossed with myeloid- and platelet-cre-expressing mice, and cellular FXIII-A mRNA expression and plasma and platelet FXIII-A levels were measured. The platelet factor 4-cre.Flox cross abolished platelet FXIII-A and reduced plasma FXIII-A to 23±3% (P<0.001). However, the effect of platelet factor 4-cre on plasma FXIII-A was exerted outside of the megakaryocyte lineage because plasma FXIII-A was not reduced in the Mpl(-/-) mouse, despite marked thrombocytopenia...
August 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/28594476/fibrinogen-mahdia-a-congenitally-abnormal-fibrinogen-characterized-by-defective-fibrin-polymerization
#12
Y Amri, H Jouini, M Becheur, R Dabboubi, B Mahjoub, T Messaoud, M T Sfar, A Casini, P de Moerloose, N E H Toumi
INTRODUCTION: Congenital dysfibrinogenemia is a rare qualitative fibrinogen deficiency. Molecular defects that result in dysfibrinogenemia are usually caused by mutations which affect fibrinopeptide release, fibrin polymerization, fibrin cross-linking or fibrinolysis. AIM: Here, we investigated the genetic basis of hypodysfibrinogenemia in two Tunisian siblings with major bleeding. METHODS: Coagulation-related tests were performed on the patients and their family members...
June 8, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28581691/recombinant-factor%C3%A2-xiii-prophylaxis-is-safe-and-effective-in-young-children-with-congenital-factor-xiii-a-deficiency-international-phase%C3%A2-3b-trial-results
#13
B A Kerlin, A Inbal, A Will, M Williams, M-L Garly, L Jacobsen, S L Kearney
Essentials Prophylaxis is the standard of care for congenital factor XIII-A (FXIII-A) deficiency. Six children with FXIII-A deficiency received once-monthly prophylaxis with recombinant FXIII-A. Prophylaxis was well tolerated and no anti-FXIII antibodies were detected. Prophylaxis was effective with an annualized bleeding rate of zero. SUMMARY: Background Factor XIII deficiency is a rare, severe congenital bleeding disorder. Monthly prophylaxis with recombinant FXIII A-Subunit (rFXIII) has demonstrated favorable safety and efficacy in patients aged ≥ 6 years, and may similarly benefit younger children...
June 5, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28578513/the-role-of-fv-1691g-a-fii-20210g-a-mutations-and-mthfr-677c-t-1298a-c-and-103g-t-fxiii-gene-polymorphisms-in-pathogenesis-of-intraventricular-hemorrhage-in-infants-born-before-32-weeks-of-gestation
#14
Dawid Szpecht, Janusz Gadzinowski, Agnieszka Seremak-Mrozikiewicz, Grażyna Kurzawińska, Krzysztof Drews, Marta Szymankiewicz
BACKGROUND: Congenital thrombophilia is associated with an increased intraventricular hemorrhage (IVH) risk among newborns, but it may also play a protective role. The role of genetic polymorphisms involved in the coagulation pathway of IVH pathogenesis is probably a consequence of an increased risk of thrombosis in the fine blood vessels in the germinal matrix region. MATERIAL AND METHODS: The aim of this study was to evaluate the possible relationship between Factor V (FV) 1691G>A, Factor II (FII) 20210G>A mutations and methylenetetrahydrofolate reductase (MTHFR) 677C>T; 1298A>C and Factor XIII (FXIII) 103G>T gene polymorphisms and the occurrence of IVH in 100 infants born from 24 + 0 to 32 + 0 weeks of gestation, born from singleton pregnancy, before 32 + 0 weeks of gestation, exposed to antenatal steroid therapy, and without congenital abnormalities...
July 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28523449/expression-of-coagulation-factor-xiii-subunit-a-correlates-with-outcome-in-childhood-acute-lymphoblastic-leukemia
#15
Bettina Kárai, Zsuzsanna Hevessy, Eszter Szánthó, László Csáthy, Anikó Ujfalusi, Katalin Gyurina, István Szegedi, János Kappelmayer, Csongor Kiss
Previously we identified B-cell lineage leukemic lymphoblasts as a new expression site for subunit A of blood coagulation factor XIII (FXIII-A). On the basis of FXIII-A expression, various subgroups of B-cell precursor acute lymphoblastic leukemia (BCP-ALL) can be identified. Fifty-five children with BCP-ALL were included in the study. Bone marrow samples were obtained by aspiration and the presence of FXIII-A was detected by flow cytometry. G-banding and fluorescent in situ hybridization was performed according to standard procedures...
May 18, 2017: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/28520207/comparison-of-f13a1-gene-mutations-in-73-patients-treated-with-recombinant-fxiii-a2
#16
V Ivaškevičius, A Biswas, M-L Garly, J Oldenburg
INTRODUCTION: Congenital factor XIII (FXIII) deficiency is a rare, autosomal recessive bleeding disorder usually caused by mutations in the F13A1 gene that produce a severe quantitative (type I) deficiency of the FXIII-A subunit. AIM: To determine the genotypes of patients with severe FXIII-A deficiency treated with recombinant FXIII-A subunit (rFXIII-A2 ) participating in three international efficacy and safety trials. METHODS: We determined the genotypes of 73 patients in total; 32 had already undergone genotype analysis and were known to carry F13A1 mutations that have been previously reported in the literature...
May 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28516512/defective-%C3%AE-2-antiplasmin-cross-linking-and-thrombus-stability-in-a-case-of-acquired-factor-xiii-deficiency
#17
Joanne L Mitchell, Sonja Wright, Sajida Kazi, Henry G Watson, Nicola J Mutch
Acquired factor XIII (FXIII) deficiency is a rare and life-threatening condition that is often misdiagnosed or missed completely. A 72-year-old woman presented with symptoms of major unprovoked bleeding but routine coagulation screening tests and platelet count were normal. Low activated FXIII (FXIIIa) activity levels and abnormal urea clot stability led to diagnosis of acquired FXIII deficiency. A modified Bethesda inhibitor titre of 1.6 Bethesda units/ml indicated the presence of a FXIII inhibitor. Bleeding responded to a single dose of FXIII concentrate and immunosuppression with prednisolone induced remission...
May 17, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28503124/factor-xiii-deficiency-and-thrombocytopenia-are-frequent-modulators-of-postoperative-clot-firmness-in-a-surgical-intensive-care-unit
#18
Sarah von Rappard, Corina Hinnen, Roger Lussmann, Manuela Rechsteiner, Wolfgang Korte
OBJECTIVE: Fibrinogen and factor XIII (FXIII) have been shown to critically influence clot firmness in the intraoperative setting and thus likely influence intraoperative bleeding. We were interested to identify potential modulators of postoperative clot firmness in a tertiary care hospital surgical intensive care unit setting, independent of their clinical course during surgery. METHODS: 272 day-shift consecutive patients were evaluated for whole blood clot firmness evaluated by the ROTEM® EXTEM thrombelastometric assay and various potential modulators of clot firmness upon arrival at the surgical intensive care unit (SICU)...
April 2017: Transfusion Medicine and Hemotherapy
https://www.readbyqxmd.com/read/28421433/modification-of-the-catalytic-subunit-of-plasma-fibrin-stabilizing-factor-under-induced-oxidation
#19
A D Vasilyeva, A V Bychkova, A E Bugrova, M I Indeykina, A P Chikunova, V B Leonova, E A Kostanova, M I Biryukova, M L Konstantinova, A S Kononikhin, E N Nikolaev, M A Rosenfeld
For the first time, by using mass-spectrometry method, the oxidation-mediated modification of the catalytic FXIII-A subunit of plasma fibrin-stabilizing factor, pFXIII, has been studied. The oxidative sites were identified to belong to all structural elements of the catalytic subunit: the β-sandwich (Tyr104, Tyr117, and Cys153), the catalytic core domain (Met160, Trp165, Met266, Cys328, Asp352, Pro387, Arg409, Cys410, Tyr442, Met475, Met476, Tyr482, and Met500), the β-barrel 1 (Met596), and the β-barrel 2 (Met647, Pro676, Trp692, Cys696, and Met710), which correspond to 3...
January 2017: Doklady. Biochemistry and Biophysics
https://www.readbyqxmd.com/read/28412719/combined-measurement-of-factor-xiii-and-d-dimer-is-helpful-for-differential-diagnosis-in-patients-with-suspected-pulmonary-embolism
#20
Ning Tang, Ziyong Sun, Dengju Li, Jun Yang, Shiyu Yin, Qing Guan
BACKGROUND: D-dimer has been used to rule out pulmonary embolism (PE). Based on previous reports of decreased concentrations of coagulation factor XIII (FXIII) in venous thromboembolism, and no change in FXIII concentration in patients with acute cardiovascular disease, we evaluated the benefit of simultaneously measuring D-dimer and FXIII concentrations for diagnosing PE. METHODS: In this prospective single-center study, we enrolled 209 patients initially suspected of having PE, and measured their D-dimer and FXIII concentrations...
April 17, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
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