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https://www.readbyqxmd.com/read/28707410/treatment-of-an-acquired-factor-xiii-inhibitor-in-an-adolescent-with-systemic-lupus-erythematosus-and-renal-failure
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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...
June 8, 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/28594476/fibrinogen-mahdia-a-congenitally-abnormal-fibrinogen-characterized-by-defective-fibrin-polymerization
#8
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-xiii-prophylaxis-is-safe-and-effective-in-young-children-with-congenital-fxiii-a-deficiency-international-phase-3b-trial-results
#9
Bryce A Kerlin, Aida Inbal, Andrew Will, Michael Williams, May-Lill Garly, Lotte Jacobsen, Susan L Kearney
BACKGROUND: Factor XIII deficiency is a rare, severe congenital bleeding disorder. Monthly prophylaxis with recombinant factor XIII (rFXIII) has demonstrated favorable safety and efficacy in patients ≥6 years and may similarly benefit younger children. OBJECTIVE: To evaluate the long-term safety and efficacy of rFXIII in children <6 years with congenital FXIII A-subunit deficiency. PATIENTS/METHODS: Six children, who previously completed a single-dose pharmacokinetic trial of rFXIII, received 35 IU kg(-1) rFXIII every 28 (± 2) days for a minimum of 52 weeks and were evaluated for bleeding and adverse events...
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
https://www.readbyqxmd.com/read/28406553/detection-of-factor-xiii-deficiency-data-from-multicentre-exercises-amongst-uk-neqas-and-pro-rbdd-project-laboratories
#17
I Jennings, S Kitchen, M Menegatti, R Palla, I Walker, M Makris, F Peyvandi
INTRODUCTION: FXIII deficiency is a rare bleeding disorders, and specific FXIII assays are recommended to detect this deficiency. We investigated the performance and accuracy of FXIII investigations in two exercises, comparing centres enrolled in the PRO-RBDD project (prospective data collection on patients with fibrinogen and Factor XIII deficiencies), and UK NEQAS BC centres. METHODS: Samples from a FXIII deficient subject and a normal donor were sent to participating centres, to investigate for FXIII deficiency, and interpret their results...
April 13, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28387755/transglutaminases-factor-xiii-a-and-tg2-regulate-resorption-adipogenesis-and-plasma-fibronectin-homeostasis-in-bone-and-bone-marrow
#18
Aisha Mousa, Cui Cui, Aimei Song, Vamsee D Myneni, Huifang Sun, Jin Jin Li, Monzur Murshed, Gerry Melino, Mari T Kaartinen
Appropriate bone mass is maintained by bone-forming osteoblast and bone-resorbing osteoclasts. Mesenchymal stem cell (MSC) lineage cells control osteoclastogenesis via expression of RANKL and OPG (receptor activator of nuclear factor κB ligand and osteoprotegerin), which promote and inhibit bone resorption, respectively. Protein crosslinking enzymes transglutaminase 2 (TG2) and Factor XIII-A (FXIII-A) have been linked to activity of myeloid and MSC lineage cells; however, in vivo evidence has been lacking to support their function...
May 2017: Cell Death and Differentiation
https://www.readbyqxmd.com/read/28380473/successful-management-of-a-patient-with-autoimmune-hemorrhaphilia-due-to-anti-factor-xiii-13-antibodies-complicated-by-pulmonary-thromboembolism
#19
Yoshiyuki Ogawa, Kunio Yanagisawa, Masayoshi Souri, Masahiro Mihara, Chiaki Naito, Makiko Takizawa, Takuma Ishizaki, Takeki Mitsui, Hiroshi Handa, Tsukasa Osaki, Yoshihisa Nojima, Akitada Ichinose
Autoimmune hemophilia-like disease (hemorrhaphilia) due to anti-factor XIII (FXIII) antibodies (AH13) is a very rare, life-threatening bleeding disorder. A 77-year-old woman developed macrohematuria and a right renal pelvic hematoma. The coagulation times were not prolonged, but FXIII activity and antigen levels were severely and moderately reduced to 9 and 29% of normal values, respectively. Accordingly, the FXIII-specific activity turned out to be low. FXIII inhibitor and anti-FXIII-A subunit autoantibodies were detected by a 1:1 crossmixing test and immunoblot and immunochromatographic assays...
2017: Acta Haematologica
https://www.readbyqxmd.com/read/28371893/tissue-transglutaminase-induction-in-the-pressure-overloaded-myocardium-regulates-matrix-remodeling
#20
Arti V Shinde, Marcin Dobaczewski, Judith J de Haan, Amit Saxena, Kang-Kon Lee, Ying Xia, Wei Chen, Ya Su, Waqas Hanif, Inderpreet Kaur Madahar, Victor M Paulino, Gerry Melino, Nikolaos G Frangogiannis
Aims: : Tissue transglutaminase (tTG) is induced in injured and remodeling tissues, and modulates cellular phenotype, while contributing to matrix cross-linking. Our study tested the hypothesis that tTG may be expressed in the pressure-overloaded myocardium, and may regulate cardiac function, myocardial fibrosis and chamber remodeling. Methods and results: In order to test the hypothesis, wildtype and tTG null mice were subjected to pressure overload induced through transverse aortic constriction...
March 28, 2017: Cardiovascular Research
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