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https://www.readbyqxmd.com/read/28728890/clinical-influence-of-preoperative-factor-xiii-activity-in-patients-undergoing-pancreatoduodenectomy
#1
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/28713823/intracardiac-hemostasis-and-fibrinolysis-parameters-in-patients-with-atrial-fibrillation
#2
Noémi Klára Tóth, Zoltán Csanádi, Orsolya Hajas, Alexandra Kiss, Edina Nagy-Baló, Kitti Bernadett Kovács, Ferenc Sarkady, László Muszbek, Zsuzsanna Bereczky, László Csiba, Zsuzsa Bagoly
AIMS: To identify intracardiac hemostasis or fibrinolysis abnormalities, which are associated with atrial fibrillation (AF) and increase the risk of thromboembolism. PATIENTS AND METHODS: Patient group consisted of 24 patients with AF and control group included 14 individuals with other supraventricular tachycardia undergoing transcatheter radiofrequency ablation. Blood samples were drawn from the femoral vein (FV), left atrium (LA), and left atrial appendage (LAA) before the ablation procedure...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28707410/treatment-of-an-acquired-factor-xiii-inhibitor-in-an-adolescent-with-systemic-lupus-erythematosus-and-renal-failure
#3
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/28704210/factor-xiii-deficiency-in-south-of-tunisia
#4
Ines Maaloul, Moez Medhaffer, Nacim Louhichi, Imen Krichen, Sofien Alibi, Sana Kmiha, Hajer Aloulou, Faiza Fakhfakh, Moez Elloumi, Choumous Kallel, Mongia Hachicha
: Factor XIII deficiency is a rare autosomal recessive disorder of hemostasis characterized by a plasmatic factor XIII level less than 1% in homozygote and bleeding as of the youth. The aim of the study is to describe the clinical features and the outcome of the patients and to determine molecular characteristics. A retrospective study, was conducted on seven patients with factor XIII deficiency in the department of hematology and pediatrics, Hedi Chaker Hospital, Sfax, Tunisia during the period of 14 years (2001-2014)...
July 12, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28688221/minimal-factor-xiii-activity-level-to-prevent-major-spontaneous-bleeds
#5
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
#6
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
#7
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/28674874/a-protocol-for-the-preparation-of-cryoprecipitate-and-cryo-depleted-plasma-for-proteomic-studies
#8
Rosemary L Sparrow, Richard J Simpson, David W Greening
Cryoprecipitate is a concentrate of high-molecular-weight plasma proteins that precipitate when frozen plasma is slowly thawed at 1-6 °C. The concentrate contains factor VIII (antihemophilic factor), von Willebrand factor (vWF), fibrinogen, factor XIII, fibronectin, and small amounts of other plasma proteins. Clinical grade preparations of cryoprecipitate are mainly used to treat fibrinogen deficiency caused by acute bleeding or functional abnormalities of the fibrinogen protein. In the past, cryoprecipitate was used to treat von Willebrand disease and hemophilia A (factor VIII deficiency), but the availability of more highly purified coagulation factor concentrates or recombinant protein preparations has superseded the use of cryoprecipitate for these coagulopathies...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28661086/selective-plasma-exchange-for-the-removal-of-pemphigus-autoantibodies-fibrinogen-and-factor-xiii-in-pemphigus-vulgaris
#9
Satoko Miyamoto, Atsushi Ohkubo, Hiroshi Seshima, Shigeto Komori, Motoki Yamamoto, Takuma Maeda, Ayako Itagaki, Hiroko Yamamoto, Kohei Nojima, Soichiro Iimori, Shotaro Naito, Naoki Kurashima, Eisei Sohara, Tatemitsu Rai, Shinichi Uchida, Tomokazu Okado
Pemphigus vulgaris is a serious autoimmune skin disorder associated with desmoglein 1 and 3. Selective plasma exchange (SePE) for pemphigus vulgaris remains unknown. We investigated the removal characteristics of pemphigus autoantibodies, immunoglobulins, and fibrinogen in three cases. When the mean processed volume for SePE was 1.2 plasma volumes, the mean percent reduction was 50.7% for desmoglein 1, 48.9% for desmoglein 3, 50.3% for IgG, 29.8% for IgA, 1.9% for IgM, and 17.6% for fibrinogen. In one case, the percent reduction after four sessions of SePE within eight days was 87...
June 2017: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/28653811/biochemical-characterization-of-medaka-oryzias-latipes-orthologue-for-mammalian-factor-xiii-and-establishment-of-gene-edited-mutant
#10
Rima Horimizu, Ryota Ogawa, Yuko Watanabe, Hideki Tatsukawa, Masato Kinoshita, Hisashi Hashimoto, Kiyotaka Hitomi
At the final process of blood coagulation, fibrin molecules are stabilized via a catalytic reaction by Factor XIIIA (FXIIIA), one of the family members of transglutaminase (TGase) that catalyzes protein cross-linking reactions. In this study, we characterized the orthologue of this enzyme in medaka (Oryzias latipes), an established model fish in which a coagulation system is also preserved. The recombinant protein of this orthologue enzyme was produced in baculovirus-infected insect cells and used for biochemical property analysis including activation by thrombin-proteolysis and calcium-dependence of the TGase enzymatic activity...
June 27, 2017: FEBS Journal
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
#11
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
#12
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
#13
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/28581691/recombinant-factor-xiii-prophylaxis-is-safe-and-effective-in-young-children-with-congenital-fxiii-a-deficiency-international-phase-3b-trial-results
#14
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/28580719/platelets-stored-at-4%C3%A2-c-contribute-to-superior-clot-properties-compared-to-current-standard-of-care-through-fibrin-crosslinking
#15
Prajeeda M Nair, Shaunak G Pandya, Shatha F Dallo, Kristin M Reddoch, Robbie K Montgomery, Heather F Pidcoke, Andrew P Cap, Anand K Ramasubramanian
Currently, platelets for transfusion are stored at room temperature (RT) for 5-7 days with gentle agitation, but this is less than optimal because of loss of function and risk of bacterial contamination. We have previously demonstrated that cold (4°C) storage is an attractive alternative because it preserves platelet metabolic reserves, in vitro responses to agonists of activation, aggregation and physiological inhibitors, as well as adhesion to thrombogenic surfaces better than RT storage. Recently, the US Food and Drug Administration clarified that apheresis platelets stored at 4°C for up to 72 h may be used for treating active haemorrhage...
July 2017: British Journal of Haematology
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
#16
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/28574019/k-time-maximum-amplitude-of-thromboelastogram-predict-post-central-venous-cannulation-bleeding-in-patients-with-cirrhosis-a-pilot-study
#17
Chandra K Pandey, Vandana Saluja, Kumar Gaurav, Manish Tandon, Vijay K Pandey, Ajeet S Bhadoria
BACKGROUND & OBJECTIVES: Coagulation and haemostasis are dynamic processes. The haemostatic changes in liver disease affect all aspects of coagulation. The prothrombin time (PT)/ international normalized ratio (INR) was developed to monitor oral anticoagulant therapy and the activated partial thromboplastin time to investigate inheritable single factor deficiencies. Viscoelastic tests such as thromboelastogram (TEG) give information about dynamics of clot formation (coagulation factor and anticoagulant activity), clot strength (platelets and fibrinogen) and clot stability (finbrinolysis and factor XIII)...
January 2017: Indian Journal of Medical Research
https://www.readbyqxmd.com/read/28542848/change-of-transfusion-and-treatment-paradigm-in-major-trauma-patients
#18
P Stein, A Kaserer, K Sprengel, G A Wanner, B Seifert, O M Theusinger, D R Spahn
Trauma promotes trauma-induced coagulopathy, which requires urgent treatment with fixed-ratio transfusions of red blood cells, fresh frozen plasma and platelet concentrates, or goal-directed administration of coagulation factors based on viscoelastic testing. This retrospective observational study compared two time periods before (2005-2007) and after (2012-2014) the implementation of changes in trauma management protocols which included: use of goal-directed coagulation management; admission of patients to designated trauma centres; whole-body computed tomography scanning on admission; damage control surgery; permissive hypotension; restrictive fluid resuscitation; and administration of tranexamic acid...
May 23, 2017: Anaesthesia
https://www.readbyqxmd.com/read/28523449/expression-of-coagulation-factor-xiii-subunit-a-correlates-with-outcome-in-childhood-acute-lymphoblastic-leukemia
#19
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
#20
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
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