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https://www.readbyqxmd.com/read/29769590/low-factor-xiii-levels-after-intravenous-thrombolysis-predict-short-term-mortality-in-ischemic-stroke-patients
#1
Edina Gabriella Székely, Katalin Réka Czuriga-Kovács, Zsuzsanna Bereczky, Éva Katona, Zoltán András Mezei, Attila Nagy, Noémi Klára Tóth, Ervin Berényi, László Muszbek, László Csiba, Zsuzsa Bagoly
In this observational study we investigated whether levels of factor XIII (FXIII) and its major polymorphisms affect the outcome of thrombolysis by recombinant tissue plasminogen activator (rtPA) in acute ischemic stroke (AIS) patients. Study cohort included 132 consecutive AIS patients undergoing i.v. thrombolysis within 4.5 h of symptom onset. Blood samples taken on admission, immediately after and 24 h after therapy were analyzed for FXIII activity and antigen levels. FXIII-A p.Val34Leu, p.Tyr204Phe, FXIII-B p...
May 16, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29747114/effect-of-double-filtration-plasmapheresis-for-antibody-mediated-rejection-on-hemostasis-parameters-and-thrombin-generation
#2
R Marlu, P Malvezzi, L Seyve, T Jouve, J Maurizi, F Defendi, P L Carron, M Christophe, A Le Gouellec, B Polack, L Rostaing
INTRODUCTION: Donor-specific alloantibodies (DSAs) cause kidney-allograft loss in chronic antibody-mediated rejection (CAMR). Treatment relies on blocking antibody-producing cells and removing DSAs by apheresis: e.g., double-filtration plasmapheresis (DFPP). MATERIALS AND METHODS: To determine the impact of DFPP (6 or 8 sessions/patient) on clotting factors and natural anticoagulants, and on thrombin generation, we performed a prospective and observational study in five CAMR kidney-transplant patients who received DFPP plus rituximab therapy...
April 20, 2018: Thrombosis Research
https://www.readbyqxmd.com/read/29738861/oxidation-induced-modifications-of-the-catalytic-subunits-of-plasma-fibrin-stabilizing-factor-at-the-different-stages-of-its-activation-identified-by-mass-spectrometry
#3
Alexandra Vasilyeva, Lyubov' Yurina, Maria Indeykina, Anna Bychkova, Anna Bugrova, Marina Biryukova, Alexey Kononikhin, Evgene Nikolaev, Mark Rosenfeld
Plasma fibrin-stabilizing factor (pFXIII) is a heterotetrameric proenzyme composed of two catalytic A subunits (FXIII-A2 ) and two inhibitory/carrier B subunits (FXIII-B2 ). The main function of the protein is the formation of cross-links between the polypeptide chains of the fibrin clot. The conversion of pFXIII into the enzymatic form FXIII-A2 * is a multistage process. Like many other blood plasma proteins, pFXIII is an oxidant-susceptible target. The influence of distinct sites susceptible to oxidation-mediated modifications on the changes in the structural-functional characteristics of the protein remains fully unexplored...
May 5, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29721968/alternative-splicing-for-activation-of-coagulation-factor-xiii-a-in-the-fish-retina-after-optic-nerve-injury
#4
Kayo Sugitani, Yoshiki Koriyama, Kazuhiro Ogai, Ayako Furukawa, Satoru Kato
Factor XIII-A (FXIII-A), which has become known as cellular transglutaminase, plays important roles in mediating cross-linking reactions in various tissues. FXIII-A acts as one of the regeneration molecules in the fish retina and optic nerve after optic nerve injury and becomes activated at the site of injury within a few hours. Previous research has shown that activated FXIII-A induces neurite outgrowth from injured retinal ganglion cells and supports elongation of the regenerating optic nerve. However, the activation mechanism of FXIII-A remains unknown...
2018: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29675848/the-role-of-barrels-1-and-2-in-the-enzymatic-activity-of-factor-xiii-a
#5
E L Hethershaw, Penelope J Adamson, Kerrie A Smith, Whitney N Goldsberry, Richard J Pease, Sheena E Radford, Peter J Grant, Robert A S Ariëns, Muriel C Maurer, Helen Philippou
BACKGROUND: Factor XIII (FXIII) is composed of an activation peptide segment, a β-sandwich domain, a catalytic core, and finally β-barrels 1 and 2. FXIII is activated following cleavage of its A-subunits by thrombin. The resultant transglutaminase activity leads to increased resistance of fibrin clots to fibrinolysis. OBJECTIVES: To assess the functional roles of β-barrels 1 and 2 in FXIII, we expressed and characterised the full-length FXIII-A subunit (FXIII-A) and variants truncated to residue 628 [truncated to β-barrel 1 (TB1)], 515 [truncated to catalytic core (TCC)] and 184 [truncated to β-sandwich (TBS)]...
April 19, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29658346/association-between-thrombophilia-gene-polymorphisms-and-recurrent-pregnancy-loss-risk-in-the-iranian-population
#6
Razieh Bigdeli, Mohammad Reza Younesi, Erfan Panahnejad, Vahid Asgary, Samaneh Heidarzadeh, Hoda Mazaheri, Samira Louni Aligoudarzi
Miscarriage is the most common complication in pregnancy. Considering the importance of the problem thrombophilia in pregnant women and its association with recurrent pregnancy loss (RPL), analysis of polymorphisms of genes involved in thrombophilia can be useful. We investigated the frequency and association between ten polymorphisms of seven thrombophilia genes and RPL in an Iranian population. This case-control study was conducted on 200 women with recurrent pregnancy loss and also on 200 women with at least one successful pregnancy as the control group...
April 15, 2018: Systems Biology in Reproductive Medicine
https://www.readbyqxmd.com/read/29657559/activation-peptide-of-the-coagulation-factor-xiii-ap-f13a1-as-a-new-biomarker-for-the-screening-of-colorectal-cancer
#7
Julien Peltier, Jean-Pierre Roperch, Stéphane Audebert, Jean-Paul Borg, Luc Camoin
Background: Colorectal cancer (CRC) remains a major cause of cancer fatalities in developed countries. The risk of death is correlated to the stage of CRC during the primary diagnosis. Early diagnosis is closely associated with enhanced survival rate. We therefore investigated the AP-F13A1 as a potential protein marker of CRC. Methods: The protein expression of FXIII in 40 serum samples was evaluated by enzyme-linked immunosorbent assays. Additionally, targeted proteomic assays (LC-PRM) were used to evaluate the expression of the activation peptide of F13A1 (AP-F13A1) in a further 113 serum samples...
2018: Clinical Proteomics
https://www.readbyqxmd.com/read/29484525/effect-of-factor-xiii-levels-and-polymorphisms-on-the-risk-of-myocardial-infarction-in-young-patients
#8
László Balogh, Éva Katona, Zoltán A Mezei, Judit Kállai, Réka Gindele, István Édes, László Muszbek, Zoltán Papp, Zsuzsanna Bereczky
Factor XIII (FXIII) stabilizes and protects the fibrin network. Its role in myocardial infarction (MI) is still to be clarified. To evaluate the association of FXIII levels with MI in young patients and to investigate how the FXIII-A p.Val34Leu, FXIII-B p.His95Arg, and IVS11, c.1952 + 144 C>G (Intron K) polymorphisms influence FXIII levels and MI risk. Patients with ST elevation MI below 40 years of age (MI, n = 119), age-matched clinical controls (CC, n = 101) without MI and coronary artery disease, and healthy controls (HC, n = 120) were investigated for FXIII activity, FXIII-A2 B2 , FXIII-B concentrations and for the polymorphisms...
February 26, 2018: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/29476647/prenatal-diagnosis-in-rare-bleeding-disorders-an-unresolved-issue
#9
REVIEW
S Tabibian, M Shams, M Naderi, A Dorgalaleh
Intracranial haemorrhage (ICH) is the most dreadful complication, and the main cause of death among patients with rare bleeding disorders (RBD) and prenatal diagnosis (PND) is a preventative lifesaving program. A total of 39 PNDs were reported in the literature through a search on PubMed, EMBASE, SCOPUS and Web of Science databases, most often for congenital factor (F) XIII and FVII deficiencies and rarely in FX, FV deficiencies and afibrinogenemia. The main cause to request a PND is ICH and related morbidity and mortality...
February 24, 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29460500/auto-and-alloantibodies-against-factor-xiii-laboratory-diagnosis-and-clinical-consequences
#10
REVIEW
L Muszbek, K Pénzes, É Katona
Acquired FXIII deficiencies caused by autoantibodies against FXIII subunits represent rare but very severe bleeding diatheses. Alloantibodies in FXIII-deficient patients also cause life-threatening bleeding complications, but they develop extremely rarely. In this review we provide an overview of the diagnosis and classification of anti-FXIII antibodies and analyze 48 patients with autoimmune FXIII deficiency and four additional FXIII-deficient patients who developed anti-FXIII alloantibody. The patients were collected from peer-reviewed publications from which relevant data could be extracted...
May 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29448295/recombinant-fxiii-rfxiii-a2-prophylaxis-prevents-bleeding-and-allows-for-surgery-in-patients-with-congenital-fxiii-a-subunit-deficiency
#11
Manuel Carcao, Carmen Altisent, Giancarlo Castaman, Katsuyuki Fukutake, Bryce A Kerlin, Craig Kessler, Riitta Lassila, Diane Nugent, Johannes Oldenburg, May-Lill Garly, Anders Rosholm, Aida Inbal
Recombinant factor XIII-A2 (rFXIII-A2 ) was developed for prophylaxis and treatment of bleeds in patients with congenital FXIII A-subunit deficiency. mentor™2 (NCT00978380), a multinational, open-label, single-arm, multiple-dosing extension to the pivotal mentor™1 trial, assessed long-term safety and efficacy of rFXIII-A2 prophylaxis in eligible patients (patients with severe [<0.05 IU/mL] congenital FXIII subunit A deficiency) aged ≥6 years. Patients received 35 IU/kg rFXIII-A2 (exact dosing) every 28 ± 2 days for ≥52 weeks...
March 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29446989/comparison-of-the-efficacy-of-two-human-fibrinogen-concentrates-to-treat-dilutional-coagulopathy-in-vitro
#12
Thorsten Haas, Melissa M Cushing, Lars M Asmis
Both congenital and acquired fibrinogen deficiency can be safely treated with administration of fibrinogen concentrate. The aim of this study was to test the efficacy of a new fibrinogen product (Fibryga) compared to a licensed product (Haemocomplettan) in an in vitro model of dilutional coagulopathy. Ten blood specimens from healthy volunteers were diluted 1:1 with balanced crystalloid solution and subsequently supplemented with each fibrinogen concentrate at a dose replicating in vivo supplementation (50 mg kg-1 )...
May 2018: Scandinavian Journal of Clinical and Laboratory Investigation
https://www.readbyqxmd.com/read/29378352/atherothrombosis-and-thromboembolism-position-paper-from-the-second-maastricht-consensus-conference-on-thrombosis
#13
H M H Spronk, T Padro, J E Siland, J H Prochaska, J Winters, A C van der Wal, J J Posthuma, G Lowe, E d'Alessandro, P Wenzel, D M Coenen, P H Reitsma, W Ruf, R H van Gorp, R R Koenen, T Vajen, N A Alshaikh, A S Wolberg, F L Macrae, N Asquith, J Heemskerk, A Heinzmann, M Moorlag, N Mackman, P van der Meijden, J C M Meijers, M Heestermans, T Renné, S Dólleman, W Chayouâ, R A S Ariëns, C C Baaten, M Nagy, A Kuliopulos, J J Posma, P Harrison, M J Vries, H J G M Crijns, E A M P Dudink, H R Buller, Y M C Henskens, A Själander, S Zwaveling, O Erküner, J W Eikelboom, A Gulpen, F E C M Peeters, J Douxfils, R H Olie, T Baglin, A Leader, U Schotten, B Scaf, H M M van Beusekom, L O Mosnier, L van der Vorm, P Declerck, M Visser, D W J Dippel, V J Strijbis, K Pertiwi, A J Ten Cate-Hoek, H Ten Cate
Atherothrombosis is a leading cause of cardiovascular mortality and long-term morbidity. Platelets and coagulation proteases, interacting with circulating cells and in different vascular beds, modify several complex pathologies including atherosclerosis. In the second Maastricht Consensus Conference on Thrombosis, this theme was addressed by diverse scientists from bench to bedside. All presentations were discussed with audience members and the results of these discussions were incorporated in the final document that presents a state-of-the-art reflection of expert opinions and consensus recommendations regarding the following five topics: 1...
February 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29344582/factor-xiii-in-plasma-but-not-in-platelets-mediates-red-blood-cell-retention-in-clots-and-venous-thrombus-size-in-mice
#14
Sravya Kattula, James R Byrnes, Sara M Martin, Lori A Holle, Brian C Cooley, Matthew J Flick, Alisa S Wolberg
The transglutaminase factor XIII (FXIII) stabilizes clots against mechanical and biochemical disruption and is essential for hemostasis. In vitro and in vivo models of venous thrombosis demonstrate that FXIII mediates clot size by promoting red blood cell (RBC) retention. However, the key source of FXIII and whether FXIII activity can be reduced to suppress thrombosis without imposing deleterious hemostatic consequences are 2 critical unresolved questions. FXIII is present in multiple compartments, including plasma (FXIIIplasma ) as a heterotetramer of A2 and B2 subunits and platelets (FXIIIplt ) as an A2 homodimer...
January 9, 2018: Blood Advances
https://www.readbyqxmd.com/read/29095761/molecular-diagnosis-of-factor-xiii-deficiency-data-from-comprehensive-coagulation-laboratory-in-iran
#15
Maryam Gheidishahran, Akbar Dorgalaleh, Shadi Tabibian, Mahmood Shams, Esmaeil Sanei Moghaddam, Sohaila Khosravi, Majid Naderi, Sara Kahraze, Fereshte Lotfi, Ahmad Kazeme, Majid Safa
: Diagnosis of factor XIII (FXIII) deficiency (FXIIID) as a rare bleeding disorder is a challenge worldwide. Thus, in the present study, we used different methods including two molecular methods for detection of FXIIID. This study was conducted on individuals suspected to FXIIID. All individuals were checked by two routinely used methods of clot solubility test in Iran and two other clot solubility tests as well as FXIII activity and antigen assays. Molecular analysis was performed by PCR-restriction fragment length polymorphism (PCR-RFLP) and tetra-primer amplification refractory mutation system (T-ARMS)-PCR for only FXIIID mutation in southeast Iran (p...
January 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29080382/factor-xiii-cotreatment-with-hemostatic-agents-in-hemophilia-a-increases-fibrin-%C3%AE-chain-crosslinking
#16
J D Beckman, L A Holle, A S Wolberg
Essentials Factor XIII (FXIII)-mediated fibrin crosslinking is delayed in hemophilia. We determined effects of FXIII cotreatment with hemostatic agents on clot parameters. FXIII cotreatment accelerated FXIII activation and crosslinking of fibrin and α2 -antiplasmin. These data provide biochemical rationale for FXIII cotreatment in hemophilia. SUMMARY: Background Hemophilia A results from the absence, deficiency or inhibition of factor VIII. Bleeding is treated with hemostatic agents (FVIII, recombinant activated FVII [rFVIIa], anti-inhibitor coagulation complex [FEIBA], or recombinant porcine FVIII [rpFVIII])...
January 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29054763/association-of-genetic-variants-of-hemostatic-genes-with-myocardial-infarction-in-egyptian-patients
#17
Amal Ahmed Abd El-Fattah, Nermin Abdel Hamid Sadik, Heba Sedrak, Ahmed Battah, Mai Nabil
Hemostatic genes polymorphisms are well known to be associated with venous thrombosis, but their association with arterial thrombosis especially myocardial infarction (MI) remains to be clarified. We investigated the role of three hemostatic gene polymorphisms, prothrombin G20210A, factor XIII (FXIII) Val34Leu (G/T), and fibrinogen-β-455G/A and their coexistence in Egyptian patients with MI. The possible correlation of these polymorphisms with plasma fibrinogen level was also evaluated. The study included 120 patients with MI and 60 healthy volunteers...
January 30, 2018: Gene
https://www.readbyqxmd.com/read/29028293/a-large-case-series-on-surgical-outcomes-in-congenital-factor-xiii-deficiency-patients-in-iran
#18
M Naderi, S Haghpanah, G Miri-Aliabad, H Tavosi, M Karimi
Essentials Data on surgery in factor XIII (FXIII) deficiency patients are scarce and lack standardized guidelines. Variable dosage of 10-50 U kg-1 was given to FXIII deficiency patients undergoing surgery. Surgical outcomes showed excellent hemostasis with a minimal risk of post-operative complications. Surgery can be performed safely in FXIII deficiency patients following FXIII administration. SUMMARY: Background The lack of accepted standardized surgical guidelines leads to dependence on the treating physicians' and centers' experiences...
December 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29027765/factor-xiii-deficiency-diagnosis-challenges-and-tools
#19
REVIEW
M Karimi, F Peyvandi, M Naderi, A Shapiro
Factor XIII deficiency (FXIIID) is a rare hereditary bleeding disorder arising from heterogeneous mutations, which can lead to life-threatening hemorrhage. The diagnosis of FXIIID is challenging due to normal standard coagulation assays requiring specific FXIII assays for diagnosis, which is especially difficult in developing countries. This report presents an overview of FXIIID diagnosis and laboratory methods and suggests an algorithm to improve diagnostic efficiency and prevent missed or delayed FXIIID diagnosis...
February 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28969316/perioperative-management-of-rare-coagulation-factor-deficiency-states-in-cardiac-surgery
#20
E R Strauss, M A Mazzeffi, B Williams, N S Key, K A Tanaka
Rare bleeding disorders (RBDs) include the hereditary deficiency of fibrinogen, factor (F)II, FV, FV + FVIII, FVII, FX, FXI or FXIII. RBDs do not confer a protective effect against atheromatous plaque formation, and thus the need for cardiovascular (CV) surgery in RBD patients is expected to increase with improved healthcare access (diagnosis and management) and longevity of the population. Clinical data regarding the management of RBDs in this setting are sparse, but the perioperative care team is obliged to gain a better understanding on available biological and pharmacological hemostatic agents...
September 1, 2017: British Journal of Anaesthesia
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