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https://www.readbyqxmd.com/read/29781127/removal-dynamics-of-autoantibodies-immunoglobulins-and-coagulation-factors-by-selective-plasma-exchange-on-three-consecutive-days
#1
Satoko Miyamoto, Atsushi Ohkubo, Hiroshi Seshima, Hiroko Yamamoto, Ayako Itagaki, Takuma Maeda, Naoki Kurashima, Takayasu Mori, Soichiro Iimori, Shotaro Naito, Eisei Sohara, Tatemitsu Rai, Shinichi Uchida, Tomokazu Okado
Selective plasma exchange has been shown to be effective in various diseases, but no studies have assessed the benefits of daily treatment. We aimed to investigate the removal dynamics of immunoglobulins, fibrinogen, and factor XIII on three consecutive days in three patients. For mean processed plasma volumes of 1.06 × plasma volume, reductions of 79.6%, 49.3%, and 8.6% were seen for immunoglobulins G, A, and M, respectively. The reductions for fibrinogen and factor XIII were 18.4% and 13.0%, respectively...
May 20, 2018: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/29769590/low-factor-xiii-levels-after-intravenous-thrombolysis-predict-short-term-mortality-in-ischemic-stroke-patients
#2
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/29750744/experience-and-prognosis-of-systemic-neonatal-thrombosis-at-a-level-iii-nicu
#3
Sevim Unal, Deniz Gönülal, Betül Siyah Bilgin, Vildan Koşan Çulha, Neşe Yarali
OBJECTIVE: The objective of this article was to evaluate neonates diagnosed systemic thrombosis and their outcomes. METHODS: We retrospectively evaluated data of neonatal systemic thrombosis between January 2011 and December 2016. RESULTS: Among 4376 hospitalized, 30 neonates (0.69%) were diagnosed systemic thrombosis. Their mean birth weight was 2422±1152 g (680 to 4750 g), gestational age was 35±5.4 weeks (25 to 41 wk). There were 25 neonates (83...
May 10, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29741294/synaptotagmin-xiii-expression-and-peritoneal-metastasis-in-gastric-cancer
#4
M Kanda, D Shimizu, H Tanaka, C Tanaka, D Kobayashi, M Hayashi, H Takami, Y Niwa, S Yamada, T Fujii, H Sugimoto, Y Kodera
BACKGROUND: Peritoneal metastasis is a frequent cause of death in patients with gastric cancer. The aim of this study was to identify molecules responsible for mediating peritoneal metastasis of gastric cancer. METHODS: Transcriptome and bioinformatics analyses were conducted to identify molecules associated with peritoneal metastasis. The therapeutic effects of intraperitoneally administered small interfering (si) RNA were evaluated using mouse xenograft models...
May 9, 2018: British Journal of Surgery
https://www.readbyqxmd.com/read/29741250/how-i-investigate-for-bleeding-disorders
#5
REVIEW
C P M Hayward
INTRODUCTION: Laboratory investigations for bleeding disorders are warranted when an individual has a personal and/or family history of bleeding, and/or laboratory findings that suggest the possibility of an inherited or acquired bleeding disorder. METHODS: This review summarizes author's experience with ordering and reporting on diagnostic investigations for common and rare bleeding disorders, with consideration of recent articles on diagnosing bleeding disorders...
May 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29721968/alternative-splicing-for-activation-of-coagulation-factor-xiii-a-in-the-fish-retina-after-optic-nerve-injury
#6
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
#7
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/29665207/recombinant-factor-xiii-a-in-a-patient-with-factor-xiii-deficiency-and-recurrent-pregnancy-loss
#8
M Al-Khabori, A Pathare, M Menegatti, F Peyvandi
Inherited factor XIII deficiency is a very rare bleeding disorder and is one of the causes of recurrent pregnancy loss. The use of plasma derived factor XIII to improve pregnancy outcomes has been reported. We report a 26-year-old lady with factor XIII-A subunit deficiency who was treated with recombinant factor XIII-A and had a successful pregnancy outcome with no pregnancy related complications. Our case illustrates that the dose of recombinant factor XIII-A can be minimized and adjusted using frequent trough level monitoring...
April 17, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29663380/transglutaminase-activity-regulates-differentiation-migration-and-fusion-of-osteoclasts-via-affecting-actin-dynamics
#9
Huifang Sun, Mari T Kaartinen
Osteoclasts, bone resorbing cells, derive from monocyte/macrophage cell lineage. Increased osteoclast activity is responsible for bone destruction in diseases such as osteoporosis, periodontitis and rheumatoid arthritis. Transglutaminases (TGs), protein crosslinking enzymes, were recently found involved in osteoclastogenesis in vivo, however their mechanisms of action have remained unknown. In this study, we have investigated the role of TG activity in osteoclastogenesis in vitro using four TG inhibitors, NC9, Z006, T101, and monodansyl cadaverine...
April 16, 2018: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/29658191/an-international-study-on-the-feasibility-of-a-standardized-combined-plasma-clot-turbidity-and-lysis-assay-communication-from-the-ssc-of-the-isth
#10
M Pieters, H Philippou, A Undas, Z de Lange, D C Rijken, N J Mutch
No abstract text is available yet for this article.
March 13, 2018: Journal of Thrombosis and Haemostasis: JTH
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
#11
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/29649768/the-level-of-circulating-fibroblast-activation-protein-correlates-with-incorporation-of-alpha-2-antiplasmin-into-the-fibrin-clot
#12
Shirley Uitte de Willige, Joyce J C M Malfliet, Shiraazkhan Abdul, Frank W G Leebeek, Dingeman C Rijken
INTRODUCTION: Circulating fibroblast activation protein (cFAP) cleaves alpha-2-antiplasmin (α2AP) N-terminally, converting native Met-α2AP into Asn-α2AP. Previous studies in purified model systems showed that Asn-α2AP is faster incorporated into a fibrin clot by activated factor XIII than Met-α2AP, making the fibrin clot more resistant to fibrinolysis. The objective was to investigate whether cFAP level in plasma associated with the amount of α2AP incorporation into fibrin in a new plasma-based clotting assay...
April 3, 2018: Thrombosis Research
https://www.readbyqxmd.com/read/29575615/spectrum-of-hemostatic-disorders-in-indian-females-presenting-with-bleeding-manifestations
#13
A Gupta, P Mishra, H P Pati, S Tyagi, M Mahapatra, T Seth, R Saxena
INTRODUCTION: Hemostatic disorders are often missed in women with bleeding particularly menorrhagia. Preexisting hemostatic disorders are now known as common risk factor for postpartum hemorrhage and prolonged bleeding in puerperium. Females with bleeding complaints constitute an important population referred to hematology clinic. Hence, we aim to evaluate the type and frequency of hemostatic disorders among females presenting with bleeding in a tertiary care hospital and a basic hemostatic laboratory...
March 25, 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29554044/systemic-hyperfibrinolysis-after-trauma-a-pilot-study-of-targeted-proteomic-analysis-of-superposed-mechanisms-in-patient-plasma
#14
Anirban Banerjee, Christopher C Silliman, Ernest E Moore, Monika Dzieciatskowa, Marguerite Kelher, Angela Sauaia, Kenneth Jones, Michael P Chapman, Eduardo Gonzalez, Hunter B Moore, Angelo D'Alessandro, Erik Peltz, Benjamin E Huebner, Peter Einerson, James Chandler, Arsen Ghasabayan, Kirk Hansen
BACKGROUND: Viscoelastic measurements of hemostasis indicate that 20% of seriously injured patients exhibit systemic hyperfibrinolysis, with increased early mortality. These patients have normal clot formation with rapid clot lysis. Targeted proteomics was applied to quantify plasma proteins from hyperfibrinolytic (HF) patients to elucidate potential pathophysiology. METHODS: Blood samples were collected in the field or at Emergency Department arrival and thrombelastography (TEG) was used to characterize in vitro clot formation under native and tissue plasminogen activator (tPA)-stimulated conditions...
March 16, 2018: Journal of Trauma and Acute Care Surgery
https://www.readbyqxmd.com/read/29536280/a-novel-ultrasonic-method-for-evaluation-of-blood-clotting-parameters
#15
Vacis Tatarunas, Algirdas Voleisis, Reimondas Sliteris, Rymantas Kazys, Liudas Mazeika, Vaiva Lesauskaite
PURPOSE: For long time, blood clot retraction was measured only by thromboelastographic or platelet contractile force measurement techniques. The purpose of the present study was development of a novel ultrasonic method based on simultaneous monitoring of variations in the ultrasound velocity and the frequency spectrum of the signal propagating in clotting blood and its application for automatic evaluation of blood clotting parameters. METHODS: Simultaneous measurement of ultrasound velocity and variations in the frequency spectrum of wideband ultrasonic signals in clotting blood samples was performed...
March 13, 2018: Journal of Medical Ultrasonics
https://www.readbyqxmd.com/read/29484525/effect-of-factor-xiii-levels-and-polymorphisms-on-the-risk-of-myocardial-infarction-in-young-patients
#16
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
#17
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
#18
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
#19
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
#20
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
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