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https://www.readbyqxmd.com/read/27913544/treatment-of-rare-factor-deficiencies-in-2016
#1
Flora Peyvandi, Marzia Menegatti
Rare bleeding disorders (RBDs) are a heterogeneous group of coagulation disorders characterized by fibrinogen, prothrombin, factors V, VII, X, XI, or XIII (FV, FVII, FX, FXI, or FXIII, respectively), and the combined factor V + VIII and vitamin K-dependent proteins deficiencies, representing roughly 5% of all bleeding disorders. They are usually transmitted as autosomal, recessive disorders, and the prevalence of the severe forms could range from 1 case in 500 000 for FVII up to 1 in 2-3 million for FXIII in the general population...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27902939/factor-xiii-deficiency-enhances-thrombin-generation-due-to-impaired-fibrin-polymerization-an-effect-corrected-by-factor-xiii-replacement
#2
Hanna H Pitkänen, Annukka Jouppila, Marja Lemponen, Minna Ilmakunnas, Jouni Ahonen, Riitta Lassila
INTRODUCTION: Factor XIII (FXIII) cross-links fibrin, completing blood coagulation. Congenital FXIII deficiency is managed with plasma-derived FXIII (pdFXIII) or recombinant FXIII (rFXIII) concentrates. AIM: As the mechanisms protecting patients with low FXIII levels (<5IU/dL) from spontaneous bleeds remain unknown we assessed the interplay between thrombin generation (TG), fibrin formation and clot kinetics before and after FXIII administration in three patients with FXIII deficiency...
November 16, 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27894217/diagnosis-clinical-manifestations-and-management-of-rare-bleeding-disorders-in-iran
#3
Akbar Dorgalaleh, Sayed Ezatolla Rafiee Alavi, Shadi Tabibian, Shahrzad Soori, Es'hagh Moradi, Taregh Bamedi, Mansour Asadi, Masumeh Jalalvand, Morteza Shamsizadeh
BACKGROUND: Rare bleeding disorders (RBDs) are heterogeneous disorders, mostly inherited in an autosomal recessive pattern. Iran is a Mideast country with a high rate of consanguinity that has a high rate of RBDs. OBJECTIVE: In this study, we present prevalence and clinical presentation as well as management and genetic defects of Iranian patients with RBDs. METHODS: For this study, all relevant publications were searched in Medlin until 2015...
November 28, 2016: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/27879471/molecular-basis-of-congenital-factor-xiii-deficiency-in-iran
#4
Akbar Dorgalaleh, Vahideh Assadollahi, Shadi Tabibian, Morteza Shamsizadeh
Factor XIII deficiency (FXIIID) is an extremely rare autosomal recessive disorder that has the highest incidence in Iran. The FXIIID is primarily due to mutations in the FXIII-A gene, most of which are unique. In the current study, we report all identified mutations among Iranian patients. Among 483 patients, 366 (75.8%) were molecularly analyzed; 11 different mutations were observed. Of 11, 8 (72.7%) are missense, whereas the remaining 3 (27.3%) are deletion/insertion. Among these patients, 347 (94.9%) had the unique mutation of c...
November 21, 2016: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/27843132/recurrent-post-tonsillectomy-secondary-hemorrhage-in-patients-with-factor-xiii-deficiency-a-case-series-and-review-of-literature
#5
Zaid Abu Rajab ALtamimi, Rashid Sheikh, Hassen Omar, Hayam Al Taweel, Shanmugam Ganesan
BACKGROUND Post-tonsillectomy hemorrhage (PTH) has been reported in the literature as a serious complication after tonsillectomy that has high morbidity and can be life threatening. In cases of recurrent secondary PTH, one should consider coagulopathies as the hidden pathology. Factor XIII deficiency is very rare, suggested to be present 1 in 2 million people. Patients with undiagnosed factor XIII deficiency with secondary PTH are extremely rare.  CASE REPORT We report on the cases of six patients (four adults and two children) who presented with recurrent attacks of secondary PTH...
November 15, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27821352/regulation-of-plasma-factor-xiii-levels-in-healthy-individuals-a-major-impact-by-subunit-b-intron-k-c-1952-144-c-g-polymorphism
#6
Zoltán A Mezei, Éva Katona, Judit Kállai, Zsuzsanna Bereczky, Éva Molnár, Bettina Kovács, Éva Ajzner, Zsuzsa Bagoly, Tünde Miklós, László Muszbek
BACKGROUND: The regulation of plasma factor XIII (FXIII) levels in healthy individuals has been only partially explored. The identification of major non-genetic and genetic regulatory factors might provide important information on the contribution of FXIII to the risk of cardio/cerebrovascular diseases. OBJECTIVES: To determine the effect of age, smoking, BMI, fibrinogen concentration on plasma FXIII activity, complex FXIII antigen (FXIII-A2B2) and total FXIII-B subunit (tFXIII-B) level, to correlate FXIII-B level with the other two FXIII parameters and to assess the variation of FXIII levels in carriers of major FXIII subunit polymorphisms...
October 27, 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27810492/molecular-initiating-events-of-the-intersex-phenotype-low-dose-exposure-to-17%C3%AE-ethinylestradiol-rapidly-regulates-molecular-networks-associated-with-gonad-differentiation-in-the-adult-fathead-minnow-testis
#7
April Feswick, Jennifer R Loughery, Meghan A Isaacs, Kelly R Munkittrick, Christopher J Martyniuk
Intersex, or the presence of oocytes in the testes, has been documented in fish following exposure to wastewater effluent and estrogenic compounds. However, the molecular networks underlying the intersex condition are not completely known. To address this, we exposed male fathead minnows to a low, environmentally-relevant concentration of 17alpha-ethinylestradiol (EE2) (15ng/L) and measured the transcriptome response in the testis after 96h to identify early molecular initiating events that may proceed the intersex condition...
December 2016: Aquatic Toxicology
https://www.readbyqxmd.com/read/27804939/-the-morphology-typical-combinations-of-polymorphic-genes-of-hemostasis-and-specific-features-of-the-pathogenesis-of-retrochorial-hematoma-in-missed-abortion
#8
A P Milovanov, N B Kuznetsova, I O Bushtyreva
AIM: to identify mutations and hemostatic gene polymorphisms typical for retrochorial hematoma (RCH) and to study its pathogenesis in missed abortion. SUBJECTS AND METHODS: A PCR assay was used to detect the genetic forms of thrombophilia in 270 patients with ultrasonographically verified RCH. Logistic regression analysis revealed that with the F7 (proconvertin, coagulation factor (CF) VII G10976A polymorphism or with the F13 (fibrinase, CF XIII) G>T, or FGB (fibrinogen β-chain) G455A polymorphism, the risk of RCH was 2...
2016: Arkhiv Patologii
https://www.readbyqxmd.com/read/27797129/alternative-agents-to-prophylactic-platelet-transfusion-for-preventing-bleeding-in-people-with-thrombocytopenia-due-to-chronic-bone-marrow-failure-a-meta-analysis-and-systematic-review
#9
REVIEW
Michael Desborough, Andreas V Hadjinicolaou, Anna Chaimani, Marialena Trivella, Paresh Vyas, Carolyn Doree, Sally Hopewell, Simon J Stanworth, Lise J Estcourt
BACKGROUND: People with thrombocytopenia due to bone marrow failure are vulnerable to bleeding. Platelet transfusions have limited efficacy in this setting and alternative agents that could replace, or reduce platelet transfusion, and are effective at reducing bleeding are needed. OBJECTIVES: To compare the relative efficacy of different interventions for patients with thrombocytopenia due to chronic bone marrow failure and to derive a hierarchy of potential alternative treatments to platelet transfusions...
October 31, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27784620/fibrinogen-splice-variation-and-cross-linking-effects-on-fibrin-structure-function-and-role-of-fibrinogen-%C3%AE-as-thrombomobulin-ii
#10
REVIEW
Cédric Duval, Robert A S Ariëns
Fibrin is an important matrix protein that provides the backbone to the blood clot, promoting tissue repair and wound healing. Its precursor fibrinogen is one of the most heterogeneous proteins, with an estimated 1 million different forms due to alterations in glycosylation, oxidation, single nucleotide polymorphisms, splice variation and other variations. Furthermore, ligation by transglutaminase factor XIII (cross-linking) adds to the complexity of the fibrin network. The structure and function of the fibrin network is in part determined by this natural variation in the fibrinogen molecule, with major effects from splice variation and cross-linking...
October 23, 2016: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/27781024/safety-of-factor-xiii-concentrate-analysis-of-more-than-20-years-of-pharmacovigilance-data
#11
Cristina Solomon, Wolfgang Korte, Dietmar Fries, Inna Pendrak, Christine Joch, Albrecht Gröner, Ingvild Birschmann
BACKGROUND: Plasma-derived factor XIII (FXIII) concentrate is an effective treatment for FXIII deficiency. We describe adverse drug reactions (ADRs) reported during pharmacovigilance monitoring of Fibrogammin®/Corifact® and review published safety data. METHODS: Postmarketing safety reports recorded by CSL Behring from June 1993 to September 2013 were analyzed. Clinical studies published during the same period were also reviewed. RESULTS: Commercial data indicated that 1,653,450,333 IU FXIII concentrate were distributed over the review period, equivalent to 1,181,036 doses for a 70 kg patient...
September 2016: Transfusion Medicine and Hemotherapy
https://www.readbyqxmd.com/read/27777753/coagulative-safety-of-epidural-catheters-after-major-upper-gastrointestinal-surgery-advanced-and-routine-coagulation-analysis-in-38-patients
#12
Owain Thomas, Hampus Rein, Karin Strandberg, Ulf Schött
BACKGROUND: The risk of spinal haematoma in patients receiving epidural catheters is estimated using routine coagulation tests, but guidelines are inconsistent in their recommendations on what to do when results indicate slight hypocoagulation. Postoperative patients are prone to thrombosis, and thromboelastometry has previously shown hypercoagulation in this setting. We aimed to better understand perioperative haemostasis by comparing results from routine and advanced tests, hypothesizing that patients undergoing major upper gastrointestinal surgery would be deficient in vitamin K-dependent coagulation factors because of malnutrition, or hypocoagulative because of accumulation of low molecular weight heparin (LMWH)...
2016: Perioperative Medicine
https://www.readbyqxmd.com/read/27759118/factor-xiii-a-transglutaminase-deficient-mice-show-signs-of-metabolically-healthy-obesity-on-high-fat-diet
#13
Vamsee D Myneni, Aisha Mousa, Mari T Kaartinen
F13A1 gene, which encodes for Factor XIII-A blood clotting factor and a transglutaminase enzyme, was recently identified as a potential causative gene for obesity in humans. In our previous in vitro work, we showed that FXIII-A regulates preadipocyte differentiation and modulates insulin signaling via promoting plasma fibronectin assembly into the extracellular matrix. To understand the role of FXIII-A in whole body energy metabolism, here we have characterized the metabolic phenotype of F13a1-/- mice. F13a1-/- and F13a1+/+ type mice were fed chow or obesogenic, high fat diet for 20 weeks...
October 19, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27749008/compaction-of-fibrin-clots-reveals-the-antifibrinolytic-effect-of-factor-xiii-comment
#14
V Gurewich
The interesting article on the antifibrinolytic effect of Factor XIII by Rijken et al [1] was predicated on the assumption that fibrinolysis is mediated only by tPA, since that is what was studied. However, there is evidence that this common assumption should be put into question. In a study published almost thirty years ago, we showed that whereas spontaneous lysis of platelet poor plasma clots was mediated by tPA, that of platelet-rich plasma clots, with which this article was concerned, could not be abolished by tPA antibody, but instead was abolished by the addition of uPA antibody...
October 17, 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27748995/compaction-of-fibrin-clots-reveals-the-antifibrinolytic-effect-of-factor-xiii-reply
#15
D C Rijken, S Abdul, J J M C Malfliet, F W G Leebeek, S Uitte de Willige
We thank Dr. Gurewich [1] for his critical evaluation of the impact of our recently published article on factor XIII [2]. This article describes that mechanical compaction or platelet-mediated retraction of plasma clots is essential to fully reveal the antifibrinolytic effect of factor XIII on tPA-induced plasma clot lysis. Dr. Gurewich argues that we cannot assume that these results are applicable to endogenous fibrinolysis in vivo, because endogenous fibrinolysis does depend not only on tPA, but also on uPA...
October 17, 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27729560/thrombophilic-gene-mutations-in-relation-to-different-manifestations-of-venous-thromboembolism-a-single-tertiary-center-study
#16
Tahir Bezgin, Cihangir Kaymaz, Özgür Akbal, Fatih Yılmaz, Hacer Ceren Tokgöz, Nihal Özdemir
BACKGROUND: Venous thromboembolism (VTE) is a common and potentially lethal disorder that manifests mainly as deep vein thrombosis (DVT) of the extremities or pulmonary embolism (PE) and occurs as a consequence of genetic and environmental risk factors. We aimed to assess the role of inherited thrombophilia as a causative or additive factor in the development of VTE. METHODS: The study included 310 patients (female: 154; mean age: 52.3 ± 16.9 years) with a first episode of VTE and 289 age- and sex-matched healthy controls...
October 11, 2016: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/27728300/an-unusual-case-of-factor-xiii-deficiency
#17
Steffy Kuriakose, K V Chandrashekhar
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27709645/selective-plasma-exchange-can-reduce-auto-antibodies-in-patients-with-bullous-pemphigoid-without-affecting-factor-xiii-and-fibrinogen
#18
Kahori Nasu, Norio Hanafusa, Masaomi Nangaku
Bullous pemphigoid (BP) is an autoimmune blistering skin disorder characterized by circulating serum IgG antibodies against two hemidesmosomal proteins: BP180 and BP230. Fundamentally, immunosuppressive therapies are administered to treat this disease, but plasmapheresis can be added for refractory patients. We experienced the case of a 63-year-old patient with refractory BP for which we administered double filtration plasmapheresis (DFPP). His skin lesions improved along with decreased IgG BP180 antibodies, but factor XIII (FXIII) and fibrinogen were also reduced by DFPP repetition...
October 6, 2016: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/27704645/investigation-of-the-anticoagulant-and-antithrombotic-effects-of-chlorogenic-acid
#19
Jun-Hui Choi, Seung Kim
Thrombosis is a leading cause of morbidity and mortality throughout the world. Thrombolytic agents are important for both the prevention and treatment of thrombosis. Fibrin clot and turbidity assays revealed that it was able to inhibit the formation of fibrin clot. Chlorogenic acid degraded blood clot and inhibited the enzymatic activity of procoagulant proteases, thrombin, activated factor X (FXa), and activated factor XIII (FXIIIa). Chlorogenic acid was found to delay activated partial thromboplastin time, prothrombin time, and thrombin time...
October 5, 2016: Journal of Biochemical and Molecular Toxicology
https://www.readbyqxmd.com/read/27665853/association-of-factor-xiii-val34leu-polymorphism-and-coronary-artery-disease-a-meta-analysis
#20
Jae Hyun Jung, Gwan Gyu Song, Jae-Hoon Kim, Young Ho Seo, Sung Jae Choi
BACKGROUND: Factor XIII plays an important role in the stabilization of the linkage between fibrins and in the pathophysiology of coronary artery disease (CAD). The association between factor XIII Val34Leu polymorphism and CAD risk remains controversial. METHODS: We conducted a meta-analysis of 36 studies involving 26,940 cases and 34,694 controls. Subgroup analyses were performed with division of data into disease (myocardial infarction [MI], CAD without MI), age, and sex...
September 26, 2016: Cardiology Journal
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