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https://www.readbyqxmd.com/read/28926188/minimal-factor-xiii-activity-level-to-prevent-major-spontaneous-bleeds-reply
#1
M Menegatti, R Palla, P Bucciarelli, F Peyvandi
We thank Dr. Dorgalaleh et al. for their interest in our report regarding the minimal Factor XIII (FXIII) activity level to prevent major spontaneous bleedings [1] and for describing their experience using a low dose prophylaxis in patients with severe FXIII deficiency [2]. As reported by the authors, primary prophylaxis is mandatory for all patients with severe congenital FXIII deficiency, since the diagnosis, to prevent spontaneous life-threatening bleeding. It is well known that the main cause of death or disability in these patients is intracranial haemorrhage (ICH), occurring either spontaneously or after minor trauma, therefore an early prophylaxis is crucial [3]...
September 19, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28915348/activation-of-factor-xiii-is-accompanied-by-a-change-in-oligomerization-state
#2
Boris A Anokhin, Vilius Stribinskis, William L Dean, Muriel C Maurer
Factor XIII A (FXIIIA) is a member of the transglutaminase enzyme family that cross-links both intra- and extracellular protein substrates. To prevent undesired cross-linking, FXIIIA is expressed as an inactive zymogen and exists intracellularly as an A2 homodimer. In plasma, FXIII A2 is complexed with two protective factor XIII B subunits (A2 B2 ) that dissociate upon activation of the zymogen. Based on limited experimental data, activated FXIII was considered a dimer of two catalytically active A subunits...
September 15, 2017: FEBS Journal
https://www.readbyqxmd.com/read/28901996/intracranial-hemorrhage-in-congenital-bleeding-disorders
#3
Shadi Tabibian, Hoda Motlagh, Majid Naderi, Akbar Dorgalaleh
: Intracranial hemorrhage (ICH), as a life-threatening bleeding among all kinds of congenital bleeding disorders (CBDs), is a rare manifestation except in factor XIII (FXIII) deficiency, which is accompanied by ICH, early in life, in about one-third of patients. Most inherited platelet function disorders (IPFDs) are mild to moderate bleeding disorders that can never experience a severe bleeding as in ICH; however, Glanzmann's thrombasthenia, a common and severe inherited platelet function disorder, can lead to ICH and occasional death...
September 9, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28898896/intracranial-hemorrhage-a-devastating-outcome-of-congenital-bleeding-disorders-prevalence-diagnosis-and-management-with-a-special-focus-on-congenital-factor-xiii-deficiency
#4
Seyed Ezatolla Rafiee Alavi, Masumeh Jalalvand, Vahideh Assadollahi, Shadi Tabibian, Akbar Dorgalaleh
No abstract text is available yet for this article.
September 12, 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/28894750/factor-xiii-subunit-a-in-the-skin-applications-in-diagnosis-and-treatment
#5
REVIEW
Lilla Paragh, Daniel Törőcsik
The role of factor XIII subunit A (FXIII-A) is not restricted to hemostasis. FXIII-A is also present intracellularly in several human cells and serves as a diagnostic marker in a wide range of dermatological diseases from inflammatory conditions to malignancies. In this review, we provide a guide on the still controversial interpretation of dermal cell types expressing FXIII-A and assess the previously described mechanisms behind their accumulation under physiological and pathological conditions of the human skin...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28871658/minimal-factor-xiii-activity-level-to-prevent-major-spontaneous-bleeds-comment
#6
Akbar Dorgalaleh, Shadi Tabibian, Majid Safa, Mahmood Shams, Majid Naderi
In a recent publication by Menegatti et al. on patients with congenital factor XIII (FXIII) deficiency, an effort was made to suggest a cut-off to start prophylaxis in order to prevent spontaneous major bleeds, including central nervous system (CNS) bleeding, gastrointestinal (GI) bleeding, hematoma and hemarthrosis (grade III bleeding according to EN-RBD classification) (1). Fifteen IU/dL FXIII coagulant activity (FXIII:C) was proposed as a cut-off for this purpose. Surprisingly, in this study, ~50% of patients with FXIII:C between the lower detection limit and 29 IU/dL, experienced these bleeds and 36% of those with FXIII:C ≥ 30% had minor spontaneous bleeding (1)...
September 4, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28868204/impact-of-low-coagulation-factor-xiii-activity-in-patients-with-chronic-subdural-hematoma-associated-with-cerebrospinal-fluid-hypovolemia-a-retrospective-study
#7
Takafumi Shimogawa, Takato Morioka, Tetsuro Sayama, Tomoaki Akiyama, Sei Haga, Toshiyuki Amano, Yoshihiko Furuta, Kei Murao, Shuji Arakawa, Iwao Takeshita
BACKGROUND: Cerebrospinal fluid hypovolemia (CSFH) is sometimes associated with chronic subdural hematomas (CSHs). Affected patients often develop enlargement and recurrence of the CSH, even if appropriate treatments such as epidural blood patch (EBP) and/or burr-hole surgery for the CSH are performed. This situation may lead to subclinical coagulopathy, including low coagulation factor XIII (CFXIII) activity. We retrospectively analyzed whether CFXIII activity was involved in the development of CSHs and post-treatment exacerbation of CSHs in patients with CSFH...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28865246/factor-xiii-levels-and-factor-xiii-b-subunit-polymorphisms-in-patients-with-venous-thromboembolism
#8
Zoltán A Mezei, Éva Katona, Judit Kállai, Zsuzsanna Bereczky, Laura Somodi, Éva Molnár, Bettina Kovács, Tünde Miklós, Éva Ajzner, László Muszbek
BACKGROUND: The association of plasma factor XIII (FXIII) level with venous thromboembolism (VTE) is still controversial and the effect of sex and FXIII B subunit (FXIII-B) polymorphisms in this respect have not been explored. OBJECTIVES: 1/ To determine FXIII activity and antigen levels in patients with a history of VTE and how they are influenced by sex and FXIII-B polymorphisms. 2/ To explore the association of FXIII levels and FXIII-B polymorphisms with the risk of VTE...
August 26, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28821815/epac1-deficient-mice-have-bleeding-phenotype-and-thrombocytes-with-decreased-gpib%C3%AE-expression
#9
Gyrid Nygaard, Lars Herfindal, Kathrine S Asrud, Ronja Bjørnstad, Reidun K Kopperud, Eystein Oveland, Frode S Berven, Lene Myhren, Erling A Hoivik, Turid Helen Felli Lunde, Marit Bakke, Stein O Døskeland, Frode Selheim
Epac1 (Exchange protein directly activated by cAMP 1) limits fluid loss from the circulation by tightening the endothelial barrier. We show here that Epac1(-/-) mice, but not Epac2(-/-) mice, have prolonged bleeding time, suggesting that Epac1 may limit fluid loss also by restraining bleeding. The Epac1(-/-) mice had deficient in vitro secondary hemostasis. Quantitative comprehensive proteomics analysis revealed that Epac1(-/-) mouse platelets (thrombocytes) had unbalanced expression of key components of the glycoprotein Ib-IX-V (GPIb-IX-V) complex, with decrease of GP1bβ and no change of GP1bα...
August 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28817543/plasma-levels-of-the-a-subunit-of-factor-xiii-in-patients-undergoing-off-pump-coronary-artery-bypass-surgery
#10
Artur Słomka, Piotr Korbal, Aleksandra Piekuś, Wojciech Pawliszak, Lech Anisimowicz, Ewa Żekanowska
No abstract text is available yet for this article.
August 9, 2017: Polish Archives of Internal Medicine
https://www.readbyqxmd.com/read/28804836/assessment-of-factor-xiii
#11
László Muszbek, Éva Katona, Adrienne Kerényi
Blood coagulation factor XIII (FXIII) is essential for maintaining hemostasis. The absence of FXIII results in severe bleeding diathesis, which without prophylaxis frequently leads to fatal bleeding. As the usual hemostasis screening tests remain normal, the diagnosis of FXIII deficiency needs specific tests. Here, we describe FXIII activity determination by the ammonia release assay, which is the first-line test in the diagnostic algorithm for FXIII deficiency. The method for another activity test, the undeservedly rarely used fibrin cross-linking assay, is also presented...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28803708/anti-phospholipid-syndrome-current-opinion-on-mechanisms-involved-laboratory-characterization-and-diagnostic-aspects
#12
REVIEW
Jean Amiral, Marie Peyrafitte, Claire Dunois, Anne Marie Vissac, Jerard Seghatchian
Anti-phospholipid syndrome is a complex and severe clinical situation, associated with symptoms such as recurrent thrombosis, arterial or venous, at any site, pregnancy loss, and other related syndromes. These clinical burdens, are highly variable from patient to patient, and are associated with biological abnormalities, such as the presence of the Lupus Anticoagulant or phospholipid dependent antibodies, confirmed on two occasions at least 12 weeks apart. From the diagnosis standpoint, both, functional (clotting) or immunological assays, are difficult to standardize and to optimize, due to the absence of reference material, or a characteristic clinical group, and international reference preparations...
July 15, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28801214/lobar-hemorrhage-induced-by-acquired-factor-xiii-deficiency-in-a-patient-with-cerebral-amyloid-angiopathy
#13
Hidetaka Arishima, Hiroyuki Neishi, Ken-Ichiro Kikuta, Mihoko Morita, Naoko Hosono, Takahiro Yamauchi, Masayoshi Souri, Akitada Ichinose
A 68-year-old man presented with intracranial hemorrhage in the right frontal lobe, which rapidly increased the day after admission. We performed hematoma removal with a biopsy of the cortex around the hematoma. The day after the operation, a subcutaneous hematoma over the craniotomy appeared, and the computed tomography showed a recurrent hemorrhage with an acute subdural hematoma. We were aware of a bleeding tendency, and a detailed hematologic examination by hematologists revealed autoimmune acquired factor XIII deficiency due to an antifactor XIII antibody...
August 8, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28766854/potential-misdiagnosis-of-dysfibrinogenaemia-data-from-multicentre-studies-amongst-uk-neqas-and-pro-rbdd-project-laboratories
#14
I Jennings, S Kitchen, M Menegatti, R Palla, I Walker, F Peyvandi, M Makris
INTRODUCTION: Mutations in fibrinogen (Fgn) genes, causing dysfibrinogenaemia, can result in either a bleeding or thrombophilic diathesis. Dysfibrinogenaemia is infrequently encountered in hospital laboratories, and the utility of different assays in the diagnosis of dysfibrinogenaemia has not previously been explored in a multicentre study. We describe here an exercise in which PRO-RBDD project (prospective data collection on patients with fibrinogen and Factor XIII deficiencies) centres, and UK NEQAS centres, performed investigations for dysfibrinogenaemia...
August 2, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28761875/inhibition-of-fibrinolysis-by-coagulation-factor-xiii
#15
REVIEW
Dingeman C Rijken, Shirley Uitte de Willige
The inhibitory effect of coagulation factor XIII (FXIII) on fibrinolysis has been studied for at least 50 years. Our insight into the underlying mechanisms has improved considerably, aided in particular by the discovery that activated FXIII cross-links α2-antiplasmin (α2AP) to fibrin. In this review, the most important effects of different cross-linking reactions on fibrinolysis are summarized. A distinction is made between fibrin-fibrin cross-links studied in purified systems and fibrin-α2AP cross-links studied in plasma or whole blood systems...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28734018/influence-of-cryoprecipitate-factor-xiii-and-fibrinogen-concentrate-on-hyperfibrinolysis
#16
Melissa M Cushing, Meghann M Fitzgerald, Rebecca M Harris, Lars M Asmis, Thorsten Haas
BACKGROUND: Hyperfibrinolysis is a potentially life-threatening condition associated with poor clot integrity and excessive bleeding. Although antifibrinolytics are an effective treatment, more liberal use of these drugs may lead to a prothrombotic risk, and an earlier and potentially safer treatment option would be desirable. Hyperfibrinolysis has been shown to be attenuated by in vitro supplementation of purified human Factor (F)XIII concentrate. Cryoprecipitate represents an alternative source of FXIII and the only approved source of concentrated FXIII in some countries...
July 21, 2017: Transfusion
https://www.readbyqxmd.com/read/28728890/clinical-influence-of-preoperative-factor-xiii-activity-in-patients-undergoing-pancreatoduodenectomy
#17
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
#18
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
#19
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
#20
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)...
September 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
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