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https://www.readbyqxmd.com/read/29095761/molecular-diagnosis-of-factor-xiii-deficiency-data-from-comprehensive-coagulation-laboratory-in-iran
#1
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...
November 1, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29080382/factor-xiii-co-treatment-with-hemostatic-agents-in-hemophilia-a-increases-fibrin-%C3%AE-chain-crosslinking
#2
Joan D Beckman, Lori A Holle, Alisa S Wolberg
BACKGROUND: Hemophilia A results from the absence, deficiency, or inhibition of factor VIII (FVIII). Bleeding is treated with hemostatic agents (FVIII, recombinant activated factor VIIa [rFVIIa], anti-inhibitor coagulation complex [FEIBA], or recombinant porcine FVIII [rpFVIII]). Despite treatment, some patients have prolonged bleeding. Factor XIII-A2 B2 (FXIII) is a protransglutaminase. During clot contraction, thrombin-activated FXIII (FXIIIa) crosslinks fibrin and α2 -antiplasmin, which promotes red blood cell retention and increases clot stability and weight...
October 28, 2017: 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
#3
Amal Ahmed Abd El-Fatah, 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...
October 17, 2017: Gene
https://www.readbyqxmd.com/read/29028293/a-large-case-series-on-surgical-outcomes-in-congenital-fxiii-deficiency-patients-in-iran
#4
Majid Naderi, Sezaneh Haghpanah, Ghasem Miri-Aliabad, Hakimeh Tavosi, Mehran Karimi
BACKGROUND: The lack of accepted standardized surgical guidelines leads to dependence on the treating physicians' and centers' experiences. AIM: Our aim is to evaluate the surgical outcomes on a large group of congenital FXIII deficiency (FXIIID) patients. METHODS: A case series study was conducted on congenital FXIIID patients in two major referral centers located in Iran from 2010 to 2016 prior to surgery. All patients were on prophylaxis using plasma factor XIII concentrate (10 U kg(-1) , every 28 days) except three patients...
October 13, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29027765/factor-xiii-deficiency-diagnosis-challenges-and-tools
#5
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...
October 13, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28969316/perioperative-management-of-rare-coagulation-factor-deficiency-states-in-cardiac-surgery
#6
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
https://www.readbyqxmd.com/read/28939369/selective-plasma-exchange
#7
REVIEW
Atsushi Ohkubo, Tomokazu Okado
Selective plasma exchange (SePE) is a new modality of simple plasma exchange that uses a selective membrane plasma separator Evacure EC-4A10 (EC-4A) (Kawasumi Laboratories Inc., Tokyo, Japan). EC-4A has a relatively small pore size of 0.03μm, which is around one-tenth that of conventional plasma separators. The sieving coefficients of albumin, immunoglobulin G (IgG), factor XIII (FXIII), and fibrinogen using EC-4A have been shown to be 0.73, 0.5, 0.17, and 0, respectively. Therefore, one session of SePE can remove approximately 50% of IgG regardless of the IgG subclasses while retaining coagulation factors, such as FXIII and fibrinogen...
August 30, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28926188/minimal-factor%C3%A2-xiii-activity-level-to-prevent-major-spontaneous-bleeds-reply
#8
LETTER
M Menegatti, R Palla, P Bucciarelli, F Peyvandi
No abstract text is available yet for this article.
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
#9
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
#10
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/28894750/factor-xiii-subunit-a-in-the-skin-applications-in-diagnosis-and-treatment
#11
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
#12
LETTER
A Dorgalaleh, S H Tabibian, M Safa, M Shams, M Naderi
No abstract text is available yet for this article.
September 4, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28865246/factor-xiii-levels-and-factor-xiii-b-subunit-polymorphisms-in-patients-with-venous-thromboembolism
#13
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/28804836/assessment-of-factor-xiii
#14
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/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...
October 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...
November 2017: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
https://www.readbyqxmd.com/read/28707410/treatment-of-an-acquired-factor-xiii-inhibitor-in-an-adolescent-with-systemic-lupus-erythematosus-and-renal-failure
#18
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...
September 2017: Transfusion
https://www.readbyqxmd.com/read/28688221/minimal-factor-xiii-activity-level-to-prevent-major-spontaneous-bleeds
#19
M Menegatti, R Palla, M Boscarino, P Bucciarelli, L Muszbek, E Katona, M Makris, F Peyvandi
Essentials A strong association between bleeding severity and FXIII activity level (FXIII:C) was shown. The range 5-30 IU dL(-1) of FXIII:C was associated with a high variability of bleeding severity. The PROspective study confirmed the association between FXIII:C activity and bleeding severity. A FXIII: C of 15 IU dL(-1) is a proposed target to start prophylaxis for prevention of major bleeding. SUMMARY: Background Congenital factor XIII (FXIII) deficiency is a rare bleeding disorder associated with significant bleeding manifestations...
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
#20
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...
October 2017: Biochimica et Biophysica Acta
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