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https://www.readbyqxmd.com/read/29310686/comparison-of-two-different-coagulation-algorithms-on-the-use-of-allogenic-blood-products-and-coagulation-factors-in-severely-injured-trauma-patients-a-retrospective-multicentre-observational-study
#1
Alexander Kaserer, Mattias Casutt, Kai Sprengel, Burkhardt Seifert, Donat R Spahn, Philipp Stein
BACKGROUND: At the University Hospital Zurich (USZ) and the Cantonal Hospital of Lucerne (LUKS) an individualized goal-directed coagulation and transfusion algorithm was introduced and implemented before 2012 (Coagulation algorithm of the USZ: USZ-Alg; of the LUKS: LUKS-Alg). Main differences between both algorithms are: 1) A target haematocrit-range of 0.21-0.24 (USZ-Alg) vs. a lower haematocrit limit only (LUKS-Alg). 2) Blind coagulation-package in selected cases (LUKS-Alg only). 3) Factor XIII substitution is considered earlier according to the USZ-Alg...
January 8, 2018: Scandinavian Journal of Trauma, Resuscitation and Emergency Medicine
https://www.readbyqxmd.com/read/29307277/pathogenicity-analysis-of-variations-and-prenatal-diagnosis-in-a-hereditary-coagulation-factor-xiii-deficiency-family
#2
Liwei Sun, Qijiang Yan, Yonghua Wang, Hualei Luo, Peng Du, Reem Hassan, Li Liu, Weiying Jiang
OBJECTIVES: Prenatal diagnosis (PND) procedure is urgent to be established for timely management and fatal consequence prevention of factor XIII deficiency (FXIIID), and variations data among Chinese are very scanty. We aimed to find a novel mutation among Chinese and establish a rapid and precise PND procedure with pathogenicity analysis to contribute to the prevention of postpartum hemorrhage in pregnant women and central nervous system bleeding in newborns. METHODS: FXIIID was diagnosed by qualitative and quantitative tests of clot solubility test and enzyme-linked immunosorbent assay, respectively...
January 7, 2018: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29165739/inhibitors-in-patients-with-congenital-bleeding-disorders-other-than-hemophilia
#3
Massimo Franchini, Giuseppe Marano, Carlo Mengoli, Vanessa Piccinini, Simonetta Pupella, Stefania Vaglio, Giancarlo Maria Liumbruno
The most worrying complication of replacement therapy for severe hemophilia A and B is currently the occurrence of inhibitory alloantibodies against infused factor VIII and factor IX, respectively. Inhibitors compromise the management of hemorrhage in affected patients, with a considerable increase in complications, disability, and costs. While these alloantibodies have been extensively studied in the past years in hemophilia A and B, those occurring in patients with other inherited bleeding disorders are less well characterized and still poorly understood, mostly due to the rarity of these hemorrhagic conditions...
November 17, 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/29095761/molecular-diagnosis-of-factor-xiii-deficiency-data-from-comprehensive-coagulation-laboratory-in-iran
#4
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-cotreatment-with-hemostatic-agents-in-hemophilia-a-increases-fibrin-%C3%AE-chain-crosslinking
#5
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])...
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
#6
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/29053871/cellular-and-morphological-characterization-of-blastoderms-from-freshly-laid-broiler-eggs
#7
N Pokhrel, E Ben-Tal Cohen, O Genin, D Sela-Donenfeld, Y Cinnamon
The pioneering study of Eyal-Giladi and Kochav (EG&K; Eyal-Giladi and Kochav, 1976) on the early developmental stages-from fertilization, through oviposition, to the gastrulation process-set the standard for characterizing chicken embryos, and has been used in numerous studies over the years. During uterine development, the chicken embryo undergoes dramatic changes, extremely rapid cell cycles, massive cell death, and axial determination processes. However, once the egg is laid, the temperature drops and the embryo enters into a diapause-like state...
October 5, 2017: Poultry Science
https://www.readbyqxmd.com/read/29028293/a-large-case-series-on-surgical-outcomes-in-congenital-fxiii-deficiency-patients-in-iran
#8
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
#9
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/28947991/plasma-protein-profiling-in-patients-undergoing-coronary-artery-bypass-grafting-surgery-and-clinical-significance
#10
Zhi-Peng Guo, Hai-Tao Hou, Rui Jing, Zhen-Guo Song, Xiao-Cheng Liu, Guo-Wei He
This study was designed to identify the protein profiling in patients with triple vessel coronary artery disease (CAD) undergoing CABG, in order to detect CAD-related differential proteins in these patients. CABG patients with triple vessel disease with/without left main stenosis (n =160) were compared to normal coronary angiographic subjects (n =160). Plasma samples of 20 males and 20 females in each group were analyzed with iTRAQ technique. ELISA test was used to test the chosen proteins from iTRAQ results in plasma samples from a new cohort of the CABG group (n=120, male/femal=61/59) and control (n =120, male/female=60/60)...
September 1, 2017: Oncotarget
https://www.readbyqxmd.com/read/28939369/selective-plasma-exchange
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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/28868204/impact-of-low-coagulation-factor-xiii-activity-in-patients-with-chronic-subdural-hematoma-associated-with-cerebrospinal-fluid-hypovolemia-a-retrospective-study
#18
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
#19
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
#20
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
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