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factor XIII

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https://www.readbyqxmd.com/read/28187047/is-extracorporeal-co2-removal-really-safe-and-less-invasive-observation-of-blood-injury-and-coagulation-impairment-during-ecco2r
#1
Johannes Kalbhenn, Nadine Neuffer, Barbara Zieger, Axel Schmutz
Extracorporeal CO2-Removal (ECCO2R) is promoted with attributes like "safe" and "less invasive" compared to (high-flow) veno-venous ECMO-Systems. With our experience in coagulation disorders during ECMO-therapy with this observational study we for the first time prospectively evaluate hemolysis and coagulation disorders during ECCO2R. Eight consecutive patients with predominant hypercapnic respiratory failure were treated with the Hemolung® Respiratory Assist System (RAS) (Alung-Technologies, Pittsburg, USA)...
February 7, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28166593/impact-of-rare-bleeding-disorders-during-pregnancy-on-maternal-and-fetal-outcomes-review-of-29-pregnancies-at-a-single-center
#2
Gokcen Orgul, Fatih Aktoz, M Sinan Beksac
Objective This study aims to give information about the relationship between different types of factor deficiencies and maternal/obstetric outcomes. Methods We retrospectively reviewed the medical records of eight women with factor deficiency disorders. The demographic and clinical features of the patients after their last pregnancies were registered retrospectively. Results There were 29 pregnancies among the 8 patients. The spontaneous abortion rate was relatively high in two patients with factor XIII deficiency (80% and 57...
February 6, 2017: Revista Brasileira de Ginecologia e Obstetrícia
https://www.readbyqxmd.com/read/28159767/ticagrelor-reversal-in-vitro-assessment-of-four-haemostatic-agents
#3
Leyla Calmette, Anne-Céline Martin, Bernard Le Bonniec, Diane Zlotnik, Isabelle Gouin-Thibault, Christilla Bachelot-Loza, Pascale Gaussem, Anne Godier
AIM: Management of ticagrelor-induced bleeding is challenging as platelet transfusion is ineffective. An effective strategy is needed. This study aimed to investigate in vitro the efficacy of four haemostatic drugs (HDs), namely recombinant activated factor VII (rFVIIa), fibrinogen concentrate (Fib), tranexamic acid (TXA) and factor XIII concentrate (FXIII) to improve the haemostatic capacity in the presence of ticagrelor. METHODS: Blood was spiked with ticagrelor then supplemented by either HD or control...
February 3, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28137667/identification-of-novel-autoantigens-via-mass-spectroscopy-based-antibody-mediated-identification-of-autoantigens-ms-amida-using-immune-thrombocytopenic-purpura-itp-as-a-model-disease
#4
Julian Kamhieh-Milz, Viktor Sterzer, Hatice Celik, Omid Khorramshahi, Reham Fadl Hassan Moftah, Abdulgabar Salama
Immune thrombocytopenic purpura (ITP) is one of the best characterized autoimmune diseases. Autoantibodies (AABs) against platelet antigens are considered as the diagnostic hallmark of ITP, but are detectable in only 50% of patients. We designed and applied a novel proteomic approach termed Mass Spectroscopy-based Antibody-Mediated Identification of Autoantigens (MS-AMIDA) for platelet antigens. Patients were separated into patients with classical AABs [ITP(+)] and patients without AABs [ITP(-)]. Altogether, 181 potential AAGs were found in ITP(+) and 135 AAGs in ITP(-), with 34 and 23 AAGs reproducibly found in two runs of MS-AMIDA...
January 27, 2017: Journal of Proteomics
https://www.readbyqxmd.com/read/28129465/evaluation-of-coagulation-during-treatment-with-directly-acting-antivirals-in-patients-with-hepatitis-c-virus-related-cirrhosis
#5
Armando Tripodi, Roberta D'Ambrosio, Lidia Padovan, Giulia Tosetti, Alessio Aghemo, Massimo Primignani, Veena Chantarangkul, Flora Peyvandi, Massimo Colombo
BACKGROUND & AIMS: The effect of direct-acting-antivirals (DAA) on coagulation of hepatitis-C-virus (HCV)-related cirrhosis is unknown. METHODS: We investigated 28 patients on DAA treatment and performed prothrombin-time, thrombin generation with and without thrombomodulin, whole-blood thromboelastometry, as well as the individual procoagulants (II, VIII, XIII, von Willebrand) and anticoagulants, antithrombin and protein-C. RESULTS: Patients had undetectable HCV-RNA at the end-of- treatment and at 12-weeks after end-of-treatment (sustained virological response)...
January 27, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28091415/advances-of-coagulation-factor-xiii
#6
REVIEW
Da-Yu Shi, Shu-Jie Wang
OBJECTIVE: To provide a comprehensive literature review on roles of coagulation factor XIII (FXIII) in coagulation, wound healing, neoplasm, bone metabolism, and pregnancy. DATA SOURCES: All articles in PubMed with key words "Coagulation factor XIII", "wound", "leukemia", "tumor", "bone," and "pregnancy" with published date from 2001 to 2016 were included in the study. Frequently cited publications before 2000 were also included. STUDY SELECTION: We reviewed the role of FXIII in biologic processes as documented in clinical, animal, and in vitro studies...
2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28035782/wonoep-appraisal-biomarkers-of-epilepsy-associated-comorbidities
#7
REVIEW
Teresa Ravizza, Filiz Y Onat, Amy R Brooks-Kayal, Antoine Depaulis, Aristea S Galanopoulou, Andrey Mazarati, Adam L Numis, Raman Sankar, Alon Friedman
Neurologic and psychiatric comorbidities are common in patients with epilepsy. Diagnostic, predictive, and pharmacodynamic biomarkers of such comorbidities do not exist. They may share pathogenetic mechanisms with epileptogenesis/ictogenesis, and as such are an unmet clinical need. The objectives of the subgroup on biomarkers of comorbidities at the XIII Workshop on the Neurobiology of Epilepsy (WONOEP) were to present the state-of-the-art recent research findings in the field that highlighting potential biomarkers for comorbidities in epilepsy...
December 30, 2016: Epilepsia
https://www.readbyqxmd.com/read/28028391/vascular-genetic-variants-and-ischemic-stroke-susceptibility-in-albanians-from-the-republic-of-macedonia
#8
Bajram Kamberi, Farije Kamberi, Mirko Spiroski
BACKGROUND: Acute first-ever ischemic stroke (FIS) is a heterogeneous, polygenic disorder. The contribution of vascular genetic variants as inherited causes of ischemic stroke has remained controversial. AIM: To examine the association of genetic variants in vascular factors with the occurrence of FIS. MATERIAL AND METHODS: The current research was performed in a group of 39 patients with FIS (study group) and 102 healthy volunteers (control group)...
December 15, 2016: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/27942785/-factor%C3%A2-xiii-pharmacodynamic-and-pharmacokinetic-characteristics
#9
E H Adam, S Kreuer, K Zacharowski, C F Weber, R Wildenauer
Factor XIII (FXIII) plays an important role in the field of blood coagulation. In the last decade, both congenital and acquired deficiencies have been investigated in clinical studies. FXIII is a versatile enzyme that leads to a covalent cross-linking of fibrin fibrils at the end of the clotting cascade and supports platelet adhesion to the damaged sub-endothelium with the result of a mechanically stable clot.Symptoms of FXIII deficiencies vary within a broad spectrum from superficial skin bleeding episodes to severe, sometimes life threatening hemorrhage, requiring prophylactic or therapeutic replacement therapy...
January 2017: Der Anaesthesist
https://www.readbyqxmd.com/read/27917497/acquired-factor-xiii-inhibitor-associated-with-mantle-cell-lymphoma
#10
Christian P Nixon, Elizabeth H Prsic, Christine A Guertin, Ryan L Stevenson, Joseph D Sweeney
BACKGROUND: Acquired Factor (F)XIII deficiency is a very rare bleeding diathesis with a potentially fatal outcome, previously described in the context of autoimmune disorders and leukemias. There is minimal information on autoantibody characterization and the role of antifibrinolytic therapy in patient management. CASE REPORT: A 79-year-old woman with a 3-month history of bruising and heavy menorrhagia presented with ongoing vaginal bleeding, symptomatic anemia, and a right thigh hematoma...
December 4, 2016: Transfusion
https://www.readbyqxmd.com/read/27913544/treatment-of-rare-factor-deficiencies-in-2016
#11
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
#12
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...
January 2017: Thrombosis Research
https://www.readbyqxmd.com/read/27894217/diagnosis-clinical-manifestations-and-management-of-rare-bleeding-disorders-in-iran
#13
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
#14
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
#15
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
#16
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...
December 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
#17
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
#18
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
#19
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
#20
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
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