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Fxi deficiency

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https://www.readbyqxmd.com/read/28492700/coagulation-factor-xi-improves-host-defence-during-murine-pneumonia-derived-sepsis-independent-of-factor-xii-activation
#1
Ingrid Stroo, Sacha Zeerleder, Chao Ding, Brenda M Luken, Joris J T H Roelofs, Onno J de Boer, Joost C M Meijers, Francis J Castellino, Cornelis van 't Veer, Tom van der Poll
Bacterial pneumonia, the most common cause of sepsis, is associated with activation of coagulation. Factor XI (FXI), the key component of the intrinsic pathway, can be activated via factor XII (FXII), part of the contact system, or via thrombin. To determine whether intrinsic coagulation is involved in host defence during pneumonia and whether this is dependent on FXII activation, we infected in parallel wild-type (WT), FXI knockout (KO) and FXII KO mice with two different clinically relevant pathogens, the Gram-positive bacterium Streptococcus pneumoniae and the Gram-negative bacterium Klebsiella pneumoniae, via the airways...
May 11, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28388959/autosomal-recessive-inherited-bleeding-disorders-in-pakistan-a-cross-sectional-study-from-selected-regions
#2
Arshi Naz, Muhammad Younus Jamal, Samina Amanat, Ikram Din Ujjan, Akber Najmuddin, Humayun Patel, Fazle Raziq, Nisar Ahmed, Ayisha Imran, Tahir Sultan Shamsi
BACKGROUND: Autosomal recessive bleeding disorders (ARBDs) include deficiencies of clotting factors I, II, V, VII, X, XI, XIII, vitamin K dependent clotting factors, combined factor V & VIII, Von Willebrand Disease (vWD) type 3, Glanzmann's thrombasthenia (GT) and Bernard-Soulier syndrome. Patients with primary bleeding disorders from all the major provincial capitals of Pakistan were screened for ARBDs. Prothrombin (PT), activated partial thromboplastin time (APTT), bleeding time (BT) and fibrinogen levels were measured...
April 7, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28373973/establishment-of-the-world-health-organization-2-nd-international-standard-for-factor-xi-plasma-human
#3
Helen Wilmot, Jason Hockley, Peter Rigsby, Elaine Gray
The 1(st) International Standard (IS) for blood coagulation factor XI (FXI), plasma, has been successfully used for potency labeling of FXI therapeutics and for diagnosis of FXI deficiency in patients. With stocks of the 1(st) IS near depletion, a replacement is required. In addition to the functional activity value, assignment of an antigen value to the 2(nd) IS would allow harmonization of antigen assay methods and differentiation of patients who have low functional activity but normal antigen FXI levels from patients who have both low functional and antigen FXI levels...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28295573/therapeutic-plasma-exchange-for-perioperative-management-of-patients-with-congenital-factor-xi-deficiency
#4
Mohamed S Alsammak, Aneel A Ashrani, Jeffrey L Winters, Rajiv K Pruthi
BACKGROUND: Factor XI (FXI) deficiency (hemophilia C [HEM-C]) is a bleeding disorder with unpredictable severity that correlates poorly with FXI coagulation activity (FXI:C). It poses a perioperative hemostatic management challenge. For US patients with severe disease, fresh frozen plasma (FFP) or, in current use, thawed plasma is the most readily available option but comes with risk of volume overload. We report our experience of using therapeutic plasma exchange (TPE) as an alternative perioperative management strategy...
March 15, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28263027/coagulation-factors-anticoagulant-proteins-and-plasminogen-in-mexican-older-adults
#5
E Hernández Zamora, L O González-Espinosa, C Zavala-Hernández, E Rosales-Cruz, E Reyes-Maldonado
INTRODUCTION: Hemostasis protects upon the occurrence of vascular endothelial damage, with involving of different factors. The interaction of these factors in older adults is poorly known, and has been associated with different disorders. Therefore, we determined the activity of coagulation factors (CF), anticoagulant proteins (AP), and plasminogen (Plg), as well as the frequency of deficiencies of these proteins in a population of healthy Mexican older adults (OA). METHODS: CF (I, II, V, VII, VIII, IX, X, and XI y XII), AP [protein C (PC), protein S (PS), and antithrombin (AT)], and Plg were determined from 244 plasma samples of OA using commercial kits in a coagulometer ACL Elite Pro...
March 6, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28092924/evaluation-of-the-use-of-rotational-thromboelastometry-in-the-assessment-of-fxi-deficency
#6
G N Pike, A M Cumming, J Thachil, C R M Hay, P H B Bolton-Maggs, J Burthem
INTRODUCTION: The absence of a reliable clinical test to predict bleeding tendency leaves factor XI (FXI)-deficient individuals at risk of overtreatment or under treatment. AIM: To assess whether rotational thromboelastometry has value in detection of FXI deficiency and identification of bleeding tendency. METHODS: Thromboelastometry was measured in whole blood and platelet-rich plasma (PRP) samples containing corn trypsin inhibitor (CTI) from controls (n = 50) and FXI-deficient individuals (n = 93) at tissue factor (TF) 0...
January 16, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28053049/genome-wide-association-study-with-additional-genetic-and-post-transcriptional-analyses-reveals-novel-regulators-of-plasma-factor-xi-levels
#7
Bengt Sennblad, Saonli Basu, Johanna Mazur, Pierre Suchon, Angel Martinez-Perez, Astrid van Hylckama Vlieg, Vinh Truong, Yuhuang Li, Jesper R Gådin, Weihong Tang, Vera Grossman, Hugoline G de Haan, Niklas Handin, Angela Silveira, Juan Carlos Souto, Anders Franco-Cereceda, Pierre-Emmanuel Morange, France Gagnon, Jose Manuel Soria, Per Eriksson, Anders Hamsten, Lars Maegdefessel, Frits R Rosendaal, Philipp Wild, Aaron R Folsom, David-Alexandre Trégouët, Maria Sabater-Lleal
Coagulation factor XI (FXI) has become increasingly interesting for its role in pathogenesis of thrombosis. While elevated plasma levels of FXI have been associated with venous thromboembolism and ischemic stroke, its deficiency is associated with mild bleeding. We aimed to determine novel genetic and post-transcriptional plasma FXI regulators.We performed a genome-wide association study (GWAS) for plasma FXI levels, using novel data imputed to the 1000 Genomes reference panel. Individual GWAS analyses, including a total of 16,169 European individuals from the ARIC, GHS, MARTHA and PROCARDIS studies, were meta-analysed and further replicated in 2,045 individuals from the F5L family, GAIT2 and MEGA studies...
February 1, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/27913544/treatment-of-rare-factor-deficiencies-in-2016
#8
REVIEW
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/27913422/factor-xi-deficiency-enhances-the-pulmonary-allergic-response-to-house-dust-mite-in-mice-independent-of-factor-xii
#9
Ingrid Stroo, Jack Yang, J Daan de Boer, Joris J T H Roelofs, Cornelis van 't Veer, Francis J Castellino, Sacha Zeerleder, Tom van der Poll
Asthma is associated with activation of coagulation in the airways. The coagulation system can be initiated via the extrinsic tissue factor-dependent pathway or via the intrinsic pathway, in which the central player factor XI (FXI) can be either activated via active factor XII (FXIIa) or via thrombin. We aimed to determine the role of the intrinsic coagulation system and its possible route of activation in allergic lung inflammation induced by the clinically relevant human allergen house dust mite (HDM). Wild-type (WT), FXI knockout (KO), and FXII KO mice were subjected to repeated exposure to HDM via the airways, and inflammatory responses were compared...
February 1, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/27862691/evaluation-of-the-use-of-global-haemostasis-assays-to-monitor-treatment-in-factor-xi-deficiency
#10
G N Pike, A M Cumming, J Thachil, C R M Hay, J Burthem, P H B Bolton-Maggs
INTRODUCTION: Previous guidelines recommend that FXI:C levels should be used to monitor FXI replacement in factor XI (FXI) deficiency. However, FXI:C levels do not correlate with bleeding tendency in this disorder and may not be the optimal test by which to monitor and determine further treatment in the postoperative period. AIM: To assess whether the thrombin generation assay (TGA) and rotational thromboelastometry can be used to monitor FXI replacement peri-operatively in FXI deficiency and to determine if changes in FXI:C levels correlate with changes in thrombin generation and clot formation parameters following treatment with solvent-detergent fresh frozen plasma (SD-FFP)...
March 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27777753/coagulative-safety-of-epidural-catheters-after-major-upper-gastrointestinal-surgery-advanced-and-routine-coagulation-analysis-in-38-patients
#11
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/27723456/molecular-genetic-analysis-of-the-f11-gene-in-14-turkish-patients-with-factor-xi-deficiency-identification-of-novel-and-recurrent-mutations-and-their-inheritance-within-families
#12
Seyma Colakoglu, Turan Bayhan, Betül Tavil, Ebru Yılmaz Keskin, Volkan Cakir, Fatma Gümrük, Mualla Çetin, Selin Aytaç, Ergul Berber
BACKGROUND: Factor XI (FXI) deficiency is an autosomal bleeding disease associated with genetic defects in the F11 gene which cause decreased FXI levels or impaired FXI function. An increasing number of mutations has been reported in the FXI mutation database, most of which affect the serine protease domain of the protein. FXI is a heterogeneous disorder associated with a variable bleeding tendency and a variety of causative F11 gene mutations. The molecular basis of FXI deficiency in 14 patients from ten unrelated families in Turkey was analysed to establish genotype-phenotype correlations and inheritance of the mutations in the patients' families...
October 4, 2016: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/27699729/the-management-of-factor-xi-deficiency-in-pregnancy
#13
REVIEW
Joanna Davies, Rezan Kadir
Management of factor XI (FXI) deficiency in pregnancy is complicated by lack of correlation between FXI level and bleeding risk. Clinicians should be vigilant about the potential for prolonged or excessive bleeding following miscarriage or termination of pregnancy, or postpartum hemorrhage (PPH). A multidisciplinary approach along with an individual care plan is recommended to prevent bleeding complications. Assessment of bleeding history, FXI level, and global tests of hemostasis can aid management decisions regarding hemostatic prophylaxis...
October 2016: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/27559095/factor-xia-specific-igg-and-a-reversal-agent-to-probe-factor-xi-function-in-thrombosis-and-hemostasis
#14
Tovo David, Yun Cheol Kim, Lauren K Ely, Isaac Rondon, Huilan Gao, Peter O'Brien, Michael W Bolt, Anthony J Coyle, Jorge L Garcia, Eric A Flounders, Thomas Mikita, Shaun R Coughlin
Thrombosis is a major cause of morbidity and mortality. Current antithrombotic drugs are not ideal in that they must balance prevention of thrombosis against bleeding risk. Inhibition of coagulation factor XI (FXI) may offer an improvement over existing antithrombotic strategies by preventing some forms of thrombosis with lower bleeding risk. To permit exploration of this hypothesis in humans, we generated and characterized a series of human immunoglobulin Gs (IgGs) that blocked FXIa active-site function but did not bind FXI zymogen or other coagulation proteases...
August 24, 2016: Science Translational Medicine
https://www.readbyqxmd.com/read/27405678/rare-coagulation-disorders-fibrinogen-factor-vii-and-factor-xiii
#15
REVIEW
P de Moerloose, J-F Schved, D Nugent
Rare coagulation disorders (RCDs) include the inherited deficiencies of fibrinogen, factor (F) II, FV, combined FV and VIII, FVII, FX, combined FVII and X, FXI, FXIII and combined congenital deficiency of vitamin K-dependent factors (VKCFDs). Despite their rarity, a deep comprehension of all these disorders is essential to really understand haemostasis. Indeed, even if they share some common features each RCD has some particularity which makes it unique. In this review, we focus on three disorders: fibrinogen, FVII and FXIII...
July 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27385629/thrombotic-events-in-asymptomatic-fxii-deficiency-versus-symptomatic-fxi-deficiency-surprising-observations
#16
COMMENT
A Girolami, E Cosi, C Santarossa, S Ferrari, A M Lombardi
OBJECTIVE: To evaluate the impact of an asymptomatic congenital clotting defect (FXII deficiency) versus that of a similar but symptomatic defect (FXI deficiency) on protection from thrombosis. PATIENTS AND METHODS: All patients with FXII or FXI deficiency and thrombosis were gathered from a time-unlimited PubMed search that was carried out twice and from personal records. Combined defects were excluded. The defect had to be proven by the demonstration of a suited hereditary pattern and by a specific clotting assay...
2016: Acta Haematologica
https://www.readbyqxmd.com/read/27305946/ischemic-strokes-in-congenital-bleeding-disorders-comparison-with-myocardial-infarction-and-other-acute-coronary-syndromes
#17
REVIEW
Antonio Girolami, Ferrari Silvia, Cosi Elisabetta, Peroni Edoardo, Girolami Bruno
OBJECTIVE: To investigate the occurrence of ischemic stroke in patients with congenital bleeding disorders. PATIENTS AND METHODS: Patients with congenital bleeding disorders who presented an objectively proven ischemic stroke were obtained by means of a time unlimited Pubmed search. Appropriate key words and Medical subject headings were used. Cross-checking of the references was also carried out. RESULTS: There were four cases of Fibrinogen defects or Factor VII deficiency; seven patients had Hemophilia (6 hemophilia A and 1 hemophilia B); eight cases of FXI deficiency and six patients with von Willebrand Disease...
2016: Cardiovascular & Hematological Disorders Drug Targets
https://www.readbyqxmd.com/read/27302213/caesarean-surgery-or-vaginal-delivery-in-a-pregnant-woman-with-homozygous-fxi-deficiency-and-fxi-level-1-this-is-the-question
#18
Giovanni Barillari, Fabio Springolo, Samantha Pasca, Anna Del Fabro, Diego Marchesoni
No abstract text is available yet for this article.
August 2016: Minerva Ginecologica
https://www.readbyqxmd.com/read/27279430/is-resistance-futile-the-role-of-activated-thrombin-activatable-fibrinolysis-inhibitor-resistance-in-bleeding-in-factor-xi-deficiency
#19
M B Boffa
No abstract text is available yet for this article.
August 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27216469/why-factor-xi-deficiency-is-a-clinical-concern
#20
REVIEW
Allison P Wheeler, David Gailani
INTRODUCTION: Inherited fXI deficiency has been an enigma since its discovery in 1953. The variable and relatively mild symptoms in patients with even the most severe form of the disorder seem out of step with the marked abnormalities in standard clotting assays. Indeed, the contribution of factor XI to hemostasis in an individual is not adequately assessed by techniques available in modern clinical laboratories. AREAS COVERED: We discuss clinical studies, genetic/genomic analyses, and advances in laboratory medicine that are reshaping our views on the role of factor XI in pathologic coagulation...
July 2016: Expert Review of Hematology
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