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https://www.readbyqxmd.com/read/28529666/a-microfluidic-model-of-hemostasis-sensitive-to-platelet-function-and-coagulation
#1
R M Schoeman, K Rana, N Danes, M Lehmann, J A Di Paola, A L Fogelson, K Leiderman, K B Neeves
Hemostasis is the process of sealing a vascular injury with a thrombus to arrest bleeding. The type of thrombus that forms depends on the nature of the injury and hemodynamics. There are many models of intravascular thrombus formation whereby blood is exposed to prothrombotic molecules on a solid substrate. However, there are few models of extravascular thrombus formation whereby blood escapes into the extravascular space through a hole in the vessel wall. Here, we describe a microfluidic model of hemostasis that includes vascular, vessel wall, and extravascular compartments...
February 2017: Cellular and Molecular Bioengineering
https://www.readbyqxmd.com/read/28523993/acquired-hemophilia-as-initial-presentation-in-a-patient-with-systemic-lupus-erythematosus
#2
Zohre Khodamoradi, Mohammad Ali Nazarinia, Somaye Bazdar
BACKGROUND: Acquired hemophilia is a rare bleeding disease but may be associated with some autoimmune diseases. Acquired hemophilia may be the result of autoantibodies against factor VIII. CASE PRESENTATION: In this study, we describe a 55 year old patient who developed hematoma and hematuria due to acquired hemophilia secondary to systemic lupus erythematosus(SLE). Then, she developed arthritis and thrombosis after some evaluation. Laboratory data showed prolonged aPTT, normal PT and platelet, low FVIII, high FVIII inhibitor, ANA, anti-ds-DNA and anti-cardiolipin...
May 19, 2017: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/28514934/a-hypothesis-king-henry-viii-s-1491-1547-personality-change-a-case-of-lead-poisoning
#3
Anne Charlton
Henry VIII (1491-1547) became King of England in 1509. He started out as a good monarch, sensible, reasonable and pleasant, but later his behaviour changed drastically. He became irascible, intolerant, violent and tyrannical. In January 1536, Henry had a serious jousting accident and was unconscious for 2 h. It is generally believed that this accident played a major role in his personality change. Letters of that time, however, indicate that the change began insidiously in 1534 and became most drastic in 1535, a year before the accident...
May 2017: Journal of Medical Biography
https://www.readbyqxmd.com/read/28512557/increased-vasoocclusive-crises-in-o-blood-group-sickle-cell-disease-patients-association-with-underlying-thrombospondin-levels
#4
M Al Huneini, S Alkindi, V Panjwani, K Al Falahi, B Al Balushi, D Gravell, C H Ho, R Krishnamoorthy, A V Pathare
OBJECTIVES: To explore the incidence of vaso-occlusive crisis (VOC) in Blood Group "O" sickle cell disease (SCD) patients, and correlate it with the blood group and thrombospondin (TSP) levels. METHODS: In 89 consecutive SCD patients, blood samples were obtained for von Williebrand factor (vWF:Ag) antigen, collagen binding activity (CBA), ristocetin binding activity (RCo), blood group typing, C-reactive protein (CRP), high performance liquid chromatography (HPLC), Serum TSP 1 and TSP 2 levels, complete blood counts (CBC), lactic dehydrogenase (LDH) levels, liver function (LFT) and renal function tests (RFT) during VOC episodes and in steady state conditions...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28508982/renal-hemorrhage-caused-by-acquired-inhibitors-to-coagulation-factors-viii-and-v-in-a-hemodialysis-patient
#5
Naoya Niwa, Tadashi Yoshida, Ryuichi Mizuno, Mototsugu Oya, Matsuhiko Hayashi
Acquired coagulation factor deficiency is a rare bleeding disorder caused by the inhibitors to coagulation factors. We report a case of an elderly hemodialysis patient who presented with the intermittent hematuria and anemia, associated with the prolonged activated partial thromboplastin time and prothrombin time. Laboratory examination revealed undetectable factor VIII activity, decreased factor V activity, and the presence of inhibitors to these coagulation factors. The patient was diagnosed to have inhibitors to coagulation factors VIII and V simultaneously...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28507083/frequency-and-epitope-specificity-of-anti-factor-viii-c1-domain-antibodies-in-acquired-and-congenital-hemophilia-a
#6
Joerg Kahle, Aleksander Orlowski, Diana Stichel, John F Healey, Ernest T Parker, Marc Jaquemin, Manuela Krause, Andreas Tiede, Dirk Schwabe, Pete Lollar, Christoph Königs
Several studies showed that neutralizing anti-factor VIII (fVIII) antibodies (inhibitors) in patients with acquired hemophilia A (AHA) and congenital hemophilia A (HA) are primarily directed to the A2 and C2 domains. In this study the frequency and epitope specificity of anti-C1 antibodies were analyzed in acquired and congenital hemophilia inhibitor patients (n=178). The domain specificity of antibodies was studied by homologue-scanning mutagenesis (HSM) with single human domain human/porcine fVIII proteins and antibody binding to human A2, C1 and C2 domains presented as human serum albumin (HSA) fusion proteins...
May 15, 2017: Blood
https://www.readbyqxmd.com/read/28495542/endothelial-function-is-preserved-in-veins-harvested-by-either-endoscopic-or-surgical-techniques
#7
Adam R Wheeler, Daniel E Kendrick, Matt T Allemang, Andre F Gosling, Ann H Kim, Alfred Hausladen, Vikram S Kashyap
OBJECTIVES: Endoscopic vein harvest for lower extremity arterial bypass grafting has been questioned due to concern for endothelial damage during procurement. We sought to compare Nitric Oxide (NO)-mediated endothelial dependent relaxation (EDR) in vein segments harvested using open surgical (OH) versus endoscopic (EH) techniques. METHODS: Saphenous vein segments were harvested for lower extremity bypass, and a single, minimally handled section of saphenous vein, free of branches, was taken from the end of the graft...
May 8, 2017: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/28494701/spindle-cell-lipoma-in-dogs
#8
Giancarlo Avallone, Valeria Pellegrino, Luisa V Muscatello, Giuseppe Sarli, Paola Roccabianca
Spindle cell lipoma (SCL) is a benign neoplasm of the adipose tissue that may resemble an undifferentiated soft tissue sarcoma (STS). This report describes the histopathological features of 6 SCLs in dogs. All SCLs were located in the subcutis and were composed of bland, occasionally vacuolated spindle cells intermixed with ropey collagen and myxoid matrix. Sudan IV stain performed in 1 case demonstrated the lipid content of vacuoles. Mature adipocytes represented less than 10% of the neoplasm in 3 cases and were absent in the remaining 3...
January 1, 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/28492697/cd32-inhibition-and-high-dose-of-rhfviii-suppress-murine-fviii-specific-recall-response-by-distinct-mechanisms-in-vitro
#9
Nadine Vollack, Julia Friese, Sabine Bergmann, Andreas Tiede, Sonja Werwitzke
Development of neutralising antibodies (inhibitors) against factor VIII (FVIII) is a frequent and severe complication of replacement therapy in haemophilia A. Previous data from haemophilia A mouse model demonstrates that both CD32 inhibition and high doses of rhFVIII prevent the differentiation of FVIII-specific memory B cells (MBCs) into antibody secreting cells (ASCs). Here, cellular targets responsible for the suppression of ASC formation by means of CD32 inhibition and high dose of rhFVIII were analysed...
May 11, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28491882/portal-vein-thrombosis-in-the-setting-of-newly-diagnosed-cushing-s-syndrome
#10
Alexandria D McDow, Anju Gurung, Rama Poola, Carmel Fratianni, Marc Garfinkel, Michael G Jakoby
The hallmark manifestations of Cushing's syndrome (CS) are well known, but hypercoagulability is perhaps least recognized. Patients with CS are at increased risk of both spontaneous and postoperative thromboembolism, with the significant majority of events occurring in the lower extremity and pulmonary venous circulations. We present a case of portal vein thrombosis (PVT) occurring in the setting of newly diagnosed CS due to a left adrenal adenoma. Factor VIII activity was approximately 2.5-fold elevated, a known mechanism by which hypercortisolemia predisposes to venous thrombosis...
April 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28486646/altered-preoperative-coagulation-and-fibrinolysis-are-associated-with-myocardial-injury-after-non-cardiac-surgery
#11
J Górka, K Polok, T Iwaniec, K Górka, A Wludarczyk, J Fronczek, P J Devereaux, J W Eikelboom, J Musial, W Szczeklik
Background: Myocardial injury after non-cardiac surgery (MINS), a complication with unclear pathogenesis, occurs within the first 30 days after surgery and worsens prognosis. Hypercoagulability induced by surgery might contribute to plaque rupture, with subsequent thrombosis and myocardial injury. This study assessed haemostatic markers before surgery and evaluated their association with MINS. Methods: This is a substudy of VISION, a prospective cohort study of perioperative cardiovascular events...
May 1, 2017: British Journal of Anaesthesia
https://www.readbyqxmd.com/read/28486277/evaluation-of-recombinant-factor-viii-fc-eloctate-activity-by-thromboelastometry-in-a-multicenter-phase-3-clinical-trial-and-correlation-with-bleeding-phenotype
#12
Frank Driessler, Maricel G Miguelino, Glenn F Pierce, Robert T Peters, Jurg M Sommer
: The aim of this study was to compare the hemostatic efficacy of recombinant factor VIII Fc (rFVIIIFc) (Eloctate) and Advate by ex-vivo rotation thromboelastometry (ROTEM) of whole blood and to explore potential ROTEM parameters that may be more predictive of a patient's bleeding tendency than plasma FVIII activity. Thirteen clinical sites were selected to perform ROTEM on freshly collected blood samples from 44 patients in the phase 3 study for rFVIIIFc, including 16 patients undergoing sequential pharmacokinetic assessment of Advate and rFVIIIFc...
May 8, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28480193/opposite-effects-of-agrimonia-pilosa-ledeb-aqueous-extracts-on-blood-coagulation-function
#13
Xianming Fei, Wufeng Yuan, Lei Jiang, Huan Wang
BACKGROUND: Agrimonia pilosa Ledeb (APL) has showed anticoagulant and antithrombotic activities in some studies, whereas its actual effects on blood coagulation are still unclear. This study was designed to observe the in vitro effects of APL aqueous extracts on blood coagulation, as well as to investigate the underlying mechanisms. METHODS: Studies were divided into four groups: 0, 4, 20, and 80 g/L of APL aqueous extracts mixed with plasma or whole blood samples...
April 2017: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28478688/gene-delivery-of-activated-factor-vii-using-alternative-aav-serotype-improves-hemostasis-in-hemophiliac-mice-with-fviii-inhibitors-and-aav-neutralizing-antibodies
#14
Junjiang Sun, Baolai Hua, R Jude Samulski, Chengwen Li
While therapeutic expression of coagulation factors from adeno-associated virus (AAV) vectors has been successfully achieved in patients with hemophilia, neutralizing antibodies to the vector and inhibitory antibodies to the transgene severely limit efficacy. Indeed, approximately 40% of mice transduced with human Factor VIII using the AAV8 serotype developed inhibitory antibodies to Factor VIII (FVIII inhibitor), as well as extremely high titers (≥ 1:500) of neutralizing antibodies to AAV8. To correct hemophilia in these mice we used AAV9, a serotype with low in vitro cross-reactivity (≤ 1:5) to anti-AAV8, to deliver mouse activated Factor VII (mFVIIa)...
May 6, 2017: Human Gene Therapy
https://www.readbyqxmd.com/read/28475272/recombinant-factor-viia-addition-to-haemophilic-blood-perfused-over-collagen-tissue-factor-can-sufficiently-bypass-the-factor-ixa-viiia-defect-to-rescue-fibrin-generation
#15
R Li, K A Panckeri, P F Fogarty, A Cuker, S L Diamond
INTRODUCTION: Factor VIII (FVIII) or factor IX (FIX)-deficient haemophilic patients display deficits in platelet and fibrin deposition under flow detectable in microfluidics. Compared to fibrin generation, decreased platelet deposition in haemophilic blood flow is more easily rescued with recombinant factor VIIa (rFVIIa), whereas rFVIIa requires FXIIa participation to generate fibrin when tissue factor (TF) is absent. AIMS: Perfusion of haemophilic whole blood (WB) over collagen/TF surfaces was used to determine whether rFVIIa/TF was sufficient to bypass poor FIXa/FVIIIa function in blood from patients with haemophilia A and B...
May 5, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28474867/the-response-of-coagulation-and-anti-coagulant-factors-of-elite-athletes-following-acute-resistance-and-high-intensity-interval-exercises
#16
Sedigheh Karampour, Abbas A Gaeini
BACKGROUND: Blood coagulation and fibrinolysis include two vital physiological systems, which are regulated by a balance between activators and inhibitors. The aim of this study is to survey the response of coagulation and anticoagulant factors following acute resistance and high intensity interval exercises. METHODS: This is an experimental study. The statistical sample was an elite group of karate males (aged 22.10±2.76 years, height 175.80±5.43 cm, mass 74...
April 26, 2017: Journal of Sports Medicine and Physical Fitness
https://www.readbyqxmd.com/read/28472252/new-insights-into-the-role-of-adipose-tissue-in-thrombosis
#17
Gemma Vilahur, Soumaya Ben-Aicha, Lina Badimon
Central obesity is independently associated with an elevated risk of cardiovascular disease, particularly thrombotic complications. Increasing data supports a link between excess body weight and the risk to suffer acute myocardial infarction, stent thrombosis after percutaneous interventions, ischemic stroke and vein thrombosis.Experimental and in vitro data have provided insights as to the mechanisms currently presumed to increase the thrombotic risk in obese subjects. Obesity is characterized by a chronic low grade inflammation and systemic oxidative stress that eventually damage the endothelium losing its antithrombotic properties...
May 3, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28470862/first-report-on-the-safety-and-efficacy-of-an-extended-half-life-glycopegylated-recombinant-fviii-for-major-surgery-in-severe-haemophilia-a
#18
K Hampton, P Chowdary, S Dunkley, S Ehrenforth, L Jacobsen, A Neff, E Santagostino, J Sathar, H Takedani, C M Takemoto, C Négrier
BACKGROUND: N8-GP (turoctocog alfa pegol) is an extended half-life glycoPEGylated recombinant factor VIII (FVIII) product developed for the prevention and treatment of bleeds in haemophilia A patients. AIM: This is a planned interim analysis of pathfinder™3, an international, open-label, Phase 3 trial evaluating the efficacy and safety (including immunogenicity) of N8-GP administered before, during and after major surgery in severe haemophilia A patients aged ≥12 years...
May 4, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28470674/acquired-hemophilia-a-updated-review-of-evidence-and-treatment-guidance
#19
REVIEW
Rebecca Kruse-Jarres, Christine L Kempton, Francesco Baudo, Peter W Collins, Paul Knoebl, Cindy A Leissinger, Andreas Tiede, Craig M Kessler
Acquired hemophilia A (AHA) is a rare disease resulting from autoantibodies (inhibitors) against endogenous factor VIII (FVIII) that leads to bleeding, which is often spontaneous and severe. AHA tends to occur in elderly patients with comorbidities and is associated with high mortality risk from underlying comorbidities, bleeding, or treatment complications. Treatment, which consists of hemostatic management and eradication of the inhibitors, can be challenging to manage. Few data are available to guide the management of AHA-related bleeding and eradication of the disease-causing antibodies...
May 3, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28458935/successful-management-of-acquired-hemophilia-a-associated-with-bullous-pemphigoid-a-case-report-and-review-of-the-literature
#20
Quentin Binet, Catherine Lambert, Laurine Sacré, Stéphane Eeckhoudt, Cedric Hermans
Background. Acquired hemophilia A (AHA) is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Symptoms include severe and unexpected bleeding that may prove life-threatening. Case Study. We report a case of AHA associated with bullous pemphigoid (BP), a chronic, autoimmune, subepidermal, blistering skin disease. To our knowledge, this is the 25th documented case of such an association...
2017: Case Reports in Hematology
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