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https://www.readbyqxmd.com/read/29148258/influence-of-medical-insurance-schemes-and-charity-assistance-projects-on-regular-prophylaxis-treatment-of-the-boys-with-severe-haemophilia-a-in-china
#1
Z Li, J Wu, Y Zhao, R Liu, K Li, Y Zhou, R Wu, R Yang, X Zhang, S Lian, Q Hu, X Li, J Gu, R Zhou, J Sun, C Li, W Xu, M-C Poon, J Xiao
OBJECTIVE: To explore the influence of medical insurance policy and charity assistance projects on the uptake and discontinuation of regular prophylaxis treatment in Chinese severe haemophilia A children. METHODOLOGY: This retrospective study was conducted on children with severe haemophilia A, who received FVIII prophylaxis treatment at 12 haemophilia centres in China from 1 November 2007 to 31 May 2013. RESULTS: The average duration of prophylaxis treatment received by haemophilia children significantly increased from 16...
November 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29146705/complement-c3-is-a-novel-modulator-of-the-anti-fviii-immune-response
#2
Julie Rayes, Mathieu Ing, Sandrine Delignat, Ivan Peyron, Laurent Gilardin, Carl-Wilhelm Vogel, David C Fritzinger, Véronique Frémeaux-Bacchi, Srinivas V Kaveri, Lubka T Roumenina, Sébastien Lacroix-Desmazes
Development of neutralizing antibodies against therapeutic factor VIII (FVIII) is the most serious complication of the treatment of hemophilia A. Increasing evidence shows the multifactorial origin of the anti-FVIII immune response, combining both genetic and environmental factors. While a role for the complement system on innate as well as adaptive immunity has been documented, the implication of complement activation on the onset of the anti-FVIII immune response is unknown. Here, using in vitro assays for FVIII endocytosis by human monocyte-derived dendritic cells and presentation to T cells, as well as animal models of in vivo complement depletion, we show a novel role for complement C3 in enhancing the immune response against therapeutic FVIII...
November 16, 2017: Haematologica
https://www.readbyqxmd.com/read/29139314/cost-utility-analysis-of-life-long-prophylaxis-with-recombinant-factor-viii-fc-vs-recombinant-factor-viii-for-the-management-of-severe-haemophilia-a-in-sweden
#3
Nathaniel Henry, Jelena Jovanović, Max Schlueter, Persefoni Kritikou, Koo Wilson, Karl-Johan Myrén
AIMS: Prophylaxis with recombinant factor VIII (rFVIII) is the standard of care for severe haemophilia A in Sweden. The need for frequent injections with existing rFVIII products may, however, result in poor adherence to prophylaxis leading to increased bleeding and long-term joint damage. Recombinant FVIIIFc (rFVIIIFc) is an extended half-life fusion protein which can offer prolonged protection and reduced dosing frequency. The objective of this study was to evaluate the cost-utility of prophylaxis with rFVIIIFc in severe haemophilia A from the perspective of the Swedish health system...
November 15, 2017: Journal of Medical Economics
https://www.readbyqxmd.com/read/29135477/thrombin-potential-and-traditional-coagulation-assay-are-they-useful-in-exploring-recurrent-pregnancy-loss-risk
#4
Ilaria Romagnuolo, Monica Attanasio, Mauro Cozzolino, Enrichetta Paladino, Giancarlo Castaman, Maria E Coccia, Cinzia Fatini
: An adequate hemostatic balance is mandatory to get successful pregnancy. Obstetric complications, such as recurrent pregnancy loss (RPL), might be due to an impairment of placental perfusion possibly related to an underlying prothrombotic status. In this study, we used the global coagulation assay, calibrated automated thrombography and traditional coagulation assay to search for a possible underlying hypercoagulable status in women with history of RPL compared with uneventful pregnancy women. Thrombin generation, Fibrinogen, factor VIII (FVIII), Plasminogen Activator Inhibitor-1 (PAI-1) and von Willebrand factor levels were analyzed in 92 not pregnant unexplained RPL and 64 uneventful pregnancy women...
November 10, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29126301/coagulation-testing-in-the-core-laboratory
#5
William E Winter, Sherri D Flax, Neil S Harris
Primary hemostasis begins with endothelial injury. VWF, produced by endothelial cells, binds to platelets and links them to subendothelial collagen. Platelet-derived ADP and thromboxane activate non-adhered platelets via their GPIIb/IIIa receptors, allowing these platelets to participate in platelet aggregation. Secondary hemostasis is initiated with the binding of factor VII to extravascular tissue factor (TF). Factors II, VII, IX and X are vitamin K-dependent factors. The role of vitamin K is to assist in the addition of gamma carboxylate groups to glutamic acids in the "GLA" domains of these factors...
November 8, 2017: Laboratory Medicine
https://www.readbyqxmd.com/read/29115006/type-2n-von-willebrand-disease-characterization-and-diagnostic-difficulties
#6
A Casonato, E Galletta, L Sarolo, V Daidone
INTRODUCTION: An abnormal factor VIII (FVIII) binding capacity of von Willebrand factor (VWF) identifies type 2N von Willebrand disease (VWD). Type 2N VWD patients are identified by means of the VWF FVIII binding (VWF:FVIIIB) assay, and especially their VWF:FVIIIB/VWF:Ag ratio (VWF:FVIIIB ratio). AIM: We report on our 15-year experience of diagnosing type 2N VWD. METHODS: We have performed 2178 VWF:FVIIIB assays in bleeders and normal subjects...
November 8, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29108420/inhibitor-risk-stratification-and-individualized-treatment-in-patients-with-nonsevere-hemophilia-a-a-single-institution-practice-audit
#7
Haowei Linda Sun, Stella Chan, Paul Yenson, Shannon Jackson
Inhibitor risk in nonsevere hemophilia A increases with cumulative factor VIII (FVIII) exposure days and high-risk mutations. A standardized approach to minimize inhibitor risk is warranted. Following establishment of a systematic approach to reduce inhibitor risk in nonsevere hemophilia, we evaluated the uptake of these strategies into clinical practice. All adult males with nonsevere hemophilia A followed by British Columbia Adult Hemophilia Program from 2004 to 2016 were included in this retrospective audit...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29108103/adamts13-and-von-willebrand-factor-a-dynamic-duo
#8
REVIEW
Kieron South, David A Lane
VWF is a key player in haemostasis, acting as a carrier for FVIII and capturing platelets at sites of vascular damage. To capture platelets, it must undergo conformational changes, both within its A1 domain and at the macromolecular level through A2 domain unfolding. Its size and this function are regulated by the metalloproteinase, ADAMTS13. Recently, it has been shown that ADAMTS13 undergoes a conformational change upon interaction with VWF and that this enhances its activity towards its substrate. This review summarises recent work on these conformational transitions, describing how they are controlled...
November 6, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29106782/expression-and-assembly-of-largest-foreign-protein-in-chloroplasts-oral-delivery-of-human-fviii-made-in-lettuce-chloroplasts-robustly-suppresses-inhibitor-formation-in-hemophilia-a-mice
#9
Kwang-Chul Kwon, Alexandra Sherman, Wan-Jung Chang, Aditya Kamesh, Moanaro Biswas, Roland W Herzog, Henry Daniell
Inhibitor formation is a serious complication of factor VIII (FVIII) replacement therapy for the X-linked bleeding disorder hemophilia A and occurs in 20-30% of patients. No prophylactic tolerance protocol currently exists. Although we reported oral tolerance induction using FVIII domains expressed in tobacco chloroplasts, significant challenges in clinical advancement include expression of the full-length CTB-FVIII sequence to cover the entire patient population, regardless of individual CD4(+) T cell epitope responses...
November 6, 2017: Plant Biotechnology Journal
https://www.readbyqxmd.com/read/29102549/lipidic-nanoparticles-comprising-of-phosphatidylinositol-mitigate-immunogenicity-and-improve-efficacy-of-recombinant-human-acid-alpha-glucosidase-in-a-murine-model-of-pompe-disease
#10
Jennifer L Schneider, Robert K Dingman, Sathy V Balu-Iyer
Enzyme replacement therapy with recombinant human acid α-glucosidase (rhGAA) is complicated by the formation of anti-rhGAA antibodies, a short circulating half-life, instability in the plasma, and limited uptake into target tissue. Previously, we have demonstrated that phosphatidylinositol (PI) containing liposomes can reduce the immunogenicity and extend plasma survival of Factor VIII (FVIII) in a mouse model of Hemophilia A. In this manuscript we investigate the ability of PI liposomes to be used as a delivery vehicle to overcome the issues that complicate therapy with rhGAA...
November 1, 2017: Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/29098718/outgrowing-the-laboratory-diagnosis-of-type-1-von-willebrand-disease-a-two-decade-study
#11
Mouhamed Yazan Abou-Ismail, Gbolahan O Ogunbayo, Michelle Secic, Peter A Kouides
Von Willebrand Factor (VWF) levels are known to increase with age in the general population, but that effect is unclear in von Willebrand's disease (VWD) patients. Thus, it is important to assess the trends of VWF levels with age, and the extent and rate of their normalization in patients with VWD. In a retrospective cohort study, we reviewed the medical records of 126 patients between 1996 and 2016 who met the NHLBI diagnostic criteria for Type 1 VWD or "Low VWF" (LVWF). We followed all their historically documented VWF antigen (VWF:Ag), VWF activity (VWF:RCo), and Factor VIII (FVIII) levels longitudinally over time, correlating data with clinical setting at time of testing...
November 3, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29094008/total-joint-replacement-in-inhibitor-positive-haemophilia-long-term-outcome-analysis-in-fifteen-patients
#12
Heidi Danielson, Riitta Lassila, Pekka Ylinen, Timo Yrjönen
AIM: To collect data from joint replacement in inhibitor patients, evaluate haemostatic and patient outcomes, and analyse the costs. METHODS: We report our 21-year, single-centre cumulative experience of 15 joint arthroplasties in six inhibitor patients. RESULTS: Two low responder inhibitor patients were in the early days treated with FVIII, whereas bypassing agents were used in the rest of the high responder patients. The primary haemostatic outcome was good in 8/15, fair in 4/15 and poor in 3/15 operations...
October 18, 2017: World Journal of Orthopedics
https://www.readbyqxmd.com/read/29080391/timing-and-severity-of-inhibitor-development-in-recombinant-versus-plasma-derived-factor-viii-concentrates-a-sippet-analysis
#13
F Peyvandi, A Cannavò, I Garagiola, R Palla, P M Mannucci, F R Rosendaal
BACKGROUND: The development of neutralizing antibodies (inhibitors) against factor VIII (FVIII) is the most severe complication in the early phases of treatment of severe haemophilia A. Recently a randomized trial, the Survey of Inhibitors in Plasma-Product Exposed Toddlers (SIPPET) demonstrated a two-fold higher risk of inhibitors development in children treated with recombinant FVIII (rFVIII) products than with plasma-derived FVIII (pdFVIII) during the first 50 exposure days (EDs). OBJECTIVE/METHODS: In this post-hoc SIPPET analysis we evaluated the rate of inhibitor incidence over time by every 5 EDs (from 0 to 50 EDs) in patients treated with different classes of FVIII product, made possible by a frequent testing regime...
October 28, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29080389/sippet-insights-into-factor-viii-immunogenicity
#14
Padraic G Fallon, Michelle Lavin, James S O'Donnell
In this issue of the Journal of Thrombosis and Haemostasis, Peyvandi et al present further insights from the Survey of Inhibitors in Plasma-Product Exposed Toddlers (SIPPET) study regarding the relative immunogenicity of recombinant- (rFVIII) versus plasma-derived FVIII (pdFVIII) in patients with haemophilia A [1]. In a post-hoc analysis of this prospective randomised controlled trial, the timing and severity of inhibitor development for previously untreated patients (PUPs) treated with rFVIII was compared with that in PUPs treated with pdFVIII...
October 28, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29080382/factor-xiii-co-treatment-with-hemostatic-agents-in-hemophilia-a-increases-fibrin-%C3%AE-chain-crosslinking
#15
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/29078111/markers-of-coagulation-activation-inflammation-and-fibrinolysis-as-predictors-of-poor-outcomes-after-pediatric-venous-thromboembolism-a-systematic-review-and-meta-analysis
#16
REVIEW
Ayesha Zia, Joy Russell, Ravi Sarode, Surendranath R Veeram, Shellie Josephs, Kendra Malone, Song Zhang, Janna Journeycake
BACKGROUND: Sequelae of venous thromboembolism (VTE) in children include recurrence, development of post thrombotic syndrome (PTS) when venous return from a limb is affected and chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary embolism. Identification of laboratory-based risk factors may be useful for individualized risk assessment for VTE sequelae. Coagulation activation and inflammation may contribute to their pathophysiology. We performed a systematic review to investigate the association between biomarkers of coagulation activation, inflammation and fibrinolysis and adverse VTE outcomes in children and young adults...
October 7, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/29070980/components-in-plasma-derived-factor-viii-but-not-in-recombinant-factor-viii-downregulate-anti-inflammatory-surface-marker-cd163-in-human-macrophages-through-release-of-cxcl4-platelet-factor-4
#17
Anne Bertling, Martin F Brodde, Mayken Visser, Janina Treffon, Michelle Fennen, Anke C Fender, Reinhard Kelsch, Beate E Kehrel
BACKGROUND: Hemarthrosis, or bleeding into the joints, is a hallmark of hemophilia. Heme triggers oxidative stress, inflammation, and destruction of cartilage and bone. The haptoglobin-CD163-heme oxygenase-1 (HO-1) pathway circumvents heme toxicity through enzymatic degradation of heme and transcription of antioxidant genes. Plasma-derived factor concentrates contain many proteins that might impact on cellular pathways in joints, blood, and vessels. METHODS: Activation of platelets from healthy volunteers was assessed by flow cytometry analysis of fibrinogen binding and CD62P expression...
September 2017: Transfusion Medicine and Hemotherapy
https://www.readbyqxmd.com/read/29070472/-breakthrough-bleeding-in-adult-patients-with-severe-hemophilia-a-receiving-low-and-intermediate-dose-fviii-for-tertiary-prophylaxis-characteristics-and-influencing-factors
#18
Shi-Qiu Qiu, Jin-Mu Zhuang, Xuan Zhou, Rui-Xue Yin, Zhu-Qin Liu, Fei Ma, Ying-Jia Li, Jing Sun
OBJECTIVE: To investigate the characteristics of breakthrough bleeding in adult patients with severe hemophilia A (SHA) receiving low- and intermediate-dose FVIII for tertiary prophylaxis and explore the factors affecting the outcomes of the treatment. METHODS: Forty-nine patients (mean age 31.53∓7.33 years) with SHA receiving tertiary prophylaxis FVIII treatment were divided into low-dose group (n=15) and intermediate-dose group (n=34). The data including clinical bleeding phenotype (Pre?AJBR), 72 h FVIII trough activity, and Functional Independence Score in Hemophilia (FISH) were recorded in all the patients, and Hemophilia Steward APP was used to record the bleeding episode and the treatment data...
October 20, 2017: Nan Fang Yi Ke da Xue Xue Bao, Journal of Southern Medical University
https://www.readbyqxmd.com/read/29063359/heparin-coumarin-protein-c-antithrombin-fibrinolysis-and-other-clotting-related-resistances-old-and-new-concepts-in-blood-coagulation
#19
REVIEW
A Girolami, E Cosi, S Ferrari, B Girolami
The concept of resistance in blood coagulation has become important. In the past it was limited to the resistance shown by some patients to heparin, coumarin or aspirin. Subsequently, it was demonstrated that a mutation in a single clotting factor, FV, showed resistance to activated protein C. Since activated protein C is supposed to downregulate aFV and aFVIII, their persistence in the circulation gives origin to a hypercoagulable state. Recently antithrombin resistance has been defined. Several prothrombin abnormalities (dysprothrombinemias) have been shown to be resistant to the action of antithrombin...
October 23, 2017: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/29051804/review-of-recombinant-anti-haemophilic-porcine-sequence-factor-viii-in-adults-with-acquired-haemophilia-a
#20
REVIEW
Emma Fosbury, Anja Drebes, Anne Riddell, Pratima Chowdary
Acquired haemophilia A (AHA) is a rare, serious bleeding disorder most often encountered in elderly patients. The mainstay of haemostatic management is with bypassing agents (BPAs) including recombinant activated factor VII (rFVIIa) and activated prothrombin complex concentrates (aPCCs). Their major limitation is incomplete efficacy, potential risk for thrombosis and the lack of routine laboratory assays for monitoring treatment response. Plasma-derived porcine FVIII (pd-pFVIII, Hyate C(®)), first used in the 1950s for the management of congenital haemophilia, has sufficient sequence homology to be haemostatic in humans, but the lack of complete homology facilitates efficacy even in the presence of human allo- and autoantibodies against human FVIII (hFVIII)...
September 2017: Therapeutic Advances in Hematology
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