keyword
MENU ▼
Read by QxMD icon Read
search

FVIII

keyword
https://www.readbyqxmd.com/read/28921445/quantification-of-coagulation-factor-viii-by-selective-reaction-monitoring
#1
Edson Galdino do Nascimento Filho, Guilherme Pauperio Lanfredi, Mário Soares Abreu-Neto, Dimas Tadeu Covas, Vitor Marcel Faça
Coagulation factor VIII (FVIII) is an important glycoprotein involved in the extrinsic coagulation cascade. Mutations in FVIII gene results in hemophilia A, a recessive coagulation disorder that is clinically managed by administration of purified FVIII from blood donors or recombinant FVIII. Because of its fundamental therapeutic application, biotechnological production of FVIII requires rigid quality control and monitoring in patients and clinical trials. Here, we describe a protocol for a mass spectrometry based approach termed selective reaction monitoring (SRM) as an important alternative tool for accurate and sensitive quantitation of purified or recombinant FVIII...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28921441/purification-and-autoactivation-method-for-recombinant-coagulation-factor-vii
#2
Vladimir Granovski, Marcela C C Freitas, Mario Soares Abreu-Neto, Dimas T Covas
Recombinant coagulation factor VII is a very important and complex protein employed for treatment of hemophiliac patients (hemophilia A/B) who develop inhibitors antibodies to conventional treatments (FVIII and FIX). The rFVII is a glycosylated molecule and circulates in plasma as zymogen of 50 kDa. When activated the molecule is cleaved to 20-30 kDa and has a half-life of about 3 h, needing to be processed fast and efficiently until freeze-drying. Here, we describe a very simple and fast purification sequence for rFVII using affinity FVII Select resin and a dialysis system that can be easily scaled up...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28921438/purification-methods-for-recombinant-factor-viii-expressed-in-human-liver-sk-hep-cells
#3
Vladimir Granovski, Mario Soares Abreu-Neto, Dimas Tadeu Covas
Coagulation factor VIII is one of the largest proteins attempted to be expressed in recombinant form. A very complex and labile protein which has a very short half-live and need a fast and efficient purification chain. Here, we describe a simple purification sequence using multimodal Capto MMC, affinity FVIII select and ion exchange SP-Fastflow chromatography steps without subjecting the target molecule to mechanical and temperature stress, separating impurities from rFVIII using net charge, hydrophobicity, and affinity of the molecules...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28920105/the-impact-of-von-willebrand-factor-on-factor-viii-memory-immune-responses
#4
Juan Chen, Jocelyn A Schroeder, Xiaofeng Luo, Qizhen Shi
Immune tolerance induction (ITI) with aggressive infusion of factor VIII (FVIII) is the current strategy used to eradicate FVIII inhibitors and restore normal FVIII pharmacokinetics in inhibitor patients. Whether the use of FVIII products containing von Willebrand factor (VWF) will affect the efficacy of ITI is still controversial. In this study, we explored the impact of VWF on FVIII memory immune responses in hemophilia A (HA) mice. A T-cell proliferation assay and cytokine profile analysis were used to study FVIII-primed CD4(+) T cells...
August 22, 2017: Blood Advances
https://www.readbyqxmd.com/read/28919830/identification-of-patients-with-congenital-hemophilia-in-a-large-electronic-health-record-database
#5
Michael Wang, Anissa Cyhaniuk, David L Cooper, Neeraj N Iyer
BACKGROUND: Electronic health records (EHRs) are an important source of information with regard to diagnosis and treatment of rare health conditions, such as congenital hemophilia, a bleeding disorder characterized by deficiency of factor VIII (FVIII) or factor IX (FIX). OBJECTIVE: To identify patients with congenital hemophilia using EHRs. DESIGN: An EHR database study. SETTING: EHRs were accessed from Humedica between January 1, 2007, and July 31, 2013...
2017: Journal of Blood Medicine
https://www.readbyqxmd.com/read/28916584/novel-insights-into-the-clinical-phenotype-and-pathophysiology-underlying-low-vwf-levels
#6
Michelle Lavin, Sonia Aguila, Sonja Schneppenheim, Niall Dalton, Kenneth L Jones, Jamie M O'Sullivan, Niamh M O'Connell, Kevin Ryan, Barry White, Mary Byrne, Marie Rafferty, Mairead M Doyle, Margaret Nolan, Roger J S Preston, Ulrich Budde, Paula James, Jorge Di Paola, James S O'Donnell
Critical clinical questions remain unanswered regarding diagnosis and management of patients with Low VWF levels (30-50 IU/dL). To address these questions, the Low VWF Ireland Cohort (LoVIC) study investigated 126 patients registered with Low VWF. Interestingly, despite their marginally reduced plasma VWF levels, ISTH BAT and Condensed MCMDM-1 VWD scores both confirmed significant bleeding phenotypes in the majority of LoVIC patients. For example, among female patients with Low VWF, 77% had ISTH BAT scores ≥6...
September 15, 2017: Blood
https://www.readbyqxmd.com/read/28905885/long-term-correction-of-hemophilia-a-mice-following-lentiviral-mediated-delivery-of-an-optimized-canine-factor-viii-gene
#7
J M Staber, M J Pollpeter, C-G Anderson, M Burrascano, A L Cooney, P L Sinn, D T Rutkowski, W C Raschke, P B McCray
Current therapies for hemophilia A include frequent prophylactic or on-demand intravenous factor treatments which are costly, inconvenient and may lead to inhibitor formation. Viral vector delivery of factor VIII (FVIII) cDNA has the potential to alleviate the debilitating clotting defects. Lentiviral-based vectors delivered to murine models of hemophilia A mediate phenotypic correction. However, a limitation of lentiviral-mediated FVIII delivery is inefficient transduction of target cells. Here, we engineer a feline immunodeficiency virus (FIV) -based lentiviral vector pseudotyped with the baculovirus GP64 envelope glycoprotein to mediate efficient gene transfer to mouse hepatocytes...
September 14, 2017: Gene Therapy
https://www.readbyqxmd.com/read/28900904/lonoctocog-alfa-a-review-in-haemophilia-a
#8
Zaina T Al-Salama, Lesley J Scott
Lonoctocog alfa (rVIII-SingleChain; Afstyla(®)) is a novel single-chain recombinant factor VIII (FVIII) molecule, with a truncated B-domain and the heavy and light chains covalently linked to form a stable and homogenous drug that binds with high affinity to von Willebrand factor (VWF). Intravenous lonoctocog alfa is approved for the prophylaxis and treatment of bleeding in patients with haemophilia A in several countries worldwide. In two pivotal, multicentre trials, lonoctocog alfa was effective in the treatment of bleeding episodes and as prophylaxis, including for perioperative management in adults, adolescents and children...
September 12, 2017: Drugs
https://www.readbyqxmd.com/read/28884611/an-evaluation-of-the-activated-partial-thromboplastin-time-waveform
#9
Takeshi Matsumoto, Hideo Wada, Naoki Fujimoto, Junki Toyoda, Yasunori Abe, Kohshi Ohishi, Yoshiki Yamashita, Makoto Ikejiri, Kei Hasegawa, Kei Suzuki, Hiroshi Imai, Kaname Nakatani, Naoyuki Katayama
The activated partial thromboplastin time (APTT) waveform includes several parameters that are related to various underlying diseases. The APTT waveform was examined in various diseases. Regarding the pattern of APTT waveform, a biphasic pattern of the first or second derivative curve (DC) was observed in patients with hemophilia and patients positive for antiphospholipid (aPL) antibodies or coagulation factor VIII (FVIII) inhibitors. The time of the first and second DC and fibrin formation at 1/2 height were prolonged in patients with hemophilia, patients with inhibitors, patients positive for aPL, patients treated with anti-Xa agents, and patients with disseminated intravascular coagulation (DIC)...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28867034/-neonatal-arterial-ischemic-stroke-which-thrombotic-biological-risk-factors-to-investigate-and-which-practical-consequences
#10
T Perez, J B Valentin, E Saliba, Y Gruel
All biological risk factors that have been previously identified to increase the risk of thrombosis in adults, have also been studied in neonates with arterial Ischemic Stroke (NAIS), but most studies were retrospective and included relatively low numbers of affected children. We therefore could not suggest recommendations with a strong level of evidence and only expert proposals potentially useful for clinical practice will be presented in this text. Despite these limitations, the extensive analysis of published data supported that factor V Leiden (FVL) and increased levels of Lp(a) could be significant risk factors for NAIS...
September 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28865249/effects-of-ultra-low-dose-versus-standard-hormone-therapy-on-fibrinolysis-and-thrombin-generation-in-postmenopausal-women
#11
Magdalena Piróg, Robert Jach, Anetta Undas
OBJECTIVE: To assess the effects of different doses of oral hormone therapy (HT) on thrombin generation and fibrinolysis. STUDY DESIGN: One hundred fifty postmenopausal women were assigned in a randomized controlled study in which the effect of standard dose (1mg 17β-estradiol/5mg dydrogesterone), ultra-low-dose HT (0.5mg 17β-estradiol/2.5mg dydrogesterone) on fibrinolysis and coagulation was compared to controls. Factors measured included plasma clot lysis time (CLT), fibrinolysis activators and inhibitors, thrombin generation (prothrombin fragments 1+2 [F1+2], endogenous thrombin potential [ETP]), normalized activated protein C sensitivity ratio (nAPCsr), and factor (F)VIII activity and were determined before and after 24 weeks of HT...
August 22, 2017: European Journal of Obstetrics, Gynecology, and Reproductive Biology
https://www.readbyqxmd.com/read/28837614/mice-deficient-in-the-anti-haemophilic-coagulation-factor-viii-show-increased-von-willebrand-factor-plasma-levels
#12
Klytaimnistra Kiouptsi, Alexandra Grill, Amrit Mann, Mareike Döhrmann, Maren Lillich, Sven Jäckel, Frano Malinarich, Henning Formes, Davit Manukyan, Saravanan Subramaniam, Avinash Khandagale, Cornelia Karwot, Serge C Thal, Markus Bosmann, Inge Scharrer, Kerstin Jurk, Christoph Reinhardt
Von Willebrand factor (VWF) is the carrier protein of the anti-haemophilic Factor VIII (FVIII) in plasma. It has been reported that the infusion of FVIII concentrate in haemophilia A patients results in lowered VWF plasma levels. However, the impact of F8-deficiency on VWF plasma levels in F8-/y mice is unresolved. In order to avoid confounding variables, we back-crossed F8-deficient mice onto a pure C57BL/6J background and analysed VWF plasma concentrations relative to C57BL/6J WT (F8+/y) littermate controls...
2017: PloS One
https://www.readbyqxmd.com/read/28836341/effect-of-late-prophylaxis-in-hemophilia-on-joint-status-a-randomized-trial
#13
Marilyn J Manco-Johnson, Bjorn Lundin, Sharon Funk, Charles Peterfy, David Raunig, Michael Werk, Christine L Kempton, Mark T Reding, Stefcho Goranov, Liana Gercheva, Luminita Rusen, Valentina Uscatescu, Marta Pierdominici, Sylvia Engelen, Jennifer Pocoski, Dale Walker, Walter Hong
BACKGROUND: Limited data exist on the impact of prophylaxis on adults with severe hemophilia A and pre-existing joint disease. OBJECTIVES: This analysis describes 3-year bleeding, joint, health-related quality-of-life (HRQoL), and other outcomes from the open-label, randomized, multinational SPINART study. PATIENTS/METHODS: Males aged 12-50 years with severe hemophilia A, ≥150 FVIII exposure days, no inhibitors, and no prophylaxis for >12 consecutive months in the past 5 years were randomized to sucrose-formulated recombinant FVIII (rFVIII-FS) prophylaxis or on-demand therapy (OD)...
August 24, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28836262/immune-status-of-patients-with-haemophilia-a-before-exposure-to-factor-viii-first-results-from-the-hemfil-study
#14
Letícia L Jardim, Daniel G Chaves, Amanda C O Silveira-Cassette, Ana Cristina Simões E Silva, Marcio P Santana, Monica H Cerqueira, Alessandra Prezotti, Claudia Lorenzato, Vivian Franco, Johanna G van der Bom, Suely M Rezende
Previous cross-sectional studies showed that some patients with haemophilia A (HA) without inhibitor presented a pro-inflammatory profile during factor VIII (FVIII) replacement therapy. Furthermore, an anti-inflammatory/regulatory state was described in HA patients after inhibitor development. However, no study investigated the levels of these biomarkers before exposure to exogenous FVIII. This study investigated the immunological profile of previously untreated patients (PUPs) with HA in comparison with non-haemophiliac boys...
August 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28830476/comparison-of-continuous-infusion-versus-bolus-injection-of-factor-concentrates-for-blood-management-after-total-knee-arthroplasty-in-patients-with-hemophilia
#15
Young Shil Park, Won-Ju Shin, Kang-Il Kim
BACKGROUND: Total knee arthroplasty (TKA) has become the treatment of choice for end-stage hemophilic arthropathy of the knee. Theoretically in hemophilia A, perioperative continuous infusion (CI) of factor VIII (FVIII) would provide a more consistent FVIII level than general bolus injections (BI) in TKA. Current study was designed to evaluate the effectiveness of CI of coagulation factor concentrates during the perioperative period compared to BI. METHODS: A total of 42 TKAs were performed in 31 patients with severe hemophilia A...
August 22, 2017: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/28815880/immunogenicity-efficacy-and-safety-of-nuwiq-%C3%A2-human-cl-rhfviii-in-previously-untreated-patients-with-severe-haemophilia-a-interim-results-from-the-nuprotect-study
#16
R J Liesner, M Abashidze, O Aleinikova, C Altisent, M J Belletrutti, A Borel-Derlon, M Carcao, H Chambost, A K C Chan, L Dubey, J Ducore, N A Fouzia, M Gattens, Y Gruel, B Guillet, N Kavardakova, M El Khorassani, A Klukowska, T Lambert, S Lohade, M Sigaud, V Turea, J K M Wu, V Vdovin, A Pavlova, M Jansen, L Belyanskaya, O Walter, S Knaub, E J Neufeld
INTRODUCTION: Nuwiq(®) (Human-cl rhFVIII) is a fourth generation recombinant FVIII, produced in a human cell line, without chemical modification or protein fusion. No inhibitors developed in studies with Nuwiq(®) in 201 previously treated patients with haemophilia A (HA). The immunogenicity, efficacy and safety of Nuwiq(®) in previously untreated patients (PUPs) with severe HA are being assessed in the ongoing NuProtect study. METHODS: The study, conducted across 38 centres worldwide, is evaluating 110 true PUPs of all ages and ethnicities enrolled for study up to 100 exposure days (EDs) or 5 years maximum...
August 16, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28804848/laboratory-testing-for-von-willebrand-factor-factor-viii-binding-for-2n-vwd
#17
Soma Mohammed, Emmanuel J Favaloro
Von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders develop due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for the VWF-related disorders requires assessment of both VWF level and VWF activity, the latter requiring multiple assays because of the many functions carried out by VWF to help prevent bleeding. The current paper describes a protocol for assessment of VWF activity by means of VWF: factor VIII binding (VWF:FVIIIB)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804835/chromogenic-factor-viii-assays-for-improved-diagnosis-of-hemophilia-a
#18
Susan Rodgers, Elizabeth Duncan
Hemophilia A is an inherited bleeding disorder caused by a reduced level of factor VIII coagulant activity (FVIII:C) in blood. Bleeding episodes may occur spontaneously in the severe form of hemophilia or after trauma in the milder forms. It is important that patients are diagnosed correctly, which includes placing them into the correct severity category of the disorder so that appropriate treatment can be given. Diagnosis is made by determination of the amount of FVIII:C in the blood, usually using a one-stage factor VIII:C assay...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804834/one-stage-factor-viii-assays
#19
Elizabeth Duncan, Susan Rodgers
Coagulation factor assays using one-stage methodology are widely used to measure factor levels for the purpose of detecting a deficiency, or to monitor replacement therapy. In this chapter, we focus on a method to measure Factor VIII coagulant activity (FVIII:C) by the one-stage assay (FVIII:C-1), with extra information provided to also allow this method to apply to Factor IX (FIX), Factor XI (FXI), and Factor XII (FXII). From the perspective of laboratory testing, these factors are components of the "intrinsic" coagulation pathway and are all measured in test systems based on the correction of the Activated Partial Thromboplastin Time (APTT)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28799202/immune-tolerance-induced-by%C3%A2-platelet-targeted-factor-viii-gene-therapy-in-hemophilia-a-mice-is-cd4%C3%A2-t-cell-mediated
#20
Y Chen, X Luo, J A Schroeder, J Chen, C K Baumgartner, J Hu, Q Shi
BACKGROUND: Immune responses are a major concern in gene therapy. Our previous studies demonstrated that platelet-targeted FVIII (2bF8) gene therapy together with in vivo drug-selection of transduced cells can rescue the bleeding diathesis and induce immune tolerance in FVIII(null) mice. OBJECTIVE: To investigate whether non-selectable 2bF8 lentiviral vector (LV) for the induction of platelet-FVIII expression is sufficient to induce immune tolerance and how immune tolerance is induced after 2bF8LV gene therapy...
August 11, 2017: Journal of Thrombosis and Haemostasis: JTH
keyword
keyword
45427
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"