• Differentiating between type 2B and platelet-type VWD, as well as between type 2N VWD and hemophilia A. Von Willebrand factor is mainly active in conditions of high blood flow and shear stress. (wikipedia.org)
  • and OBIZUR [Antihemophilic Factor (Recombinant), Porcine Sequence] for bleeding episodes in adults with acquired hemophilia A (acquired Factor VIII [FVIII] deficiency), approved in 2014. (genengnews.com)
  • Hemophilia is a rare genetic disorder in which blood doesn't clot normally because it lacks sufficient blood-clotting proteins known as clotting factors. (pharmiweb.com)
  • 1 patient had hemophilia B and the other von Willebrand disease. (cdc.gov)
  • Recombinant factor VIIa (rFVIIa) has become available for treating people with hemophilia with inhibitors who experience bleeding or require surgery. (medscape.com)
  • Originally, rFVIIa was developed for the treatment of bleeding complications in patients with hemophilia with alloantibodies (inhibitors) against exogenous factor VIII or IX. (medscape.com)
  • We describe the uses of rFVIIa in conditions unrelated to hemophilia and the treatment of acquired inhibitors of factors VIII and IX. (medscape.com)
  • In the past two years, several new coagulation factor products have been approved in the United States to treat hemophilia and von Willebrand disease. (marketingresearchbureau.com)
  • This included four new factor VIII products, three new factors IX products and the first recombinant von Willebrand factor, as well as a product to treat acquired hemophilia. (marketingresearchbureau.com)
  • Since 1993, the Marketing Research Bureau has surveyed and analyzed the hemophilia care market, annually, focusing on the most important issues including the conversion rate and rationale to new products, in particular recombinant factors, the adoption of prophylaxis and immune tolerance, the evolving use of plasma-derived products, the emergence of new companies and therapies, treatment preferences and pricing and reimbursement trends. (marketingresearchbureau.com)
  • CSL Behring announced today that the U.S. Food and Drug Administration has accepted for review the company's Biologics License Application (BLA) for its novel investigational recombinant factor VIII single-chain (rVIII-SingleChain) for the treatment of hemophilia A. In the pivotal clinical trial, rVIII-SingleChain met all primary endpoints. (csl.com)
  • Today, we have the only recombinant single-chain factor VIII product in late-stage development for the management of hemophilia A, and we are excited to be one step closer to providing this innovative treatment to patients in the U.S. (csl.com)
  • ADYNOVATE is an injectable medicine that is used to help treat and control bleeding in children and adults with hemophilia A (congenital Factor VIII deficiency). (adynovate.com)
  • ADVATE is a medicine used to replace clotting factor (factor VIII or antihemophilic factor) that is missing in people with hemophilia A (also called "classic" hemophilia). (adynovate.com)
  • New data to be presented from the ATLAS program for fitusiran, an investigational therapy for the prophylactic treatment of people with hemophilia A or B, with or without inhibitors, will showcase for the first time the hemostatic equivalency of antithrombin lowering in hemophilia A. Hemostatic equivalency is used to better understand how non-factor therapies, such as fitusiran, can help correct the clotting defect in hemophilia. (sanofi.com)
  • Hemophilia A is an X-linked, recessive disorder caused by deficiency of functional plasma clotting factor VIII (FVIII), which may be inherited or arise from spontaneous mutation. (medscape.com)
  • On September 5, 1995, a 13-year-old boy with mild hemophilia A (factor VIII deficiency) became acutely ill with nausea and vomiting after a 2-week period of fatigue, poor appetite, and low-grade fever. (cdc.gov)
  • In response to this bulletin, two brothers with hemophilia A (aged 6 and 7 years) who had received this clotting factor concentrate were identified and tested for anti-HAV on November 17. (cdc.gov)
  • Second, it binds and stabilizes the procoagulant protein factor VIII (FVIII). (medscape.com)
  • The main treatment options for patients with vWD are desmopressin (DDAVP), recombinant vWF, and vWF/factor VIII (vWF/FVIII) concentrates. (medscape.com)
  • Through total or partial funding, we support external investigators who initiate, design and implement high-quality hematological research that is focused on our recombinant products, Coagulation Factor IX (Recombinant) Albumin Fusion Protein and Antihemophilic Factor (Recombinant) Single Chain, and our plasma products, Human Plasma Derived von Willebrand Factor (HP-vWF/FVIII) concentrate, Fibrinogen, and Prothrombin complex concentrate [human]. (cslbehring.ch)
  • Depending on your specific situation, your healthcare provider will decide if you may use VONVENDI by itself, or if you require additional recombinant FVIII (rFVIII). (vonvendi.com)
  • VONVENDI is infused into your blood stream, where it acts like your body's natural von Willebrand factor (VWF), helping to form clots, and helping your own FVIII to work. (vonvendi.com)
  • von Willebrand disease patients lack both von Willebrand factor (VWF) and factor VIII (FVIII), which are critical for normal haemostasis. (bvsalud.org)
  • The conventional treatment for VWD includes desmopressin and replacement therapy with plasma derived FVIII with VWF concentrates or recombinant VWF. (bvsalud.org)
  • CAMBRIDGE, Massachusetts, January 31, 2022 - Takeda Pharmaceutical Company Limited ("Takeda") today announced that the U.S. Food & Drug Administration (FDA) approved VONVENDI ® [von Willebrand factor (Recombinant)] for routine prophylaxis to reduce the frequency of bleeding episodes in patients with severe Type 3 von Willebrand disease (VWD) receiving on-demand therapy. (hemophiliafed.org)
  • reduce the number of bleeding episodes when used regularly (prophylaxis) in adults with severe Type 3 von Willebrand disease receiving on-demand therapy. (hemophiliafed.org)
  • ALTUVIIIO is not indicated for the treatment of von Willebrand disease. (altuviiiohcp.com)
  • Von Willebrand disease (VWD) is the most common hereditary blood-clotting disorder in humans. (wikipedia.org)
  • In 2008 a new diagnostic category of "Low VWF" was proposed to include those individuals whose von Willebrand factor levels were below the normal reference range but not low enough to be von Willebrand disease (levels in the 30-50 IU/dL range). (wikipedia.org)
  • Genetic testing is typically not part of the initial workup for von Willebrand disease, and is not needed for people diagnosed with type 1 VWD based on clinical history and laboratory tests. (wikipedia.org)
  • Patients with von Willebrand disease typically display a normal prothrombin time and a variable prolongation of partial thromboplastin time. (wikipedia.org)
  • Vonvendi treats bleeding episodes in patients with von Willebrand disease. (rxwiki.com)
  • Vonvendi is a prescription medication used to treat and control bleeding episodes in patients with von Willebrand disease (VWD). (rxwiki.com)
  • Vonvendi is used to replace von Willebrand factor that is missing in people with von Willebrand disease. (rxwiki.com)
  • Von Willebrand disease (vWD) is a common, inherited, genetically and clinically heterogeneous hemorrhagic disorder caused by a deficiency or dysfunction of the protein termed von Willebrand factor (vWF). (medscape.com)
  • Mutations in von Willebrand factor and to a lesser extent, CD42b/GP1Ba, that affect the binding of VWF to the GP1B receptor are the primary cause of the hereditary bleeding disorder known as Type 2 von Willebrand disease (VWD). (peprotech.com)
  • von Willebrand factor (vWF), the macromolecular plasma glycoprotein named for its contribution to the hereditary bleeding disorder known as von Willebrand disease (vWD), functions as a key regulator of primary hemostasis. (biomedcentral.com)
  • INTRODUCTION: von Willebrand disease (VWD) is the common bleeding disorder with a clinically relevant bleeding prevalence of 1:10,000. (bvsalud.org)
  • Second-line therapy for von Willebrand disease (vWD). (intercept-usa.com)
  • von Willebrand disease (VWD) is the most commonly reported inherited bleeding disorder and may alternatively occur as an acquired von Willebrand syndrome (AVWS). (edu.au)
  • ADYNOVATE is not used to treat von Willebrand disease. (adynovate.com)
  • A medicine used to treat some factor deficiencies and von Willebrand disease. (curegt.org)
  • On October 20, during a hospital visit to evaluate vaginal bleeding 1 month postpartum, a 28-year-old woman with type 2 von Willebrand disease was found to have elevated liver enzymes and was IgM anti-HAV positive. (cdc.gov)
  • It arises from a deficiency in the quality or quantity of von Willebrand factor (VWF), a multimeric protein that is required for platelet adhesion. (wikipedia.org)
  • Vonvendi belongs to a group of drugs called recombinant protein treatments. (rxwiki.com)
  • Recombinant Human sCD42b/GP1Ba is a 54.6 kDa protein containing 496 amino acid residues that correspond to the extracellular portion of CD42b/GP1Ba, plus a C‐terminal His‐Tag. (peprotech.com)
  • Von Willebrand issue (VWF) is a 500- to 15 000-kDa multimeric protein circulating within the blood. (holliseden.com)
  • The F8(scFv)-DAAO-Q144R recombinant protein is expressed by each CHO-S and E. coli cells. (holliseden.com)
  • The full length protein consists of four modulesModule I shares partial identity with the N-terminal part of the Insulin-like Growth Factor Binding Proteins (IGFBPs). (neuromics.com)
  • Module IV resembles the CT domain of several extracellular protein including, Von Willebrand's factor and mucins. (neuromics.com)
  • CTGF is comprised of four distinct structural domains (modules), which are identified as IGF-Binding Protein (IGF-BP), von Willebrand Factor C (VWFC), Thrombospondin type I (TSP type I), and C-terminal cysteine knot-like (CTCK) domains. (peprotech.com)
  • Recombinant Human CTGF is an 11.0 kDa protein consisting of 97 amino acid residues. (peprotech.com)
  • Proteinet visade sig t o m ha en isopeptidbinding, vilket är den första som observerats i ett protein uttryckt av en Gram-negativ bakterie. (umu.se)
  • It acts as a carrier protein for Factor VIII and promotes platelet adhesion and aggregation. (nsjbio.com)
  • A partial recombinant human protein was used as the immunogen for this anti-von Willebrand Factor antibody. (nsjbio.com)
  • VWD/AVWS develops from defects and/or deficiency in the adhesive plasma protein von Willebrand factor (VWF). (edu.au)
  • Das entsprechende Protein weist einige Charakteristika auf, die die Identität mit einer von J. H. Waite aus dem Byssusfaden isolierten Catecholoxidase wahrscheinlich machen. (uni-bayreuth.de)
  • Coagulation Factor VIII Human, Recombinant Protein is produced in CHO cells and is a glycosylated polypeptide chain with 2322 amino acids. (ilexlife.com)
  • Transcript variant 2 encodes a putative small protein, isoform b, which consists primarily of the phospholipid binding domain of factor VIIIc. (ilexlife.com)
  • The hemostatic system consists of platelets, coagulation factors, and the endothelial cells lining the blood vessels. (medscape.com)
  • Under physiological circumstances, the resistance of the endothelial cell lining to interactions with platelets and coagulation factors prevents thrombosis. (medscape.com)
  • Platelets play a primary role in this process, interacting with subendothelium-bound von Willebrand factor (vWf) via the membrane glycoprotein (GP) Ib complex. (medscape.com)
  • The binding of von Willebrand factor (VWF) to its platelet receptor initiates the primary mechanism for the adhesion of platelets to a site of vascular injury and subsequent platelet activation. (peprotech.com)
  • Bitiscetin has recently been shown to induce von Willebrand factor (vWF)-dependent aggregation of fixed platelets (Hamako J, et al, Biochem Biophys Res Commun 226:273, 1996). (ashpublications.org)
  • V ON WILLEBRAND FACTOR (vWF) is a multimeric glycoprotein (GP) that plays a key role, under high-shear conditions, in the initial attachment of platelets to the extracellular matrix of the endothelial cells after injury of the vessel wall. (ashpublications.org)
  • von Willebrand Factor (vWF) is a multimeric glycoprotein that is found in endothelial cells, plasma and platelets. (nsjbio.com)
  • This increase in the thrombin burst occurs after direct rFVIIa activation of factors IX and X on the surface of activated platelets (even in the absence of factor VIII or IX). (medscape.com)
  • The rFVIIa seems to work in a TF-independent manner directly on factors IX and X on the phospholipid surface of activated platelets. (medscape.com)
  • rFVIIa is able to activate factor X on phospholipid vesicles, activated platelets, or monocytes independent of TF, although the TF-independent generation of thrombin is much less efficient than the TF-dependent thrombin generation by rFVIIa. (medscape.com)
  • We searched PubMed without time restriction using key words: bone marrow and fibrosis as the main stem against the terms: growth factors, cytokines and chemokines, morphology, megakaryocytes and platelets, myeloproliferative disorders, myelodysplastic syndrome, collagen biosynthesis, mesenchymal stem cells, vitamins and minerals and hormones, and mechanism of tissue fibrosis. (bvsalud.org)
  • Platelet adhesion (ie, of platelets to exposed vascular subendothelium) requires von Willebrand factor (VWF) and the platelet glycoprotein Ib/IX complex. (msdmanuals.com)
  • A very rare disorder in which a person's platelets do not have enough of the receptor (glycoprotein Tb/TX) needed to bind von Willebrand factor. (curegt.org)
  • IHC: Formalin-fixed, paraffin-embedded human tonsil stained with anti-von Willebrand Factor antibody (clone SPM577). (nsjbio.com)
  • This anti-von Willebrand Factor antibody is available for research use only. (nsjbio.com)
  • The optimal dilution of the anti-von Willebrand Factor antibody for each application should be determined by the researcher. (nsjbio.com)
  • Store the anti-von Willebrand Factor antibody at 2-8oC (with azide) or aliquot and store at -20oC or colder (without azide). (nsjbio.com)
  • VONVENDI is the only recombinant von Willebrand factor (VWF) replacement therapy, and the first and only treatment to reduce the frequency of bleeding episodes for severe Type 3 VWD approved by the FDA for routine prophylactic use. (hemophiliafed.org)
  • Your first dose of VONVENDI for each bleeding episode may be administered with a recombinant factor VIII as instructed by your healthcare provider. (hemophiliafed.org)
  • Your healthcare provider will instruct you whether additional doses of VONVENDI with or without recombinant factor VIII are needed. (hemophiliafed.org)
  • Vonvendi is the first FDA approved recombinant von Willebrand factor. (rxwiki.com)
  • VONVENDI is the first and only recombinant von Willebrand factor (rVWF) treatment approved for on-demand and surgery for adults with all types of VWD (Type 1, Type 2, and Type 3). (vonvendi.com)
  • VONVENDI is recombinant, meaning it's made without using human blood. (vonvendi.com)
  • Have been told that you have inhibitors to von Willebrand factor (because VONVENDI may not work for you). (vonvendi.com)
  • The role of vascular endothelial growth factor (VEGF) in differential vWF expression was investigated using cultured human umbilical vein endothelial cells (HUVECs). (biomedcentral.com)
  • Immune thrombotic thrombocytopenic purpura (iTTP) is an acute, rare life-threatening condition associated with antibodies to ADAMTS13, resulting in severe enzyme deficiency, failure of von Willebrand factor (VWF) cleavage, excess platelet-VWF binding and microthrombi formation, resulting in multi-organ damage. (haematologica.org)
  • For management of patients with vWD or factor XIII deficiency, Pathogen Reduced Cryoprecipitated Fibrinogen Complex should not be used if recombinant or specific virally-inactivated factor preparations are available. (intercept-usa.com)
  • The pharmacokinetics of rVIII-SingleChain compared with recombinant human antihemophilic factor VIII (octocog alfa) was also studied. (csl.com)
  • Antihemophilic factor. (curegt.org)
  • Activity assays comprise collagen binding (VWF:CB) and a ristocetin-based recombinant glycoprotein Ib-binding (VWF:GPIbR) assay that reflects a contemporary alternative to classical ristocetin cofactor (VWF:RCo). (edu.au)
  • Glycoprotein IIb/IIIa receptor antagonists block a receptor on the platelet for fibrinogen and von Willebrand factor. (wikidoc.org)
  • Transcript variant 1 encodes a large glycoprotein, isoform a, which circulates in plasma and associates with von Willebrand factor in a noncovalent complex. (ilexlife.com)
  • Formation of neutralizing antibodies (inhibitors) to Factor VIII is possible following administration of ALTUVIIIO. (altuviiiohcp.com)
  • Your doctor will perform blood tests to check your von Willebrand factor activity and the presence of any inhibitors. (rxwiki.com)
  • It emerged that bone marrow fibrosis is the outcome of complex interactions between growth factors, cytokines, chemokines and hormones together with their facilitators and inhibitors. (bvsalud.org)
  • if less than 5 Bethesda Units, human factor VIII may be effective, but higher titer inhibitors usually respond only to porcine factor VIII, recombinant factor VIIa or activated prothrombin complex concentrates. (nih.gov)
  • Your body may form inhibitors to factor VIII. (adynovate.com)
  • Talk with your HCP to make sure you are carefully monitored with blood tests for the development of inhibitors to factor VIII. (adynovate.com)
  • Testing for inhibitors is indicated when bleeding is not controlled after adequate amounts of factor concentrate are infused during a bleeding episode. (medscape.com)
  • Infused the same way as other Factor VIII replacement therapies but taken less frequently. (altuviiiohcp.com)
  • Biotechnology leader CSL Behring offers the broadest range of quality plasma-derived and recombinant therapies in our industry. (csl.com)
  • Grifols, S.A. has collaboration agreements with Canadian Blood Services for the processing of other plasma-derived products, and with GIANT and GigaGen to develop recombinant polyclonal immunoglobulin therapies. (yahoo.com)
  • Background: Arterial injury is immediately followed by platelet adhesion at the site of injury, a process that requires the interaction of subendothelial von Willebrand factor with the platelet GP1b receptor. (bgu.ac.il)
  • Free hemoglobin increases von Willebrand factor-mediated platelet adhesion in vitro: implications on circulatory devices. (bcm.edu)
  • Platelet adhesion involves a novel interaction between vimentin and von Willebrand factor under high shear stress. (bcm.edu)
  • The alpha granules contain hemostatic proteins such as fibrinogen, vWf, and growth factors (eg, platelet-derived growth factor and transforming growth factors). (medscape.com)
  • Limitations of Use: Pathogen Reduced Cryoprecipitated Fibrinogen Complex should not be used for replacement of factor VIII. (intercept-usa.com)
  • Other coagulation factor assays may be performed depending on the results of a coagulation screen. (wikipedia.org)
  • Platelet disorders lead to defects in primary hemostasis and produce signs and symptoms different from coagulation factor deficiencies (disorders of secondary hemostasis). (medscape.com)
  • The extracellular region contains one von Willebrand factor-like A domain and four cysteine-rich repeats. (bio-techne.com)
  • Connective Tissue Growth Factor belongs to the CCN family of proteins. (neuromics.com)
  • The CCN family presently consists of six members in human also known as: Cyr61 (Cystein rich 61), CTGF (Connective Tissue Growth Factor), Nov (Nephroblastoma Overexpressed gene), WISP-1, 2 and 3 (Wnt-1 Induced Secreted Proteins). (neuromics.com)
  • Determined by its ability to bind recombinant human von Willebrand Factor-A2 in a functional ELISA. (peprotech.com)
  • CD151 binding may actually stabilize VLA-3, enabling it to bind to additional factors (12). (bio-techne.com)
  • The manipulation of endothelial cells to secrete protective factors could enhance cardioprotection. (mdpi.com)
  • Following cleavage of the precursor prepro-vWF form, the mature vWF is stored in Weibel-Palade bodies until its release is stimulated by various secretagogues or pathological stimuli, including inflammatory factors. (biomedcentral.com)
  • vWF undergoes a variety of posttranslational modifications that influence the affinity and availability for Factor VIII, including cleavage of the propeptide and formation of N-terminal disulfide bonds. (nsjbio.com)
  • This has become the case for treatment of hemorrhage with recombinant factor VIIa (rFVIIa). (medscape.com)
  • At the site of injury, tissue factor (TF) and factor VIIa activate factors X and IX. (medscape.com)
  • The foreign substance could be a virus or germ or even factor concentrate. (curegt.org)
  • Hepatitis A outbreaks associated with receipt of clotting factor concentrate previously have been recognized in Europe but not in the United States (1-5). (cdc.gov)
  • During September-November 1995, three cases of hepatitis A in recipients of Alphanate (TM) * factor VIII concentrate (Alpha Therapeutic Corporation, Los Angeles, California) from lot number AP5014A were reported to CDC. (cdc.gov)
  • In addition, one case of hepatitis A in a recipient of AlphaNine S-D (TM) factor IX concentrate (Alpha Therapeutic Corporation) has been reported and is under investigation. (cdc.gov)
  • During the 6 weeks preceding illness, the patient had used 68 vials (approximately 34,000 units) from the implicated lot (i.e., lot number AP5014A) of Alphanate (TM) and nine vials from four lots of another brand of factor VIII concentrate. (cdc.gov)
  • During 1995, her only exposure to factor concentrate was use of 48 vials (approximately 24,000 units) of Alphanate (TM) from the implicated lot on September 19. (cdc.gov)
  • Log your first ADYNOVATE infusion to activate the FACTOR METER . (adynovate.com)
  • Module II includes a stretch of 70amino acid residues - which shares sequence identity with the Von Willebrand Factor Type C repeat (VWC). (neuromics.com)
  • Plasminogen activators (PA) Tissue-type plasminogen activators (alteplase, tenecteplase) are produced by recombinant technology. (wikidoc.org)
  • If assessment of plasma Factor VIII activity is needed, it is recommended to use a validated one-stage clotting assay. (altuviiiohcp.com)
  • Vascular endothelial cell (EC)-derived factors play an important role in endothelial-cardiomyocyte crosstalk and could save cardiomyocytes (CMs) from injury. (mdpi.com)
  • No other factor products had been used during this interval, and no other sources of infection were identified. (cdc.gov)
  • von Willebrand factor (vWF) is a potent regulator of angiogenesis, tumor growth, and metastasis. (biomedcentral.com)
  • Research on the GC-hemostasis association has revealed that the increased expression of tissue factor (TF) promotes the pathogenic conditions of coagulation, tumor growth, and angiogenesis [ 7 ]. (biomedcentral.com)