• It is deficient and/or defective in von Willebrand disease and is involved in many other diseases, including thrombotic thrombocytopenic purpura, Heyde's syndrome, and possibly hemolytic-uremic syndrome. (wikipedia.org)
  • Therefore, VWF deficiency or dysfunction (von Willebrand disease) leads to a bleeding tendency, which is most apparent in tissues having high blood flow shear in narrow vessels. (wikipedia.org)
  • Recent research also suggests that von Willebrand Factor is involved in the formation of blood vessels themselves, which would explain why some people with von Willebrand disease develop vascular malformations (predominantly in the digestive tract) that can bleed excessively. (wikipedia.org)
  • A study by Ladikou et al indicated that patients with coronavirus disease 2019 (COVID-19) commonly have high levels of vWF and factor VIIIc, which may play a role in the hypercoagulable state and increased venous thromboembolism (VTE) rate encountered in COVID-19. (medscape.com)
  • In type III von WiUebrand disease (vWD) patients, the bleeding time was only partially corrected or not modified after cryoprecipitate infusion, although the levels and the multimeric structure of plasma von WiUebrand factor (vWF) were normal. (karger.com)
  • Plasma von Willebrand factor in connective tissue disease. (bmj.com)
  • Examination of the entire von Willebrand factor (VWF) gene for mutations, particularly in types 1 and 3 von Willebrand disease (VWD) is becoming more widely practised. (nih.gov)
  • 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)
  • There have been recent advances in von Willebrand disease (vWD) testing as well as recent updates in the diagnosis and management of vWD. (siemens-healthineers.com)
  • He has been involved and continues to be involved in the training of pathology residents in the Washington DC area and has current research interests in heparin induced thrombocytopenia, von Willebrand disease testing, therapeutic apheresis outcomes, and pediatric reference intervals. (siemens-healthineers.com)
  • Mild forms of von Willebrand disease may become apparent only when abnormal bleeding occurs following surgery or a serious injury. (medlineplus.gov)
  • In severe cases of von Willebrand disease, heavy bleeding occurs after minor trauma or even in the absence of injury (spontaneous bleeding). (medlineplus.gov)
  • Symptoms of von Willebrand disease may change over time. (medlineplus.gov)
  • Von Willebrand disease is divided into three types. (medlineplus.gov)
  • Von Willebrand disease is estimated to affect 1 in 100 to 10,000 individuals. (medlineplus.gov)
  • Most researchers agree that von Willebrand disease is the most common genetic bleeding disorder. (medlineplus.gov)
  • Variants in the VWF gene cause von Willebrand disease. (medlineplus.gov)
  • Abnormally slow blood clotting causes the prolonged bleeding episodes seen in people with von Willebrand disease. (medlineplus.gov)
  • The three types of von Willebrand disease are classified based on the amount of von Willebrand factor that is produced or its ability to function. (medlineplus.gov)
  • Variants in the VWF gene that reduce the amount of von Willebrand factor cause type 1 von Willebrand disease. (medlineplus.gov)
  • People with type 1 von Willebrand disease have varying amounts of von Willebrand factor in their bloodstream. (medlineplus.gov)
  • Variants that impair the function of von Willebrand factor cause the four subtypes of type 2 von Willebrand disease. (medlineplus.gov)
  • People with type 2 von Willebrand disease can have bleeding episodes of varying severity depending on the extent of the von Willebrand factor abnormalities, but the bleeding episodes are typically similar to those seen in type 1. (medlineplus.gov)
  • An abnormal gene can make some kids not have enough vWF, or a defective version of it, causing the bleeding disorder von Willebrand disease . (hopkinsallchildrens.org)
  • Doctors order the vWF antigen test to help diagnose or monitor the treatment of von Willebrand disease. (hopkinsallchildrens.org)
  • 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)
  • Structure and function of von Willebrand factor (vWF) in relation to von Willebrand disease. (thebloodproject.com)
  • This test allows identifying qualitatively abnormal variants of the vWF, also known as type II von Willebrand disease (vWD). (medscape.com)
  • Wiseguy reports clinical trial report, "Von Willebrand Disease (Factor VIII Deficiency) Global Clinical Trials Review, H2, 2016' provides an overview of Von Willebrand Disease (Factor VIII Deficiency) clinical trials scenario. (marketersmedia.com)
  • This report provides top line data relating to the clinical trials on Von Willebrand Disease (Factor VIII Deficiency). (marketersmedia.com)
  • ALTUVIIIO is not indicated for the treatment of von Willebrand disease. (altuviiiohcp.com)
  • Von Willebrand Disease (VWD) is the most prevalent bleeding disorder, causing excessive bleeding and bruising in 1% of the population. (isth.org)
  • For a long time, autosomal recessive inheritance has been considered a unique feature of type 3 von Willebrand disease (VWD), which is characterized by the virtual absence of von Willebrand factor (VWF) in plasma and storage compartments. (karger.com)
  • Castaman G, Federici AB, Rodeghiero F, Mannucci PM: Von Willebrand's disease in the year 2003: towards the complete identification of gene defects for correct diagnosis and treatment. (karger.com)
  • Rodeghiero F, Castaman G: von Willebrand disease type 1: definition, phenotypes, clinical and laboratory assessment. (karger.com)
  • Eikenboom JCJ, Reitsma PH, Peerlinck KMJ, Briët E: Recessive inheritance of von Willebrand's disease type I. Lancet 1993;341:982-986. (karger.com)
  • Eikenboom JC: von Willebrand disease type 3: clinical manifestations, pathophysiology and molecular biology. (karger.com)
  • Eikenboom JCJ, Castaman G, Vos HL, Bertina RM, Rodeghiero F: Characterization of the genetic defects in recessive type 1 and type 3 von Willebrand disease patients of Italian origin. (karger.com)
  • Castaman G, Novella E, Castiglia E, Eikenboom JCJ, Rodeghiero F: A novel family with recessive von Willebrand disease due to compound heterozygosity for a splice site mutation and a missense mutation in the von Willebrand factor gene. (karger.com)
  • Castaman G, Bertoncello K, Bernardi M, Eikenboom JC, Budde U, Rodeghiero F: Autosomal recessive von Willebrand disease associated with compound heterozygosity for a novel nonsense mutation (2908 del C) and the missense mutation C2362F: definite evidence for the non-penetrance of the C2362F mutation. (karger.com)
  • Tjernberg P, Castaman G, Vos HL, Bertina RM, Eikenboom JCJ: Homozygous C2362F von Willebrand factor induces intracellular retention of mutant von Willebrand factor resulting in autosomal recessive severe von Willebrand disease. (karger.com)
  • Impaired production, control or secretion of VWF leads to the clinical symptoms referred to as von Willebrand disease, named the most frequent inherited blood loss disorder23. (buyresearchchemicalss.net)
  • DelveInsight's, " Von Willebrand's Disease - Pipeline Insight, 2022 ," report provides comprehensive insights about 4+ companies and 4+ pipeline drugs in Von Willebrand's Disease pipeline landscape. (marketresearch.com)
  • People with von Willebrand disease (VWD) have a problem with a protein called von Willebrand factor (VWF). (marketresearch.com)
  • Von Willebrand's Disease - Pipeline Insight, 2022"" report by DelveInsight outlays comprehensive insights of present scenario and growth prospects across the indication. (marketresearch.com)
  • A detailed picture of the Von Willebrand's Disease pipeline landscape is provided which includes the disease overview and Von Willebrand's Disease treatment guidelines. (marketresearch.com)
  • The assessment part of the report embraces, in depth Von Willebrand's Disease commercial assessment and clinical assessment of the pipeline products under development. (marketresearch.com)
  • In the report, detailed description of the drug is given which includes mechanism of action of the drug, clinical studies, NDA approvals (if any), and product development activities comprising the technology, Von Willebrand's Disease collaborations, licensing, mergers and acquisition, funding, designations and other product related details. (marketresearch.com)
  • The companies and academics are working to assess challenges and seek opportunities that could influence Von Willebrand's Disease R&D. The therapies under development are focused on novel approaches to treat/improve Von Willebrand's Disease. (marketresearch.com)
  • This segment of the Von Willebrand's Disease report encloses its detailed analysis of various drugs in different stages of clinical development, including phase II, I, preclinical and Discovery. (marketresearch.com)
  • BT 200 is a pegylated synthetic RNA oligonucleotide is being developed by Band Therapeutics for the prevention of arterial thrombosis and treatment of Von Willebrand's Disease. (marketresearch.com)
  • The drug is currently in phase II stage of clinical trial evaluation for the treatment of Von Willebrand's Disease. (marketresearch.com)
  • 4+ key companies which are developing the therapies for Von Willebrand's Disease. (marketresearch.com)
  • The companies which have their Von Willebrand's Disease drug candidates in the most advanced stage, i.e. phase II include, Band Therapeutics. (marketresearch.com)
  • Von Willebrand's Disease pipeline report provides the therapeutic assessment of the pipeline drugs by the Route of Administration. (marketresearch.com)
  • 2004). "The diagnosis of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization" . (wikipedia.org)
  • In our cohort of von Willebrand disease (VWD) patients, we found four missense substitutions located either in the A1 domain linker (p.Cys1227Arg), A1 region (p.Leu1288Arg and p.Leu1340Arg), or A2 domain (p.Val1524Gly). (isth.org)
  • Yadegari H, Biswas A, Sadangi S, Oldenburg J. Investigating Pathomolecular Mechanisms von Willebrand Disease Variants Located in a Domains of the von Willebrand Factor [abstract]. (isth.org)
  • Von Willebrand Disease is caused by low levels of functional von Willebrand factor. (emoryhealthcare.org)
  • There are at least 6 types of von Willebrand Disease. (emoryhealthcare.org)
  • Guidelines and recommendations for the acute and prophylactic treatment of bleeding in von Willebrand disease (VWD) patients with von Willebrand factor (VWF)/factor VIII (FVIII) concentrates should be based on the analysis of the content of VWF/FVIII concentrates and on pharmacokinetic studies in patients with different severity of VWD (type 1, type 2 or type 3). (uantwerpen.be)
  • Since then, the VWF:CB has continued to be used to help diagnose von Willebrand disease (VWD) ( correctly) and also to help assign the correct subtype, as well as to assist in the monitoring of VWD therapy, especially desmopressin (DDAVP). (edu.au)
  • 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)
  • Early diagnosis of von Willebrand disease (VWD) is important to make sure that you're treated and can live a normal, active life. (hoacny.com)
  • Bleeding disorders, such as hemophilia and von Willebrand disease (VWD) , are conditions in which the blood does not clot properly due to a lack of specific clotting factor proteins in the blood. (cdc.gov)
  • Published findings from a Community Counts study that estimates the number of people with rare bleeding disorders, other than hemophilia and von Willebrand disease, receiving care at Hemophilia Treatment Centers in the United States. (cdc.gov)
  • Von Willebrand disease (VWD) is a hereditary quantitative deficiency or functional abnormality of von Willebrand factor (VWF), which causes platelet dysfunction. (msdmanuals.com)
  • Acquired von Willebrand disease is rare and is characterized by low levels of VWF due to decreased production or increased clearance of VWF from the circulation but is not inherited. (msdmanuals.com)
  • Bleeding manifestations in the most common type I von Willebrand disease (VWD) include bruising, mucosal bleeding, bleeding from small skin cuts that may stop and start over hours, increased menstrual bleeding, and sometimes bleeding after surgical procedures (eg, tooth extraction, tonsillectomy). (msdmanuals.com)
  • 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)
  • A von Willebrand factor (vWF) antigen test measures the amount of a clotting factor called von Willebrand factor. (hopkinsallchildrens.org)
  • vWF antigen, vWF activity, and factor VIII activity are required before performing this test. (medscape.com)
  • Blood was sampled for thrombin-antithrombin complex (TAT), prothrombin activation fragments 1 + 2 (F(1 + 2)), von Willebrand factor antigen (VWF:Ag), D-dimer, and interleukin-6 (IL-6) at baseline and after 2 h. (nih.gov)
  • The effect of vibration on back discomfort and serum levels of von Willebrand factor antigen: a preliminary communication. (cdc.gov)
  • Diagnosis is based on low levels of von Willebrand factor antigen and von Willebrand factor activity (ristocetin cofactor activity). (msdmanuals.com)
  • Another form of the disorder, acquired von Willebrand syndrome, is not caused by inherited gene variants (also called mutations). (medlineplus.gov)
  • Objective: To review the use of Von Willebrand Factor (VWF) concentrate for treatment of acquired Von Willebrand syndrome (VWS)-related bleeding in adult extracorporeal membrane oxygenation (ECMO) patients and determine if it was associated with improved VWF laboratory parameters. (gwu.edu)
  • 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)
  • Important in the maintenance of hemostasis, it promotes adhesion of platelets to the sites of vascular injury by forming a molecular bridge between sub-endothelial collagen matrix and platelet-surface receptor complex GPIb-IX-V. Also acts as a chaperone for coagulation factor VIII, delivering it to the site of injury, stabilizing its heterodimeric structure and protecting it from premature clearance from plasma. (rcsb.org)
  • Draw specimen before coagulation factor replacement therapy. (medscape.com)
  • Herein, we summarize our experience with oligonucleotide antithrombotic agents and their complementary antidotes, targeting the platelet adhesive protein von Willebrand factor and the pivotal coagulation protease factor IXa. (duke.edu)
  • Overview of Hemostasis Hemostasis, the arrest of bleeding from an injured blood vessel, requires the combined activity of Vascular factors Platelets Plasma coagulation factors Regulatory mechanisms counterbalance. (msdmanuals.com)
  • 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)
  • A partial recombinant human protein was used as the immunogen for this anti-von Willebrand Factor antibody. (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)
  • 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)
  • 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)
  • The extent of factor deficiency determines the probability and severity of bleeding. (msdmanuals.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)
  • Title : Evaluation of von Willebrand factor phenotypes and genotypes in Hemophilia A patients with and without identified F8 mutations Personal Author(s) : Boylan, Brian;Rice, Anne S.;De Staercke, Christine;Eyster, M. Elaine;Yaish, Hassan M.;Knoll, Christine M.;Bean, Christopher J.;Miller, Connie H. (cdc.gov)
  • Treatment with investigational efanesoctocog alfa (formerly BIVV001) -- a new class of factor VIII replacement therapy -- prevented bleeding episodes in patients with severe hemophilia A, according to results from the phase III XTEND-1 trial . (medpagetoday.com)
  • Collectively, these results show that by maintaining high sustained factor VIII activity, once-weekly efanesoctocog alfa provided substantial improvements in clinical outcomes and quality of life for patients with severe hemophilia A," the authors wrote. (medpagetoday.com)
  • The ability to provide high, sustained factor VIII activity in patients with hemophilia A has been constrained by the von Willebrand factor-imposed half-life ceiling. (medpagetoday.com)
  • While normalizing factor VIII levels helps protect patients with hemophilia A from spontaneous and traumatic bleeding, thus preserving joint health, von Drygalski and colleagues pointed out that the interaction between factor VIII and endogenous von Willebrand factor limits the half-life of current factor VIII replacement products to 8 to 19 hours. (medpagetoday.com)
  • 50 IU per deciliter) with currently available factor VIII therapies requires frequent administration, which confers a substantial treatment burden on people with hemophilia and their caregivers," they wrote. (medpagetoday.com)
  • In a crowded field of transformative therapies for hemophilia, efanesoctocog alfa stands out as a winner -- a major therapeutic advance that achieves highly protective factor VIII levels with a once-weekly infusion," Leissinger concluded. (medpagetoday.com)
  • A reconstitution device (a device used to "reconstitute" or mix factor with a liquid [usually water], called a diluent, before it can be infused or given to a person with hemophilia). (cdc.gov)
  • In type 2 N, there is impaired binding of VWF to factor VIII and significant reduction in factor VIII levels (ie, to 1 to 5%) similar to that seen in hemophilia A. (msdmanuals.com)
  • Hemophilia Hemophilias are common hereditary bleeding disorders caused by deficiencies of either clotting factor VIII or IX. (msdmanuals.com)
  • 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)
  • 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)
  • von Willebrand factor type C domain) Monomers are subsequently N-glycosylated, arranged into dimers in the endoplasmic reticulum and into multimers in the Golgi apparatus by crosslinking of cysteine residues via disulfide bonds. (wikipedia.org)
  • The plasma von Willebrand factor (vWF) multimer analysis provides a qualitative visual assessment of the size spectrum and the banding pattern of VWF multimers present in plasma compared with a normal control. (medscape.com)
  • Von Willebrand multimers = look to see if the reason it is not functioning is because the larger multimers are not present. (wikibooks.org)
  • The ability of von Willebrand factor (VWF) to initiate platelet adhesion depends on the number of monomers in individual VWF multimers and on the self - association of individual VWF multimers into larger structures. (bvsalud.org)
  • Von Willebrand factor (VWF) (German: [fɔn ˈvɪləbʁant]) is a blood glycoprotein involved in hemostasis, specifically, platelet adhesion. (wikipedia.org)
  • Von Willebrand Factor's primary function is binding to other proteins, in particular factor VIII, and it is important in platelet adhesion to wound sites. (wikipedia.org)
  • It acts as a carrier protein for Factor VIII and promotes platelet adhesion and aggregation. (nsjbio.com)
  • Savage, B., Saldivar, E. & Ruggeri, Z.M. Initiation of platelet adhesion by arrest onto fibrinogen or translocation on von Willebrand factor. (nature.com)
  • Endothelial secretion of von Willebrand factor (VWF) from intracellular organelles referred to as Weibel-Palade bodies (WPBs) is necessary for platelet adhesion towards the hurt vessel wall. (buyresearchchemicalss.net)
  • High-density lipoprotein modulates thrombosis by preventing von Willebrand factor self-association and subsequent platelet adhesion. (bvsalud.org)
  • 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)
  • This has become the case for treatment of hemorrhage with recombinant factor VIIa (rFVIIa). (medscape.com)
  • Ishler B, Dickerson M, Lee A, Hale A, Moskowitz K. Lyophilized Human Platelets Show Hemostatic Function Independent of von Willebrand Factor [abstract]. (isth.org)
  • A standard nomenclature for von Willebrand factor gene mutations and polymorphisms. (nih.gov)
  • In addition, hypoxia induces the endothelial expression of adhesion molecules, including von Willebrand factor (VWF). (usp.br)
  • Treatment is with infusion of von Willebrand factor into the vein or by releasing stored von Willebrand factor using desmopressin. (emoryhealthcare.org)
  • The VWF gene provides instructions for making a blood clotting protein called von Willebrand factor, which is essential for the formation of blood clots . (medlineplus.gov)
  • the A3 domain, which binds to collagen (von Willebrand factor type A domain) the C4 domain, in which the RGD motif binds to platelet integrin αIIbβ3 when this is activated (von Willebrand factor type C domain) the other C domains, which may interact in ER dimers: the larger protein show six beads of (C and C-like) domains under cryo-EM. (wikipedia.org)
  • Every monomer contains particular binding domains that provide them the capacity to interact and bind other proteins specifically, factor VIII, collagen, and some platelet receptors. (medscape.com)
  • The von Willebrand factor (VWF) collagen binding (VWF:CB) assay was first reported for use in von Willebrand diagnostics in 1986, by Brown and Bosak. (edu.au)
  • von Willebrand Factor (vWF) is a multimeric glycoprotein that is found in endothelial cells, plasma and platelets. (nsjbio.com)
  • von Willebrand factor (vWF) is a large multimeric glycoprotein synthesized as a 2050 amino acid monomer in endothelium, megakaryocytes, and the subendothelial connective tissue. (medscape.com)
  • Structure-function relationships of platelet glycoprotein Ib as a receptor for von Willebrand factor. (elsevierpure.com)
  • 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)
  • The role of vascular endothelial growth factor (VEGF) in differential vWF expression was investigated using cultured human umbilical vein endothelial cells (HUVECs). (biomedcentral.com)
  • Von Willebrand factor is synthesized and secreted by vascular endothelium to form part of the perivascular matrix. (msdmanuals.com)
  • Von Willebrand Factor Concentrate Administration for Acquired Von Will" by Michael Mazzeffi, Allison Bathula et al. (gwu.edu)
  • Testing for inhibitors is indicated when bleeding is not controlled after infusion of adequate amounts of factor concentrate during a bleeding episode. (medscape.com)
  • 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)
  • Other factor deficiencies are more rare but can include factor V, factor VII, factor X, factor XI, and fibrinogen. (emoryhealthcare.org)
  • In COVID-19+ patients, we found pronounced increases in plasma levels of von Willebrand factor (vWF) and fibrinogen. (lu.se)
  • The key factors in clot formation are the chemical and physical nature of the surfaces and their interactions with the blood components, such as platelets and plasma proteins. (springer.com)
  • Von Willebrand factor has two main functions: 1) connecting platelets to sites of injured blood vessels and 2) protecting factor VIII from normal breakdown in the blood circulation. (emoryhealthcare.org)
  • 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)
  • 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)
  • In addition, vWF is a carrier protein for factor VIII and prevents its proteolytic degradation in plasma. (medscape.com)
  • Cloudy plasma may result in underestimation of vWF:Ag levels, while the presence of rheumatoid factor in the plasma can produce overestimation. (medscape.com)
  • If assessment of plasma Factor VIII activity is needed, it is recommended to use a validated one-stage clotting assay. (altuviiiohcp.com)
  • VWF is also required to maintain normal plasma factor VIII levels. (msdmanuals.com)
  • L'agrégation plaquettaire induite par le collagène dans des échantillons de plasma riche en plaquettes de 14 lapins sains a été mesurée par turbidimétrie en utilisant un agrégomètre, avant et une heure après une injection intra- veineuse d'alun. (who.int)
  • Testing can be complex and requires tests of protein levels as well as the function of von Willebrand factor and often examination of the von Willebrand factor gene. (emoryhealthcare.org)
  • These disorders are also are diagnosed by measuring the factor levels and often evaluation of the corresponding gene. (emoryhealthcare.org)
  • She also pointed out that while trials of factor VIII gene therapies also show prolonged production of factor VIII, the latest results suggest that factor VIII production in most patients gradually declines over a few years to trough levels demonstrated with weekly efanesoctocog alfa. (medpagetoday.com)
  • Formation of neutralizing antibodies (inhibitors) to Factor VIII is possible following administration of ALTUVIIIO. (altuviiiohcp.com)
  • Your body can make neutralizing antibodies which inhibit von Willebrand factor and/or factor VIII from working correctly. (rxwiki.com)
  • In addition to checking for neutralizing antibodies, your doctor will also check your von Willebrand factor levels to make sure they are not too high, which could increase your risk of blood clots. (rxwiki.com)
  • Proteins called clotting factors are needed for blood to clot properly and help prevent too much bleeding. (hopkinsallchildrens.org)
  • Among 133 patients, almost all males, who received once-weekly prophylaxis with efanesoctocog alfa 50 IU/kg for 52 weeks (group A), the median annualized bleeding rate was 0 (interquartile range 0-1.04), and the estimated mean annualized bleeding rate was 0.71 (95% CI 0.52-0.97), reported Annette von Drygalski, MD, PharmD, of the University of California San Diego, and colleagues. (medpagetoday.com)
  • In an editorial accompanying the study , Cindy Leissinger, MD, of Tulane University School of Medicine in New Orleans, said a major advantage of efanesoctocog alfa "is its value as a true factor VIII replacement that can be used to treat acute bleeding and can be measured by standard laboratory assays to allow for monitoring and dose adjustments when needed. (medpagetoday.com)
  • Injections of treatment products, called clotting factor concentrates (or factor), into a vein - a process known as infusion - can help manage and treat bleeding disorders . (cdc.gov)
  • elements of note are: the D'/D3 domain, which binds to factor VIII (von Willebrand factor type D domain). (wikipedia.org)
  • In type 1C, the amount of von Willebrand factor is low because the protein is removed from the body more quickly than usual. (medlineplus.gov)
  • In type 2A, von Willebrand factor is not the right size and cannot help form clots. (medlineplus.gov)
  • In type 2B, von Willebrand factor forms clots too easily. (medlineplus.gov)
  • We report our experience with 12 families with clearly recessive inheritance, but definitely measurable factor VIII and VWF, which is not typical for severe type 3 VWD. (karger.com)
  • The two in indistinguishable clinically = they have to determined by specific factor assays. (wikibooks.org)
  • If these assays are used, divide the result by 2.5 to approximate the patient's ALTUVIIIO Factor VIII activity level. (altuviiiohcp.com)
  • Particular secretory granules inside the endothelial cell referred to as Weibel-Palade body (WPBs) contain several biologically active substances, although von Willebrand element (VWF) is definitely the most abundant17,18. (buyresearchchemicalss.net)
  • Von Willebrand factor acts as a glue that holds blood clots together and prevents the breakdown of other blood clotting proteins. (medlineplus.gov)
  • In types 2M and 2N, von Willebrand factor is unable to interact with other structures or proteins needed to form blood clots. (medlineplus.gov)
  • 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)
  • Your doctor will perform blood tests to check your von Willebrand factor activity and the presence of any inhibitors. (rxwiki.com)
  • The Human Von Willebrand Factor ELISA kit can be used to identify samples from the human species. (elisakit.cc)
  • The ALTUVIIIO Factor VIII activity level is overestimated by the chromogenic assay and a specific ellagic acid-based aPTT reagent in one-stage clotting assay by approximately 2.5-fold. (altuviiiohcp.com)
  • This test checks the clotting activity of factor VIII. (hoacny.com)
  • Some people who have VWD have low levels of factor VIII activity, while others have normal levels. (hoacny.com)
  • Of note, von Drygalski and team said that once-weekly prophylaxis with efanesoctocog alfa provided mean factor VIII activity of more than 40 IU/dL for approximately 4 days after administration and of 15 IU/dL at day 7. (medpagetoday.com)
  • Prostate cancer-secreted CCN3 uses the GSK3β and β-catenin pathways to enhance osteogenic factor levels in osteoblasts. (nih.gov)
  • Von Willebrand issue (VWF) is a 500- to 15 000-kDa multimeric protein circulating within the blood. (holliseden.com)