Extractions: About ZLB Behring Media Room U.S. Products ... eNEWS Updates von Willebrand disease (VWD) is a hereditary disorder characterized by abnormally slow clotting of the blood. Patients with von Willebrand disease may have spontaneous and prolonged bleeding from the nose and gums. VWD occurs in about one in 100 persons and affects both males and females. von Willebrand disease occurs when one of the coagulation factors, called von Willebrand factor, does not work properly or when there is not enough in the blood. The lower the level of active von Willebrand factor in a person's blood, the more severe the disease. In many patients with von Willebrand disease, another coagulation factor, factor VIII, is also affected. People with von Willebrand disease need to boost the level of von Willebrand factor and factor VIII in their blood. People with mild or moderate type 1 von Willebrand disease may be infused with the chemical DDAVP (desmopressin acetate) or use a high-concentrate nasal spray containing DDAVP. DDAVP causes the release of von Willebrand factor from storage sites in the body and increases the level in the blood. Patients with type 2 or type 3 von Willebrand disease may need replacement von Willebrand factor and factor VIII.
Summary Of Von Willebrand Disease von willebrand disease is the most common inherited bleeding disorder. People with von willebrand disease may have low levels of or a defective clotting http://www.patientlinx.com/blooddisorders/sumvw.cfm
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Haemophilia Foundation Australia // Knowledge A Many people with von willebrand disorder don t have any symptoms but those Some children have von willebrand disorder because there was a mutation, http://www.haemophilia.org.au/knowledge/cid/29/category/von Willebrand Disorder/
Extractions: Q: 01. What are the symptoms of vWD? A: Many people with von Willebrand disorder don't have any symptoms but those who do may experience: frequent nosebleeds, easy bruising, heavy menstrual (period) flow, excessive bleeding in the mouth (from tongue and gums). Bleeding usually occurs only after injury although people with the severe type of von Willebrand disorder may bleed spontaneously. Q: 02. Is von Willebrands only an inherited condition? A: There is not always a family history. Some children have von Willebrand disorder because there was a mutation, or change in the gene, during the foetal stage. When this happens the person's chances of passing von Willebrand disorder on to his/her children are the same as those of a parent who has inherited the condition. Von Willebrands can also be acquired rather than inherited, but this is very rare. This means the person is not born with the disorder but develops it at a later time. For example, someone with serious immune system problems such as rheumatoid arthritis, types of kidney failure or certain cancers might develop acquired von Willebrands. The problem is usually caused by antibodies produced against the persons own clotting factors that destroy or hinder the function of the von Willebrand factor or factor VIII.
Haemophilia Foundation Australia // Knowledge von willebrand Disorder (vWD) is due to a deficiency of von willebrand Factor The abnormal gene in von willebrand disorder is on one of the regular http://www.haemophilia.org.au/knowledge/cid/9/category/von Willebrand Disorder/p
Extractions: 02. How is von Willebrand Disorder inherited? Von Willebrand disorder is mostly a genetic disorder. This means it is passed down through the genes from parent to child. The abnormal gene in von Willebrand disorder is on one of the regular chromosomes, not on one of the sex chromosomes (like haemophilia).
Von Willebrand Factor In particular, the von willebrand Factor (vWF) Ag determination is essential for The IL Test von willebrand Factor is a latex particle enhanced http://www.beckman.com/products/testdetail/hemostasis/ILTestvonWillebrandFactor.
Extractions: Related Product Information Select Features Test Menu Lab Resources Literature MSDS Link Press Releases Support Overview Hemostasis Home Hemostasis Pubs The diagnosis of Von Willebrand disease (VWD)likely the most prevalent congenital bleeding disorderrequires specialized laboratory tests. In particular, the von Willebrand Factor (vWF) Ag determination is essential for attaining a reliable diagnosis. The IL Test⢠von Willebrand Factor is a latex particle enhanced immunoturbidimetric assay for quantification of vWF:Ag in plasma, specially designed to be used on IL's fully automated coagulation analyzers. It offers the advantage of being less labor intensive and more readily automated than the ELISA assay, which results in an easier and more cost effective managementwith use of stored calibrationof Von Willebrand disease. Features Note: Not all products are available in all countries.
Extractions: helpful? yes no The conditions covered in this entry are haemophilia A, haemophilia B (Christmas disease), von Willebrand disease and some other coagulation defects. The haemophilias (hemophilias - US) are a group of inheritable blood disorders characterised by various defects in the blood clotting system. The clotting factors present in blood were initially known by Roman numerals, numbered from I to XIII. More recently described factors have been given names and some deficiencies are associated with excessive, rather than reduced, blood coagulation. Factor VIII is deficient in classical haemophilia, also known as haemophilia A. Factor IX is deficient in haemophilia B (also known as Christmas disease). Von Willebrand factor is deficient in von Willebrand disease and this results in a failure of platelet and vessel wall function with an associated reduction in factor VIII. In haemophilia A and B it is almost always males who are affected, with haemophilia A being five times as frequent as haemophilia B. Thus there are currently about 5,000 people with haemophilia A and 1,000 people with haemophilia B in the UK. Von Willebrand disease occurs equally in males and females and about 5,000 patients are registered in the UK but the real number of affected people is almost certainly much greater. These conditions affect all racial groups and occur worldwide.
Extractions: Indication for Use: For use in adult patients for treatment and prevention of bleeding in hemophilia A (classic hemophilia) and in adult and pediatric patients for treatment of spontaneous and trauma-induced bleeding episodes in severe von Willebrand disease and in mild and moderate von Willebrand disease where use of desmopressin is known or suspected to be inadequate
FIRST BIOLOGIC APPROVED FOR CLOTTING DISORDER or hardto-treat cases of von willebrand s disease (vWD), a bleeding disorder. or defective in vWD disease, factor VIII and von willebrand factor. http://www.fda.gov/bbs/topics/ANSWERS/ANS00947.html
Extractions: Public Health Service 5600 Fishers Lane Rockville, MD 20857 FDA Talk Papers are prepared by the Press Office to guide FDA personnel in responding with consistency and accuracy to questions from the public on subjects of current interest. Talk Papers are subject to change as more information becomes available. T99-15 Print Media: 301-827-6242 April 1, 1999 Consumer Inquiries: 888-INFO-FDA FIRST BIOLOGIC APPROVED FOR CLOTTING DISORDER FDA today approved a new indication for a plasma-derived product called Antihemophilic Factor/von Willebrand Factor Complex (Human), marketed as Humate-P. The product is now approved to treat severe or hard-to-treat cases of von Willebrand's disease (vWD), a bleeding disorder. It was previously approved for adult patients with hemophilia A. The disease vWD is the most common hereditary bleeding disorder, affecting approximately 1% of the U.S. population. It is caused by a deficiency or defect in certain plasma proteins critical to blood clotting. In most people the disease is mild and treatment is usually not required to control bleeding. However, approximately 2,000 people in the U.S. each year suffer from severe forms of the disease where bleeding can be excessive if not treated. A drug, desmopressin, is an effective treatment for some forms of vWD, but for about 600 patients, plasma-derived products are needed. Humate-P is the only approved plasma-derived treatment for vWD.
Extractions: A NTIBODIES (anti-Human and others as indicated) RDI Divison of Fitzgerald Industries Intl offers a wide line of antibodies. Since no one antibody works best for all applications (neutralization, blotting, ELISA, etc), we offer many different types of antibodies to help solve this problem. Please inquire for other applications or types of antibodies not listed below. von Willebrand Factor MATCHED PAIR ANTIBODIES for Elisa of von Willebrand Factor -human, dog, rat, rabbit and pig cat:RDI-VWF-KT $625.00/set -consists of two matched pair polyclonals which can be used to form a sandwich Elisa, approx 5 plates per set . VWF in plasma and other fluids can be measured by capturing onto a microplate plate coated with a affinity absorbed polyclonal antibody to VWF. After washing , the captured VWF is detected with goat anti-VWF IgG conjugated to HRP. After washing bound antibody, conjugate activity is expressed by incubaton with OPD (or TMB). The color generated is proportional o the concentration of VWF present in the sample. Consists of: a) Affinity Purfied Goat anti-VWF IgG 1 vial b) HRP-conjugated Goat anti-vWF 1 vial -sample protocol also included with antibodies For Research Use Only Goat anti-human von Willebrand Factor IgG fraction cat#RDI-VWFabG $312.00/vial 2.5mg
Extractions: The Hemophilia and Thrombosis Center of Nevada 2020 W. Palomino Lane Suite 110 Las Vegas, Nevada 89106 since September 1 Last updated September 1, 2005 webmaster RIS The Hemophilia and Thrombosis Center of Nevada (HTCN) was founded in 1997 as the first treatment center in the state of Nevada dedicated to the diagnosis and treatment of patients with bleeding or clotting disorders. The HTCN is a non profit corporation. We support ourselves with educational grants and research grants. All team members are employed through community organizations who donate their time to the treatment center. We currently care for patients and families throughout Nevada and the neighboring underserved regions. While clinics are held in Las Vegas, we attempt to coordinate care through the primary care physician's office locally. We have an active advisory board composed only of affected and concerned persons. This board promotes the mission of the HTCN and helps to guide the activities of the treatment center staff. If you are interested in volunteering at the HTCN or helping support us, please call the center at the number below. We always need help with our newsletters published three times per year and special events.
UniProt Knowledgebase Keyword: Von Willebrand Disease Protein which, if defective, causes von willebrand disease, a hemorrhagic disorder in which the von willebrand factor is either quantitatively or http://www.expasy.org/cgi-bin/get-entries?KW=von Willebrand disease
Learn More About Von Willebrand Disease Learn More about von willebrand Disease UMass Memorial Medical Center. http://www.umassmemorial.org/ummhc/hospitals/med_center/services/bleeding-clotti
Extractions: Von Willebrand Disease The most common inherited bleeding disorder is von Willebrand disease. It is genetically transmitted from generation to generation, and is caused by a defect or deficiency in an essential blood clotting protein called the von Willebrand factor. While hemophilia occurs only in males, von Willebrand disease affects both males and females. It is estimated that between one and three percent of the U.S. population has von Willebrand disease. Normally, proteins called clotting factors in blood act as a patch when a blood vessel is injured. Von Willebrand factor is what allows the platelets to stick to the blood vessel wall. When this factor is deficient, bleeding lasts longer. Easy and excessive bruising, nosebleeds, heavy or prolonged menstrual bleeding, or prolonged or excessive bleeding following a tooth extraction are the most common symptoms of von Willebrand disease. Unlike hemophilia, von Willebrand disease is more likely to cause bleeding under skin and manifests itself as severe bruising or nosebleeds. People with von Willebrand disease are less likely to bleed into the joints and muscles than those with hemophilia. Patients with mild to moderate von Willebrand disease (known as Type 1) may remain undiagnosed until they have surgery or a serious injury. Most often it is a mild disorder. It may be diagnosed at any age and be associated with few, if any, symptoms. In fact, affected individuals may not be diagnosed until a family member is identified with the condition.
Von Willebrand's Disease von willebrand s disease (vWD) is a congenital, extrinsic platelet defect resulting in Endothelial cells with stored von willebrand factor (red) in the http://www.vet.uga.edu/vpp/clerk/anderson/
Extractions: Von Willebrand's Disease Julie B. Anderson, DVM; Kenneth S. Latimer, DVM, PhD; Perry J. Bain, DVM, PhD; Heather L. Tarpley, DVM Class of 2004 (Anderson), College of Veterinary Medicine, University of Tennessee, Knoxville, TN 37996 and the Department of Pathology (Latimer, Bain, Tarpley), College of Veterinary Medicine, University of Georgia, Athens, GA 30602-7388 Introduction Von Willebrand's disease (vWD) is a congenital, extrinsic platelet defect resulting in platelet dysfunction. It is characterized by a deficiency of von Willebrand factor (vWF), a glycoprotein that is involved in platelet adhesion to the vessel wall during formation of the primary hemostatic plug. Although vWD is most commonly reported in dogs, it also has been observed in swine, horses, cattle, and cats. Disease Mechanism vWD is characterized by a lack of functional vWF, resulting in abnormal primary hemostasis (platelet plug formation) and prolongation of bleeding time in vivo Figure 1. Small, medium, and large multimers of vWF exist. The largest ones (high molecular weight) are most active in hemostasis because they presumably have increased numbers of binding sites per molecule or because their physical characteristics alter under certain conditions of blood flow. vWF circulates bound to factor VIII and appears to prolong the half life of factor VIII. In dogs with vWD, factor VIII concentrations usually remain at 20% or more of normal values. The role of vWF in the release and production of factor VIII has not been clarified.
NEJM -- Treatment Of Von Willebrand's Disease Review Article from The New England Journal of Medicine Treatment of von willebrand s Disease. http://content.nejm.org/cgi/content/extract/351/7/683
Extractions: Full Text PDF PDA Full Text CME Exam ... PubMed Citation Von Willebrand's disease is an inherited bleeding disorder with a prevalence as high as 1 to 2 percent in the general population, according to screening studies. In contrast, estimates based on referral for symptoms of bleeding suggest a prevalence of 30 to 100 cases per million, which is similar to the prevalence of hemophilia A. The disease was first described in 1926 by the Finnish pediatrician Erik von Willebrand, who used a rowboat to make house calls to patients with the disease in deficiency Full Text of this Article Classification Laboratory Diagnosis General Principles of Treatment Autologous Replacement Therapy Dosage and Routes of Administration Clinical Efficacy Response and Phenotypes Adverse Effects Allogeneic Replacement Therapy Replacement Products Dosage of Concentrate Laboratory Monitoring Antifibrinolytic Amino Acids Reproductive Health Menorrhagia Management of Delivery Alloantibodies Acquired von Willebrand Syndrome The Future Conclusions
Extractions: Full Text PDF PDA Full Text PowerPoint Slide Set ... PubMed Citation ABSTRACT Background Aortic-valve stenosis can be complicated by bleeding that is associated with acquired type 2A von Willebrand syndrome. However, the prevalence and cause of the hemostatic abnormality in aortic stenosis are unknown. Methods We enrolled 50 consecutive patients with aortic stenosis, who completed a standardized screening questionnaire to detect a history of bleeding. Forty-two patients with severe aortic stenosis underwent valve replacement. Platelet function under conditions of high shear stress, von Willebrand factor collagen-binding activity and antigen levels, and the multimeric structure of von Willebrand factor were assessed at base line and one day, seven days, and six months postoperatively. Results Skin or mucosal bleeding occurred in 21 percent of the patients with severe aortic stenosis. Platelet-function abnormalities under conditions of high shear stress, decreased von Willebrand
Von Willebrand's Disease von willebrand s Disease. by Jennie Bullock. von willebrand s disease is a bleeding disorder found in many breeds of dog as well as other animals. http://www.barkbytes.com/medical/med0036.htm
Extractions: von Willebrand's Disease by Jennie Bullock von Willebrand's disease is a bleeding disorder found in many breeds of dog as well as other animals. It is characterized by hematomas, nosebleeds, and intermittent limping (due to bleeding into the joints). Similarly to hemophilia A, dogs with this disorder are deficient in clotting factor VIII activity. One of the primary distinctions of von Willebrand's disease however, is that this disorder is not sex-linked. von Willebrand's disease appears in two variations: inherited and acquired. In its acquired presentation von Willebrand's is a complication of thyroid deficiency. With proper treatment and stabilization of the dog's thyroid levels the von Willebrand's is negated. Hereditary von Willebrand's disease is far more complex. Each breed of dog will have a different set of "typical symptoms" of the disease. Symptoms can range from abnormally long bleeding time due to a cut toenail, to hemorrhaging during minor surgery, to spontaneous nosebleeds, with a wide assortment of presentations between. Injuries that are followed by bleeding may or may not require a transfusion. Even a small wound may necessitate veterinary treatment. Carriers of this disorder can live their entire lives with no outward indications of this disease. von Willebrand's disease can be fatal. There is no cure and no effective treatment. It appears that DNA screening is the most accurate means of testing currently.
Von Willebrand's Disease There has been considerable discussion about von willebrand s Disease (vWD) Type III is a mutation that stops production of the von willebrand s Factor http://www.bmd.org/health/vonwillebrands.html
Extractions: There has been considerable discussion about von Willebrand's Disease (vWD) in Berners over the last two years. Very recent developments now give Berner owners and breeders the opportunity to evaluate their dogs and with prudent breeding practices eliminate this disease from our breed. VetGen has recently developed a DNA test for vWD in Berners. This test is identical to the test for Doberman Pinschers. A great deal of thanks goes to Cindy Stauch for serving as the vital laison between VetGen and dog owners. We also need to thank VetGen for doing this study on 30-50 dogs free of charge to determine if vWD in Berners has a mutation that is already known in another breed. The owners of the dogs that submitted samples to VetGen need to be thanked. We as a breed fancy should be mindful of how lucky we are. If vWD in Berners was not due to one of the known mutations it would have cost us literally hundreds of thousands of dollars to find the mutation. There are already a few breeds that are not so lucky. A little background is in order. There are three types of vWD, based on DNA mutations, so far found in dogs. Type I is autosomal recessive and the one seen in Doberman Pinschers as well as Manchester Terriers, Poodles, and Pembroke Welsh Corgis. It is a relatively mild disease with infrequent spontaneous bleeds and will be discussed in more detail. Type II is found in German Shorthaired and Wirehaired Pointers. Type III is a mutation that stops production of the von Willebrand's Factor (vWF) completely. This type is seen in Scottish Terriers and Shetland Sheepdogs and is severe, often resulting in spontaneous bleeds and death in puppyhood for affected dogs. Of note is that the mutation for these two breeds is different in the Type III disease but both result is no vWF production. This is important because the test for Scottish Terriers won't work on Shetland Sheepdogs.
Extractions: Hemophilia Factor VII Deficiency Factor XI Defiency Women's Disorders ... BDANENY HOME Bleeding Disorders Association of Northeastern New York (BDANENY), Inc. What is von Willebrand Disease? Although less widely known than hemophilia, von Willebrand disease (vWD) is actually the most common inherited bleeding disorder affecting 1 in 100 individiuals. It affects women and men in equal numbers. People with vWD take longer to stop bleeding due to defects or deficiencies in a clotting factor called von Willebrand factor (vWF). In most cases, the problem arises from low levels of vWF in the bloodstream. In other cases, there may be enough vWF, but it does not work properly. An abnormal gene in vWD is located on a chromosome called an autosomal chromosome or autosome, rather than a sex-linked (X) chromosome. Therefore, both men and women may pass the vWD gene to their offspring. A parent with von Willebrand disease has a 50% chance of passing the disease onto each of his or her children, whether they are male or female. vWD can also be the result of a spontaneous, genetic mutation in the child. Individuals whose vWD is the result of such a mutation may also pass the disease on to their children. Not everyone who has vWD shows symptoms. For those who do, typical signs include nosebleeds, easy bruising, heavy menstrual flow, and excessive or unusual bleeding from the mouth or gums. Sometimes bleeding is caused by injury. At other times there is no obvious cause. Gastrointestinal or urinary tract bleeding may also occur in persons with vWD. It is rare for persons with vWD to have joint or muscle bleeds.
Von Willebrand Disease von willebrand Disease Updated March 29, 2005 von willebrand Disease KidsHealth; von willebrand Disease KidsHealth Article for teens http://www.noah-health.org/en/blood/hemophilia/types/vonwillebrand.html
