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  2. Jul 29, 2022 · The data presented by Dhami and colleagues bring yet another piece to the puzzle, linking VWF to malignant diseases, this time in metastatic breast cancer. Animal studies confirming such results could extend and strengthen even further the relevance of this study.

    • 10.1111/jth.15810
    • 2022/10
  3. Apr 29, 2021 · In conclusion, yes, there are some efficient treatments for VWD, but, no, this does not mean that we should stop trying to improve these treatments with the goals of reaching superior efficacy, improving quality of life of the patients, and finally, curing the disease.

    • Cécile V Denis, Sophie Susen, Peter J Lenting
    • 2021
    • What Every Physician Needs to Know
    • Are You Sure Your Patient Has VWD? What Should You Expect to find?
    • Beware of Other Conditions That Can Mimic VWD
    • More Definitive Therapies?
    • What Other Therapies Are Helpful For Reducing Complications?
    • What Should You Tell The Patient and The Family About Prognosis?
    • What If Scenarios
    • Pathophysiology
    • What’s The Evidence?

    Von Willebrand factor (VWF) is a huge multimeric protein comprised of identical 280,000 Dalton monomeric subunits. VWF is critical in the initiation of hemostasis. VWF multimers are produced and secreted by endothelial cells (and platelets) and, because they bind to platelet glycoprotein (GP) I receptors, induce platelet adherence to long VWF multi...

    VWD is usually an inherited bleeding disorder. Only rarely is it acquired. A family history of bleeding, especially affecting both males and females, suggests VWD. It is a mild bleeding disorder in many patients. Diagnostic evaluation is often initiated to determine the cause of easy bruising, excessive bleeding with dental extractions or surgery, ...

    Hemophilia A and either quantitative or qualitative platelet disorders can resemble VWD clinically. Hemophilia A is an X-linked deficiency of FVIII that primarily affects males. VWD is an autosomal disorder (discussed further in “Pathophysiology”). Platelet-type (or “pseudo”-) VWD is a gain-of-function defect in platelet GP Ib with similar clinical...

    Humate-P is a concentrate that contains all VWF multimers and FVIII, and is given either by rapid IV infusion or (sometimes) continuous infusion. The product is labeled for both RCoF and FVIII dosing. (Other VWF-containing products are described and compared by Favoloro 2016 in the references.) For a bleeding episode, Humate-P is usually administer...

    The anti-fibrinolytics, aminocaproic acid (Amicar; 50 mg/kg [up to a maximum of 5 grams] given 4 times daily) or tranexamic acid (25 mg/kg given 3 times daily) are especially useful in the management of oral/dental bleeding. Estrogen or oral contraceptive pills can sometimes be effective in the management of bleeding (including menorrhagia) in wome...

    The prognosis for most patients with VWD is favorable, provided their bleeding is treated and prevented appropriately. Many individuals with VWD are followed routinely at a hemophilia treatment center. Individuals with VWD should be discouraged from participating in high-impact sports/activities (football, boxing, rugby, wrestling, rodeo-type event...

    Most cases of VWD are inherited. Rarely, however, VWD is acquired in association with: (1) multiple myeloma, lymphoproliferative or myeloproliferative disorders (acquired VWD caused by anti-VWF autoantibodies to, or cell absorption of, large VWF multimers); (2) hypothyroidism (caused by decreased VWF synthesis); or (3) serious cardiac conditions as...

    The gene encoding the VWF monomer is on chromosome #12. Expression of the mRNA for the VWF monomer is translated into a 280,00 Dalton protein that contains a series of different domains. These include contiguous non-identical regions (A domains) that: (a) bind to platelet GP Ib (the A1 domain); (b) contain the peptide cleavage site for ADAMTS-13, t...

    James, AH, Manco-Johnson, MJ, Yawn, BP, Dietrich, JE, Nichols, WL. “Von Willebrand disease: key points from the 2008 National Heart, Lung, and Blood Institute guidelines”. Obstet Gynecol. vol. 114. 2009. pp. 674-8. (Summary of management in Ob-Gyn journal.) Ruggeri, ZM, Ware, J. “von Willebrand factor”. FASEB J. vol. 7. 1993. pp. 308-16. (Classic p...

  4. Dec 6, 2019 · Abstract. von Willebrand disease (VWD) is the most common autosomal inherited bleeding disorder, with an estimated prevalence of 1 in 1000 individuals. VWD is classified into quantitative and qualitative forms. Diagnosis of VWD is complex and requires (1) a personal history of bleeding symptoms, (2) family history of bleeding or VWD, and (3 ...

    • Ruchika Sharma, Sandra L. Haberichter
    • 10.1182/hematology.2019000064
    • 2019
    • 2019/12/12
  5. Synopsis. Von Willebrand disease (VWD) is a common bleeding disorder, affecting males and females equally, that often manifests in mucosal bleeding. VWD can be secondary to a quantitative (Type 1 and Type 3) or qualitative (Type 2) defects in Von Willebrand factor.

    • Angela C. Weyand, Veronica H. Flood
    • 2021/12
    • 10.1016/j.hoc.2021.07.004
  6. Several studies have demonstrated the independent prognostic value of VWF:Ag, with high VWF levels correlating with poorer survival in patients with colorectal cancer, ovarian cancer, glioblastomas, and esophageal and lung cancer. 14-18 Moreover, plasma proteomic analysis reported VWF as a biomarker for the early detection of colon cancer. 19 ...

  7. May 29, 2023 · There’s no cure for von Willebrand disease, but it can be treated and/or managed. A key to managing this disorder is to reduce the risk of bleeding before it starts. This means avoiding certain...

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  2. Learn about VONVENDI® [von Willebrand factor (Recombinant)] clinical trial results.

  3. We're dedicated to conquering childhood cancer through research funding & patient support. Help save childhood dreams by donating to help fund childhood cancer research.

  4. Visit the official VONVENDI® [von Willebrand factor (Recombinant)] HCP website.

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