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Von Willebrand disease (VWD) is the most common hereditary blood-clotting disorder in humans. An acquired form can sometimes result from other medical conditions. [ 1 ] It arises from a deficiency in the quality or quantity of von Willebrand factor (VWF), a multimeric protein that is required for platelet adhesion .
Type 1 von Willebrand Disease in dogs. Type 1 von Willebrand Disease is the most common type, and also the mildest. It occurs when dogs have a mild deficiency in all the proteins making up their ...
Vonicog alfa, sold under the brand names Vonvendi and Veyvondi, is a medication used to control bleeding in adults with von Willebrand disease (an inherited bleeding disorder). [6] [5] [7] [8] It is a recombinant von Willebrand factor. [6] [5] The most common adverse reactions are generalized itching, vomiting, nausea, dizziness, and vertigo. [6]
Desmopressin (DDAVP) is usually the first line treatment for mild to moderate type 1 von Willebrand disease. [1] It is not recommended in severe disease or in those with abnormal factor VIII. [1] Usefulness in type 2A, 2M, or 2N von Willebrand disease is variable. [1] Generally not recommended in 2B and type 3 von Willebrand disease. [1]
Von Willebrand factor (VWF) (German: [fɔn ˈvɪləbʁant]) is a blood glycoprotein that promotes hemostasis, specifically, platelet adhesion.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. [5]
Platelet storage pool deficiency is a family of clotting disorders characterized by deficient granules in platelets.Individuals with these disorders have too few or abnormally functioning alpha granules, delta granules, or both alpha and delta granules and are therefore unable to form effective clots, which leads to prolonged bleeding.
Various types of hemophilia and von Willebrand disease are the major genetic disorders associated with coagulopathy. Rare examples are Bernard–Soulier syndrome, Wiskott–Aldrich syndrome and Glanzmann's thrombasthenia. Gene therapy treatments may be a solution as they involve in the insertion of normal genes to replace defective genes ...
Because von Willebrand factor helps stabilize circulating levels of factor VIII, the vasopressin analog desmopressin can be used to stimulate the AVPR2 receptor and increase levels of circulating factor VIII. This is useful in the treatment of hemophilia A as well as Von Willebrand disease.