Von willebrand’s disease

A hereditary blood disease, occurring in both sexes, in which the mucous membrane starts to bleed without any apparent reason. It is caused by a deficiency of a clotting factor in the blood, called von Willebrand’s factor. [Described 1926. After E. A. von Willebrand (1870-1949), Finnish physician.]


A protein substance in plasma involved in platelet aggregation.


An inherited chronic bleeding disorder. Von Willebrand disease (VWD) is a platelet disorder caused by a defect in a clotting factor known as von Willebrand factor (a protein that is essential for the formation of blood clots). A deficiency of the factor impairs the aggregation of platelets at the site of a wound, which delays or prevents the formation of blood clots needed to stop bleeding. Factor VIII, a substance in blood that is necessary for clotting, may be reduced in people with VWD because von Willebrand factor serves as a carrier for factor VIII.


A genetically determined blood disorder in which the affected person suffers episodes of spontaneous bleeding similar to that occurring in people with haemophilia. It may be associated with a lack of factor VIII in the blood. The disorder is inherited as an autosomal dominant gene.


A congenital bleeding disorder caused by either the mismanufacture of or a deficiency of a protein that helps platelets stick to injured blood vessels during the formation of blood clots. Inadequate amounts of the protein (“von Willebrand’s factor”) result in easy bruising or bleeding. Common symptoms of the disorder are frequent bleeding from the gums after toothbrushing, frequent or prolonged nosebleeds, heavy menstrual periods, or heavy bleeding after childbirth or surgery.


A hereditary, lifelong condition characterized by abnormal gene inheritance, akin to hemophilia. This genetic anomaly leads to reduced levels of von Willebrand’s factor (vWF), a crucial substance involved in blood clotting, within the bloodstream.


Signs of a lack of this factor include profuse bleeding from the gums, cuts, and nosebleeds. Women may experience heavy menstrual flows. In extreme instances, bleeding can occur within the joints and muscles.


The condition is identified through blood clotting tests and by measuring the levels of von Willebrand factor in the blood. To prevent or manage bleeding episodes, desmopressin, which is similar to ADH, can be used. Additionally, concentrated von Willebrand factor or Factor VIII may be administered for bleeding treatment.


 


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