Von Willebrand Disease

 Von willebrand disease

What is Von Willebrand Disease?

Von Willebrand’s disease is a hereditary bleeding disorder that occurs in males and females and is characterized by a deficiency of or a defect in a protein termed von Willebrand factor. The disorder prevents platelets to adhere to damaged endothelium. The Von Willebrand factor is a protein also serves as a carrier protein for factor VIII. It is characterized by an increase tendency to bleed from mucous membranes. The Von Willebrand’s gene is located on chromosome no-12.

Causes

A genetic mutation causes Von Willebrand disease.

Symptoms

  • Epistaxis
  • Gum bleeding.
  • Easy bruising.
  • Excessive menstrual bleeding.

Diagnosis

Von Willebrand disease is diagnosed by blood sample tests in laboratory.

Treatment of Von Willebrand Disease

Medicine Desmopressin Acetate is given to treat people with milder forms of Von Willebrand disease. It works by making the body release more Von Willebrand factor into the blood. It also helps increase the level of factor VIII in the blood.

In severe case the Von Willebrand factor replacement therapy is given.

People with Von Willebrand disease follow bleeding precaution. Provide emotional support to the child and parents especially if the child is experiencing bleeding episodes.

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