Von the deficiency of the Von- Willebrand factor

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Last updated: December 25, 2019

Von Willebrand diseaseVon-Willebrand is a bleeding disorder which affects the clotting mechanism in the body. During injury excess of bleeding occurs which may lead to internal organ damage rarely it may also lead to death. The clotting mechanism is altered due to the deficiency of the Von- Willebrand factor which is a key factor in the clotting process and they also carry the clotting factor VIII which is also an important clotting factor.

The Von-Willebrand factor sticks to the platelets and it forms a clot when the blood vessels are injured and the bleeding is arrested or stopped. 1Types of von Willebrand Disease 1The three major types of VWD are Type 1, Type 2, and Type 3. Type Levels of Von-willebrand factor Levels of factor VIII Condition I Low Low Common (60%-80%) II Low-altered function Low Moderate (15%-30%) III No factor present Low Rare (5%-10%) Acquired Von willebrand disease   Low   Low Acquired in later stage of life Rare  CLINICAL SYMPTOMS 2·         Excessive bleeding during injury or surgery or dental work·         Excessive Nosebleeds ·         Heavy menstrual bleeding·         Blood in urine or stools·         Bruising DIAGNOSIS ·         Von Willebrand factor antigen: The level of Von Willebrand factor is measured.·         Ristocetin cofactor activity: Ristocetin is an antibiotic used in measuring Von Willebrand factor works in the clotting process·         Factor VIII clotting activity:  low levels and activity of factor VIII are measured·         Von Willebrand factor multimers: this helps in evaluating the specific structure of von Willebrand factor in blood, its protein complexes (multimers) and its molecules break down. This helps to identify the type of Von Willebrand disease.

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·         Platelet function test: measures the functions of the platelets. 1TREATMENT 2 CLASS DRUGS BRAND NAME Anti-diuretic hormones Desmopressin DDAVP,Minirin,Stimate,Noctiva Antifibrinolytic or Clot-stabilizing medications Aminocaproic acid  Tranexamic acid Amicar Cyklokapron, Lysteda Replacement therapies Combination of von Willebrand factor and factor VIII Humate-P Contraceptives Levonorgestrel Ethnyl estrodiol   Beyaz, Safyral Altavera , Amethyst, Aviane, Canrese                                               

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