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von Willebrand's Disease von Willebrand's Disease -By A. Dasgupta ,MBBS(Std) What is it ? von Willebrand's Disease is a very common hereditary bleeding disorder caused by Qualitative or Ouantitative defects involving the factor VIII-von Willebrand Factor complex. What is the occurance of this disease ? 1 in 800-1000 individuals of either sex. Pathophysiology : vWD is an Autosomal disorder mainly due to Platelet abnormalities and in severe cases,due toCoagulation Disorders as well. vWF is a protein consisting of a series of multimers.It is synthesized by the Macrophages &Mononuclear Phagocytic System being coaded by a gene in Chromosome 12.It serves important functions in blood: 1)It serves as a plasma carrier of Factor VIII by forming the complex. 2)It increases the half-life of Factor-VIII in blood. 3)It binds with Collagen & Platelets via receptors IB-IX & IIb-IIIa to favor Platelet Adhesion & Aggregation so,deficiency or defect in vWF causes -------- 1)Platelet function Disorders giving rise to vWD. 2)Decreased Half-life[from 12 hours to 2.4 hours] of F-VIII which may cause F-VIII Deficiency leading to graver conditions. Clinical manifestations: 1)in mild cases,Bleeding occurs only after surgery or trauma. 2)Moderate to severe cases are characterized by petechie,purpura,epistaxis,oral mucosal & g.i.bleeding,menorrhagia etc. 3)in very severe cases,F-VIII deficiency may cause Haematoma Formation,Haemarthrosis etc. Types: about 20 variants of vWD have been detected,which are broadly categorized into 3 types:Type-I,Type-II & Type-III. Type-III & Type-IIC are autosomal recessive while the rest are autosomal dominant. TYPE-I: it's a mild disorder due to missensed genetic mutation & characterized by mild to moderate reduction in plasma vWF.[activity 50%]Synthesis of vWF is normal but release of multimers is inhibited. TYPE-II: it's a qualitative disorder due to missensed genetic mutation & characterized by normal or near normal levels ofdysfunctional vWF. TYPE-IIa: has a deficiency of High & Medium mol.wt. vWF multimers due to secretory inability or increased proteolysis. TYPE-IIb: due to inappropriate binding of vWF to Platelets. TYPE-IIN: due to defect in F-VIII Binding Site of vWF. TYPE-III: It's a very sveere but least common disorder due to Deletion & Frame-shift mutation. The patient shows no detectable vWF activity & may have Sufficiently low f-VIII levels. Laboratory diagnosis: i)Bleeding time: Prolonged ii)Prothrombin time:Normal iii)Platelet Count: Normal iv)Clotting time: Prolonged v)Activated Partial Thromboplastin Time: Prolonged vi)Plasma vWF:Reduced Treatment of this disease : 1)Mild bleeding is managed with DDAVP or Desmopressin. 2)Severe bleeding can be controlled by intravenous cryoprecipitates. 3)for oral surgical procedures,EACA is given orally. 4)Menorrhagia,if present,is managed with Hormonal suppression. |
