<prompt> Which of the following is an Autosomal Dominant disease?Von Willebrand diseaseWilsons diseaseCystic fibrosisFriedrichs ataxia  <response>: Von Willebrand disease VWD - Autosomal dominant disorder The main source of plasma von-Willebrand factor is endothelial cells. von Willebrand factor Vwf circulates in the plasma in association with factor VIII. Patients with VWD have defects in platelet function despite a normal platelet count Deficiency of von Willebrand factor vWF -promote platelet adherence and activation causes platelet adhesion BT and Intrinsic pathway activity aPTT. CF Spontaneous mucosal bleeding - Petechiae purpura epistaxis melena excessive bleeding from wounds menorrhagia Lab findings - Platelet count - Normal Prothrombin time - Normal Bleeding time - Increased Activated paial thromboplastin time aPTT-Increased Rx - Desmopressin for mild form - Cryoprecipitate for severe form 