Tăng bilirubin, phosphatase kiềm, GGT và Stones Nhìn thấy trong bùn hoặc sỏi bàng quang túi mật của Mỹ phổ biến Pigment nHẤT lên đến 10 năm; sỏi cholesterol nHẤT chung ở thanh thiếu niên Mỹ là xét nghiệm chẩn đoán tốt nhất | Chapter 7 Hematologic Disorders 143 Inherited Bleeding Disorders of Procoagulants Essentials of Diagnosis Factor VIII deficiency hemophilia A X-linked recessive. Factor VIII activity 1 causes spontaneous bleeding in skin joints muscles and viscera. Mild disease with 5-40 activity has bleeding only with trauma. Prothrombin time PT normal. Prolonged activated partial thromboplastin time aPTT Factor IX deficiency hemophilia B genetics and presentation similar to factor VIII. PT normal. aPTT variably prolonged Factor XI deficiency hemophilia C autosomal recessive with mild symptoms. Bleeding after surgery or trauma. Severity of bleeding does not correlate well with factor activity level von Willebrand disease autosomal dominant or recessive. von Willebrand factor binds factor VIII and is a cofactor in endothelial adhesion of platelets. Mild symptoms. Menorrhagia may be the first manifestation Rare genetic deficiencies of other factors in the clotting cascade have been described and are detected by specific factor analysis Differential Diagnosis Severe liver disease causes decreased synthesis of prothrombin fibrinogen factors V VII IX X XII and XIII plasminogen antithrombin III AT III protein C and protein S. There may be both bleeding and thrombotic tendencies Vitamin K deficiency. Vitamin K is a cofactor in synthesis of factors II VII IX and X. Newborn infants and children with poor intake or fat malabsorption are at risk DIC Treatment In mild factor VIII deficiency factor VIII and vWF released from endothelium after desmopressin administration are adequate to prevent bleeding Most factor VIII deficient patients require exogenous factor VIII to treat hemorrhage. Some require prophylactic factor VIII to prevent progressive arthropathy Exogenous factor IX used for factor IX deficiency Peri-operative or peripartum treatment with fresh frozen plasma FFP and platelets for factor XI deficiency. Desmopressin also used Intravenous or intranasal desmopressin used for bleeding
