Disorders of bleeding and thrombosis

Disorders of bleeding and thrombosis - • X-linked •...

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Disorders of bleeding and thrombosis Congenital coagulation disorders Coagulation Biological amplification system results in the generation of thrombin (converts fibrinogen to fibrin) Intrinsic pathway - Activated by subendothelial collages and tested for by the activated partial thromboplastin time (APTT) Extrinsic pathway - Activated by tissue factor and tested for by prothrombin time (PT). International normalizes ratio (INR) = patients PT / mean normal PT. Congenital coagulation disorders Haemophilia (factor VIII deficiency, haemophilia A) Almost confined to males (X-linked) Often not noticed until 6 months old Haemarthrosis, (knees, elbows, ankles) Haematuria, epistaxis, GI bleed less common Normal PT, prolonged APTT Treat by replacing factor VIII – half life of 12 hours (needs to be given twice daily) Factor IX deficiency (haemophilia B, Christmas disease)
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Unformatted text preview: • X-linked • Clinically indistinguishable from haemophilia A • Normal PT, prolonged APTT • Treat by replacing factor IX Von Willebrand’s disease • Usually autosomal dominant • Prolonged APTT, normal PT • Desmopressin can be used to increase factor VIII and VWF • Otherwise, Factor VIII and VWF concentrates are given Acquired coagulation disorders • Hepatocytes synthesise all coagulation factors other than factor VIII. • Liver disease results in decreased factor synthesis, and reduced clearance of activated factors and t-PA. • Disseminated intravascular coagulation / increased fibrinolysis. • Coumarin’s (Warfarin) inhibit vitamin K-dependant enzyme. • Factors produced by this enzyme (II, VII, IX and X) less biologically active. • Same observed in neonates, and individuals deficient in vitamin K....
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This note was uploaded on 12/04/2011 for the course ANTHRO 2000 taught by Professor Monicaoyola during the Fall '10 term at Broward College.

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Disorders of bleeding and thrombosis - • X-linked •...

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