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2013-07-16 07:11:52
thrombotic disorders

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  1. do any of the inherited thrombotic disorders lead to arterial thromboses?
  2. which 2 inherited clotting disorders increase VTE?
    • Homozygous factor V leiden
    • antithrombin deficiency
  3. do you treat inherited thrombotic disorders?
    • no unless:
    • preg -> heparin
    • active thrombus -> LMWH and warfarin
  4. what is coumarin?
  5. what is the prognosis of homozygous antithrombin deficiency?
    death in utero
  6. genetics of antithrombin deficiency?
    autosomal dominant
  7. people with antithrombin deficiency get a VTE before the age of what?
  8. how much does the risk of thrombosis increase in antithrombin deficiency?
  9. when do you treat, and with what, antithrombin deficiency?
    • if preg -> LMWH
    • if heart surg using cardiopulmonary bypass -> antithrombin transfusion pre-op
  10. what does antithrombin do?
    • inactivates activated factors
    • -> hinders thrombosis
  11. how do heparin and fondaparinux work?
    increase antithrombin
  12. what do deficiencies in protein C or protein S do?
    • -> prothrombotic state
    • X5 risk of thrombosis
  13. talk about protein C/S?
    dependent on which vit
    role in coag
    • need vit K for synthesis
    • they are natural anticoagulants by:
    • -reducing thrombin generation
    • -deactivating factors
  14. what percentage of the following have factor V leiden:
    a) europeans
    b) asians/americans
    c) chinese
    • a) 5%
    • b) 0.5%
    • c) v rare
  15. what is the genetic change in factor V leiden?
    • gain of function mutation
    • stops cleavage and thus inactivation of factor Va
  16. what is the risk increase of thrombosis in factor V leiden?
    X5 homozygotes

    X50 in rare heterozygotes
  17. what is APS?
    antiphospholipid syndrome
  18. what is the cause of APS?
    antiphospholipid antibodies vs the attached proteins
  19. what tests are used to diagnose APS?
    • ELISA for the autoAb
    • APPT
    • DRVVT (?lupus anticoagulant)
  20. what two autoAbs do you search for with ELISA testing in APS?
    • anticardiolipin Ab
    • anti-beta2 glycoprotein I Ab
  21. what complication of preg is assoc with APS?
    pre eclampsia
  22. name a non-inherited cause of increased risk of VTE and arterial thrombi?
  23. complications of APS?
    • strokeĀ 
    • VTE and art thrombosis
    • pre eclampsia
    • livedo reticularis
    • transverse myelitis
    • skin necrosis
    • chorea
  24. normally if someone has a Hx of stroke they are put on aspirin, what do you give them if they have APS?
  25. APS is linked to what autoimmune conditions?
    • SLE
    • RA
    • systemic sclerosis
    • temporal arteritis
    • sjogren's
  26. what is sjogren's?
    autoimmune attack of exocrine glands
  27. what is the DRVVT used in testing for lupus anticoagulant for APS?
    Dilute russell viper venom test
  28. what is DIC?