Kind en Jongere Deel 4

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  1. DD for primary clotting disorders
    • Acquired:
    • -Asprin use
    • -Uremia (no idea why)
    • -Idiopathic Thrombocytopenia Purpora
    • Congenital:
    • -TAR (Thrombocytopenia with Absent Radius)
    • -VonWillebrand's Sickness
  2. DD for secondary clotting disorders
    • Acquired:
    • -Vitamin K def.
    • -Use of Cumarine (spice which is converted to anticoagulant via some fungi)
    • -Liver disfunction
    • Congenital
    • -Hemophelia A(fVIII def)/B(fIX def)
  3. Role of Antithrombin III
    Inactivates thrombin (IIa), factors IXa and Xa
  4. Role of Protein C
    Inactivates Va and VIIIa
  5. Role of Protein S
    Activates Protein C
  6. Factors requiring vitamin K for proper function
    II, VII, IX, X, C, S
  7. What is factor V Leiden?
    A defect in factor V means it is not inactivated by Protein C - it is the most common hypercoagulability disorder among Eurasians and is especially dangerous for PREGNANT women and women who take OCPs
  8. What is ITP
    Idiopathic Thrombocytopenia Purpura - abnormally low platelet count (nl 150-400,000/uL - ITP occurs when less than 20,000/uL). Occurs in two forms - acute (usually begins in children) and chronic (usually begins in older children). Usually due to auto-immune reaction following a viral infection.
  9. When is bone marrow testing necessary with ITP?
    When Thrombopoetine is elevated (recall: Thrombopoetine is the protein which stimulates production of platelets by the bone marrow)
  10. Tx for ITP
    • Grade 1: Observe and if necessary, give a fibrinolysis blocker
    • Grade 2: Corticosteroids and IV IgG
  11. Function of von Willebrand's factor
    Connecting exposed collagen to platelets and deactivating factor VIII
  12. Types of von Willebrand disease
    • Type 1: Reduction in amount of vWF
    • Type 2: Reduction in action of vWF
    • Type 3: Total lack of vWF
  13. Diagnosis of vWF disease
    Measure vWF levels and activity - by type 1 and type 3 is the level lower and by type 2 is there a discrepancy between the two. Also measure APTT (Activated Partial Thromboplastin Time - measures intrinsic and common pathways) to test for lack of factor VIII (because in activated form, it leaves the circulation)
  14. Tx for vWF disease
    Desmopressine for type 1 and 2 (form of vasopressin) which induces release of stored vWF. Also, for some f**king reason, give Hepatitis B vaccine.
  15. Diagnosis of DIC
    Low platelets, fibrinogen and a higher APTT
  16. Tx of DIC
    Treat underlying cause if possible - give blood clotting factors, platelets and eventually heparin (potent anticoagulant)
  17. Diff btwn PT and APTT
    • PT (Prothrombin Time) measures extrinsic pathway
    • APTT (Activated Partial Thromboplastin Time) meausures intrinsic pathway
  18. Transmission of Hemophilia
  19. Hemophilia A vs. B (problem and frequency)
    • A - probs with factor VIII - 85% of cases
    • B - probs with factor IX - 15% of cases
  20. Clinical presentation of hemophilia
    Prolonged APTT (don't think on this one, just memorized - it does NOT make sense)
  21. Vitamin K deficiency's effect on PT, APTT, BT and Platelet Count
    • PT: prolonged
    • APTT: normal
    • BT: normal
    • PC: normal

    Thus Vitamin K affects only eKtrinsic (PT)

    Factors II, VII, IX and X low
  22. DIC's effect on PT, APTT, BT and Platelet Count
    • PT: prolonged
    • APTT: prolonged
    • BT: prolonged
    • PC: decreased

    Thus DIC affects first three (b/c DIC has three letters) and last is decreased beacuse all platelets are being used up
  23. Von Willebrand's disease effect on PT, APTT, BT and Platelet Count
    • PT: normal
    • APTT: prolonged
    • BT: prolonged
    • PC: normal

    Thus vWFd affects middle two (because middle two letters are capitalized)
  24. Hemophilia's effect on PT, APTT, BT and Platelet Count
    • PT: normal
    • APTT: prolonged
    • BT: normal
    • PC: normal

    Thus Hemophelia affects only ACTIVE PTT (because homos are really active)
  25. Thrombocytopenia's effect on PT, APTT, BT and Platelet Count
    • PT: normal
    • APTT: normal
    • BT: prolonged
    • PC: decreased

    This one should be obvious - thrombocytopenia by definition means fewer platelets, and BT is a direct measure of platelet function
  26. Tx for hemophilia
    Prophilactic clotting factors after first episode of bleeding, no aspirin and subcutaneous (as opposed to intramuscular) vaccines
  27. Two illnesses associated with vitamin K deficiency
    • CF
    • alpha-1-antitrypsin deficiency
  28. Differentiate Kroep (Diphtheria), Pseudokroep (Croup) and Epiglottitis
    • Remember ABCDEF
    • Age (Epiglottitis only occurs over age of 2, others from 6months to 3 yrs)
    • Body temp (Pseudokroep does not cause fever, Kroep causes mild fever and Epiglottitis causes >39 deg fever)
    • Cough (Epiglottitis causes a muted cough while others cause a barking cough)
    • Drooling (Only caused by Epiglottitis)
    • whEezing (Pseudokroep does not cause this, others cause it upon inhalation) - also, Epiglottitis victims want to sit up
  29. Prevention of Epiglottitis
    H. Influenza vaccine
  30. Classic presentation of bronchiolitis
    Hyperexpanded chest with intercostal "intrekkingen" (withdrawls)
  31. What is a Westley score
    Method of categorizing Croup (Laryngotracheobronchitis) - less than 5 no worries, less than 10 treat with corticosteroids, more than 10 treat with adrenaline and intubation
  32. Classic presentation of croup
    Diaphragm dome at position of 6th rib
  33. What is shock
    Low blood pressure, rapid heart beat and poor end organ perfusion (i.e. confusion and low urine output)
  34. What is hypovolemic shock
    Shock due to low circulatory volume (usually due to hemmorhage or loss of fluid from system). This is the most common form of shock in children and is usually due to vomiting or diarrhea.
  35. What is distributive shock?
    Shock due to impaired use of oxygen by cells which leads to vasodilation. Examples are anaphylactic and septic shock.
  36. What is cardiogenic shock?
    Shock due to malfunctioning heart. In children, this is usually caused by cardiac defects, but may also be cause by viral myocarditis.
  37. What is obstructive shock?
    Shock due to obstruction of vasculature. In children, this is usually caused by cardiac defects.
  38. What is dissociative shock?
    Shock caused by an impaired dissociation of oxygen from hemoglobin. In children this is typically caused by hemoglobin disorders (e.g. methemoglobinemia)
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Kind en Jongere Deel 4
Kind en Jongere Deel 4
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