Kind en Jongere Deel 4

• Acquired:
• -Asprin use
• -Uremia (no idea why)
• -Idiopathic Thrombocytopenia Purpora
• Congenital:
• -TAR (Thrombocytopenia with Absent Radius)
• -VonWillebrand's Sickness

• 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)

Inactivates thrombin (IIa), factors IXa and Xa

Inactivates Va and VIIIa

Activates Protein C

II, VII, IX, X, C, S

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

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.

When Thrombopoetine is elevated (recall: Thrombopoetine is the protein which stimulates production of platelets by the bone marrow)

• Grade 1: Observe and if necessary, give a fibrinolysis blocker
• Grade 2: Corticosteroids and IV IgG

Connecting exposed collagen to platelets and deactivating factor VIII

• Type 1: Reduction in amount of vWF
• Type 2: Reduction in action of vWF
• Type 3: Total lack of vWF

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)

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.

Low platelets, fibrinogen and a higher APTT

Treat underlying cause if possible - give blood clotting factors, platelets and eventually heparin (potent anticoagulant)

• PT (Prothrombin Time) measures extrinsic pathway
• APTT (Activated Partial Thromboplastin Time) meausures intrinsic pathway

X-linked

• A - probs with factor VIII - 85% of cases
• B - probs with factor IX - 15% of cases

Prolonged APTT (don't think on this one, just memorized - it does NOT make sense)

• PT: prolonged
• APTT: normal
• BT: normal
• PC: normal

Thus Vitamin K affects only eKtrinsic (PT)

Factors II, VII, IX and X low

• 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

• PT: normal
• APTT: prolonged
• BT: prolonged
• PC: normal

Thus vWFd affects middle two (because middle two letters are capitalized)

• PT: normal
• APTT: prolonged
• BT: normal
• PC: normal

Thus Hemophelia affects only ACTIVE PTT (because homos are really active)

• 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

Prophilactic clotting factors after first episode of bleeding, no aspirin and subcutaneous (as opposed to intramuscular) vaccines

• CF
• alpha-1-antitrypsin deficiency

• 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

H. Influenza vaccine

Hyperexpanded chest with intercostal "intrekkingen" (withdrawls)

Method of categorizing Croup (Laryngotracheobronchitis) - less than 5 no worries, less than 10 treat with corticosteroids, more than 10 treat with adrenaline and intubation

Diaphragm dome at position of 6th rib

Low blood pressure, rapid heart beat and poor end organ perfusion (i.e. confusion and low urine output)

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.

Shock due to impaired use of oxygen by cells which leads to vasodilation. Examples are anaphylactic and septic shock.

Shock due to malfunctioning heart. In children, this is usually caused by cardiac defects, but may also be cause by viral myocarditis.

Shock due to obstruction of vasculature. In children, this is usually caused by cardiac defects.

Shock caused by an impaired dissociation of oxygen from hemoglobin. In children this is typically caused by hemoglobin disorders (e.g. methemoglobinemia)