Procoagulants and hemophilia drugs

Question 1

2 types of hemophilia

Answer 1

A and B
A is a deficiency in factor VIII
B is a deficiency in factor IX (Christmas factor)

Question 2

Hemophilia tx

Answer 2

Replacing clotting factors

Severity of hemophilia depends on how much clotting factor is lacking

Question 3

Hemophilia drugs

Answer 3

Desmospressin - releases factor VIII from endothelium. Does not release IX therefore only for A
Aminocaproic acid - antifibrinolytic agent which works by inhibition of plasminogen activators

Question 4

Hemophilia and NSAIDS

Answer 4

Pts should avoid ASA as they suppress platelet aggregation and promote GI ulceration and bleeding
Second gen NSAIDS (COX 2 inhibitors) are considered to be safer

Question 5

TXA inhibits

Answer 5

Plasmin (the enzyme that dissolves the fibrin meshwork of blood clots)
TXA binds to lysine receptor sites on plasmin and prevents plasmin from binding to lysine molecules in fibrin

Question 6

TXA class TRANexamic acid

Answer 6

Antifibrinolytic for traumatic hemorrhage

Question 7

TXA contras

Answer 7

Hyper sensitivity, thromboembolic disease (DVT, CVA, PE)

Injury over 3 hours

Question 8

TXA criteria

Answer 8

Traumatic injury under 3 hours ago with a pt over 16, pressure under 90 or heart rate over 110

Question 9

TXA dose

Answer 9

1g IV/IO in 250mL D5W or NS over 10 minutes (10gtts = 4 per second) no repeat

Question 10

Hemophilia is seen in

Answer 10

males mostly. 70% from defective gene from mama, 30% from spontaneous gene mutation

Question 11

Hemophilia A deficiency in

Answer 11

VIII (antihemophilic factor)

Question 12

Hemophilia B deficiency in

Answer 12

IX (Christmas factor) A is 6X more prevalent than B.

1 in 5000 to 1 in 30000 males

Question 13

Patho of hemophilia

Answer 13

Platelet aggregation is normal, but fibrin production is inhibited.
VIII and IX are part of contact (intrinsic) activation coagulation pathway and are needed to catalyze X to Xa which in turn catalyzes prothrombin to thrombin.

Question 14

Desmporessin

Answer 14

Analog of ADH. Works be releasing stored factor VIII from vascular endothelium. Good for mild hemophilia A.

Question 15

Antifibrinolytic Agents

Answer 15

Most useful for preventing recurrent bleeding less useful for stopping ongoing bleed.
Aminocaproic acid and TXA

Question 16

TXA MOA

Answer 16

A derivative of lysine, it inhibits plasmin (the enzyme that dissolves the fibrin meshwork of blood clots) by binding to lysine receptors sites on plasmin and preventing plasmin from binding to lysine molecules in fibrin.