Tiffany's Basic Science

Question 1

What proteins are affected in hemophilia? What is the inheritance pattern?

Answer 1

Hemophilia A: factor 8
Hemophilia B: factor 9
X-linked recessive

Question 2

What joint is more is most commonly affected in hemophilia?

Answer 2

Knee hemarthrosis

Question 3

Hemophiliac presents w paresthesia in the L4 nerve distribution but is otherwise neuro intact. What do suspect?

Answer 3

Iliacus hematoma. Compresses femoral n
Hemophiliacs can also bleed in the spine, but would have other neuro deficits

Question 4

True or False: hemophilia can cause LLD

Answer 4

True
Causes epiphyseal overgrowth

Question 5

List typical XR findings in hemophiliacs (7)

Answer 5

1. Epiphyseal overgrowth
2. Generalized osteopenia
3. Irregular joint spaces
4. Jordan’s sign: square patella and femoral condyles
5. Ballooning of distal femur
6. Wide intercondylar notch
7. Patella appears long and thin on lateral view

Question 6

How are PT and PTT affected in hemophilia?

Answer 6

Prolonged PTT, normal PT

Question 7

What are the indications for factor 8 or 9 administration in hemophilia? What levels should be obtained?

Answer 7

Indications - low factor level of high Bethesda assay (high inhibitor titre)
Goal level depends on potential bleeding
- Acute hematoma: 30%
- Acute hemarthrosis or soft tissue surgery: 50%
- Skeletal surgery: 100% 1st wk postop, >50% 2nd wk postop