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HEMOPHILIA

HEMOPHILIA. John O’Sullivan 24 May 2001. Outline. Definition Epidemiology Diagnosis Factor Replacement Hemophilic Arthropathy Treatment Other Topics. Hemophilia. Disorder of hemostasis, a coagulopathy Hemophilia A - Factor VIII deficiency Hemophilia B – Factor IX deficiency

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HEMOPHILIA

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  1. HEMOPHILIA John O’Sullivan 24 May 2001

  2. Outline • Definition • Epidemiology • Diagnosis • Factor Replacement • Hemophilic Arthropathy • Treatment • Other Topics

  3. Hemophilia • Disorder of hemostasis, a coagulopathy • Hemophilia A - Factor VIII deficiency • Hemophilia B – Factor IX deficiency • Prevalence: 13.4 cases per 100,000 males • Incidence: 1 in 5032 live male births

  4. Diagnosis • Atypical bleeding at circumcision or bruising at neonatal vaccines • Toddlers with lip bleeding or unusual bruising when learning to walk • Hx of affected males on mother’s side • Elevated PTT • Factor assays

  5. Factor Deficiencies • Severe: <1% • Hemarthrosis with minimal trauma or ADLs • Moderate: 1 to 5% • Intermediate symptoms with fewer hemarthroses • Mild: >5% • Joint bleeds rarely develop except with significant trauma

  6. Factor Replacement • For routine muscle or joint bleed give 25 U/kg to maintain levels >1% for 48 hours • Preoperatively: • Screen for inhibitors • Elevate F VIII levels to >100% 1 hr preop • Maintain at 60% • If vigorous PT required, transfuse to 50% pretreatment

  7. Preoperative Evaluation • Accurate diagnosis • Accurate quantification • Adequate reserves of factor • Lab must be able to perform assays • HIV, Hepatitis status

  8. Hemophilic Arthropathy • As blood is catabolized, it is absorbed by synovium • Iron is toxic to cells – synovial cells disintegrate releasing lysosomes which destroy cartilage and inflame synovium • Hypertrophic, hypervascular synovium • Chondrocytes also affected • FIBROSIS

  9. Radiographic Changes • Like RA • Soft tissue swelling • Osteopenia • Overgrowth of epiphysis • Erosions • Cysts

  10. Treatment • Prophylactic transfusions must be started at age 2 to 3 • Need central access • Risk of bacteremia • Costly • Aspiration: lowers blood load

  11. Treatment • Arthroscopic synovectomy • Radiosynovectomy • 32P chromic phosphate • 75% good to excellent results

  12. Knee • Arthroscopic synovectomy • Open debridement • TKA • Arthrodesis

  13. ELBOW • Open synovectomy with excision of radial head • Contraindicated in children • ANKLE and SUBTALAR JOINT • Arthrodesis

  14. Hip • Two modes of onset • Rapid progression due to increased intracapsular pressure • Chronic synovitis • THA • 33% aseptic failure rate

  15. Shoulder • Abandoned open synovectomy • Arthroscopy limited • Arthrodesis • Arthroplasty

  16. Other Topics • Muscle bleeds • Pseudotumor • HIV

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