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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
     5  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
     6  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
     7  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
     8  Preoperative Evaluation Accurate diagnosis Accurate quantification Adequate reserves of factor Lab must be able to perform assays HIV, Hepatitis status
     9  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
     11  Radiographic Changes Like RA Soft tissue swelling Osteopenia Overgrowth of epiphysis Erosions Cysts
     15  Treatment Prophylactic transfusions must be started at age 2 to 3 Need central access Risk of bacteremia Costly Aspiration: lowers blood load
     16  Treatment Arthroscopic synovectomy Radiosynovectomy 32P chromic phosphate 75% good to excellent results
     17  Knee Arthroscopic synovectomy Open debridement TKA Arthrodesis
     18  ELBOW Open synovectomy with excision of radial head Contraindicated in children ANKLE and SUBTALAR JOINT Arthrodesis
     19  Hip Two modes of onset Rapid progression due to increased intracapsular pressure Chronic synovitis THA 33% aseptic failure rate
     20  Shoulder Abandoned open synovectomy Arthroscopy limited Arthrodesis Arthroplasty
     21  Other Topics Muscle bleeds Pseudotumor HIV