Hemophilia - HEMOPHILIA John O’Sullivan 24 May 2001...

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Unformatted text preview: 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 Prevalence: 13.4 cases per 100,000 males Incidence: 1 in 5032 live male births Diagnosis Atypical bleeding at circumcision or Atypical bruising at neonatal vaccines bruising Toddlers with lip bleeding or unusual Toddlers bruising when learning to walk bruising Hx of affected males on mother’s side Elevated PTT Factor assays Factor Deficiencies Severe: <1% Hemarthrosis with minimal trauma or ADLs Moderate: 1 to 5% Intermediate symptoms with fewer Intermediate hemarthroses hemarthroses Mild: >5% Joint bleeds rarely develop except with Joint significant trauma significant Factor Replacement For routine muscle or joint bleed give 25 U/kg to For maintain levels >1% for 48 hours maintain Preoperatively: Screen for inhibitors Elevate F VIII levels to >100% 1 hr preop Maintain at 60% If vigorous PT required, transfuse to 50% If pretreatment pretreatment Preoperative Evaluation Accurate diagnosis Accurate quantification Adequate reserves of factor Lab must be able to perform assays HIV, Hepatitis status Hemophilic Arthropathy As blood is catabolized, it is absorbed by As synovium synovium Iron is toxic to cells – synovial cells Iron disintegrate releasing lysosomes which destroy cartilage and inflame synovium destroy Hypertrophic, hypervascular synovium Chondrocytes also affected FIBROSIS Radiographic Changes Like RA Soft tissue swelling Osteopenia Overgrowth of epiphysis Erosions Cysts Treatment Prophylactic transfusions must be started at Prophylactic age 2 to 3 age Need central access Risk of bacteremia Costly Aspiration: lowers blood load Treatment Arthroscopic synovectomy Radiosynovectomy 32P chromic phosphate 75% good to excellent results Knee Arthroscopic synovectomy Open debridement TKA Arthrodesis ELBOW Open synovectomy with excision of Open radial head radial Contraindicated in children ANKLE and SUBTALAR JOINT Arthrodesis Hip Two modes of onset Rapid progression due to increased Rapid intracapsular pressure intracapsular Chronic synovitis THA 33% aseptic failure rate Shoulder Abandoned open synovectomy Arthroscopy limited Arthrodesis Arthroplasty Other Topics Muscle bleeds Pseudotumor HIV ...
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This note was uploaded on 12/24/2011 for the course STEP 1 taught by Professor Dr.aslam during the Fall '11 term at Montgomery College.

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