1. February 4 th, 2011

2. The Child With Pain Single Joint Involvement Multiple Joint Involvement With Fever Septic arthritis/Osteomyelitis Sympathetic arthritis Foreign body with infection Traumatic arthritis Soft Tissue infection Rheumatic Diseases Reactive arthritis Toxic synovitis Diskitis Sarcoidosis Hemoglobinopathy Malignancies Without Fever Trauma Mechanical derangement Rheumatic Diseases Toxic synovitis Avascular necrosis Subacuteosteomyelitis Diskitis Hemoglobinopathy Reflex sympathetic dystrophy Malignancies Psychogenic pain With Fever Bacterial infections Sepsis Viral infections Lyme disease Reactive arthritis Rheumatic diseases Post immunization Immune deficiencies Serum sickness Inflammatory bowel disease Sarcoidosis Familial Mediterranean fever Malignancies Without Fever Rheumatic diseases Joint hypermobility Growing pains Post immunization Immune deficiencies Guilain-Barre Lyme Disease Fibromyalgia syndrome Chronic fatigue syndrome Psychogenic pain Malignancies

3. Evaluation of Bleeding Disorder Am I dealing with a bleeding disorder? What is the clinical phenotype? Is it congenital or acquired? Systemic disease or drug causing exacerbation?

4. Am I dealing with a bleeding disorder? With bruising Trauma is most common Abuse is more common than hemophilia

5. Am I dealing with a bleeding disorder? Bruising Typical areas: boney protuberances of extremities More pronounced, more numerous, recurrent Larger than quarter-size Associated hematoma Intramuscular hematoma or hemarthrosis Out of proportion to mechanism

6. Am I dealing with a bleeding disorder? Epistaxis ER visit Both nostrils simultaneously Associated with other signs of bleeding Family history of similar bleeding

7. Am I dealing with a bleeding disorder? Menorrhagia Associated with anemia Frequent pad changes (<q2hrs) Menses >7days

8. Am I dealing with a bleeding disorder? Surgical Bleeding Uncontrolled bleeding in surgical field Other sites (drains, lines) Unexpected need for transfusion

9. What is the clinical phenotype? Primary hemostasis (platelets, vWF, vessel) Easy bruisability Petechiae Epistaxis Menorrhagia Surgical wound oozing Coagulation factor (Hemophilia) Hematomas Hemarthrosis Delayed surgical bleeding

10. Inheritance Patterns Hemophilia X-Linked Glanzmann thrombasthenia Factor XIII Autosomal recessive Von Willebrand Autosomal Dominant

11. Acquired Bleeding Abnormality Underlying medical illness Liver disease Vitamin K deficiency Disseminated intravascular coagulation Medications

12. Lab Evaluation Screening Studies CBC, smear, PT, PTT, Bleeding time/PFA PT: extrinsic and common pathways PTT: intrinsic and common pathways Factor XIII can have normal PT and PTT Incidental prolonged PTT Lupus Anticoagulant

13. Hemophilia X-Linked Female carriers may be symptomatic Presentation Circumcision With ambulation Labs: prolonged PTT Hemophilia A: Factor VIII 1:5,000 Hemophilia B: Factor IX 1:30,000

14. Hemophilia: Treatment Infusion of factor concentrates Prophylactically Minor hemorrhage 50% correction Major hemorrhage 100% correction

15. What’s going on? 16y/o athlete with h/o hemophilia Cc: Left groin pain, limp No h/o trauma Exam: decreased sensation anterior L thigh Painful extension L hip Decreased strength with flexion L hip

16. What to do? 3y/o with h/o hemophilia Was playing on back of couch and fell to floor Struck head Acts drowsy