February 4 th, 2011

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

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?

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

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

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

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

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

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

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

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

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

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

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

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

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