1. Step 6
1.1. Review session 1
1.2. Report new knowledge
1.2.1. bleeding disorders in children
1.2.1.1. acquired Vs inherited
1.2.1.1.1. acquired
1.2.1.2. purpuric disorder
1.2.1.2.1. Von willebrand disease
1.2.2. coagulation disorder
1.2.2.1. heeamophilia
1.2.2.1.1. x-linked disease .. dificiancy of clotting factor ... types are A,B and C
1.2.2.1.2. life long bleeding disorder ... can be because of recent mutation
1.2.2.1.3. SEVERE ( activity less than 1% .. MILD ( activity more than 5%)
1.2.2.1.4. heamophilia A occurs because of either deleation, fram shift or mis sense mutation
1.2.2.1.5. if untreated it results in knee swellen which will lead to limping .. usually known because of family history
1.2.2.1.6. S&S include heamarthrosis, bruising and internal bleeding .. cause of death now is infection
1.2.2.1.7. diagnosed by PPT time which will be delayed
1.2.3. physiology of coagulation
1.2.3.1. intrinsic pathway
1.2.3.2. extrinsic pathway
1.2.3.2.1. after tissue factor 7 is activated to 7a which activate tissue factor >> then factor 10 is activated
1.2.3.3. common pathway
1.2.3.4. diagnosis by prothrombin time .. platlets count ..
1.2.3.5. there are primary and secondary hemostasis and the fibrinolysis
1.3. 30 minutes
2. Step 8
2.1. Diagnostic decision
2.1.1. heamophilia type A (severe)
2.2. Mechanism
2.2.1. inhireted disorder .. x-linked .. factor 8 is reduced or absenant
2.3. Presentation
2.3.1. right knee swelling
2.3.2. scattered bruising over the body
2.4. Supporting data
2.4.1. Physical examination
2.4.1.1. vital signs
2.4.1.1.1. 36.8
2.4.1.1.2. RESP rate is 28
2.4.1.1.3. BP is not mention
2.4.1.1.4. weight and height are not mention
2.4.1.1.5. nothing mention about myelstones
2.4.1.2. when crying he is holding he is right knee
2.4.1.3. the knee is warm, tense and tender
2.4.1.4. ROM is restricted
2.4.1.5. bruising is scattered over the body
2.4.1.6. normal heart sound
2.4.1.7. oral mucosa is normal
2.4.2. tests results
2.4.2.1. low Hb
2.4.2.2. high WBC
2.4.2.3. high APTT
2.4.2.4. x-ray shows soft tissue swelling
2.4.2.5. factor 8 is low (1%)
2.4.2.6. normal TT and PT
2.4.2.7. normal mixing time
2.4.2.8. normal factor 9 and VWf
2.5. 10 minutes
3. Step 7
3.1. Inquiry plan and info gathering
3.1.1. History of presenting complain
3.1.1.1. bruising was noticed during the last year
3.1.1.2. swollen started 2 hours after the bath
3.1.1.3. bruising was taught to be normal cuz the child is active
3.1.1.4. he was crying
3.1.1.5. nothing mention about falling
3.1.2. Previous medical / surgical history
3.1.2.1. no previous bleeding
3.1.2.2. past history is clear
3.1.2.3. uncircumsized
3.1.3. Drug history / allergy
3.1.3.1. no drugs
3.1.3.2. no allergy
3.1.3.3. no use of anti-coagulant
3.1.4. Family history
3.1.4.1. as one sister and one brother .. they are both normal ...
3.1.4.2. his cousin dies of bleeding after tonsilectormy
3.1.4.3. north saudi .. the mother migrated from Syria
3.1.4.4. the father is lawyer .. the mother is part time school teacher
3.1.4.5. no history of the mother and father
3.1.4.6. no nose bleeding. no mouth bleeding
3.1.5. Social / occupational history
3.1.5.1. nutririon is not mention
3.1.5.2. delivary is not mention
3.1.5.3. no smokers in the family
3.1.6. Systemic review
3.1.7. Physical examination
3.1.7.1. vital signs
3.1.7.1.1. 36.8
3.1.7.1.2. RESP rate is 28
3.1.7.1.3. BP is not mention
3.1.7.1.4. weight and height are not mention
3.1.7.1.5. nothing mention about myelstones
3.1.7.2. when crying he is holding he is right knee
3.1.7.3. the knee is warm, tense and tender
3.1.7.4. ROM is restricted
3.1.7.5. bruising is scattered over the body
3.1.7.6. normal heart sound
3.1.7.7. oral mucosa is normal
3.1.8. tests results
3.1.8.1. low Hb
3.1.8.2. high WBC
3.1.8.3. high APTT
3.1.8.4. x-ray shows soft tissue swelling
3.1.8.5. factor 8 is low (1%)
3.1.8.6. normal TT and PT
3.1.8.7. normal mixing time
3.1.8.8. normal factor 9 and VWf