Clinical approach to ableeding child

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This is appt presentation done by me and my colleagues Bahaa Alsayed and Anas mohammed ( agroup of medical undergarduates , school of Medicine, Ain-shams university , Cairo , Egypt ) ...This work was presented at the end of our Pediatrics clinical round ..

I Hope every one to get the best out of the presentaion ..Any commentaries are even more appreciated :)

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Clinical approach to ableeding child

  1. 1. Objectives :
  2. 2. Hemostasis BV Injury Thromboxane Platelet A2 ADP Phospolipid Serotonin Platelet Activation Stable Hemostatic Plug
  3. 3. Vascular Platelet defect defect
  4. 4. Primary Hemostatic Disorders Vascular defect : Congenital : Hereditary hemorrhagic telangectasia Acquired :  Vitamin C deficiency (scurvy)  Connective tissue disorders  Henoch schonlein purpura  Infections : Meningiococcemia Platelet defect :  platelets (thrombocytopenia) : 1. Decreased platelet production 2. Abnormal distribution 3. Increased destruction 4. Decrease production and increased destruction  Platelet Dysfunction ( Thrombasthenia ) :
  5. 5. Primary Hemostatic Disorders  platelets (thrombocytopenia) :  Congenital : TAR Syndrome , Fanconi anemia  Acquired : BM cavity abnormality , Stem cell abnormality  Splenomegaly Giant hemangioma  Immune mediated : (ITP, drugs, infections : EBV, HIV)  Non immune mediated : (DIC , TTP , HUS )  Congenital infections
  6. 6. Primary Hemostatic DisordersPlatelet Dysfunction ( Thrombasthenia ) :  Glanzmann’s (normal platelet morphology , defect of platelet aggregation )  Bernard sloer ( qualitative and mild quantitative platelet disorder , failure of platelet adhesion) platelets (thrombocytopenia) Von willebrand disease ( quantitative or qualitative deficiency of vWF molecule which: - binds to exposed subendothelial collagen - mediates initial platelet adhesion  Aspirin  Renal failure  Heparin in high doses
  7. 7. Secondary Hemostatic DisordersCoagulopathies :  Hemophilia A (factor VIII defeciency , classic hemophilia )  Hemophilia B (factor IX defeciency , christmas disease )  Hemophilia C (factor XI defeciency ) Von willebrand disease ( Vascular emophilia ) Congenital afibrinogenemia  Vit. K defeciency  Liver disease  DIC ( consumptive coagulopathy )  Anticoagulants : Oral anticoagulants – Heparin
  8. 8. • Age• Sex• Trauma• Past bleeding history (tooth ,circumcision)• Distribution• Associated symptoms ( Fever and weight loss ), Abdominal pain and arthritic pain ( HSP)• Review of other systems• Medications• Family history of bleeding
  9. 9. Typical Presentations
  10. 10. Typical Presentations cont.
  11. 11. Clinical approach in making a diagnosis Clinical Examination• Petechiae ( less than 2 mm)• Purpura ( more than 2 mm and less than 20 mm)• Ecchymosis ( more than 20 mm)• Joint bleed• Hepatosplenomegaly• Lymphadenopathy• Evidence of underlying liver disease
  12. 12. Petechiae PurpuraEcchymosis Hemarthrosis
  13. 13. Vascular causes :Vascular causes : Platelet defect Coag.fact defect Site of bl. Skin and mm. Deep in soft Petechiae Yes No Echymosis Small , superficial Large , deep Hemarthrosis Rare Common Hematomas Rare Common Bl.after cuts Early Late Bl.post surgery Early Late
  14. 14. Clinical approach in making a diagnosisGeneral Specific tests tests • CBC • Blood film • Platelet f. tests • BT • Fibrinolysis tests • Cl. factor assay • PT ,PTT • Mixing test • vWF assay • TT • PFA
  15. 15. CBC Blood Film PT PTT
  16. 16. Data Interpretation • Liver disease • Warfarin therapy • Vit. K def. • DIC ( early ) • Factor 7 def. • Factor 7 inhibitor Dose the patient has factor def. ? Do mixing testsCorrected Not correctedFactor def. Inhibitors or lupus anticoagulant
  17. 17. Data Interpretation• Heparin therapy• Factor 8 , 9 ,11 , 12 def.• vWD• Factor inhibitor• Lupus anticoagulant• Common pathway def.• Heparin or warfarin overdose• Late liver disease• DIC• Factors inhibitors• Primary fibrinolysis• Dysfibrinoginemia
  18. 18. Data Interpretation Liver disease DIC Primary fibrinolysis Factor 8 normal Factor 8 low Platelet normalD- dimer test normal Abnormal D - D- dimer test dimer normal
  19. 19. Data InterpretationConsider evaluating for:• Platelet disorder• Mild factor deficiency• Factor XIII• Monoclonal gammopathy• Abnormal fibrinolysis• a2 anti-plasmin deficiency• Vascular disorders• Dysfibrinogenemia
  20. 20. Data Interpretation Normal PT , PTT Think of platelet Normal platelet Thrombocytopenia• Do PFA-100 Look for the causeThink of• vWD• Factor 13 def.• Vasculopathy
  21. 21. Current treatment strategiesTreatment IndicationPlatelet concentrate Low pt , platelet dysfunctionFresh frozen plasma “ FFP ” Multi-factor coagulopathy Factors V, X (XI, XIII) deficiencyCryoprecipitate Low fibrinogen, FXIII deficiency Platelet dysfunctionProthrombin concentrate Multiple Vitamin K deficiency Liver DiseaseAminocaproic Acid Excessive fibrinolysisDDAVP Platelet dysfunctionFactor concentrate-rFVIIa FVII deficiency-FVIII FVIII deficiency-FIX FIX deficiency

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