Approach to bleeding disorder

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Approach to a bleeding disorder: These presentation has the approach for a patient of bleeding disorder. it has History, physical finding, Investigations.

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  • Approach to bleeding disorder

    1. 1. Approach to a Bleeding Disorder By: Hailemariam Bekele Hayelom Michael
    2. 2. Outline 2 Introduction History taking Physical finding Investigation References
    3. 3. Introduction Bleeding, technically known as hemorrhaging, is the loss of blood escaping from the circulatory system. Bleeding can occur internally, where blood leaks from blood vessels inside the body, or externally, either through a natural opening such as the mouth, nose, ear, urethra, vagina or anus, or through a break in the skin. 3
    4. 4. 4 Bleeding arises due to either traumatic injury, underlying medical condition, or a combination. 'Medical bleeding' denotes hemorrhage as a result of an underlying medical condition Blood can escape from blood vessels as a result of 3 basic patterns of injury: Intravascular changes - changes of the blood within vessels (e.g. ↓ clotting factors)
    5. 5. 5 Intramural changes - changes arising within the walls of blood vessels (e.g. aneurysms) Extravascular changes - changes arising outside blood vessels (e.g. infection) Certain medical conditions can also make patients susceptible to bleeding. These are conditions that affect the normal "hemostatic" functions of the body. Hemostasis involves several components. The main components of the hemostatic system include platelets and the coagulation system.
    6. 6. Bleeding disorders Inherited disorders Acquired disorders Inherited disorders: Coagulation Factor Deficiencies Platelete disorders Acquired disorders: Liver disease , Vitamin- k deficiency, Anticoagulants,Platelet abnormalities 6
    7. 7. 7
    8. 8. History taking 8 For most hemorrhagic conditions, history plays an important role in diagnosing the cause. For a hemorrhagic condition the history should determine the site or sites of bleeding, the severity and duration of hemorrhage, and the age at symptom onset.
    9. 9. 9 Was the bleeding spontaneous or after trauma? Does bruising occur spontaneously? One should determine if symptoms correlate with the degree of injury or trauma. Are there lumps with bruises for which there is minimal trauma? Was there a previous personal or family history of similar problems? recent transfusion?
    10. 10. Cont’d… 10 If there has been joint pain, swelling or limitation of movement If there has been bleeding from umbilical stump If there has been previous surgery or significant dental procedures, was there any increased bleeding? Delayed or slow healing of superficial injuries may suggest a hereditary bleeding disorder. In postpubertal females, it is important to take a careful menstrual history. Medications such as aspirin, other non-
    11. 11. 11 Note: Once the child is beyond the neonatal period, thrombotic symptoms are relatively rare until adulthood. In the neonate, physiologic deficiencies of procoagulants and anticoagulants cause the hemostatic mechanism to be dysregulated Even in the absence of a family history, the presence of thrombosis in the child or teenager should trigger an evaluation of the individual for a hereditary or acquired predisposition to
    12. 12. 12 Non-steroidal anti-inflammatory drugs such as ibuprofen, mefenamic acid, etc. and aspirin inhibit platelet function whereas anticonvulsants, antihistaminics, antituberculous drugs especially rifampicin are known to cause thrombocytopenia. The overall health of the patient also is a clue to the cause of bleeding. Congenital bleeding disorders and ITP usually occur in children who are otherwise well.
    13. 13. Physical Examination 13 Physical examination offers further clues to the diagnosis Is it bleeding? e.g. fixed drug eruption, erythema nodosum, viral exanthem and mosquito bites The examination should determine the presence of petechiae, ecchymoses, hematomas, hemarthroses, or mucous membrane bleeding.
    14. 14. 7/19/2014 approach to pediatrics bleeding disorders 14 Patients with defects in platelet/blood vessel wall interaction usually have mucous membrane bleeding; petechiae on the skin and mucous membranes; and small, ecchymotic lesions of the skin sometimes associated with hematomas. Individuals with a clotting factor deficiency such as factor VIII or factor IX deficiency have symptoms of deep bleeding into muscles and joints with
    15. 15. 15
    16. 16. 16
    17. 17. 17
    18. 18. 18 Petechiae are pathognomonic of platelet-related bleeding Bruises in any area that appear excessively large for the degree of trauma or those with underlying palpable hematomas may be seen in patients with significant bleeding disorders Swelling of any joint without a history of significant trauma is definitely abnormal. Similarly, deep tissue and intramuscular bleeds should prompt the diagnosis of a
    19. 19. 19
    20. 20. 20 If it is bleeding, then is it localized or generalized? A single site involved, it is more likely to be a localized bleeding rather than a generalized bleeding disorder If generalized, is it platelet type or coagulation type of bleeding? Is it congenital/hereditary or acquired disorder? Symptoms of a longer duration are indicative of a congenital disorder such as von Willebrand Disease (vWD) or coagulation-factor deficiencies
    21. 21. 7/19/2014 approach to pediatrics bleeding disorders 21 Look for hepatosplenomegaly Do a rectal exam for evidence of GI bleeding Look for physical signs and symptoms of diseases related to capillary fragility: Petechiae secondary to coughing, sneezing, Valsalva maneuver, blood pressure measurement. Note: The possibility of physical abuse must be considered in the evaluation of any child with unusual patterns of bruising or bleeding.
    22. 22. Laboratory Evaluation
    23. 23. Laboratory Investigation Tests for Platelet I. Platelet count II. Bleeding Time(BT) Tests for coagulation factors I.ProthrombinTi me(PT) II. Activated Partial Thromboplastin Time(aPTT) III.Thrombin
    24. 24. Platelet count must be done in a suspected bleeding disorder. Bleeding Time (BT) Significance Assess Primary Hemostatic defect(vessel wall or platelet). Dependent on adequate functioning of plt. & Bl.Vs. Range 4-8 min Platelet count & Bleeding Time
    25. 25. Causes of prolonged BT I. Thrombocytopenia. II. VWD. III. Platelet function disorder IV. Disorder of blood vesseles. Interpretation
    26. 26. Significance  Reflects overall activity of the Extrinsic Pathway.  Most sensitive to changes in Factor V,VII,X.  Lesser to Factor I & II. Principle Platelet poor plasma+Tissue Thromboplastin+Calcium In Presence of F VII Extrinsic pathway is activated & clot formed Prothrombin Time(PT)
    27. 27. Causes of prolonged PT 1. Deficiency of Factor VII,X,V,II,I 2. Vit K deficiency 3. Liver disease 4. Oral anticoagulants Interpretatio n
    28. 28. Significance  Reflects efficiency of Intrinsic Pathway.  Sensitive to changes in Factor VIII,IX,XI,XII.  Also sensitive to heparin & circulating anticoagulants. The test measures the clotting time of plasma after the activation of contact So it indicates the overall efficiency of the Intrinsic pathway Normal range 26 to 40 seconds. Activated Partial Thromboplastin Time (aPTT)
    29. 29. Causes of prolonged aPTT 1. Deficiency of Factor VIII(Hemophilia A). 2. Deficiency of Factor IX(Hemophilia B). 3. Heparin therapy. 4. Circulating anticoagulants. 5. Liver disease. Interpretation
    30. 30. Significance Asses the final step of coagulation i.e. conversion of fibrinogen to fibrin in presence of thrombin. Bypasses Extrinsic & Intrinsic pathway. Principle Thrombin is added to plasma and the clotting time is measured. TT is affected by the concentration and reaction of fibrinogen and by the presence of inhibitory substances. Normal range A patient’s TT should be within 2 s of the control Thrombin Time(TT)
    31. 31. Causes of prolonged TT 1. Disorders of fibrinogen- Afibrinogenaemia. Hypofibrinogenaemia. Dysfibrinogenaemia. 2. Liver disease. 3. heparin therapy. . Interpretati on
    32. 32. Relevant 2nd line investigations are carried out with each of the patterns of abnormalities in first line tests. 2nd Line Investigation
    33. 33. PT-Normal PTT-Normal TT-Normal Fibrinogen -Normal Platelet count-Normal Interpretation 1. Primary hemostasis disorder. 2. Disorders of platelet function(cong or acquired). 3. Vascular disorders of hemostasis. 4. Factor XIII deficiency( fibrin stablizing factor) Scenario #1 ?
    34. 34. PT-Eleveted PTT-Normal TT-Normal Fibrinogen -Normal Platelet count-Normal Interpretation 1. Factor VII deficiency. 2. Liver disease. 3. Vit K deficiency. 4. At the start of oral anticoagulant therapy. 5. Mild deficiency of Factor II, V, X. Scenario # 2 ?
    35. 35. 2nd line investigations 1. Mixing test. 2. Factor VII assay. 3. Liver function test.
    36. 36. Mixing study Correction test using PT or aPTT PRINCIPLE Unexplained prolongation of PT or aPTT can be investigated with simple correction test by mixing the pt`s plasma with normal plasma. Correction (should be within few seconds) indicates a possible factor deficiency, whereas failure to correct suggests the presence of an inhibitor. METHOD Perform a PT and/or aPTT on control, patient`s,and a 50:50 mixture of the control and pt`s plasma.
    37. 37. PT-Normal PTT-Eleveted TT-Normal Fibrinogen -Normal Platelet count-Normal Interpretation 1. Congenital deficiency of F VIII, FIX,. 2. vWD 3. Heparin 4. Circulating anticoagulants- Specific (Anti factor VIII). Second line investigations 1. Mixing test. 2. Factor VIII & factor ix assay Scenario # 3 ?
    38. 38. PT-Eleveted PTT-Eleveted TT-Normal Fibrinogen -Normal Platelet count-Normal Interpretation 1. Vit k def. 2. On oral anticoagulants. 3. Liver dis. 4. Rare congenital or acq. Deficiency of factor v,x,ii 5. Combined factor V+VIII deficiency. 2nd line investigations 1. Mixing test. Scenario # 4 ?
    39. 39. PT-Eleveted PTT-Eleveted TT-Eleveted Fibrinogen –Normal/Abnormal Platelet count-Normal Interpretation 1. Unfractionated heparin 2. Hypofibinogenaemia 3. Afibrinogenemia 4. Dysfibrinogenemia 5. Systemic hyperfibrinolysis 6. Some cases of liver disease. 2nd line investigations Reptilase or ancord time Scenario # 5 ?
    40. 40.  Reptilase & Ancord are purified enzymes from snake.  May be used to replace Thrombin in TT test.  Snake venom is not inhibited by heparin  Normal time for clotting in presence of Heparin.  Clotting time will be prolonged in presence of decreased Fibrinogen. Reptilase or Ancord Time
    41. 41. PT-Eleveted PTT-Eleveted TT-Normal Fibrinogen –Normal/low Platelet count-Low Interpretation Chronic liver disease esp.cirrohsis. 2nd line investigations 1. Specific factor assay. 2. Peripheral blood smear. 3. Bone marrow aspirate. Scenario # 6 ?
    42. 42. PT-Normal PTT-Normal TT-Normal Fibrinogen -Normal Platelet count- low Interpretation 1. Thrombocytopenia. 2. Heparin use. 2nd line investigations 1. Peripheral blood smear. 2. Bone marrow aspirate. Scenario # 7 ?
    43. 43. References 43 1. American Academy of pediatrics (http://pedsinreview.aappublications.org/cgi/ content/full/29/4/121) 2. Nelson Textbook of Pediatrics, 18th ed. 3. Current Pediatric Diagnosis & Treatment , 18th edition 4. Pediatrics on call journal, may 2005 vol.2 issue 5 5. Up to date
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