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How to approach a child with bleeding disorder
This document provides guidance on evaluating and treating children with bleeding disorders. It outlines the key steps in the clinical approach: obtaining a medical history, conducting a physical exam, and ordering laboratory tests. Common inherited disorders include hemophilia A/B and von Willebrand disease, while acquired disorders include DIC and vitamin K deficiency. Based on the history and exam findings, specific laboratory tests are interpreted to identify coagulation factor deficiencies or other causes of bleeding. Common treatments target the underlying disorder, such as intravenous immunoglobulins for ITP or DDAVP for von Willebrand disease.
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How to approach a child with bleeding disorder
- 2. How To ApproachA child with Bleeding Disorder DR. KAMRAN AKBAR PGR PEDIATRICS UNIT 2
- 3. Approach includes: History Clinical Examination Laboratory Findings
- 4. INHERITED DISORDERS ACQUIREDDISORDERS Early age of presentation Family history positive More sever Bleeding is the dominant feature Single factor defect Later age of presentation Family history usually negative Less sever Clinical picture is dominated by the underlying disorder e g. DIC Multiple hemostatic defect
- 5. HISTORY: If childbleeds from superficial cut with profuse bleed? If fever and neurological manifestations? If history of viral prodrome ? Drug related purpura Meningococce mia/TTP If patient taking aspirin , ibuprofen ,antibiotics. Coagulation disorder Platelet Disorder HUS/ITP If child bleeds from injury , does the bleeding stop and resume?
- 6. If patient is neonate , with history of traumatic delivery or low APGAR. If patient is neonate , and mother took phenytoin . If patient receive multiple blood transfusions in the past. If other family members have similar problem of abnormal bleeding or if repeated episodes of bleeding gums , prolonged bleeding as a result of circumcision. DIC Vitamin K Deficiency Congenital Disorder Post transfusional purpura or alloimunizational HISTORY: continued
- 7. Clinical Examination Theexamination should determine the presence of : petechiae, ecchymosis, hematomas, hemarthroses, or mucous membrane bleeding.
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- 10. Look For : Easy Bruising Individuals with disorders of the collagen matrix and vessel wall may have loose joints and lax skin associated with easy bruising (Ehlers-Danlos syndrome). Mucocutaneous Bleed Patients with defects in platelet-blood vessel wall interaction (VWD or platelet function defects) Deep Bleed (muscle or joints) Individuals with a clotting factor deficiency of factor VIII or IX (hemophilia A or B)
- 11. Lymphadenopathy + hepatosplenomegaly Leukemia Lymphoma Infection Symmetric Purpura on Legs and buttocks Henoch– schonlein Purpura Ecchymotic Lesions Extensive and in various stages of revolutions Physical Abuse
- 12. Bleeding Disorders VesselWall Disorder Platelet Disorder Coagulation Factor Abnormalities
- 13. Vessel wall disorder Acquired Congenital 1. SENILE PURPURA 2. VASCULAR PURPURA 3. HENOCH SCHONLEIN PURPURA 1. HEREDITARY HEMORRHAGIC TELENGIECTASIA 2. EHLERS DANLOS SYNDROME
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- 15. HEREDITARY HEMORRHAGIC TELENGIECTASIA HEMOPHILIA
- 16. Platelet Disorders QualitativeQuantitative • THROMBASTHENIA • BERNARD-SOULIER SYNDROME • DRUGS(ASPIRIN,TXA2,INDOMATHACIN • THROMBOCYTOPENIA • THROMBOCYTHEMIA
- 17. Coagulation Factor Abnormalities Hereditary Acquired 1. HEMOPHILIA A(factor VIII deficiency) 2. HEMOPHILIA B(factor IX deficiency) 3. von WILLEBRAND DISEASE 4. DISORDERS OF FIBRINOGEN-HEREDITARY AFIBRINOGENAEMIA HYPOFIBRINOGENAEMI DYSFIBRINOGENAEMIA 1. DISSEMINATED INTRAVASCULAR COAGULATION(DIC) 2. LIVER DISEASE 3. VIT K DEFICIENCY 4. MASSIVE TRANSFUSION OF STORED BLOOD 5. ACQUIRED INHIBITORS OF COAGULATION 6. HEPARIN OR ORAL ANTICOAGULANT THERAPY 7. RENAL DISEASE
- 18. 1. Full bloodcount and blood film 2. Bleeding time 3. Prothrombin time with INR – measure factors II, V, VII, X. 4. Activated Partial thromboplastin time measures II, V, VIII, IX, X, XI and XII. 5. Mixing studies 6. Thrombin Time 7. Quantitative fibrinogen assay 8. D-Dimers 9. Biochemical Screen for renal and Liver function tests 10. Platelet aggregation studies
- 19. CBC complete bloodcount, F factor PFA platelet function analyzer, PT prothrombin time, PTT activated partial thromboplastin time, RIPA ristocetin -induced platelet aggregation, vWD von Willebrand disease, vWF von Willebrand factor, vWF : R Co ristocetin cofactor activity.
- 20. Interpretation of Labresults: Factor VIII Factor IX vWD Thrombocyto penia DIC Complete blood examination Normal Normal Normal Decrease platelets counts Dec. Hb Dec. Platelets Peripheral film Schistocytes Bleeding time Normal Normal Increase Increase Increase PT Normal Normal Normal Normal Prolong APTT Prolong Prolong Prolong Normal Prolong Mixing studies Correct PT, APTT Correct PT, APTT Correct PT, APTT Correct PT, APTT Clotting factors assay VIII deficiency IX deficien cy vWF deficiency - Thrombin Time - - - - Increase
- 21. Treatment of commonbleeding disorders. Bleeding disorder Idiopathic thrombocytopenic purpura Disseminated Intravascular coagulation Henoch-schonlein purpura Platelet function abnormalities Treatment Intravenous immunoglobulins Steroids Anti D immunoglobulins Continuous platelet transfusion (for life threatening hemorrhage). Fresh frozen plasma Platelet and packed red blood cell transfusion. Steroids Platelet transfusion(for life threatening hemorrhage)
- 22. Treatment of commonbleeding disorders (continued) Bleeding disorder Von willebrand disease (vWD) Treatment Desmopressin acetate (DDAVP)