Bleeding child

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Bleeding in children

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  • Jump to: navigation, searchHenoch-SchönleinpurpuraClassification and external resourcesTypical purpura on lower legs and buttocksICD-10D69.0(ILDS D69.010)ICD-9287.0DiseasesDB5705MedlinePlus000425eMedicinederm/177emerg/767emerg/845ped/3020MeSHD011695Henoch–Schönlein purpura (HSP, also known as anaphylactoidpurpura[1], purpurarheumatica[1], and Schönlein–Henochpurpura)[1] is a disease of the skin and other organs that most commonly affects children. In the skin, the disease causes palpable purpura (small hemorrhages); often with joint and abdominal pain. With kidney involvement, there may be a loss of small amounts of blood and protein in the urine, but this usually goes unnoticed; in a small proportion of cases, the kidney involvement proceeds to chronic kidney disease. HSP is often preceded by an infection, such as pharyngitis.HSP is a systemic vasculitis (inflammation of blood vessels) and is characterized by deposition of immune complexes containing the antibody IgA; the exact cause for this phenomenon is presently unknown. It usually resolves within several weeks and requires no treatment apart from symptom control, but may relapse in a third of the cases and cause irreversible kidney damage in about one in a hundred cases.Henoch-Schönleinpurpura is a small-vessel vasculitis in which complexes of immunoglobulin A (IgA) and complement component 3 (C3) are deposited on arterioles, capillaries, and venules. As with IgA nephropathy, serum levels of IgA are high in HSP and there are identical findings on renal biopsy; however, IgA nephropathy has a predilection for young adults while HSP is more predominant among children. Further, IgA nephropathy typically only affects the kidneys while HSP is a systemic disease. HSP involves the skin and connective tissues, scrotum, joints, gastrointestinal tract and kidneys.[7]
  • Bleeding child

    1. 1. Tutorial 10: Bleeding disorders in children  Physiology of haemostasis & coagulation pathways  Common bleeding disorders
    2. 2. Haemostasis of Blood 1. Vascular spasm and vasoconstriction 2. Formation of platelets plug 3. Blood coagulation 4. Formation of fibrin mesh
    3. 3. Blood Coagulation
    4. 4.  Abnormal /normal bleeding?  The onset of bleeding - neonatal period (intrauterine or postnatal infection) - 2nd to 5th day of life (hemorrhagic disease of newborn) - Prolonged bleeding after trauma and dental extraction (congenital or acquired disorders)  The site of bleeding - Bleeding into joints or muscles (Hemophilia A & B) - The nose (epistaxis) mainly due to von-willebrand’s disease - Abnormal bleeding involves gums, skin and under periosteum of long bones (scurvy) - GIT hemorrhage in neonatal period (hemorrhagic disease)
    5. 5.  The past history - Easy bruising, bruising at abnormal sites, prolonged bleeding after trauma or invasive procedure  The family history - Hemophilia A, Christmas disease (sex- linked recessive) - Von- Willebrand’ s disease & hemorrhagic hereditary telangiectasia (autosomal dominant)
    6. 6.  Drug ingestion (warfarin, heparin & aspirin)  Diet ( vitamin C and Vitamin K)  Splenomegaly (hypersplenism) – underlying causes like leukemia  Miscellaenous ( Wiskott-Aldrich Syndrome characterized by eczema, thrombocytopenia and immunodeficiency & Ehlers-Danlos syndrome due to platelet aggregation failure)
    7. 7. Bleeding disorder in children  Defects / abnormalities in : 1. Vascular OR 2. Platelets ( qualitative/quantitative) OR 3. Coagulation
    8. 8. Bleeding Due to Vascular Defect Anaphylactoid Purpura; Henoch- Schonlein purpura Infective states; meningococca emia & dengue haemorrhaic fever Nutrition Deficiency; Vitamin C (Scurvy) Miscellaenous; hereditary haemorrhagic telangiectasia, polyarteritis nodosa &Ehlers- Danlos Syndrome
    9. 9. Anaphylactoid purpura  Small vessel vasculitis by immune complexes  Combination of skin rashes (purpura), athralgia and abdominal pain.  Usually occur at age between 3-10 year old  Common in boys-preceded by pharyngitis
    10. 10.  -Haemophilia  -Von Willibrand Disease
    11. 11. -is a group of hereditary genetic disorders that impair the body's ability to control blood clotting or coagulation Classification : -Haemophilia A is a recessive X-linked genetic disorder involving a lack of functional clotting Factor VIII and represents (80%) -Haemophilia B is a recessive X-linked genetic disorder involving a lack of functional clotting Factor IX. (20%) -X-linked recessive,male predominance,qualitative/quantitative defect
    12. 12. Signs and symptoms Present towards the end of first year of life(vary)  Recurrent spontaneous bleeding into joints and muscles  Large bruises and haematomas  Internal or external bleedings Three levels of hemophilia are recognized, according to the level of clotting factor amounts in the blood. These are often expressed as percentages of normal:  Above 5% - mild hemophilia  1% to 5% - moderate hemophilia  Less than 1% - severe hemophilia
    13. 13. Diagnosis  History and signs  Activated partial thromboplastin time (prolonged)  Factor VIII assay <10 IU  Prenatal test-genetic testing have been made available
    14. 14. Management • Regular infusions of recombinant FVIII or FIX concentrates • IM injections, aspirin and NSAIDs and trauma should be avoided in patients with haemophilia. • Desmopressin (DDAVP) and cryoprecipitate for mild or moderate haemophilia A • Specialised physiotherapy-pain,reabsorption and prevent/treat injury
    15. 15. Complications • Deep internal bleeding -Joint damage from haemarthrosis -Intracranial haemorrhage • Transfusion transmitted infection
    16. 16. -deficiency of von Willebrand factor. Von Willebrand factor facilitates platelet adhesion to damaged endothelium and also acts as the carrier protein for FVIII. most common hereditary bleeding disorder, present in 1–2% of the general population. Is usually inherited as an autosomal dominant trait and rarely as an autosomal recessive trait. -Affects men and women equally - Classification Type 1- partial quantitative decrease of qualitatively normal von Willebrand factor Type 2-qualitatively abnormal Type 3-absent
    17. 17. Signs and Symptoms  Varying degree of easy bleeding  Mucosal bleeding such as epitaxis and gum bleeding  Bruising  Menorrhagia  Severe internal or joint bleeding uncommon
    18. 18. Diagnosis • Bleeding time (is prolonged) • APTT (prolonged) • Ristocetin cofactor test (to measure vWF activity which uses the antibiotic ristocetin to induce vWF to bind to platelets. ) • von Willebrand factor level (level is reduced also act as APR) Von Willebrand disease may be hard to diagnose.
    19. 19. Treatment • DDAVP for milder form of vWD ( increase secretion of FVIII and vWD into plasma) • Plasma derived FVIII concentrate for more severe types of vWD • For women with heavy menstrual bleeding, the combined oral contraceptive pill may be effective in reducing bleeding or in reducing the length or frequency of periods
    20. 20. Contraindications  Bleeding (hemorrhaging) may occur after surgeries or other invasive procedures.  If you have von Willebrand disease, do not take nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin or ibuprofen
    21. 21. Platelet Disorders  Leads to defects in primary haemostatic mechanism  Increased bleeding time  Purpura and petechiae
    22. 22. Types of platelet disorders  Qualitative (dysfunction)  Glanzman disease  Quantitative reduced (Thrombocytopenia)  Decreased production: aplastic anemia, leukemia  Increased destruction: ITP, drugs, collagen disorders  Increased consumption: DIC, septicaemia
    23. 23. IMMUNE THROMBOCYTOPENIA Classified according to antigenic stimulus  Autoimmune  Immune/idiopathic thrombocytopenic purpura (ITP)  Alloimmune  Posttransfusion purpura  Neontal alloimmune thrombocytopenia  Drug-induced  Splenic destruction of antibody coated platelet
    24. 24. Acute ITP  Affects young children (> 90% of cases)  Acute in onset, usually self-limiting  Majority of cases follow vaccinations or viral infections  ♂ : ♀ = 1:1  About 10% fail to recover within 6 months → chronic ITP
    25. 25. Signs & Symptoms <20,000/μl-admitted  Spontaneous formation of purpura and petechiae  Epistaxis  Bleeding gums  Hematomas in mouth/mucous membranes <5,000 /μl  Subarachnoid/intracerebral hemorrhage  Lower GI bleeding/internal bleeding
    26. 26. How to diagnose? Low platelet count No other blood abnormalities Exclude 2⁰ causes: leukemia, medications, lupus, cirrhosis, HIV etc
    27. 27. Findings No splenomegaly Prolonged bleeding time Bone marrow examination: increased megakaryocytes
    28. 28.  Full blood count  Peripheral blood film  Bleeding time (thrombocytopenia and dysfunction)  Prothrombin time  Activated Partial Thromboplastin Time  Coagulation factor assays (FXIII & FIX)  aPTT & PT mixing studies  Platelet funtion test & flow cytometry  Thrombin time  Liver & Renal Function Test  D-dimers

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