1. Hemorrhagic diatheses in children. Gastrointestinal bleedings. Sakharova I. Ye., MD, PhD

2. 2 The stopping of a bleeding is carried out due to three parts of a hemostasis: 1.Vascular integrity. 2.Qualitative and quantitative characteristics of platelets. 3.Presence of coagulation factors in blood.

3. 3 According to this all hemorrhagic diatheses are divided into 3 groups: 1. Vasopathies 2. Thrombopathias 3. Coagulopathies

4. 4 Schönlein-Henoch purpura (synonims - anaphylactoid purpura, allergic angiitis, small-vessel vasculitis, hemorrhagic vasculitis, Henoch-Schönlein disease) is one of the collagen vascular diseases in which basis lays immune complex mechanism of small vessels wall damaging with skin, joints, intestine and kidneys affection.

5. 5 Clinical features. Papular- macular hemorrhagic purpuric lesions. Typical places of rush localization: extensor surfaces of legs, on the feet, over the joints, on the buttocks; occasionally, they may occur on the hands, extensor surfaces of arms, elbows, and face, but very rarely on the trunk.

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10. 10 Clinical features of the Sch ö nlein- Henoch purpura.  joints involvement  acute abdominal pain, vomiting, melena  renal involvements (microscopic hematuria, with or without proteinuria)  scrotal involvement (epydidimitis, orchitis, and scrotal bleeding)

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12. 12 Idiopathic thrombocytopenic purpura (ITP, primary immune thrombocytopenic purpura, autoimmune thrombocytopenic purpura) describes an autoimmune disorder in which the number of circulating platelets is less than 150 G/l.

13. 13 Clinical features. The onset of the disease is usually sudden. The symptoms of intoxication and fever usually are absent. Skin purpura, which arises either spontaneously or secondary to trauma. The type of rush is petechial- bruise. The second frequent clinical sign are bleedings. In the beginning of the disease can be nose bleeding (epistaxis), bleeding from gums, mucous membranes, gastrointestinal tract, kidneys and metrorrhagias (uterinal bleedings). Hemorrhage into the central nervous system, the most serious complication of thrombocytopenia.

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16. 16 Hemophilia A and B are inherited bleeding disorders caused by deficiencies of clotting factor VIII (F VIII - antigemophilic globulin (AHG)) and factor IX (F IX - plasma thromboplastin component (PTC) or Christmas factor) correspondingly.

17. 17 Mechanism of hemophilia inheritance XyXy xx xy XxXx XxXx XyXy xxXxXx XyXy XXxXx xy XyXy

18. 18 Prophylactic treatment The aim of this treatment is to maintain 5 % factor activity in patient’s blood. Start from the age of 1-2 years. Use monoclonal-antibody purified F VІІІ, F ІХ and recombinant F VІІІ, F ІХ 3 times in week in hemophilia A and 2 times in week in hemophilia B 25-40 IU/kg.

19. 19 Treatment of acute bleeding episodes Hemophilia A –Fresh frozen plasma (100 ml=80IU АHG) Dose is 10-15 ml/kg IV during 30-60 min, repeat after 8-12 hours. –Cryoprecipitate – Monoclonal-antibody purified F VІІІ and recombinant F VІІІ Hemophilia B – Fresh frozen plasma – Monoclonal-antibody purified F IX and recombinant F IX

20. 20 During severe or dangerous (e.g. CNS, retroperitoneal) bleeds need to obtain 50-100% factor activity for 7-10 days. For less critical situations (e.g. dental extractions, haematuria, soft tissue bleeds), 20-50% factor activity for 2-7 days are generally sufficient. For uncomplicated haemarthroses or superficial muscle or soft tissue bleeds, 20-30% for 1-2 days.

21. 21 Laboratory differential diagnostics of hemorrhagic diatheses Count of platelets Time of bleeding Coagula tion time Clot retrac- tion Coagulo gram Hem. vaskul. ITP Trombopathia Hemophilia nnnnnn nnnnnnnn nnnnnn nnnnnn nnnnnn

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42. 42 Success is not final, failure is not fatal. It is the courage to continue that counts. Winston Churchill

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