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Hematologic dysfunction - Coggle Diagram

- - - - Alpha globin decreased
    - - Beta globin decreased
  - - - enlarged heart, an enlarged liver and spleen, fluid in the chest and abdomen, edematous skin
        
        often die while in the womb.
    - - Pale in first year and died
  - - - Alpha thalassemia major
      - Hb E
      - Beta thalasemia major
    - - VRecive blood when have indication : Very pale , infection
  - - - Continuously received penicillin after splenectomy until 5 year
      - Received Vaccine to prevent Pneumococcal และ Hemophilus influenza vaccine (Hib vaccine) before splenectomy 2 weeks
    - - serum ferritin>1,000 ng/ml
        ( 2 time within 1-3 Months)
      - received blood transfusion > 1 year
      - Iron in the liver is high level {(Magnetic
        Resonance Imaging (MRI T2*) >7 มก./ก.ของน้้ําหนักเนอื้ ตับที่แห้ง
      - Desferoxamine (Desferal) 20-60 mg/kg/day SC, IV
    - - ➢ Low transfusion: for supportive Hb <7 gm/dl
      - ➢ Hypertransfusion/High transfusion
        
        treatment Hb >10 gm/dl and provide new blood (not more than7 days) and combination with Iron chelation
      - Leucocyte depleted packed red cells (LDPRC)
        
        decrease Febrile non-hemolytic transfusion reaction
      - Check HCV, HBV and HIV
      - pre-medication :antipyretic (acetaminophen; 10 มก./กก.),antihistamine 0.1-0.2 มก./กก.
      - Diuretics (furosemide; 1 มก./กก.)
        ➢ Rate 4 ml/kg/hr ( 2.5- 4 hrs.)
    - - Advice to caregivers and patients
      - Get enough food and vitamins but avoid high iron foods
      - Avoid infection because it can cause hemolysis
- - - - Bleeding with severe trauma or surgery
    - - Bleeding with trauma
    - - Normal
    - - Spontaneous Bleeding without trauma
  - - - Subcutaneous and intramuscular hemorrhage are common
        
        Hemarthrosis (knees, elbows and ankles)
        
        ❖hemorrhage of GI
        
        Intracranial hemorrhage
    - - feeling of stiffness, tingling (เข็ม ทิ่ม), or ache and followed by a decrease in the ability to move the joint, warmth, redness, swelling, and severe pain
  - - - ➢Hx of family
      - ➢Hx of bleeding
      - ➢ level of Factor VIII, IX ➢ Lab
      - aPTT (Activated Partial Thromboplastin Time) เพื่อดูกํารท้ํางําน ของ factor ทุกตัวยกเว้น F7
      - -prolong VCT (Venous Clotting Time)
  - - - Desmopressin : DDAVP) 0.3-0.4 mcg/kg (increase factor VIII)→in mild symptom of
        hemophilia
      - Corticosteroids: prednisolone 1-2 mg/kg/day 3-5 วัน :
        hematuria or acute hemarthrosis
      - Fresh frozen plasma (FFP) : contain all types of coagulation factors
        
        Cryoprecipitate: Prepared from FFP contains factor VIII & fibrinogen
        
        -factor VIII concentrate 1 unit/kg เพิ่ม factor VIII ร้อยละ 2 -factor IX concentrate 1 unit/kg เพิ่ม factor IX ร้อยละ 1
      - NSAIDS: Ibuprofen avoid aspirin (inhibit platelet
        function)
    - - I-Ice
      - C-Compression
      - R-Rest
      - E-Elevation
  - - - Observe signs of bleeding such as bleeding spots, bloody stools. consciousness, joint pain
        
        2.Avoid activities that may cause bleeding, such as hard toys and organize a harmless environment.
        
        Bed rest and raise the bed guard
        
        Avoid brushing teeth if there is blood in your mouth.
        
        Avoid inject intramuscularly or subcutaneous injection.
        
        Provided blood components or DDAVP (desmopressin) according to the treatment plan and observed for complications.
        
        Provide primary care in case of bleeding, such as RICE technique
        
        Follow-up with laboratory tests for aPTT, VCT, factor level