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Sickle Cell (Variants (HbSC: not disease ↓Hgb, but no Sx 1 gene, trait…

- - - - Acute
        
        Acute chest
        
        Exchange transfusion
        
        urgent
        
        Priapism :eggplant:
        
        drainage of penis before → exchange transfusion
      - Chronic:
        
        auto infarct spleen
        
        risk of encapsulated organisms
        
        PPx penicillin :pencil2: until 5y
        
        Pneumococcal Vaccine
        
        bone ischemia
        
        ↑OM
        
        always S. Aureus
        
        if Salmonella → SCD
        
        Avascular Necrosis of Hip
        
        can be any bone
        
        but hip we care about
        * conservative management (crutches)
        
        then Sx :hocho:
    - - :explode: →UB released into blood →liver conjugation → stool 💩
        
        always some jaundice at baseline
        
        Bili 1-2 (↑ than nl)
        
        pigmented gallstones → cholecystectomy
        
        anemia
        
        obtain CBC & bili for baseline
        
        Anemia → poor O2 carrying cap. → EPO in kidneys
        
        elevated baseline reticulocyte
        
        want to know this
        
        Tx:
        
        folate
        
        Fe
        
        Deferoxamine
        
        Fe overload from pRBC transfusions