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Coagulopathies in childhood (INVESTIGATION (PT - activity of factor 2, 5,…

- - - - Aquired:
        
        Purpuras
      - Inherited:
        
        Ehler Danlos Sy
        
        Collagen synth. disease
        
        Marfans Sy
        
        A.Dom, assoc. with aortic distention, long limbs and lens dislocation.
        
        Rendu Osler Weber Sy
        
        Hereditary hemorrhagic telangiectasia
    - - Thrombocytopenia
        
        Central(decr. prod)
        
        Peripheral(increased destr)
        
        Immune Thrombocytopenia(IPT)
        
        Most common in childhood.
        
        Acute, benign and self limiting in 6-8weeks.
      - Thrombocytopathy
        
        Aquired(drugs, uremia, liver disease)
        
        Inherited. Rare group of disorders.
    - - x-linked recessive
        
        Hemophilia A, Factor 8 def, 1/5000 males.
        
        Treatment: recomb. factors. 5-20% develop antibodies to the coag.factor which inhibits effect of factor admin. Desmopressin stimulate F8 prod in Hem. A.
        
        Hemophilia B, Factor 9 def, 1/30.000 males.
        
        I.M.admins, NSAIDS and aspirin must be avoided in hemophilias&vWF.
        
        vWF disease; quaility or quantity loss.
        
        Treat: Desmopressin, vWF concentrates.
      - Aquired:
        
        vit. k def.
        
        Liver disease.
- - - - Add normal plasma to a mixture of 50:50. Then analyse factors/inhibitors.