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Hemophilia

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  • 1. hemophilia
    Dr shabeelpn
  • 2. A group of hereditarygenetic disorders  that impair the body's ability to control blood clotting or coagulation .
    The effects of this sex-linked, X chromosome disorder are manifested almost entirely in males
     Females are almost exclusively carriers of the disorder, and may have inherited it from either their mother or father.
  • 3. Causes
    Haemophilia A is an X-linked genetic disorder involving a lack of functional clotting Factor VIII and represents 90% of hemophilia cases.[3]
    Haemophilia B is an X-linked genetic disorder involving a lack of functional clotting Factor IX It is more severe but less common than Hemophilia A.
    Haemophilia C is an autosomal recessive genetic disorder involving a lack of functional clotting Factor XI.
  • 4. HEMOPHILIA a
    Mild : 6 – 50 % of normal factor viii level
    Moderate : 1 -5 %
    Severe : < 1% ( joint synovitis , hemophilic arthropathies , IM bleeding, hemarrhagic cyst)
  • 5. HEMOPHILIA b
    Genetic bckgrnd , factor level, clinical
    symptoms are same as HEM A
    Distinction – 1940
    specific factor concentrate
  • 6.
  • 7. management
    Severity , type and site of hemorrhage
    Commercially prepared factor viii & ix complex conc ,desmopressin acetate , cryoppt, ffp….
    Chance of viral transmission (dry heat treated concentrate)
    High purity factor viii products (monoclonal antibody purification technique) - improved viral safety
    DDAVP (desmopressin acetate) provide transient increase in coag factor. Absence of viral risk and lower cost
  • 8. ddavp
    Dose : .3 micro gm/ kg iv or sc prior to tx
    Result in 2-5 fold increase in factor viii , vwf antigen
    For children : intranasal spray
    Stimulate endogenous release f viii&vwf
    Prolongd use exhaust f viii& dimnishdhemostatcactvty..antifibrinolytcs as adjuncts to ddavp
  • 9. Oral findngs
    Petechiae,ecchymosis
    Gingival bleedng+enlargmnt
    Continousbleedng…stain tooth
    Labial frenum>tongue>buccal mucosa
    Bleedng increase…trauma
    Acute/chronic hemarthrosis
  • 10.
  • 11. Treatment scenario OMFS
    -hypnosis,diazepam therapy
    L.A---block 20-30%,,hematoma frmn
    Oral Endotrachealprfrd over nasal
    50-100% preopfactr level
    Addtnl Post op factor maintainence
    3-5 days aftr surgery—fibrinolysis
    Local hemostatic agents—pressure,packs,sutures,top thrombin ,stents,fibrin glues
  • 12. perio
    Hyperemic ging:spont & Induced bleedng
    Periodontitis ---extraction
    Probng,supraging scaling
    Careful subging—replacement therapy
    Severely inflmd& swollen---chlorhex/gross debridement/hand instrument….ging shrinkage
    Deep scaling-quadrant –reduce area of bleed
    Local hemostats /antifibrinolytics
    Block—raise to 30% of normal
    Periosrgry warrants 50% circltngfactor+aflytcs
  • 13. conserv & endo
    Rubber dam
    Rct best in place of xtrn—not beyond apex
  • 14. pedo & ortho
    Factor conc----extraction
    Thorough scaling-reduce inflmn-xtract –pressure
    Bleeding from minor cuts—respond to local pressure
    Use extra oral force+short duration
  • 15. Now be ready to treat A Hemophiliac
  • 16. Let’s identify 9 faces in this?????????!!!!!!!!