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Hemophilia

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Hemophilia

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

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