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
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
