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HEM
OP
HIL
IA Dr shabeel pn
A group of hereditary genetic 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.
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.
HEMOPHILIA A Mild : 6 – 50 % of normal factor viii level Moderate : 1 -5 % Severe : < 1% ( joint synovitis ,
hemophilic arthropathies , IM bleeding, hemarrhagic cyst)
HEMOPHILIA B
Genetic bckgrnd , factor level, clinical
symptoms are same as HEM A
Distinction – 1940
specific factor concentrate
MANAGEMENT Severity , type and site of hemorrhage Commercially prepared factor viii & ix
complex conc ,desmopressin acetate , cryo ppt, 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
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& dimnishd
hemostatc actvty..antifibrinolytcs as adjuncts to ddavp
ORAL FINDNGS Petechiae,ecchymosis Gingival bleedng+enlargmnt Continous bleedng…stain tooth Labial frenum>tongue>buccal mucosa Bleedng increase…trauma Acute/chronic hemarthrosis
TREATMENT SCENARIO OMFS -hypnosis,diazepam therapy L.A---block 20-30%,,hematoma frmn Oral Endotracheal prfrd over nasal 50-100% preop factr level Addtnl Post op factor maintainence 3-5 days aftr surgery—fibrinolysis Local hemostatic agents—
pressure,packs,sutures,top thrombin ,stents,fibrin glues
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 Perio srgry warrants 50% circltng
factor+aflytcs
CONSERV & ENDO Rubber dam Rct best in place of xtrn—not beyond
apex
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
NOW BE READY TO TREAT A
HEMOPHILIAC
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LET’S IDENTIFY 9 FACES IN THIS?????????!!!!!!!!