hemophilia

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?????????!!!!!!!!