hemophiliaDr shabeelpn
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.
   CausesHaemophilia 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 aMild : 6 – 50 % of normal factor viii levelModerate : 1 -5 % Severe : < 1% ( joint synovitis , hemophilic arthropathies , IM bleeding, hemarrhagic cyst)
HEMOPHILIA bGenetic bckgrnd , factor level, clinical    symptoms are same as HEM ADistinction – 1940 specific factor concentrate
managementSeverity , 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 safetyDDAVP (desmopressin acetate) provide transient increase in coag factor. Absence of viral risk and lower cost
ddavpDose : .3 micro gm/ kg   iv or sc prior to txResult in 2-5 fold increase in factor viii , vwf antigen For children : intranasal spray Stimulate endogenous release f viii&vwfProlongd use exhaust f viii& dimnishdhemostatcactvty..antifibrinolytcs as adjuncts to ddavp
Oral findngsPetechiae,ecchymosisGingival bleedng+enlargmntContinousbleedng…stain toothLabial frenum>tongue>buccal mucosaBleedng increase…traumaAcute/chronic hemarthrosis
             Treatment scenario                        OMFS-hypnosis,diazepam therapyL.A---block 20-30%,,hematoma frmnOral Endotrachealprfrd over nasal50-100% preopfactr levelAddtnl Post op  factor maintainence3-5 days aftr surgery—fibrinolysisLocal hemostatic agents—pressure,packs,sutures,top thrombin ,stents,fibrin glues
perioHyperemic ging:spont & Induced bleedngPeriodontitis ---extractionProbng,supraging scalingCareful subging—replacement therapySeverely inflmd& swollen---chlorhex/gross debridement/hand instrument….ging shrinkageDeep scaling-quadrant –reduce area of bleedLocal hemostats /antifibrinolyticsBlock—raise to 30% of normalPeriosrgry warrants 50% circltngfactor+aflytcs
conserv & endoRubber damRct best in place of xtrn—not beyond apex
pedo & orthoFactor conc----extractionThorough scaling-reduce inflmn-xtract –pressure Bleeding from minor cuts—respond to local pressureUse extra oral force+short duration
  Now be ready        to  treat                   A         Hemophiliac
Let’s identify 9 faces in this?????????!!!!!!!!

Hemophilia

  • 1.
  • 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.
    CausesHaemophilia 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 aMild :6 – 50 % of normal factor viii levelModerate : 1 -5 % Severe : < 1% ( joint synovitis , hemophilic arthropathies , IM bleeding, hemarrhagic cyst)
  • 5.
    HEMOPHILIA bGenetic bckgrnd, factor level, clinical symptoms are same as HEM ADistinction – 1940 specific factor concentrate
  • 7.
    managementSeverity , typeand 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 safetyDDAVP (desmopressin acetate) provide transient increase in coag factor. Absence of viral risk and lower cost
  • 8.
    ddavpDose : .3micro gm/ kg iv or sc prior to txResult in 2-5 fold increase in factor viii , vwf antigen For children : intranasal spray Stimulate endogenous release f viii&vwfProlongd use exhaust f viii& dimnishdhemostatcactvty..antifibrinolytcs as adjuncts to ddavp
  • 9.
    Oral findngsPetechiae,ecchymosisGingival bleedng+enlargmntContinousbleedng…staintoothLabial frenum>tongue>buccal mucosaBleedng increase…traumaAcute/chronic hemarthrosis
  • 11.
    Treatment scenario OMFS-hypnosis,diazepam therapyL.A---block 20-30%,,hematoma frmnOral Endotrachealprfrd over nasal50-100% preopfactr levelAddtnl Post op factor maintainence3-5 days aftr surgery—fibrinolysisLocal hemostatic agents—pressure,packs,sutures,top thrombin ,stents,fibrin glues
  • 12.
    perioHyperemic ging:spont &Induced bleedngPeriodontitis ---extractionProbng,supraging scalingCareful subging—replacement therapySeverely inflmd& swollen---chlorhex/gross debridement/hand instrument….ging shrinkageDeep scaling-quadrant –reduce area of bleedLocal hemostats /antifibrinolyticsBlock—raise to 30% of normalPeriosrgry warrants 50% circltngfactor+aflytcs
  • 13.
    conserv & endoRubberdamRct best in place of xtrn—not beyond apex
  • 14.
    pedo & orthoFactorconc----extractionThorough scaling-reduce inflmn-xtract –pressure Bleeding from minor cuts—respond to local pressureUse extra oral force+short duration
  • 15.
    Nowbe ready to treat A Hemophiliac
  • 16.
    Let’s identify 9faces in this?????????!!!!!!!!