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Hemophilia

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  • 1. Dr/ Hytham NafadyDr/ Hytham NafadyHemophiliaHemophilia
  • 2. HemophiliaX-linked recessive disorder characterized by deficiency ofcoagulation factor.HemophiliaHemophilia AHemophilia B(Chrismas disease)Deficiency ofcoagulation factorVIIIDeficiency ofcoagulation factorIX
  • 3. X linked recessiveMales ♂ Female ♀Patient Carrier
  • 4. Queen Victoria
  • 5. The RomanovsRussia’s last royal familyHemophilia = Royal disease
  • 6. Bleeding:Intraarticular,Intramuscular,Intraosseous,Subperiosteal.Life threatening hemorrhage:Intracranial.Throacic.Abdominal.
  • 7. HemophiliaHemophilicarthropathyHemophilicpseudotumor
  • 8. Hemophilic arthropathyEtiology:Repeated hemarthrosis.Pathology:Repeated hemarthrosis chronic synoviitis, synovial hemo-siderosis synovial proliferation & pannus formation cartilage destruction bone erosions and subcortical cysts.
  • 9. Hemophilic arthropathyDemographics:Age: 1st or 2nd decade.Sex: it is a disease of males.
  • 10. Hemophilic arthropathyClinical presentation:3 types of arthropathy
  • 11. Acute arthropathyRedhottenderjoint swelling.FeverLeukocystosis.DDx: septic arthritis
  • 12. Subacute arthropathySubacute arthropathyLimitation of joint motion & muscle wasting
  • 13. Chronic arthropathyChronic arthropathySever joint contracture.DDx: juvenile rheumatoid arthritis.
  • 14. Hemophilic arthropathyHemophilic arthropathyLocation:Knee  elbow  ankle  hip  shoulder.Distribution:Unilateral or bilateral asymmetrical (DDx with JRA).
  • 15. Radiological manifestiation ofhemophilic arthropathyDense joint effusion HemoarthrosisJuxta-articular osteoporosis. Hyperemia of theepiphysisEnlargement of the epiphysis Hyperemia of theepiphysisDiffuse narrowing of the jointspaceCartilage destructionIrregular articular surfaces Bone erosionSubchondral sclerosis, cysticchanges and marginalosteophytic lipping.Premature 2ryosteoarthritis.
  • 16. MRIMRILow signal intensity of the hypertrophied synovium on allpulse sequences due to siderotic synoviitis.
  • 17. KneeKnee16 +Bulbous femoral condyles with flattened inferior surface.Slanting of the tibial plateau.Widening of the intercondylar notch (due to repeatedhemorrhage in the attachments of the cruciate ligaments).Squaring of the lower pole of the patella.
  • 18. ElbowElbow16 +Widening of the trochlear & ulnar notches.Enlargement of the radial head.
  • 19. Dense joint effusionDense joint effusion (sail sign)
  • 20. AnkleAnkle16 +Talar tilt: medial slanting of the tibiotalar joint withalteration of ankle mortise.
  • 21. MRIMRI1. Hemorrhagic joint effusion.2. Synovial hemosiderin deposition (low signal intensity onall pulse sequences with blooming artifact on gradientecho images).3. Destruction of the articular surface.4. Bone marrow edema of the epiphysis.
  • 22. Complications of hemophilicComplications of hemophilicarthropathy:arthropathy:1. Premature 2ry osteoarthritis.2. Premature fusion of the epiphysis with limb shortening.3. Avascular bone necrosis.4. Septic arthritis.
  • 23. DD of hemophiliaJRANeuromuscular disorders.Neuromuscular disordresimmobilizationSynovial atrophy Vascular stasisEpiphysealenlargementDecreasedcartilage nutrition
  • 24. ParaplegiaParaplegia
  • 25. Poliomyelitis
  • 26. Hemophilic pseudotumorHemophilic pseudotumorRepeated intramusular, subperiosteal or intraosseoushemorrhage.
  • 27. Pathology:Subperiosteal hemorrhage  destruction of the underlyingcortex & medulla & elevation of the overlying periosteum.Intraosseous hemorrhage  intraosseous lytic lesion.
  • 28. Location:Iliac wings.Femoral diaphysis.Calcaneus.
  • 29. Special CaseFemale patient with Turnersyndrome (XO)Inherited hemophilia B from herfather.X ray ankle shows talar tilt.
  • 30. Bleeding into kidney
  • 31. Intracranial hemorrhageIntracranial hemorrhage1/3 of deaths.