Hemophilia

*Hemophilia A is X_linked recessive disease so,it affects males and females are carriers *Cause: Deficiency of factor VIII *Clinical pictures: _ bleeding from umbilical stump -CNS hemorrhage in neonates -ecchymosis either spontaneously or after minor trauma -Excessive bleeding after minor surgeries like tooth extraction or cicumcision -Intramuscular hematomas -Orificial bleeding (hematuria..epistaxis..bleeding per gum..Git bleeding) -Hemoarthrosis and may lead to ankylosis *Investigations" -prolonged coagulation time -prolonged APTT -Deficient factor VIII by specific factor assay -Radiologic examination of joints with hemarthrosis -prenatal diagnosis by amniocentesis *Treatment: -Avoid trauma, IM injection -Cold compresses and application of thrombin gel on small wounds -cold packs and physiotherapy for affected joints -The main treatment is replacement therapy to increase level of factor VIII in plasma by using fresh frozen plasma or cryoprecepitate ir factor VIII concentrate