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Royal Disease
“Hemophilia”


                Andal, Katrina
                    Joie R.
                    DMD2D
Definition
       Hemophilia is a coagulation
        disorder arising from a genetic defect of
        the X chromosome.
       Depending on the degree of the disorder
        in the affected individual, uncontrolled
        bleeding may occur spontaneously with
        no known initiating event, or occur
        after specific events such as surgery,
        dental procedures, immunizations, or
        injury.
       Any of several hereditary blood-
        coagulation disorders in which the blood
        fails to clot normally because of a
        deficiency or abnormality of one of the
        clotting factors.
       Hemophilia, a recessive trait associated
        with the X-chromosome, is manifested
        almost exclusively in males.
Signs and Symptoms
   External bleeding may include:

      Bleeding in the mouth from a cut or bite or from cutting
       or losing a tooth.

      Nosebleeds for no obvious reason.

      Heavy bleeding from a minor cut.

      Bleeding from a cut that resumes after stopping for a
       short time.



   Internal bleeding may include:

      Blood in the urine (from bleeding in the kidneys or
       bladder).

      Blood in the stool (from bleeding in the intestines or
       stomach).

      Large bruises (from bleeding into the large muscles of the
       body).
Signs and Symptoms
   Bleeding in the Joints

      Bleeding in the knees, elbows, or other joints is another
       common form of internal bleeding in people who have
       hemophilia.

      The bleeding causes tightness in the joint with no real pain
       or any visible signs of bleeding. The joint then becomes
       swollen, hot to touch, and painful to bend.

   Bleeding in the Brain

      Long-lasting, painful headaches or neck pain or stiffness

      Repeated vomiting

      Sleepiness or changes in behavior

      Sudden weakness or clumsiness of the arms or legs or
       problems walking

      Double vision

      Convulsions or seizures
Dental Correlation
      Tooth extraction in patients with hereditary
       hemorrhagic disorders can lead to severe bleeding
       , often life threatening. The association of general
       anti hemorrhagic treatment with local haemostatic
       measures for bleeding control is mandatory with
       these patients. The therapy protocol is the result of
       an interdisciplinary collaboration, between a
       hematologist and an oral surgeon, in order to
       control and to reduce the severity of postoperative
       bleeding.


      Controlling haemorrhage by using periodontal
       cement packing in patients with bleeding disorders
       is beneficial and cost effective in dental extraction.
       Local techniques still requires specific replacement
       therapy.The adopted protocol produced a reliable
       outcome of dental extractions on an out -patient
       basis, with postsurgical comfort due to a
       combination of systemic treatment and local
       improved hemostasic measures.
Classifications
     Hemophilia A           Hemophilia B        Hemophilia C
      It is the most        It is the second    It is a mild form
     common type of         most common          of hemophilia.
       hemophilia.        type of hemophilia.
                              (Moderate)
         (Severe)                                   (Mild)
    It is also known as    It was originally     Deficiency of
            factor        named “Christmas        factor XI.
    VIII deficiency or         disease”.
    classic hemophilia.
8. andal   royal disease

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8. andal royal disease

  • 1. Royal Disease “Hemophilia” Andal, Katrina Joie R. DMD2D
  • 2. Definition  Hemophilia is a coagulation disorder arising from a genetic defect of the X chromosome.  Depending on the degree of the disorder in the affected individual, uncontrolled bleeding may occur spontaneously with no known initiating event, or occur after specific events such as surgery, dental procedures, immunizations, or injury.  Any of several hereditary blood- coagulation disorders in which the blood fails to clot normally because of a deficiency or abnormality of one of the clotting factors.  Hemophilia, a recessive trait associated with the X-chromosome, is manifested almost exclusively in males.
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  • 4. Signs and Symptoms External bleeding may include:  Bleeding in the mouth from a cut or bite or from cutting or losing a tooth.  Nosebleeds for no obvious reason.  Heavy bleeding from a minor cut.  Bleeding from a cut that resumes after stopping for a short time. Internal bleeding may include:  Blood in the urine (from bleeding in the kidneys or bladder).  Blood in the stool (from bleeding in the intestines or stomach).  Large bruises (from bleeding into the large muscles of the body).
  • 5. Signs and Symptoms Bleeding in the Joints  Bleeding in the knees, elbows, or other joints is another common form of internal bleeding in people who have hemophilia.  The bleeding causes tightness in the joint with no real pain or any visible signs of bleeding. The joint then becomes swollen, hot to touch, and painful to bend. Bleeding in the Brain  Long-lasting, painful headaches or neck pain or stiffness  Repeated vomiting  Sleepiness or changes in behavior  Sudden weakness or clumsiness of the arms or legs or problems walking  Double vision  Convulsions or seizures
  • 6. Dental Correlation  Tooth extraction in patients with hereditary hemorrhagic disorders can lead to severe bleeding , often life threatening. The association of general anti hemorrhagic treatment with local haemostatic measures for bleeding control is mandatory with these patients. The therapy protocol is the result of an interdisciplinary collaboration, between a hematologist and an oral surgeon, in order to control and to reduce the severity of postoperative bleeding.  Controlling haemorrhage by using periodontal cement packing in patients with bleeding disorders is beneficial and cost effective in dental extraction. Local techniques still requires specific replacement therapy.The adopted protocol produced a reliable outcome of dental extractions on an out -patient basis, with postsurgical comfort due to a combination of systemic treatment and local improved hemostasic measures.
  • 7. Classifications Hemophilia A Hemophilia B Hemophilia C It is the most It is the second It is a mild form common type of most common of hemophilia. hemophilia. type of hemophilia. (Moderate) (Severe) (Mild) It is also known as It was originally Deficiency of factor named “Christmas factor XI. VIII deficiency or disease”. classic hemophilia.