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Congenital Afibrinogenemia Causes and Risks:
This rare disease is caused by an autosomal recessive gene. There may be either a lack of fibrinogen or defect in the function of fibrinogen (a substance necessary for clotting of blood). It can occur in both sexes.
Symptoms:
Note: This condition usually does not produce severe, spontaneous bleeding.
Signs and Tests:
If a tendency to bleed is suspected, laboratory tests are performed to determine the type and extent.
Tests include:
Treatment:
Treatment is only necessary if there is severe bleeding or in preparation for surgery. Patients may be transfused with plasma (the liquid portion of the blood), concentrated fibrinogen , or cryoprecipitate (a blood product containing concentrated fibrinogen). Individuals with this condition should be immunized with the hepatitis B vaccine because of the increased risk of developing hepatitis due to transfusion of blood products.
Prognosis:
The prognosis is good when patients seek proper treatment.
Complications:
Complications usually only occur with trauma:
Call Your Healthcare Provider:
Call your health care provider or seek emergency care if excessive bleeding occurs.
Notify your surgeon before you have surgery if you know or suspect you have a blood disorder.
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