What is the definition of Congenital Fibrinogen Deficiency?
Congenital fibrinogen deficiencies are very rare genetic disorders of blood coagulation. There are several types of congenital fibrinogen deficiencies, which are characterized by the amount of decreased quantity or quality of circulating fibrinogen in the blood, such as afibrinogenemia (lack of fibrinogen), hypofibrinogenemia (low fibrinogen), and dysfibrinogenemia (abnormal fibrinogen). Fibrinogen is a protein that creates blood clots and stops bleeding. Congenital fibrinogen deficiencies can range from mild to severe.
What are the symptoms for Congenital Fibrinogen Deficiency?
Symptoms of congenital fibrinogen deficiencies depend on the type.
Symptoms of afibrinogenemia (lack of fibrinogen) may include umbilical cord bleeding, nosebleed, bruising easily, bleeding in the mucous membranes, bleeding in the joints, gastrointestinal bleeding, excessive menstrual bleeding, miscarriage, excessive bleeding after trauma or surgery, and intracranial bleeding.
Symptoms of hypofibrinogenemia (low fibrinogen) may include milder bleeding after trauma or surgery.
Dysfibrinogenemia (abnormal fibrinogen) may not have any symptoms or may include abnormal bleeding or clotting.
What are the current treatments for Congenital Fibrinogen Deficiency?
Treatment for congenital fibrinogen deficiencies involves the administration of fresh frozen plasma, cryoprecipitate (fibrinogen and blood clotting factors), or fibrinogen (RiaSTAP).
Individuals with congenital fibrinogen deficiencies should also receive the hepatitis B vaccine to reduce the risk of acquiring the disease through transfusions.