Name
AFIBRINOGENEMIA
DESCRIPTION
DETAIL
OTHER TESTS - PLASMA FIBRINOGEN LEVELS - DECREASED PT, PTT & THROMBIN TIME - INCREASED
TYPENOTES
Medical Care Hemorrhage: For patients with clinical bleeding associated with afibrinogenemia or dysfibrinogenemia, replacement of fibrinogen to a level of more than 0.8 g/L is usually adequate to maintain hemostasis, although levels greater than 1 g/L have been recommended for CNS hemorrhage. Plasma-derived fibrinogen concentrates have the advantage of virus inactivation. The usual starting dose for adults is 1-2 g intravenously administered. The pediatric dose is 30-100 mg/kg intravenously administered, depending on the severity and site of bleeding. Cryoprecipitate has been used as a source of fibrinogen; each bag of cryoprecipitate contains 100-250 mg of fibrinogen. Thrombosis: Patients presenting with thrombosis associated with dysfibrinogenemia should receive anticoagulation therapy. The duration of therapy has not been established for this particular group of patients; the decision depends on the clinical situation and the presence of other contributing factors. If the patient has had multiple thromboembolic events, a single life-threatening event, or has additional inherited risk factors, protracted anticoagulation therapy is recommended. Spontaneous abortion: Recurrent spontaneous abortion may be prevented by routine prophylaxis with fibrinogen concentrates starting early in pregnancy. 2. Cryoprecipitate : Can be used when fibrinogen concentrates are not available. Unlike fibrinogen concentrates, it does not undergo virus inactivation. The precipitate formed when fresh frozen plasma (FFP) is slowly thawed. It contains factor VIII, factor XIII, fibrinogen, von Willebrand factor (vWF), and fibronectin. Each bag provides 100-250 mg fibrinogen. DRUG TREATMENT: 1. Antifibrinolytics : These are useful in conjunction with fibrinogen replacement for the treatment of mucosal bleeding, particularly bleeding involving the oronasopharynx. Inhibition of local fibrinolysis allows maintenance of the clot and decreases the frequency of rebleeding. - AMINOCAPROIC ACID (HAMOSTAT) : Lysine analogue that inhibits fibrinolysis by blocking binding of plasmin or plasminogen activators to lysine residues on fibrin. - TRANEXAMIC ACID ( CAPILOC, CAPITRAX ) : Alternative to aminocaproic acid. Inhibits fibrinolysis by displacing plasminogen from fibrin. Surgical Care To prevent excessive bleeding during surgical procedures, prophylactic treatment to raise fibrinogen levels to 1.0-1.5 g/L during the procedure is recommended. Replacement should be continued for 4-14 days following surgery, depending on the nature of the surgical procedure and time to complete healing.
RELATED DISEASE
Not Available Disease
DISEASE
INVESTIGATION
PROTHROMBIN TIME, PARTIAL THROMBOPLASTIN TIME, THROMBIN TIME, COMPLETE BLOOD COUNT