dysfibrinogenemia


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dys·fi·brin·o·ge·ne·mi·a

(dis'fī-brin'ō-jĕ-nē'mē-ă), [MIM*134820]
An autosomal dominant disorder of qualitatively abnormal fibrinogens of various types; each type is named for the city in which the abnormal fibrinogen was discovered. Examples include: 1) Amsterdam, Bethesda II, Cleveland, Los Angeles, Saint Louis, Zurich I and II: major defect, aggregation of fibrin monomers; thrombin time prolonged; inhibitory effect on normal clotting; asymptomatic; 2) Bethesda I and Detroit: major defect, fibrinopeptide release; thrombin time prolonged; inhibitory effect on normal clotting; abnormal bleeding; 3) Baltimore: major defect, fibrinopeptide release; thrombin time prolonged; no inhibitory effect on normal clotting; bleeding and thrombosis; 4) Leuven: major defect, questionable aggregation of fibrin monomers; thrombin time prolonged; slight inhibitory effect on normal clotting; abnormal bleeding; 5) Metz: major defect unreported; thrombin time infinite; effect on normal clotting unreported; abnormal bleeding; 6) Nancy: major defect, aggregation of fibrin monomers; thrombin time prolonged; slight inhibitory effect on normal clotting; asymptomatic; 7) Oklahoma: major defect unreported; thrombin time normal; no effect on normal clotting; abnormal bleeding; 8) Oslo: major defect unreported; thrombin time shortened; effect on normal clotting unreported; abnormal thrombosis; 9) Parma: major defect unreported; thrombin time infinite; no inhibitory effect on normal clotting; abnormal bleeding; 10) Paris I: major defect unreported; thrombin time infinite; inhibitory effect on normal clotting; asymptomatic; 11) Paris II: major defect unreported; thrombin time prolonged; inhibitory effect on normal clotting; asymptomatic; 12) Troyes: major defect unreported; thrombin time prolonged; effect on normal clotting unreported; asymptomatic; 13) Vancouver: major defect unreported; thrombin time prolonged; no effect on normal clotting; abnormal bleeding; 14) Wiesbaden: major defect, aggregation of fibrin monomers; thrombin time prolonged; inhibitory effect on normal clotting; bleeding and thrombosis.

dysfibrinogenemia

/dys·fi·brin·o·ge·ne·mia/ (dis-fi-brin″o-jĕ-ne´me-ah) the presence in the blood of abnormal fibrinogen.

dysfibrinogenemia

A group of qualitative, usually AD, fibrinogen defects ranging in severity from innocuous to hemorrhagic diathesis; most are asymptomatic and detected by presurgical screens, given the abnormalities in coagulation parameters; these subjects suffer frequent spontaneous abortion, bleeding, poor wound healing, and thrombosis Lab Normal fibrinogen and clotting times; ↑ PT, ↑ thrombin time, ↑ reptilase time. See Fibrinogen.

dys·fi·brin·o·ge·ne·mi·a

(dis'fī-brin'ō-jĕ-nē'mē-ă)
An autosomal dominant disorder of qualitatively abnormal fibrinogens of various types, resulting in abnormalities of coagulation tests (bleeding time, clotting time, thrombin time); symptoms vary from none to abnormal bleeding and excessive clotting.
Synonym(s): dysfibrinogenaemia.

dysfibrinogenemia

the presence of abnormal fibrinogens in the body. An inherited dysfibrinogenemia occurs in humans and has been reported in a collie dog.
References in periodicals archive ?
A 49-year-old African-American female with history of hepatitis C and hypertension was investigated for a possible dysfibrinogenemia when an abnormal thrombin time (TT) and reptilase time (RT) were reported by the laboratory.
8,9) Other risk factors reported to be common to both arterial and venous thrombosis include the presence of antiphospholipid antibodies, dysfibrinogenemia, hyperhomocysteinemia, and elevated levels of fibrinogen, lipoprotein (a) and factor VIII.
Drugs (see Table 4) Wiskott-Aldrich syndrome, May-Hegglin anomaly Systemic illness (liver disease, myeloproliferative disorder, infection) Platelet dysfunction Drugs Myeloproliferative disorder Von Willebrand disease Thrombocytopenia Absent Radius (TAR) syndrome Bernard-Soulier Glanzmann thrombasthenia Storage disease Renal disease Coagulation protein disorders Hemophilia (factor VIII, IX, XI deficiency) Von Willebrand disease Dysfibrinogenemia Factor XIII Plasmin or plasminogen deficiency or inhibitor Circulating anticoagulant or inhibitor Vitamin K deficiency, warfarin therapy Systemic illnesses (liver disease, amyloidosis) Table 2.
Patients with a history of afibrinogenemia, dysfibrinogenemia, or hypofibrinogenemia were excluded from this study.
Other inherited thrombophilic disorders include heparin cofactor II deficiency, plasminogen deficiency, dysfibrinogenemia, factor XII deficiency, and increased factor VIII coagulant activity.
Predisposing Risk Factors for Venous Thromboembolism[1,6,25] Primary Risk Factors Antithrombin III deficiency Deficit of Factor XII Dysfibrinogenemia Plasminogen disorders Protein C and S deficiency Secondary Risk Factors Age over 40 years Cerebrovascular disease or congestive heart failure Immobilization (confinement in bed, postoperative state) Inflammatory bowel disease Leg edema, ulcers, varicose veins, venous stasis Long bone or pelvic fracture Lupus anticoagulant Malignancy Nephrotic syndrome Obesity Polycythemia rubra vera Pregnancy Prior thromboebolism Sepsis
Acquired deficiency is most common and causes include disseminated intravascular coagulation, (4) liver disease, (5) hemodilution, (6) and acquired dysfibrinogenemia.
Coagulation tests including for lupus antico-agulant, dysfibrinogenemia, resistance to activated protein C, deficiencies of protein S, C or antithrom-bin, and mutations of factor V were all detected as normal.
These assays are recommended for detecting hypofibrinogenemia or dysfibrinogenemia and for evaluating the risk of bleeding (9).
Severe end-stage bleeding reflects markedly decreased coagulation factor production, excessive fibrinolysis, dysfibrinogenemia, thrombocytopenia, and occasionally DIC.
It is affected by abnormal fibrinogen, dysfibrinogenemia and the presence of circulating anticoagulants including heparin and FDPs.