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 ?
Predisposing risk factors associated with patient (assign score 1 unless otherwise noted) Clinical setting Inherited Acquired (score (score 3) 3) Age 40-60 years Factor V Lupus Leiden/ anticoagulant activated Age > 60 years protein C Antiphospholipid (score 2) resistance antibodies History DVT/PE Antithrombin Myeloproliferative (score 3) III deficiency disorders Pregnancy or Dysfibrinogenaemia Disorders of postpartum plasminogen and (< 1 month) plasmin activation Malignancy Homocysteinaemia Heparin (score 2) thrombocytopenia Varicose 20210A Hyperviscosity veins prothrombin syndromes mutation Inflammatory Homocysteinaemia bowel disease Obesity (> 20% ideal body weight) Combined oral contraceptive/HRT Total additional predisposing risk factors associated with patient: Step 3.
The test therefore only detects disturbances in the final stages of coagulation, especially dysfibrinogenaemia or the presence of thrombin inhibitors (Koch and Biber, 2007).
Fibrinogen Milano VI: a heterozygous dysfibrinogenaemia (Aa16 Arg-His) with bleeding tendency.