<症例>フィブリノーゲン異常症の1例

概要

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A dysfibrinogenemia was found in an eleven-year-old male patient with infectious mononucleosis through routine blood clotting tests. Clotting times were prolonged. The plasma fibrinogen level was shown to be normal by the turbidity method but to be markedly reduced by the thrombin method. Such discrepancy led us to investigate fibrinogen further. Other tests such as coagulation factors and fibrinopeptides release were normal. In family studies the propositus' brother had similar abnormalities. Fibrinogen purified from the patient plasma was similar to normal fibrinogen in the following respects ; SDS-PAGE analysis, Ouchterlony method, immunoelectrophoresis method and the affinity of radiolabeled urokinase and tissue-type plasminogen activator to fibrin clot. In contrast, this fibrinogen showed a defect in polymerization of fibrin monomer and of whole plasma. In addition, differences in charge of the molecule were revealed by disc electrophoresis and iso-electrophoresis ; the isoelectric point of normal fibrinogen was PI 5.65-5.60 and 5.50,and that of patient's fibrinogen was PI 5.60-5.40. Furthermore fibrinogens from the patient and normal control plasma were analyzed by two-dimensional electrophoresis of O'Farrell ; the β chains were different in charge, but identical in M.W. These results suggest that the patients fibrinogen may have molecular abnormality.
近畿大学の論文
1986-06-25