Vila V, Regañón E, Aznar J, Navarro G, Salas M
Thromb Res. 1987 Mar 1;45(5):437-49. doi: 10.1016/0049-3848(87)90307-0.
A congenital dysfibrinogenemia, fibrinogen Barcelona I, was detected in a 28 year-old woman with no prior history of bleeding. The thrombin induced clotting of plasma and purified fibrinogen was much prolonged. Fibrin monomer aggregation was impaired. The abnormal fibrinogen polymerized in the presence of calcium and can be further cross-linked by factor XIIIa. The turbidity of fibrin gels obtained from fibrinogen Barcelona was much lower than normal fibrinogen. The kinetic constant Km for fibrinogen Barcelona plus normal fibrinogen gelation was similar to normal fibrinogen gelation. The release rate of fibrinopeptide A by thrombin was slower than that of normal fibrinogen. However, two mol of fibrinopeptide A was released per mol of fibrinogen in 30 min. SDS-PAGE of abnormal and normal fibrinogens and of reduced fibrinogens showed identical patterns. Sialic acid content was markedly decreased in fibrinogen Barcelona. Plasmin digestion of two fibrinogens showed identical patterns in SDS-PAGE as regards X fragment formation. The kinetics of fibrinogen degradation showed a decrease in the formation rate of D and E fragments. The fact that the patient was in threat of abortion and developing a haemorrhagic syndrome may indicate that the defect in the fibrinogen was important in the pathogenesis of haemorrhage in this patient.
在一名无出血既往史的28岁女性中检测到一种先天性纤维蛋白原异常血症——纤维蛋白原巴塞罗那I型。凝血酶诱导的血浆和纯化纤维蛋白原的凝血时间显著延长。纤维蛋白单体聚集受损。异常纤维蛋白原在钙存在的情况下聚合，并可被因子XIIIa进一步交联。从纤维蛋白原巴塞罗那获得的纤维蛋白凝胶的浊度远低于正常纤维蛋白原。纤维蛋白原巴塞罗那加正常纤维蛋白原凝胶化的动力学常数Km与正常纤维蛋白原凝胶化相似。凝血酶释放纤维蛋白肽A的速率比正常纤维蛋白原慢。然而，每摩尔纤维蛋白原在30分钟内释放两摩尔纤维蛋白肽A。异常和正常纤维蛋白原以及还原纤维蛋白原的SDS-PAGE显示出相同的模式。纤维蛋白原巴塞罗那中的唾液酸含量显著降低。两种纤维蛋白原的纤溶酶消化在SDS-PAGE中关于X片段形成显示出相同的模式。纤维蛋白原降解的动力学显示D和E片段的形成速率降低。患者面临流产威胁并出现出血综合征这一事实可能表明纤维蛋白原缺陷在该患者出血的发病机制中起重要作用。
