Jobin F, Vu L, Delage J M
Acta Haematol. 1978;59(2):119-27. doi: 10.1159/000207753.
Two new families of congenital dysfibrinogenemia originating from French Canada are reported. The dysfibrinogenemia in the first family is characterized by an abnormal aggregation of the fibrin monomers; the defect in the second family is due to a faulty release of fibrinopeptides during the proteolytic phase of the thrombin-fibrinogen reaction.
据报道,来自法裔加拿大的两个先天性异常纤维蛋白原血症新家族。第一个家族的异常纤维蛋白原血症的特征是纤维蛋白单体异常聚集;第二个家族的缺陷是由于在凝血酶-纤维蛋白原反应的蛋白水解阶段纤维蛋白肽释放错误。