Marchi R, Lundberg U, de Bosch N B, Arocha-Piñango C L
Instituto Venezolano de Investigaciones Científicas, Caracas, Venezuela.
Blood Coagul Fibrinolysis. 1998 Nov;9(8):733-9.
Fibrinogen Caracas I is a dysfibrinogenemia with a mild bleeding diathesis and a defective wound healing. We have characterized this abnormal fibrinogen using transmission electron microscopy (TEM) in combination with turbidity and permeation studies. Turbidometric and permeability analysis showed that the abnormal fibrin had a significantly decreased mass:length ratio and fiber diameter. In addition, the permeability studies of plasma fibrin clots showed that the gel porosity of the abnormal fibrinogen was reduced. Images of the abnormal fibrin structure obtained using TEM showed that the fibers were thinner, much less branched and less ordered than normal fibers. Diminished fibrin fiber diameter and reduced fibrin gel porosity have been taken as hallmarks of thrombophilic dysfibrinogenemias. The results of the present study show that these features are not necessarily predictive of thrombophilia. Further studies performed on a larger number of dysfibrinogenemias need to be conducted in order to establish the implications of these parameters on the clinical outcome.
纤维蛋白原加拉加斯I型是一种伴有轻度出血倾向和伤口愈合缺陷的异常纤维蛋白原血症。我们已结合比浊法和渗透研究，使用透射电子显微镜（TEM）对这种异常纤维蛋白原进行了表征。比浊法和渗透性分析表明，异常纤维蛋白的质量：长度比和纤维直径显著降低。此外，血浆纤维蛋白凝块的渗透性研究表明，异常纤维蛋白原的凝胶孔隙率降低。使用TEM获得的异常纤维蛋白结构图像显示，与正常纤维相比，这些纤维更细、分支更少且排列更无序。纤维蛋白纤维直径减小和纤维蛋白凝胶孔隙率降低被视为血栓形成性异常纤维蛋白原血症的标志。本研究结果表明，这些特征不一定能预测血栓形成倾向。需要对更多数量的异常纤维蛋白原血症进行进一步研究，以确定这些参数对临床结果的影响。
