Rocha E, Lasierra J, Narvaiza M J, Vilades E, Palacios E, Fernandez J
Ric Clin Lab. 1984 Oct-Dec;14(4):663-72. doi: 10.1007/BF02906307.
An abnormal fibrinogen was discovered in a 9-year-old male subject without history of hemorrhagic diathesis. Coagulation time, prothrombin time and Reptilase time were prolonged. The thrombin time was corrected using increasing concentrations of normal plasma and bovine thrombin; there was a partial correction at pH 6.5 and ionic strength 0.05. A study of the family showed that the mother and a brother of the propositus presented the same abnormalities. Analysis of the purified fibrinogen showed normal fibrinopeptide release and normal levels of sialic acid and hexosamines. However, coagulation index, polymerization of fibrin monomers, isoelectric point and sedimentation coefficient were abnormal. In view of the abnormalities described and by comparison with the data reported in the literature, we believe that this should be considered a new variant of the fibrinogen molecule and we have designated it 'fibrinogen Logroño'.
在一名无出血素质病史的9岁男性受试者中发现了一种异常纤维蛋白原。凝血时间、凝血酶原时间和爬虫酶时间延长。使用浓度递增的正常血浆和牛凝血酶校正凝血酶时间;在pH 6.5和离子强度0.05时出现部分校正。对该家族的研究表明,先证者的母亲和一个兄弟有相同的异常情况。对纯化纤维蛋白原的分析显示纤维蛋白肽释放正常,唾液酸和己糖胺水平正常。然而,凝血指数、纤维蛋白单体聚合、等电点和沉降系数均异常。鉴于所描述的异常情况并与文献报道的数据进行比较,我们认为这应被视为纤维蛋白原分子的一种新变体,我们将其命名为“洛格罗尼奥纤维蛋白原”。