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在一名患有与先天性低纤维蛋白原血症(纤维蛋白原马尔默型)相关的复发性血栓形成的患者中观察到纤维连接蛋白水平升高的发作。

Episodes of increased fibronectin level observed in a patient suffering from recurrent thrombosis related to congenital hypodysfibrinogenaemia (fibrinogen Malmoe).

作者信息

Soria J, Soria C, Hedner U, Nilsson I M, Bergqvist D, Samama M

出版信息

Br J Haematol. 1985 Dec;61(4):727-38. doi: 10.1111/j.1365-2141.1985.tb02887.x.

Abstract

A study has been conducted in a Swedish patient with severe thrombotic disease and repeated miscarriages related to a hypodysfibrinogenaemia with defective thrombin binding to the abnormal fibrin. The hypodysfibrinogenaemia was found in several members of the family. The patient also had an increased concentration of fibronectin in her plasma at two different occasions. This would appear to be unrelated to the abnormal fibrinogen since a normal concentration of fibronectin has been found in her relatives presenting the same fibrinogen anomaly, and in the patient at two other times. In conclusion, the thrombotic disorder in this patient presenting a congenital hypodysfibrinogenaemia may be explained by the defective thrombin binding to fibrin.

摘要

对一名患有严重血栓性疾病且反复流产的瑞典患者进行了一项研究,该患者患有低纤维蛋白原血症,凝血酶与异常纤维蛋白的结合存在缺陷。在该家族的几名成员中发现了低纤维蛋白原血症。该患者在两个不同时间点血浆中的纤连蛋白浓度也有所升高。这似乎与异常纤维蛋白原无关,因为在表现出相同纤维蛋白原异常的亲属以及该患者的另外两个时间点都发现了正常浓度的纤连蛋白。总之,该患有先天性低纤维蛋白原血症患者的血栓形成障碍可能是由于凝血酶与纤维蛋白的结合缺陷所致。

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