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一种异常纤维蛋白原(哥本哈根II型),其唾液酸含量增加,与血栓形成倾向相关且肝功能正常。

An abnormal fibrinogen (Copenhagen II) with increased sialic acid content associated with thrombotic tendency and normal liver function.

作者信息

Hansen M S, Schousboe I

出版信息

Scand J Haematol. 1984 Jul;33(1):9-14. doi: 10.1111/j.1600-0609.1984.tb02203.x.

Abstract

An increased sialic acid content of the fibrinogen molecule is found in foetal fibrinogen and as an acquired disorder in hepatic disease. A qualitatively abnormal fibrinogen was detected in the plasma of a 25-year-old man with a thrombotic tendency. The purified fibrinogen had a significantly increased content of sialic acid, an abnormal fibrin monomer polymerization, and a changed mobility in crossed affinity-immunoelectrophoresis using immobilized helix pomatia lectin. The patient had no biochemical or clinical signs of liver disease. The occurrence of a thrombotic tendency and an increased fibrinogen sialic acid content without signs of liver disease may represent a new variant of congenital dysfibrinogenaemia.

摘要

胎儿纤维蛋白原以及作为肝病的一种后天性病症中,可发现纤维蛋白原分子的唾液酸含量增加。在一名有血栓形成倾向的25岁男性的血浆中检测到一种定性异常的纤维蛋白原。纯化后的纤维蛋白原唾液酸含量显著增加,纤维蛋白单体聚合异常,并且在使用固定化蜗牛凝集素的交叉亲和免疫电泳中迁移率发生改变。该患者没有肝病的生化或临床体征。出现血栓形成倾向且纤维蛋白原唾液酸含量增加但无肝病体征,可能代表先天性异常纤维蛋白原血症的一种新变体。

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