Stormorken H, Brosstad F, Seim H
Thromb Haemost. 1983 Apr 28;49(2):120-2.
A family with dysfibrinogenemia is described. The abnormal fibrinogen occurred in three successive generations indicating a dominant hereditary pattern. Thrombin and reptilase times were about twice the normal value. This was shown to be caused by a polymerization defect, fibrinopeptide release being normal. Platelet aggregation was undisturbed, indicating normal platelet-fibrinogen binding. The bleeding time was normal and there was no bleeding tendency. However, an obscure recurrent pulmonary ailment may, or may not, be related to the dysfibrinogenemia. The abnormal fibrinogen was tentatively termed Oslo IV.
本文描述了一个患有异常纤维蛋白原血症的家族。异常纤维蛋白原在连续三代人中出现,表明其遗传模式为显性遗传。凝血酶时间和爬虫酶时间约为正常值的两倍。研究表明,这是由聚合缺陷引起的,纤维蛋白肽释放正常。血小板聚集未受影响,表明血小板与纤维蛋白原的结合正常。出血时间正常,且无出血倾向。然而,一种不明原因的复发性肺部疾病可能与异常纤维蛋白原血症有关,也可能无关。这种异常纤维蛋白原暂被命名为奥斯陆IV型。