Lehmer R R, Elias A N, Capdeville M J, Brown D R, Branson H E
J Natl Med Assoc. 1985 Jul;77(7):561-5.
A slow clotting dysfibrinogen with delayed anodal immunoelectrophoretic mobility and impaired fibrinopeptide A release has been identified in a patient with recurrent portal vein and deep venous thrombosis. Affected family members tested in the initial screening were asymptomatic. The proband's father died of pulmonary embolism at age 44 years and had mesenteric thrombosis at necropsy. The association of a plasma protein abnormality with visceral thrombosis is unusual and has never been observed previously with a dysfibrinogen. The qualitative abnormality is transmitted as an autosomal codominant and is tentatively designated, fibrinogen Irvine.
在一名复发性门静脉和深静脉血栓形成的患者中,发现了一种凝血缓慢的异常纤维蛋白原,其阳极免疫电泳迁移延迟且纤维蛋白肽A释放受损。在初步筛查中接受检测的受影响家庭成员无症状。先证者的父亲在44岁时死于肺栓塞,尸检时发现有肠系膜血栓形成。血浆蛋白异常与内脏血栓形成的关联并不常见,以前从未在异常纤维蛋白原中观察到。这种定性异常以常染色体共显性方式遗传,暂命名为纤维蛋白原欧文型。