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一名患有蛋白C缺乏症的患者出现复发性多支视网膜小动脉阻塞。

Recurrent multiple-branch retinal arteriolar occlusions in a patient with protein C deficiency.

作者信息

Nelson M E, Talbot J F, Preston F E

机构信息

Department of Ophthalmology, Royal Hallamshire Hospital, Sheffield, United Kingdom.

出版信息

Graefes Arch Clin Exp Ophthalmol. 1989;227(5):443-7. doi: 10.1007/BF02172896.

Abstract

A 34-year-old woman is presented who has recurrent, multiple, retinal arteriolar occlusions associated with a deficiency in protein C, a vitamin K-dependent anticoagulant. Protein C is a naturally occurring anticoagulant and if there is a deficiency, it can lead to episodes of thrombosis; the deficiency can be acquired or congenital. This is the first documented case of retinal arteriolar occlusion associated with a congenital deficiency of this protein. The mechanism for arteriolar occlusion in this patient is discussed, and whilst the role of protein C deficiency remains presumptive, it is strongly implicated.

摘要

本文报告了一名34岁女性,她反复出现多发性视网膜小动脉闭塞,与蛋白C缺乏有关,蛋白C是一种维生素K依赖的抗凝剂。蛋白C是一种天然存在的抗凝剂,如果缺乏,可导致血栓形成发作;这种缺乏可以是后天获得的,也可以是先天性的。这是第一例有文献记载的与这种蛋白先天性缺乏相关的视网膜小动脉闭塞病例。本文讨论了该患者小动脉闭塞的机制,虽然蛋白C缺乏的作用仍为推测性,但有很强的关联性。

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