Majid Zainab, Tahir Faryal, Ahmed Jawad, Bin Arif Taha, Haq Anwarul
Internal Medicine, Dow University of Health Sciences, Karachi, PAK.
Neurology, Baylor University Medical Center, Dallas, USA.
Cureus. 2020 Mar 30;12(3):e7472. doi: 10.7759/cureus.7472.
Protein C (PC) is a 62-kD vitamin K dependent glycoprotein produced by the liver as a zymogen and is activated by binding to the thrombin-thrombomodulin complex, with protein S (PS) acting as a cofactor. Among its various functions, PC acts as a naturally occurring anticoagulant and its deficiency, either homozygous or heterozygous, predisposes the individual to a state of thrombosis, particularly venous thromboembolism, and mainfests as myocardial infarction (MI), deep venous thrombosis, pulmonary embolism, or stroke. This review discusses the pathophysiology of the anticoagulatory effect of PC, mode of inheritance of its deficiency, the arterial and venous involvement in patients with stroke, and its risk factors. A detailed analysis of published case reports on PC deficiency as a causative agent of stroke in young adults has also been included along with the management of such patients.
蛋白C(PC)是一种由肝脏产生的62kD维生素K依赖性糖蛋白,以酶原形式存在,通过与凝血酶-血栓调节蛋白复合物结合而被激活,蛋白S(PS)作为辅助因子。在其多种功能中,PC作为一种天然存在的抗凝剂,其缺乏,无论是纯合子还是杂合子,都会使个体易处于血栓形成状态,尤其是静脉血栓栓塞,并表现为心肌梗死(MI)、深静脉血栓形成、肺栓塞或中风。本综述讨论了PC抗凝作用的病理生理学、其缺乏的遗传方式、中风患者的动脉和静脉受累情况及其危险因素。还包括对已发表的关于PC缺乏作为年轻成年人中风病因的病例报告的详细分析以及此类患者的管理。