Koester B H, Köveker G B, Pötsch B, Becker H D
Allgemeinchirurgische Klinik und Poliklinik, Universität Tübingen.
Chirurg. 1993 Oct;64(10):809-12.
We report a case of hereditary protein S and protein C deficiency which are a rare defects of the anti-coagulation-system. Protein S is a vitamin K dependent glycoprotein that functions as a cofactor to activated Protein C in the inactivation of coagulation factors V and VIIIa. A deficiency of these proteins caused by a genetic defect increase the risk of recurrent thrombosis at a younger age. Acquired decreases in protein S and C concentration have been reported in connection with age, sex, pregnancy and with oral anticonception. The higher risk for thrombotic diseases of patients with thrombophilia requires a sufficient treatment and prophylaxis, e.g. with fresh frozen plasma or a protein C concentrate.
我们报告一例遗传性蛋白S和蛋白C缺乏症，这是抗凝系统罕见的缺陷。蛋白S是一种维生素K依赖的糖蛋白，在凝血因子V和VIIIa的失活过程中作为活化蛋白C的辅因子发挥作用。由遗传缺陷导致的这些蛋白缺乏会增加年轻时复发性血栓形成的风险。据报道，蛋白S和C浓度的后天性降低与年龄、性别、妊娠以及口服避孕药有关。血栓形成倾向患者患血栓性疾病的风险较高，需要进行充分的治疗和预防，例如使用新鲜冷冻血浆或蛋白C浓缩物。
