Gris J C, Schved J F, Martinez P, Bengler C, Lemesle F, Arich C, Sarlat C
Laboratoire d'Hématologie et Immunologie, CHU, Nîmes, France.
Blood Coagul Fibrinolysis. 1992 Oct;3(5):673-7. doi: 10.1097/00001721-199210000-00027.
Serial studies of the plasma protein C-protein S system were performed during the clinical course of a pregnant woman with meningococcaemia who recovered under therapy. The patient had limited purpura fulminans skin lesions and hereditary C4b-binding protein deficiency was suspected. This diagnosis was confirmed in the patient 1 year after delivery and also by family studies. During the meningococcaemia, an initial mild and transient acquired protein C deficiency was seen but no protein S deficiency was observed despite consumption of the latter protein. As C4b-binding protein partial deficiency is associated with high free protein S and protein S activity, this may have protected against acquired protein S deficiency during meningococcaemia.
在一名患脑膜炎球菌血症的孕妇临床病程中，对其血浆蛋白C-蛋白S系统进行了系列研究，该孕妇在治疗后康复。患者有局限性暴发性紫癜皮肤病变，怀疑存在遗传性C4b结合蛋白缺乏症。分娩1年后在患者身上以及通过家族研究证实了这一诊断。在脑膜炎球菌血症期间，最初发现有轻度短暂的获得性蛋白C缺乏，但尽管后者蛋白消耗，未观察到蛋白S缺乏。由于C4b结合蛋白部分缺乏与高游离蛋白S和蛋白S活性相关，这可能在脑膜炎球菌血症期间预防了获得性蛋白S缺乏。
