Rivard G E, David M, Farrell C, Schwarz H P
Division of Hematology-Oncology, Hôpital Sainte-Justine, Université de Montréal, Québec, Canada.
J Pediatr. 1995 Apr;126(4):646-52. doi: 10.1016/s0022-3476(95)70369-1.
To evaluate the clinical and laboratory effects of protein C concentrate as an adjunct to conventional therapy in the treatment of meningococcemia with purpura fulminans.
Case series (pilot study).
Intensive care unit in a tertiary care pediatric hospital.
Four children (aged 3 months to 15 years) requiring intensive treatment for meningococcemia with shock, disseminated intravascular coagulation, and purpura fulminans.
Intravenous administration of a protein C concentrate (100 IU/kg every 6 hours).
Plasma protein C amidolytic activity, fibrinogen, and D-dimers; evolution of skin and limb lesions.
Treatment with protein C concentrate led to a rise in plasma protein C activity levels to within normal limits in all patients, associated with an increase in plasma fibrinogen and a bimodal decrease in D-dimers. No adverse effects were noted. All patients had reversal of organ dysfunction despite the severity of the initial illness. Two patients recovered completely with no sequelae; two required amputations.
These encouraging clinical and laboratory results and the absence of side effects warrant the initiation of a double-blind, randomized controlled multicenter trial to determine the role of protein C replacement in the treatment of meningococcemia-associated purpura fulminans.
评估蛋白C浓缩物作为传统疗法辅助手段治疗暴发性紫癜型脑膜炎球菌血症的临床及实验室效果。
病例系列（初步研究）。
一家三级护理儿科医院的重症监护病房。
4名儿童（年龄3个月至15岁），因暴发性紫癜型脑膜炎球菌血症伴休克、弥散性血管内凝血而需要强化治疗。
静脉注射蛋白C浓缩物（每6小时100 IU/kg）。
血浆蛋白C酰胺水解活性、纤维蛋白原和D-二聚体；皮肤和肢体病变的演变情况。
使用蛋白C浓缩物治疗后，所有患者血浆蛋白C活性水平均升至正常范围，同时血浆纤维蛋白原增加，D-二聚体呈双峰下降。未观察到不良反应。尽管初始病情严重，但所有患者的器官功能障碍均得到逆转。2名患者完全康复，无后遗症；2名患者需要截肢。
这些令人鼓舞的临床和实验室结果以及无副作用的情况，使得开展一项双盲、随机对照多中心试验以确定蛋白C替代疗法在治疗脑膜炎球菌血症相关暴发性紫癜中的作用成为必要。
