Derkx H H, Kuijper E J, Fijen C A, Jak M, Dankert J, van Deventer S J
University of Amsterdam, Department of Paediatrics, The Netherlands.
Eur J Pediatr. 1995 Sep;154(9):735-8. doi: 10.1007/BF02276718.
We evaluated the complement system in 29 children (mean age: 4.5 years) who survived fulminant meningococcal septic shock. No terminal complement deficiencies were found. One patient, who experienced the most dramatic disease course, had a decreased haemolytic activity in the haemolysis-in-gel test for the alternative pathway. The properdin concentration in serum of this patient was < 0.1 microgram/ml (n = 17.1-27.7 micrograms/ml). Coagulation studies revealed a heterozygeous type I protein C deficiency as well. He was the only patient with a Neisseria meningitidis group Y infection.
Fulminant meningococcal disease due to uncommon serogroups should lead to screening of the alternative pathway of complement activation.
我们评估了29名暴发性脑膜炎球菌性败血症休克幸存者（平均年龄：4.5岁）的补体系统。未发现终末补体缺陷。一名经历了最严重病程的患者，其替代途径的凝胶内溶血试验中的溶血活性降低。该患者血清中的备解素浓度<0.1微克/毫升（正常范围为17.1 - 27.7微克/毫升）。凝血研究还发现了杂合子I型蛋白C缺乏。他是唯一一例感染Y群脑膜炎奈瑟菌的患者。
由不常见血清群引起的暴发性脑膜炎球菌病应进行补体激活替代途径的筛查。
