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散发性脑膜炎球菌病患者先天性或后天性补体缺陷的患病率。

Prevalence of congenital or acquired complement deficiency in patients with sporadic meningococcal disease.

作者信息

Ellison R T, Kohler P F, Curd J G, Judson F N, Reller L B

出版信息

N Engl J Med. 1983 Apr 21;308(16):913-6. doi: 10.1056/NEJM198304213081601.

Abstract

We evaluated the complement system in 20 patients presenting with a first episode of meningococcal meningitis, meningococcemia, or meningococcal pericarditis. Assays of total serum complement activity were performed prospectively in 12 patients and retrospectively in 8. Six of the twenty patients had a complement deficiency (CH50 greater than 2 S.D. below the normal mean). Three of these six had a deficiency of a terminal-pathway protein (C6 in two and C8 in one), and the other three had deficiencies of multiple complement proteins associated with underlying systemic lupus erythematosus or multiple myeloma. Patients with decreased amounts of complement were similar to normal patients in terms of sex, age, type of infection, and meningococcal serogroup, but 3 of the 6 patients with a complement deficiency were black, as compared with none of the 14 patients with normal function (P = 0.018). Complement deficiency is common in patients with a first episode of meningococcal disease and may be due to either a deficiency in a single terminal protein or a complement-depleting underlying illness.

摘要

我们评估了20例首次发作的脑膜炎球菌性脑膜炎、脑膜炎球菌血症或脑膜炎球菌性心包炎患者的补体系统。对12例患者前瞻性地进行了总血清补体活性检测,对8例患者进行了回顾性检测。20例患者中有6例存在补体缺陷(CH50低于正常均值2个标准差以上)。这6例中的3例存在终末途径蛋白缺陷(2例C6缺陷,1例C8缺陷),另外3例存在与潜在系统性红斑狼疮或多发性骨髓瘤相关的多种补体蛋白缺陷。补体含量降低的患者在性别、年龄、感染类型和脑膜炎球菌血清群方面与正常患者相似,但6例补体缺陷患者中有3例为黑人,而14例功能正常的患者中无一例是黑人(P = 0.018)。补体缺陷在首次发作的脑膜炎球菌病患者中很常见,可能是由于单一终末蛋白缺陷或消耗补体的潜在疾病所致。

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