Zerzri Yusr, Kallel-Sellami Maryam, Abdelmalek Rim, Laadhar Lilia, Ben Chaabane Tawfik, Makni Sondés
Laboratoire d'immunologie, Hôpital Rabta, Tunis, Tunisie.
Tunis Med. 2010 Apr;88(4):269-76.
The complement system is one of the main effectors of both innate and adaptive immunity. Hereditary complement deficiency, mainly those of the terminal pathway (C5-C9), is at increased risk for septic meningitides particularly meningococcal ones.
to assess clinical and biochemical features of 3 Tunisian adults with C5 hereditary complement deficiency (C5D), with a familial study performed for two of them.
Functional activity of the classical and the alternative pathway of complement (CH50 and AP50 respectively) were measured according to standards haemolytic procedures. Serum concentration of complement components were determined by nephelemetry and ELISA. C5D was diagnosed when CH50, AP50 and C5 antigenic level were highly decreased.
Our patients were 2 men and one woman. All these patients presented clinical symptoms of septic meningitides. Meningococcal orign was confirmed in one case. C5 level varies between 0 and 0.4%. Levels of other complement components: Clq, C3, C4, properdine, C6, C8 and C9 were normal. Antigenic C7 level was 50% in the female patient. Familial study revealed no similar hereditary complement deficiency in relatives.
Only 27 cases with C5D were reported in the literature. The description of 3 cases in our series demonstrates that: * C5D is not rare in Tunisia, ** C5D is clinically commonly complicated by meningitides with unconstant severity, *** C5D is biologically caracterised by a variable level of the plasmatic C5 component.
补体系统是固有免疫和适应性免疫的主要效应器之一。遗传性补体缺陷，主要是终末途径（C5 - C9）的缺陷，患化脓性脑膜炎尤其是脑膜炎球菌性脑膜炎的风险增加。
评估3名患有C5遗传性补体缺陷（C5D）的突尼斯成年人的临床和生化特征，并对其中2人进行家族研究。
根据标准溶血程序测量补体经典途径和替代途径的功能活性（分别为CH50和AP50）。通过散射比浊法和酶联免疫吸附测定法测定补体成分的血清浓度。当CH50、AP50和C5抗原水平显著降低时诊断为C5D。
我们的患者为2名男性和1名女性。所有这些患者均出现化脓性脑膜炎的临床症状。其中1例确诊为脑膜炎球菌感染。C5水平在0至0.4%之间变化。其他补体成分：C1q、C3、C4、备解素、C6、C8和C9水平正常。女性患者的抗原性C7水平为50%。家族研究显示亲属中无类似的遗传性补体缺陷。
文献中仅报道了27例C5D病例。我们系列中3例病例的描述表明：* C5D在突尼斯并不罕见，** C5D在临床上通常并发脑膜炎，严重程度不一，*** C5D在生物学上的特征是血浆C5成分水平可变。
