镰状细胞贫血中补体系统B因子的缺乏
Deficiency of factor B of the complement system in sickle cell anaemia.
作者信息
Wilson W A, Hughes G R, Lachmann P J
出版信息
Br Med J. 1976 Feb 14;1(6006):367-9. doi: 10.1136/bmj.1.6006.367.
Abstract
Factors B and D as well as the total activity of the alternative pathway of complement activation were measured using a functional assay in sera from 29 patients with sickle cell anaemia and 18 normal controls. Total alternative pathway activity was reduced in the patients compared with controls. In patients with abnormally low total alternative pathway activity factor D levels were normal, whereas factor B levels were significantly depressed to a mean level of about half of normal. Regression analysis in patients also showed a significant relation between total alternative pathway activity and factor B levels. A deficiency of factor B is the likely cause of the defect in the complement system in patients with sickle cell anaemia. Such a defect may contribute to the excessive proneness of such patients to severe infection.
摘要
采用功能分析法检测了29例镰状细胞贫血患者和18名正常对照者血清中的B因子、D因子以及补体激活替代途径的总活性。与对照组相比，患者的替代途径总活性降低。在替代途径总活性异常低的患者中，D因子水平正常，而B因子水平显著降低，平均降至正常水平的一半左右。对患者的回归分析还显示，替代途径总活性与B因子水平之间存在显著相关性。B因子缺乏可能是镰状细胞贫血患者补体系统缺陷的原因。这种缺陷可能导致这些患者极易发生严重感染。
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