Solal-Celigny P, Laviolette M, Hebert J, Atkins P C, Sirois M, Brun G, Lehner-Netsch G, Delâge J M
Clin Exp Immunol. 1982 Jan;47(1):197-205.
We report a complete C3b inactivator deficiency in a 28-year-old patient referred for bronchiectasis and recurrent pneumonia. In addition to these recurrent infections, previously described with this deficiency, he also had several immune complex manifestations (rheumatoid factor, circulating immune complexes and one episode of serum sickness). The consequences of C3b inactivator deficiency on complement activation, chemotaxis and opsonization, clearance of immune complexes, and on red blood cell sensitization are discussed. The study of the parents showed an autosomal dominant transmission.
我们报告了一名28岁因支气管扩张症和复发性肺炎前来就诊的患者存在完全性C3b灭活因子缺乏症。除了此前报道的与该缺乏症相关的复发性感染外，他还出现了多种免疫复合物表现（类风湿因子、循环免疫复合物以及一次血清病发作）。本文讨论了C3b灭活因子缺乏对补体激活、趋化作用和调理作用、免疫复合物清除以及红细胞致敏的影响。对其父母的研究显示为常染色体显性遗传。
