Borzy M S, Gewurz A, Wolff L, Houghton D, Lovrien E
Department of Pediatrics, School of Medicine, Doernbecher Memorial Hospital for Children, Oregon Health Sciences University, Portland 97201.
Am J Dis Child. 1988 Jan;142(1):79-83. doi: 10.1001/archpedi.1988.02150010089032.
A 10-year-old Laotian boy had homozygous deficiency of the third component of complement and recurrent bacterial infections beginning at age 5 months. Cellular and humoral immunity were normal, as were polymorphonuclear leukocyte chemotaxis and bactericidal activities. Serum complement-mediated hemolytic, chemotactic, and opsonic activities were deficient. In vitro addition of purified C3 to patient serum restored hemolytic complement to normal levels, and plasma infusion during each of four episodes of pneumonia significantly enhanced serum opsonic activity for as long as 36 hours. A renal biopsy specimen revealed mesangiopathic glomerulonephritis, although significant levels of circulating IgG immune complexes were not detected. These findings further support the association of C3 deficiency with immune-complex disease and suggest that plasma infusion may be an adjunct to antibiotic therapy in the management of severe pyogenic infections in patients with C3 deficiency.
一名10岁的老挝男孩患有补体第三成分纯合性缺乏症，自5个月大时起便反复出现细菌感染。细胞免疫和体液免疫正常，多形核白细胞趋化性和杀菌活性也正常。血清补体介导的溶血、趋化和调理活性均有缺陷。在患者血清中体外添加纯化的C3可使溶血补体恢复至正常水平，并且在四次肺炎发作期间每次进行血浆输注可使血清调理活性显著增强长达36小时。肾活检标本显示为系膜增生性肾小球肾炎，尽管未检测到显著水平的循环IgG免疫复合物。这些发现进一步支持了C3缺乏与免疫复合物疾病之间的关联，并表明在C3缺乏患者严重化脓性感染的管理中，血浆输注可能是抗生素治疗的辅助手段。
