Rambausek M, van den Wall Bake A W, Schumacher-Ach R, Spitzenberg R, Rother U, van Es L A, Ritz E
Department of Internal Medicine, University of Heidelberg, FRG.
Nephrol Dial Transplant. 1987;2(4):208-11.
C3 and Bf alleles were examined in the general population, in 67 patients with biopsy-confirmed mesangial IgA nephropathy and 81 patients with other types of glomerulonephritis, from the Heidelberg and Leiden renal programmes respectively. In both populations, a significant excess of homozygous phenotype C3FF (3.4% in controls; 10.4% in IgA nephropathy) and a deficit of C3FS heterozygous phenotype (35.8% in controls; 19.4% in IgA nephropathy) were observed in patients with IgA nephropathy, but not in other types of glomerulonephritis. No difference of C3 gene frequencies was found. C3FF was associated with an adverse clinical outcome (a higher prevalence of renal failure and hypertension). A significant excess of Bf-F gene frequency was noted (0.20 in controls; 0.33 in IgA nephropathy). In addition, an excess of phenotype BfFF was found (none in controls; 10.4% in IgA nephropathy). BfFF homozygotes also carried a higher risk of an adverse outcome (renal failure and hypertension). The data suggest a role for genetically coded (presumably) immunological factors in the genesis and course of IgA nephropathy.
分别从海德堡和莱顿肾脏研究项目中选取了普通人群、67例经活检确诊为系膜增生性IgA肾病的患者以及81例其他类型肾小球肾炎患者,检测其C3和Bf等位基因。在这两组人群中,IgA肾病患者出现纯合子表型C3FF显著增多(对照组为3.4%;IgA肾病患者为10.4%)以及C3FS杂合子表型减少(对照组为35.8%;IgA肾病患者为19.4%)的情况,而其他类型肾小球肾炎患者则未出现这种情况。未发现C3基因频率存在差异。C3FF与不良临床结局相关(肾衰竭和高血压的患病率更高)。观察到Bf - F基因频率显著增多(对照组为0.20;IgA肾病患者为0.33)。此外,还发现表型BfFF增多(对照组无;IgA肾病患者为10.4%)。BfFF纯合子也具有更高的不良结局风险(肾衰竭和高血压)。这些数据表明,基因编码的(可能)免疫因素在IgA肾病的发生和发展过程中发挥了作用。