Quérin S, Noël L H, Grünfeld J P, Droz D, Mahieu P, Berger J, Kreis H
Clin Nephrol. 1986 Mar;25(3):134-40.
Twelve of 767 renal allograft recipients developed linear fixation of IgG along the glomerular basement membrane (GBM) by direct immunofluorescence technique. This was associated with linear fixation along the tubular basement membrane in 7 of them. Circulating anti-GBM antibodies were not detected by indirect immunofluorescence or radioimmunoassay in any patient whereas anti-TBM antibodies were found in 2 of 4 with linear TBM fixation. Among the 12 patients with linear GBM fixation, 5 had Alport's syndrome; the 7 others had various renal diseases, excluding anti-GBM nephritis. Among the 767 patients, 34 had Alport's syndrome or variants (i.e., 4.5%). The incidence of linear GBM fixation is much higher in Alport's syndrome than in other renal diseases. Linear GBM fixation was not clearly related to anti-GBM antibodies and was not accompanied by significant deterioration of graft function. These findings may be relevant, however, to the missing GBM antigen in Alport's syndrome.
在767例肾移植受者中,有12例通过直接免疫荧光技术在肾小球基底膜(GBM)上出现IgG线性沉积。其中7例同时伴有肾小管基底膜的线性沉积。所有患者经间接免疫荧光或放射免疫分析均未检测到循环抗GBM抗体,而在4例出现肾小管基底膜线性沉积的患者中,有2例检测到抗TBM抗体。在12例出现GBM线性沉积的患者中,5例患有Alport综合征;另外7例患有各种肾脏疾病,但不包括抗GBM肾炎。在767例患者中,有34例患有Alport综合征或其变异型(即4.5%)。Alport综合征患者中GBM线性沉积的发生率远高于其他肾脏疾病。GBM线性沉积与抗GBM抗体无明显关联,且未伴有移植肾功能的显著恶化。然而,这些发现可能与Alport综合征中缺失的GBM抗原有关。