Pettersson E, Törnroth T, Wieslander J
Karolinska Institute, Department of Renal Medicine, Huddinge Hospital, Sweden.
Clin Nephrol. 1990 Mar;33(3):105-9.
Using indirect immunofluorescence, cryostat sections from renal biopsy specimens of 14 adult patients showing marked diffuse thinning of the glomerular basement membrane (GBM) on ultrastructural analysis were examined for the presence of the Goodpasture (GP) epitope M2 using anti-M2 antiserum. In no case was a total absence of M2 noted. The fluorescence pattern was fine but homogeneously linear along the GBM in 12 cases, intensity varying from +-++, as compared with for the control specimen GBM. A faint, broken line of stain, intensity+, was observed in biopsy specimens of two patients, one of whom had family members with progressive hereditary nephritis, type Alport's syndrome. Clinical presentation was dominated by hematuria (10/14 patients) but also included three patients with isolated proteinuria. Two patients had nephrotic range proteinuria. Other than the GBM changes, histological findings were sparse, with either no abnormalities or only slight mesangial increase in most. One case of focal segmental sclerosis and hyalinosis was also found. The findings from this study suggest that the abnormally thin GBM does not lack the GP epitope, but it may be reduced.
采用间接免疫荧光法，使用抗M2抗血清对14例成年患者肾活检标本的低温切片进行检查，这些患者在超微结构分析中显示肾小球基底膜（GBM）明显弥漫性变薄，以检测Goodpasture（GP）表位M2的存在。在所有病例中均未发现M2完全缺失。12例患者的荧光模式沿GBM呈细微但均匀的线性，强度从±到++不等，与对照标本GBM相比。在两名患者的活检标本中观察到微弱的、间断的染色线，强度为+，其中一名患者有患进行性遗传性肾炎（Alport综合征）的家庭成员。临床表现以血尿为主（10/14例患者），但也包括3例孤立性蛋白尿患者。两名患者有肾病范围的蛋白尿。除GBM改变外，组织学表现较少，大多数病例无异常或仅有轻微的系膜增生。还发现1例局灶节段性硬化和玻璃样变。本研究结果表明，异常变薄的GBM并不缺乏GP表位，但可能减少。
