FOCAL SEGMENTAL GLOMERULAR SCLEROSIS - THE CELLULAR LESION
FOCAL SEGMENTAL GLOMERULAR SCLEROSIS - THE CELLULAR LESION
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DOI：
10.1038/ki.1985.225
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发表时间：
1985-01-01
影响因子：
19.6
通讯作者：
LEWIS, EJ
中科院分区：
文献类型：
--
作者：
SCHWARTZ, MM;LEWIS, EJ
对59例局灶节段性肾小球硬化（FSG）肾活检标本的病理特征与患者活检时的临床和实验室检查结果进行了相关性分析。两种形态学模式进行了鉴定：39活检标本只有局灶性节段性瘢痕，这是经常与透明沉积。在20例FSG活检标本中，肾小球也含有细胞病变。它包括肾小球受累部分的细胞过多，周围Bowman's间隙中的细胞增加，以及相关肾小球内脏上皮细胞的反应性和增殖性变化。可见细胞病变覆盖肾小球毛细血管，有极轻微的组织学异常，并邻近瘢痕。然而，它通常叠加在节段性瘢痕或肾小球的一部分上，毛细血管塌陷，基底膜褶皱。免疫荧光和电子显微镜检查没有显示出显着的免疫反应物或电子致密沉积物在肾小球。39例仅有节段性瘢痕的患者中有19例蛋白尿等于或大于3.0 g/天，9例患有肾病综合征。20例细胞病变患者中有18例蛋白尿≥ 3.0 g/d，14例有肾病综合征。在有细胞病变的患者中，蛋白尿发作和活检之间的间隔时间短得多（3.4 ± 0.01）。3对71.9 ±。平均87个月±。当仅比较肾病范围蛋白尿时，这种差异也是显著的（3.4 ± 0.01）。3对45.6 ±。平均73个月±。SD P < 0.01）。有或无细胞病变的FSG患者在男性/女性比例、年龄、血清肌酐和舒张压方面相似。从这些观察，我们认为肾小球损伤，证明了节段性增生性病变的迹象，上皮细胞损伤，是一个重要的因素，在FSG的发病机制。细胞病变和肾病综合征的临床表现之间的时间关系表明，这些病理变化不仅是继发于慢性蛋白尿，并可能代表特定的损害的病因。
The pathological features of 59 renal biopsy specimens with focal segmental glomerular sclerosis (FSG) were correlated with the patient''s clinical and laboratory findings at the time of biopsy. Two morphologic patterns were identified: thirty-nine biopsy specimens had only focal segmental scars which were frequently associated with hyaline deposits. In 20 biopsy specimens with FSG, the glomeruli also contained a cellular lesion. It consisted of hypercellularity in the involved portion of the glomerulus, increased cells in the surrounding Bowman''s space, and reactive an proliferative changes in the associated glomerular visceral epithelial cells. The cellular lesion was seen overlying glomerular capillaries with minimal histologic abnormalities and adjacent to scars. However, it was usually superimposed upon a segmental scar or a portion of the glomerulus with collapsed capillaries and wrinkled, folded basement membranes. Immunofluorescence and electron microscopy did not demonstrate significant immune reactants or electron-dense deposits in the glomeruli. Nineteen of 39 patients with only segmental scars had proteinuria equal to or greater than 3.0 g/day, and nine had the nephrotic syndrome. Eighteen of 20 patients with the cellular lesion had proteinuria equal to or greater than 3.0 g/day, and 14 had the nephrotic syndrome. The interval between the onset of proteinuria and biopsy was much shorter in patients with the cellular lesion (3.4 .+-. 3 vs. 71.9 .+-. 87 months, mean .+-. SD, P < 0.01).This difference was also significant when only those with nephrotic range proteinuria were compared (3.4 .+-. 3 vs. 45.6 .+-. 73 months, mean .+-. SD P < 0.01). Patients with FSG, with or without the cellular lesion, were similar with respect to male/female ratio, age, serum creatinine, and diastolic blood pressure. From these observations, we believe that glomerular injury, evidenced by segmental proliferative lesions with signs of epithelial cell injury, is a significant factor in the pathogenesis of FSG. The temporal relationship between the cellular lesion and the onset of clinical manifestations of the nephrotic syndrome suggests that these pathologic changes are not merely secondary to chronic proteinuria and may represent specific damage to the etiologic insult.