Sherman R L, Susin M, Mouradian J
Perspect Nephrol Hypertens. 1977;6:175-86.
There seems to be little doubt that FGS is a nonspecific lesion that represents one way in which the renal glomerulus responds to a variety of injuries. This is illustrated by the large number of diverse conditions with which the lesion is associated including various forms of glomerulonephritis, pyelonephritis, hereditary nephritis, and heroin usage. Nevertheless, there remains a relatively large isiopathic group which, though possibly heterogeneous, displays a number of characteristic clinical and pathologic features including the following: 1. Steroid-resistant nephrotic syndrome; 2. Hematuria and hypertension; 3. Normal serum complement; 4. Progressive renal insufficiency; 5. Typical pathologic lesion most common in or restricted to juxtamedullary cortex; 6. Absence of clearly defined evidence of immune complex deposition by immunofluorescent or electron microscopic studies; 7. Recurrence of the lesion following renal transplantation. The pathogenesis of these changes is unclear, the evidence for an immune complex mechanism meager, and the suggestion that the disease is mediated by a humoral mechanism remains to be explored. The potential recurrence of this lesion in the transplanted kidney affords a unique opportunity to study the disease early in its course by a variety of techniques that may help to clarify this still poorly understood entity.
毫无疑问,局灶节段性肾小球硬化(FGS)是一种非特异性病变,代表了肾小球对多种损伤的一种反应方式。与该病变相关的大量不同病症说明了这一点,这些病症包括各种形式的肾小球肾炎、肾盂肾炎、遗传性肾炎以及海洛因使用。然而,仍有一个相对较大的特发性群体,尽管可能具有异质性,但表现出一些特征性的临床和病理特征,包括以下几点:1. 激素抵抗型肾病综合征;2. 血尿和高血压;3. 血清补体正常;4. 进行性肾功能不全;5. 典型的病理病变最常见于或局限于近髓质皮质;6. 免疫荧光或电子显微镜研究未发现明确的免疫复合物沉积证据;7. 肾移植后病变复发。这些变化的发病机制尚不清楚,免疫复合物机制的证据不足,疾病由体液机制介导的说法仍有待探索。这种病变在移植肾中潜在的复发为通过各种技术在病程早期研究该疾病提供了独特的机会,这些技术可能有助于阐明这个仍了解甚少的实体。