Ramirez F, Travis L B, Cunningham R J, Cavallo T, Rajarman S, Brouhard B H, Ellis E N
Int J Pediatr Nephrol. 1982 Sep;3(3):175-8.
The usual clinical course of focal segmental glomerulosclerosis is marked by progressive decrease in renal functional and sclerosis on biopsy over a period of months to years. We report a variant exemplified by 2 children who developed chronic renal failure within 12 weeks of the onset of nephrotic syndrome; repeat renal biopsies demonstrated extensive extracapillary glomerular proliferation and crescent formation. Both were males, less than 5 years of age, presenting with nephrotic syndrome resistant to steroid therapy and normal renal function. Renal biopsies done after 8 weeks of therapy, demonstrated findings compatible with focal segmental glomerulosclerosis. Within 12 weeks of onset of nephrotic syndrome, both patients experienced a decrease in renal function requiring dialysis. Repeat renal biopsies revealed extensive extracapillary glomerular proliferation with crescent formation. These patients represent a variant of focal segmental glomerulosclerosis characterized by rapid progression to renal failure with extensive extracapillary glomerular proliferation and crescent formation.
局灶节段性肾小球硬化的通常临床病程表现为在数月至数年的时间里，肾功能逐渐下降，活检显示硬化。我们报告了一个变体病例，有2名儿童在肾病综合征发病后12周内发展为慢性肾衰竭；重复肾活检显示广泛的毛细血管外肾小球增生和新月体形成。两人均为男性，年龄小于5岁，表现为对类固醇治疗耐药的肾病综合征且肾功能正常。治疗8周后进行的肾活检显示结果与局灶节段性肾小球硬化相符。在肾病综合征发病12周内，两名患者的肾功能均下降至需要透析。重复肾活检显示广泛的毛细血管外肾小球增生并伴有新月体形成。这些患者代表了局灶节段性肾小球硬化的一种变体，其特征是迅速进展至肾衰竭，并伴有广泛的毛细血管外肾小球增生和新月体形成。
