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  • Title: Focal segmental glomerulosclerosis, crescent, and rapidly progressive renal failure.
    Author: Ramirez F, Travis LB, Cunningham RJ, Cavallo T, Rajarman S, Brouhard BH, Ellis EN.
    Journal: Int J Pediatr Nephrol; 1982 Sep; 3(3):175-8. PubMed ID: 7141789.
    Abstract:
    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.
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