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In this report, we describe the case of a boy who developed the nephrotic syndrome at one and one-half years of age, remained steroid-responsive for nine years, but then became steroid-resistant, associated with the development of progressive glomerular sclerosis and renal insufficiency. We suggest that this evolution is evidence in favor of the view that minimal change disease and focal glomerulosclerosis have a common pathogenesis.

Two HLA identical siblings developed focal glomerulosclerosis at about 15 years of age. To our knowledge, this is a unique case of the familial nephrotic syndrome because this is both the latest onset ever reported and the first description of HLA identity. We suggest that the pathogenesis of renal disease in these siblings involved an interaction of a shared genetic susceptibility and a common environment.