A patient with a single functioning kidney presented with the nephrotic syndrome. On the basis of highly selective proteinuria, a diagnosis was made of lipoid nephrosis. Steroid therapy over a 2-year period did not control the disease. The patient eventually developed end-stage renal failure and malignant hypertension. Nephrectomy was performed to control the hypertension. Histological examination showed congenital dysplasia in one kidney and sclerosing glomerulonephritis, malignant nephrosclerosis, as well as dysplastic changes in the other.
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