Bilateral Renal Hypoplasia With Oligomeganephronia

Abstract

The syndrome of congenital bilateral renal hypoplasia with hypertrophy of nephrons has been well documented in the French literature, but has attracted little attention in North America. We have described two patients with clinical and histological features of the disease. The clinical picture is one of progressive chronic renal failure with symptoms of vomiting, fever, and dehydration occurring in early infancy. The essential pathologic features are a reduction in the size of both kidneys, and a decrease in the number but a marked increase in size of the remaining glomeruli. The possible causes of glomerular hypertrophy are briefly discussed.