Bilateral renal hypoplasia with oligonephronia

This is the first case report in the English literature of bilateral renal hypoplasia with oligonephronia. Shortly after birth the patient had vomiting, polydipsia, polyuria, hyposthenuria, salt wasting, azotemia, and subsequently renal osteodystrophy and growth retardation. She had demonstrably deficient renal ammonia production. The kidneys were small; the glomeruli were few in number, larger than normal, and had no evidence of inflammatory disease or preceding glomerular destruction. The tubules were dilated. Peritubular, periglomerular, and interstitial fibrosis were widespread. No other congential anomalies were found. The identical twin of the patient had no evidence of renal disease. Although this disease closely resembles nephronophthisis, it may be distinguished from it by careful consideration of clinical and morphologic features.