Congenital oligonephronic renal hypoplasia with hypertrophy of nephrons (oligonephronia)

Four patients with oligonephronia are described. The clinical picture was that of a progressive renal insufficiency, but the time of onset and severity varied from one case to another. One patient had oligonephronia of one kidney and aplasia of the other kidney. In another patient a familial syndrome of multiple congenital anomalies was observed. In a sib of the latter patient, a simple unilateral renal hypoplasia was discovered.