[Familial medullary cystic kidney with progressive kidney failure].

The reported case of 2 brothers suffering from medullary sponge kindeys is unique in that uremia developed in spite of the absence of urinary tract obstruction, infection or hypertension. With the exception of congenital nystagmus and psoriasis, none of the extrarenal malformations often associated with medullary sponge kidneys was observed.