Familial hypoplastic glomerulocystic kidney. A new entity?

Two pairs of female siblings of French and Italian origin presented with the histological picture of glomerulocystic kidneys. The cases differ from the patients previously described with glomerulocystic kidneys by the absence of major extrarenal malformations, the reduction of kidney size with absence of renal papillae and by the presence of stable chronic renal failure, starting during the first months of life. Both mothers of the patients also had chronic renal failure with similar urographic abnormalities.