This report describes a kindred with progressive hereditary nephropathy. The disease is characterized by an insidious onset in the fourth and fifth decades of life, slow progression, normal results of urinalysis, and renal corticomedullary cysts. There is no salt wasting or severe anemia. Genetic transmission appears to be autosomal dominant. The family reported here may represent a new hereditary nephropathy or a variant of medullary cystic disease.
Wrigley KA, Sherman RL, Ennis FA, Becker EL. Progressive Hereditary NephropathyA Variant of Medullary Cystic Disease?. Arch Intern Med. 1973;131(2):240–244. doi:10.1001/archinte.1973.00320080076010