All are true about nephrnophthisis except

Coical tubulointerstitial damage occurs in nephrnophthisis nephrnophthisis (uremic medullary cystic diseases complex) it is a group of progressive renal disorders that usually have onset in childhood . 4 variants of diseases are; Sporadic /non familial Familial juvenile nephrnophthisis Renal retinal dysplasia Adult onset medullary cystic disease Affected children present first with polyuria and pilydipsia which reflects a marked tubular defect in concentrating ability Refer robbins 8/959