<prompt> All are true about nephrnophthisis exceptInterstitial fibrosisCoical tubular hyperophyCysts in the medulla20 cases are non-familial  <response>: 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 8959 