Nephronophthisis - uremic medullary cystic disease (UMCD) complex

This is a group of Progressive renaldisorders that usualy have their onset in childhood. The common characteris- tic is the peresence of a variable number of cysts in the medulla associated with significant cortical tubular atro- phy and interstitial fibrosis. Although the presence of medullary cysts is important, the corticaltubulointerstitial dam- age is the causeof the eventual renal incufficiency. İt has four variants: 1- Sporodic, nonfamilial (% 20); 2- Familial juvenile nephronophytisis (% 50), inherited asa recessive disease; 3- Renal - retinal dysplasia (% 15), recessively inherited and associated with retinitis pigmentosa; and 4-Adult onset medullary cystic disease, dominantly inherited (%15). The case had shown the view of sporadic nonfamilial variant of UMCD and was presented because of the rarity in literatüre it.