A range of renal and urinary tract malformations that vary from complete renal agenesis , to renal hypodysplasia, MULTICYSTIC DYSPLASTIC KIDNEY, duplex renal collecting system, ureteropelvic junction obstruction (UPJO), megaureter, posterior urethral valves (PUV), and vesicoureteral reflux (VUR). Up to 10% of cases are familial (autosomal dominant), although these are frequently asymptomatic or less severe; thus, PENETRANCE is incomplete. CAKUT occurs in 1 in 500 live births, and is lethal 1 in 2,000 births, it can also occur with other congenital anomalies, such as papillorenal syndrome (OMIM: 120330). OMIM: 610805
