Polycystic disorders of kidneys
Renal dysplasia Relatively common, often acquired, presents in infancy; unilateral or bilateral and segmental, focally irregular cystic kidneys, related to mesenchymal immaturity, accompanied by obstruction
Infantile polycystic kidneys Uncommon, AR, seen in infants with massively enlarged kidneys and aberrant bile ducts.
Adult polycystic kidneys Common (1-2:1000, US), AD, located to a gene on chromosome 16, affects adults, large bumpy kidneys, cysts in liver, lung, pancreas, berry aneurysms in brain
Medullary sponge kidneys Common, bilateral, uncertain pattern of heredity, affects adults, inability to concentrate urine, hypercalcemia, nephrolithiasis, pyelonephritis, distal renal tubular acidosis and cystic dilation of collecting ducts; renal function and life span may be normal
Uremic medullary sponge kidney Rare, inherited, first seen in young adults; bilateral corticomedullary junction cysts and functional tubular defects, Fanconi syndrome and uremia.