polycystic kidney (redirected from Uremic medullary sponge kidney)

polycystic kidney

n.

An inherited progressive disease characterized by formation of multiple cysts of varying size scattered diffusely throughout both kidneys and resulting in destruction of the kidney parenchyma, hypertension, bloody urine, and uremia. Also called polycystic disease of kidneys.

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polycystic kidney

Polycystic renal disease Nephrology An inherited disease characterized by the development of innumerable cysts in the kidneys filled with fluid–urine that replace much of the mass of the kidneys and reduce kidney function leading to kidney failure. See Autosomal dominant polycystic kidney disease, Multilocular cyst of the kidney.

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