- Polycystic kidney disease
- Genetic disorder characterized by the development of innumerable cysts in the kidneys. These cysts are filled with fluid, and replace much of the mass of the kidneys. This reduces kidney function, leading to kidney
* * *polycystic kidney disease n either of two hereditary diseases characterized by gradually enlarging bilateral cysts of the kidney which lead to reduced renal functioning:a) a disease that is inherited as an autosomal dominant trait, is usu. asymptomatic until middle age, and is marked by side or back pain, hematuria, urinary tract infections, and nephrolithiasisb) a disease that is inherited as an autosomal recessive trait, usu. affects infants or children, and results in renal failure
* * *either of two unrelated heritable disorders marked by cysts scattered throughout both kidneys. Autosomal dominant polycystic kidney disease (ADPKD, formerly called the adult form) is the most common renal cystic condition, with deterioration of renal function that may not be detectable until the third or fourth decade of life; it runs a variable course, with only some cases progressing to renal failure. Autosomal recessive polycystic kidney disease (ARPKD, formerly called the infantile form) may be congenital or may appear at any time during childhood and has a high perinatal mortality rate; almost all cases lead to hypertension. In older children it may be associated with cystic and fibrotic disease of the liver. A similar acquired disease sometimes accompanies end-stage renal disease and is called acquired cystic disease of kidney. Called also polycystic kidneys and polycystic renal d.
Autosomal dominant polycystic kidney disease viewed from the external surface (A) and bisected (B), the kidney showing marked enlargement and numerous dilated cysts. (C), Autosomal recessive polycystic kidney disease, characterized by smaller cysts and dilated channels at right angles to the cortical surface.
Medical dictionary. 2011.