Prognosis in cystic kidney disorders

There are 2 inherited cystic kidney diseases that progress to kidney failure. Both are "ciliopathies," a relatively new category of disorders consequent to single gene mutations in the sensory cilia that are present on a host of epithelial cells. Autosomal dominant polycystic kidney disease (ADPKD) is a major cause of end stage kidney disease in adults; although the disorder has its onset in childhood, kidney failure in children from ADPKD is rare.

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