Section Editor: Dharam M. Ramnani, MD

Virginia Urology, Richmond, VA, USA

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Introduction: Autosomal dominant polycystic kidney disease (ADPKD) is a hereditary disease in which large number of cysts develop in the kidneys which progressively destroy the renal parenchyma causing renal failure. It is quite common and among the major causes of end-stage renal disease, accounting for 5%-10% of patients on renal dialysis or requiring kidney transplantation. The underlying mechanism is mutations in two genes, PKD1 on chromosome 16p encoding polycystin-1 and PKD2 on chromosome 4q encoding polycystin-2.

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