Section Editor: Dharam M. Ramnani, MD

Virginia Urology, Richmond, VA, USA

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Image Description

Epidemiology: Autosomal dominant polycystic kidney disease (ADPKD) is a common cystic kidney disease and the most common hereditary kidney disease. It occurs in 1 to 2 per 1000 live births. It is one of the leading causes of renal failure and accounts for 5% to 10% of patients on dialysis or renal transplantation list. Most patients are between 40-50 yrs. of age at presentation. It is inherited as an autosomal dominant disorder with complete penetrance (by age 80 years). About 25% of patients have no family history and represent new mutations. There is no sex or racial predilection. This specimen photograph shows external (right) and cut surface (left) of a kidney that is completely replaced by numerous cysts.

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