Autosomal Dominant Polycystic Kidney Disease with Liver and Pancreatic Involvement.

ABSTRACT

Autosomal dominant polycystic kidney disease (adult polycystic kidney disease, Potter Type III disease) is the fourth-most common cause of end-stage renal disease.(1) Polycystic Liver Disease, the most common extrarenal manifestations of Polycystic Kidney Disease is seen in 75-90% of cases, characterized by multiple biliary cystic lesions localized in over 50% of the hepatic parenchyma. In Polycystic kidney disease, hepatic cysts develop later than the renal cysts.(2) It is associated with hypertension in about 70% of cases, cyst in pancreas in 9%, saccular berry aneurysm of cerebral arteries in 3-13%, and mitral valve prolapse.