[Case of autosomal dominant polycystic kidney disease presenting hepatic encephalopathy]

We report a 71-year-old woman with autosomal dominant polycystic kidney disease (ADPKD), who presented with hepatic encephalopathy. She was diagnosed as having ADPKD at 61 years of age. Thereafter, her renal function gradually worsened and she was admitted to our hospital because of encephalopathy and end-stage renal failure. The main laboratory findings were as follows: BUN 77 mg/dl; creatinine 9.0 mg/d; ammonia 573 microg/dl. Hepatic encephalopathy was improved after hemodialysis and administration of lactulose. The liver demonstrated multiple cysts on computed tomography. Angiography demonstrated that the peripheral branch of the portal vein was stenotic and a spleno-renal shunt was detected. We considered that portal hypertension was caused by multiple liver cysts, and that hepatic encephalopathy was caused by the spleno-renal shunt. It is generally considered that severe hepatic complications are rare in ADPKD, but this case suggested the need to screen for the development of hepatic lesions in ADPKD.