A patient with autosomal dominant polycystic kidney disease who developed severe hepatic insufficiency during chronic hemodialysis

Abstract

The case involved a 70-year-old female with end-stage autosomal dominant polycystic kidney disease (ADPKD) who had been on hemodialysis for 2 years. She had been diagnosed with hepatic encephalopathy at the time of the initiation of dialysis. An abdominal computed tomography (CT) scan demonstrated mechanical compression of portal venous flow due to liver cysts, which exacerbated the patient’s portal hypertension. In November 2014, she was emergently admitted to our hospital with upper abdominal pain, disdialysis syndrome, and liver dysfunction. A CT scan showed that the inferior vena cava had been almost completely compressed by the liver cysts. Based on her laboratory data and magnetic resonance imaging findings, she was diagnosed with liver cyst infection. Enterococcus faecalis was detected in a blood culture. Antibiotics were not effective, and the patient’s general condition gradually worsened over time. Finally, she died of liver failure 35 days after admission. Although it is known that severe hepatic complications are rare in ADPKD, a previous study showed that hepatic cyst infections are more serious than renal cyst infections and that some ADPKD patients in Japan die of refractory hepatic cyst infections. Thus, this case suggests that physicians should pay careful attention to the management of liver cysts in ADPKD patients.