[Two cases of autosomal dominant polycystic kidney disease treated with hemodialysis associated with polycystic liver complications related to the cause of death].
As long-term survival has become possible in patients with autosomal dominant polycystic kidney disease (ADPKD) with progress in hemodialysis (HD), complications by various extrarenal diseases has presented new problems. Recent experience of two rare cases of ADPKD ending fatally due to complications by polycystic liver is presented. Case 1: A 60-year-old female with a family history of ADPKD without a past history of liver disease, was diagnosed as ADPKD at the age of 45 years. Hemodialysis was started at the age of 58 years. From 6 months prior to her death, abdominal circumference increased (body height: 149 cm, abdominal circumference: 100 cm). Dyspnea, abdominal pain and anorexia appeared and she died of hepatic failure leading to cachexia. Case 2: A 76-year-old female with a family history of ADPKD without a past history of liver disease, was started on HD at the age of 73 years. Abdominal circumference was 84 cm (body height: 138 cm). She was repeatedly admitted to and discharged from the hospital due to febrile episodes. Infection of polycystic liver was complicated by DIC and she died of gastrointestinal hemorrhage. Autopsy revealed abscess in some of the cysts in the liver. Hepatic cysts most frequently complicating ADPKD so far have presented with scarcely any clinical problems. Recently, however, cases of infection of hepatic cysts, portal hypertension and hepatic insufficiency have been reported. The relationship between these hepatic diseases and the prognosis of ADPKD has received attention. Increase in the number of cases of complications similar to the present cases is anticipated.
Saima
S
S
International Medical Center, Division of Nephrology, Tokyo, Japan.
Suzuki
Y
Y
Yoshimoto
K
K
Nakamura
Y
Y
Munakata
A
A
D000368:Aged; D003560:Cysts / Q000209:etiology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008100:Liver Abscess / Q000209:etiology*; D008107:Liver Diseases / Q000209:etiology*; D008875:Middle Aged; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications* / Q000628:therapy*; D006435:Renal Dialysis