부산대학교 도서관

Autosomal recessive polycystic kidney disease (ARPKD) is known to be quite variable in its clinical presentation and disease progression pattern. We studied the clinical course and outcome of our 10 ARPKD patients. The median age at last follow-up is 9.9 years. All patients were diagnosed within the 1st year of life. The clinical presentations during their neonatal periods were flank mass (80.0%), respiratory insufficiency (50.0%), and hyponatremia (55.6%). Eight of the patients showed hepatic complications such as hepatic fibrosis (100%), and intrahepatic biliary duct dilatation was seen in six out of the eight (75.0%) patients, whereas none of them had cholangitis. Six patients required renal replacement therapy at a median age of 7.6 years, and the five of them subsequently underwent kidney transplantation. Enlarged organs had to be removed, and severe pancytopenia should be corrected by splenectomy before renal transplantation. In conclusion, clinical treatments for kidney and hepatic complications in ARPKD should be designed appropriately depending on individual patient conditions.