Longterm Follow-up of Autosomal Recessive Polycystic Kidney Disease

ABSTRACT

PURPOSE: This study was aimed to assess the clinical manifestations and courses of autosomal recessive polycystic kidney disease (ARPKD). METHODS: The medical records of 10 children diagnosed as infantile or juvenile ARPKD at Seoul National University Children's Hospital between January, 1984 and December, 1996, were reviewed, retrospectively. RESULTS: The average age at diagnosis was 3 8/12 years (4months-7 3/12 years) and sex ratio was 1 1. The mean follow-up duration was 8 5/12 years. Family history of renal cystic disease or hepatic fibrosis was not detected in any cases. Bilateral enlarged kidneys were noted in 2 cases and hepatosplenomegaly in all cases. Renal function had been maintained normally in all cases during follow-up. Urinary abnormalities were revealed in 4 cases and hypertension in 2 cases. Although liver enzyme levels were normal in all cases, esophageal varix was detected in 6 cases by gastrofiberscopy, and two of them received Warren shunt operation. The shunt operation did not affect the progression of the renal lesion. In 2 patients, who were diagnosed initially as congenital hepatic fibrosis, the renal cystic changes of ARPKD were detected 3 years and 6 years later. CONCLUSION: Because of the heterogeneous clinical spectrum and variable rate of progression of renal and hepatic lesion in ARPKD, the detection rate may vary. So patients, suspected to have ARPKD or congenital hepatic fibrosis, should be regularly followed up to detect latent renal lesion through radiological or pathological studies.