A 56-year-old woman in chronic hemodialysis had been suffering from uncontrollable fever for the past 7 months. Her original disease was diagnosed as familial polycystic kidney and three of her five brothers were found to have the same disease. Her chromosome was 46,XX,21P+ and laboratory examination revealed severe anemia, malnutrition, liver dysfunction, pyuria and candidiasis of urine. Abdominal echogram and CT scan revealed polycystic kidneys and multiple liver cysts. She was admitted to our hospital and was diagnosed as having pyelonephritis of the right kidney. As her condition was not improved by conservative therapy right nephrectomy was performed. One month later, spiking fever and left tenderness reappeared. Those symptoms could not be controlled by conservative therapy and left nephrectomy was performed again. Pathological examination on nephrectomized kidneys showed interstitial nephritis, hyaline degeneration and proliferative change of glomeruli, microabscess, colloid of tubules and calcification of parts of Henle's loops. Nephrectomy has been performed in 1.6 to 10.0% of polycystic kidneys due to references since 1952. Eight of the 22 polycystic kidneys (36.3%) seen at our hospital during the past 10 years have been removed.
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