Recurrent bacteremia with enteric pathogens in recessive polycystic kidneydisease

Eight children with autosomal recessive polycystic kidney disease (ARPKD) a nd recurrent bacteremia with enteric pathogens are described. Typical clini cal features of bacterial cholangitis were absent, although in five patient s histological and/or microbiological data indicated that the bacteremic ep isodes originated in the biliary tree. Bacteremia with enteric pathogens or recurrent culture-negative febrile illness in a child with ARPKD should ra ise suspicion of cholangitis, even in the absence of typical clinical findi ngs.