Section Editor: Dharam M. Ramnani, MD

Virginia Urology, Richmond, VA, USA

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Epidemiology: Autosomal recessive polycystic kidney disease (ARPKD) is a disease of infants and was previously referred to as infantile or childhood polycystic kidney disease. Most cases become apparent at birth. The incidence is around 1 in 20,000 live births. Almost 50% of the affected newborns die soon after birth. The parents are usually unaffected. The siblings have 1 in 4 chance of being affected. Image courtesy of Dr. Jean-Christophe Fournet, Paris, France; humpath.com; Used with permission

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