ARPKD With Shrinking Kidneys and Progressive Loss of Kidney Function

This is the authors' abstract. We don't add key points for this paper.

Autosomal recessive polycystic kidney disease (ARPKD) is a rare inherited disorder that often presents in the antenatal period with bilaterally enlarged echogenic kidneys, oligohydramnios, and an empty fetal bladder. Children diagnosed postnatally may have enlarged, echogenic kidneys, and later develop dilated intrahepatic bile ducts (Caroli disease) or congenital hepatic fibrosis. With advances in supportive care, many now survive into adulthood, yet the long-term natural history remains incompletely defined.