PKD is characterized by progressive cystic dilation of the renal tubules, which results in
nephromegaly and often culminates in end-stage renal disease. PKD can be inherited as
either an autosomal dominant trait (ADPKD) or autosomal recessive trait (ARPKD). Since
our last review on this subject in 2002 (1), there have been tremendous advances in the
understanding of the genetics and pathogenesis of PKD. The localization of the PKD
proteins polycystin-1, polycystin-2, and fibrocystin in the primary cilium has renewed …