Predictors of progression in autosomal dominant and autosomal recessive polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) are characterized by bilateral cystic kidney disease leading to progressive kidney function decline. These diseases also have distinct liver manifestations. The range of clinical presentation and severity of both ADPKD and ARPKD is much wider than was once recognized. Pediatric and adult nephrologists are likely to care for individuals with both diseases in their lifetimes. This article will review genetic, clinical, and imaging predictors of kidney and liver disease progression in ADPKD and ARPKD and will briefly summarize pharmacologic therapies to prevent progression.
Author(s): Eric G. Benz [sup.1] [sup.2] , Erum A. Hartung [sup.1] [sup.2] Author Affiliations: (1) grid.239552.a, 0000 0001 0680 8770, Division of Nephrology, Children's Hospital of Philadelphia, , 3401 Civic [...]