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

Pediatr Nephrol. 2021 Sep;36(9):2639-2658. doi: 10.1007/s00467-020-04869-w. Epub 2021 Jan 21.

Abstract

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.

Keywords: Children; Outcomes; Polycystic kidney disease; Progression; Stage 5 chronic kidney disease.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Adult
  • Child
  • Disease Progression
  • Humans
  • Polycystic Kidney, Autosomal Dominant* / diagnostic imaging
  • Polycystic Kidney, Autosomal Dominant* / drug therapy
  • Polycystic Kidney, Autosomal Dominant* / genetics
  • Polycystic Kidney, Autosomal Dominant* / pathology
  • Polycystic Kidney, Autosomal Recessive* / diagnostic imaging
  • Polycystic Kidney, Autosomal Recessive* / drug therapy
  • Polycystic Kidney, Autosomal Recessive* / genetics
  • Polycystic Kidney, Autosomal Recessive* / pathology