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

TitlePredictors of progression in autosomal dominant and autosomal recessive polycystic kidney disease.
Publication TypeJournal Article
Year of Publication2021
AuthorsBenz EG, Hartung EA
JournalPediatr Nephrol
Date Published2021 Jan 21
ISSN1432-198X
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.

DOI10.1007/s00467-020-04869-w
Alternate JournalPediatr Nephrol
PubMed ID33474686
Grant ListK23-DK109203 / DK / NIDDK NIH HHS / United States
T32-DK007785 / DK / NIDDK NIH HHS / United States