title: Predictors of autosomal dominant polycystic kidney disease progression.
abstract: Autosomal dominant polycystic kidney disease is a genetic disorder associated with substantial variability in its natural course within and between affected families. Understanding predictors for rapid progression of this disease has become increasingly important with the emergence of potential new treatments. This systematic review of the literature since 1988 evaluates factors that may predict and/or effect autosomal dominant polycystic kidney disease progression. Predicting factors associated with early adverse structural and/or functional outcomes are considered. These factors include PKD1 mutation (particularly truncating mutation), men, early onset of hypertension, early and frequent gross hematuria, and among women, three or more pregnancies. Increases in total kidney volume and decreases in GFR and renal blood flow greater than expected for a given age also signify rapid disease progression. Concerning laboratory markers include overt proteinuria, macroalbuminuria, and perhaps, elevated serum copeptin levels in affected adults. These factors and others may help to identify patients with autosomal dominant polycystic kidney disease who are most likely to benefit from early intervention with novel treatments.
authors.affiliation: Division of Renal Diseases and Hypertension, University of Colorado School of Medicine, Aurora, Colorado; and robert.schrier@ucdenver.edu.
authors.forename: Robert W
authors.initials: RW
authors.lastname: Schrier
authors.affiliation: Division of Renal Diseases and Hypertension, University of Colorado School of Medicine, Aurora, Colorado; and.
authors.forename: Godela
authors.initials: G
authors.lastname: Brosnahan
authors.affiliation: Division of Renal Diseases and Hypertension, University of Colorado School of Medicine, Aurora, Colorado; and.
authors.forename: Melissa A
authors.initials: MA
authors.lastname: Cadnapaphornchai
authors.affiliation: Division of Renal Diseases and Hypertension, University of Colorado School of Medicine, Aurora, Colorado; and.
authors.forename: Michel
authors.initials: M
authors.lastname: Chonchol
authors.affiliation: Cardiorenal Department, Medical Affairs, Otsuka America Pharmaceutical, Inc., Princeton, New Jersey.
authors.forename: Keith
authors.initials: K
authors.lastname: Friend
authors.affiliation: Division of Renal Diseases and Hypertension, University of Colorado School of Medicine, Aurora, Colorado; and.
authors.forename: Berenice
authors.initials: B
authors.lastname: Gitomer
authors.affiliation: Cardiorenal Department, Medical Affairs, Otsuka America Pharmaceutical, Inc., Princeton, New Jersey.
authors.forename: Sandro
authors.initials: S
authors.lastname: Rossetti
mesh_terms: D018450:Disease Progression; D006801:Humans; D007668:Kidney / Q000473:pathology* / Q000503:physiopathology*; D016891:Polycystic Kidney, Autosomal Dominant / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D011237:Predictive Value of Tests; D050396:TRPP Cation Channels / Q000235:genetics*
chemical_list: D050396:TRPP Cation Channels
keywords: PKD1/PKD2; autosomal dominant polycystic kidney disease; disease progression; hypertension; renal blood flow; total kidney volume