title: Predictors of rapid disease progression in autosomal dominant polycystic kidney disease.
abstract: Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common genetic diseases with a reported prevalence of 1:400 to 1:1000. Since the intact kidneys can compensate for the loss of glomerular filtration in ADPKD patients, renal insufficiency usually remains undetected until almost the fourth decade of life. Hereafter, reliable diagnostic and prognostic biomarkers to identify ADPKD progression are urgently needed. Several studies and systematic reviews tried to identify markers or predictors of rapid disease progression of ADPKD. The aim of this study is to review predictors of rapid disease progression of ADPKD that can be useful to the clinician. We will describe several factors associated with rapid progression of ADPKD derived from retrospective or cross-sectional studies, suggesting the best and most useful predictors that may help to patients management in clinical practice. We will attempt to identify the most useful predictors of rapid disease progression of ADPKD: established TKV growth rate >5% per year, annual estimated glomerular filtration rate decline >5 mL/min/1.73 m2, truncating PKD1 mutations and elevated plasma copeptin level. The combination of several factors that can predict the rapid ADPKD progression is more accurate than a single-marker strategy. The "PRO-PKD" risk scoring system combined with TKV, can be useful in order to evaluate the ADPKD patients and they appear to be appropriate predictors of progression disease. Moreover levels of copeptin and some urinary markers can be matched to these factors for improved patient assessment in rapid progression.
authors.affiliation: Department of Nephrology, Dialysis and Transplantation, San Bortolo Hospital, Vicenza, Italy - valentina.corradi@ulssvicenza.it.|International Renal Research Institute (IRRIV), San Bortolo Hospital, Vicenza, Italy - valentina.corradi@ulssvicenza.it.
authors.forename: Valentina
authors.initials: V
authors.lastname: Corradi
authors.affiliation: Department of Nephrology, Dialysis and Transplantation, San Bortolo Hospital, Vicenza, Italy.
authors.forename: Fiorella
authors.initials: F
authors.lastname: Gastaldon
authors.affiliation: International Renal Research Institute (IRRIV), San Bortolo Hospital, Vicenza, Italy.
authors.forename: Carlotta
authors.initials: C
authors.lastname: Caprara
authors.affiliation: Department of Nephrology, Dialysis and Transplantation, San Bortolo Hospital, Vicenza, Italy.
authors.forename: Anna
authors.initials: A
authors.lastname: Giuliani
authors.affiliation: Department of Nephrology, Dialysis and Transplantation, San Bortolo Hospital, Vicenza, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Martino
authors.affiliation: International Renal Research Institute (IRRIV), San Bortolo Hospital, Vicenza, Italy.
authors.forename: Fiorenza
authors.initials: F
authors.lastname: Ferrari
authors.affiliation: Department of Nephrology, Dialysis and Transplantation, San Bortolo Hospital, Vicenza, Italy.|International Renal Research Institute (IRRIV), San Bortolo Hospital, Vicenza, Italy.
authors.forename: Claudio
authors.initials: C
authors.lastname: Ronco
mesh_terms: D065092:Antidiuretic Hormone Receptor Antagonists / Q000627:therapeutic use; D001552:Benzazepines / Q000627:therapeutic use; D015415:Biomarkers / Q000097:blood / Q000652:urine; D002986:Clinical Trials as Topic; D003430:Cross-Sectional Studies; D018450:Disease Progression; D005260:Female; D005919:Glomerular Filtration Rate; D006801:Humans; D007668:Kidney / Q000473:pathology; D008297:Male; D009929:Organ Size; D016891:Polycystic Kidney, Autosomal Dominant / Q000188:drug therapy / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D012084:Renin-Angiotensin System / Q000187:drug effects; D012189:Retrospective Studies; D050396:TRPP Cation Channels / Q000235:genetics; D000077602:Tolvaptan
chemical_list: D065092:Antidiuretic Hormone Receptor Antagonists; D001552:Benzazepines; D015415:Biomarkers; D050396:TRPP Cation Channels; D000077602:Tolvaptan