title: The PROPKD Score: A New Algorithm to Predict Renal Survival in Autosomal Dominant Polycystic Kidney Disease.
abstract: The course of autosomal dominant polycystic kidney disease (ADPKD) varies among individuals, with some reaching ESRD before 40 years of age and others never requiring RRT. In this study, we developed a prognostic model to predict renal outcomes in patients with ADPKD on the basis of genetic and clinical data. We conducted a cross-sectional study of 1341 patients from the Genkyst cohort and evaluated the influence of clinical and genetic factors on renal survival. Multivariate survival analysis identified four variables that were significantly associated with age at ESRD onset, and a scoring system from 0 to 9 was developed as follows: being male: 1 point; hypertension before 35 years of age: 2 points; first urologic event before 35 years of age: 2 points; PKD2 mutation: 0 points; nontruncating PKD1 mutation: 2 points; and truncating PKD1 mutation: 4 points. Three risk categories were subsequently defined as low risk (0-3 points), intermediate risk (4-6 points), and high risk (7-9 points) of progression to ESRD, with corresponding median ages for ESRD onset of 70.6, 56.9, and 49 years, respectively. Whereas a score ≤3 eliminates evolution to ESRD before 60 years of age with a negative predictive value of 81.4%, a score >6 forecasts ESRD onset before 60 years of age with a positive predictive value of 90.9%. This new prognostic score accurately predicts renal outcomes in patients with ADPKD and may enable the personalization of therapeutic management of ADPKD.
authors.affiliation: Department of Nephrology, University Hospital, Brest, France; European University of Brittany, Brest, France; National Institute of Health and Medical Sciences, INSERM U1078, Brest, France;
authors.forename: Emilie
authors.initials: E
authors.lastname: Cornec-Le Gall
authors.affiliation: National Institute of Health and Medical Sciences, INSERM U1078, Brest, France; Department of Molecular Genetics, University Hospital, Brest, France;
authors.forename: Marie-Pierre
authors.initials: MP
authors.lastname: Audrézet
authors.affiliation: Department of Pharmacology, INSERM U850, University Hospital, Limoges, France;
authors.forename: Annick
authors.initials: A
authors.lastname: Rousseau
authors.affiliation: Department of Nephrology, University Hospital, Nantes, France;
authors.forename: Maryvonne
authors.initials: M
authors.lastname: Hourmant
authors.affiliation: Department of Nephrology, Broussais Hospital, Saint Malo, France;
authors.forename: Eric
authors.initials: E
authors.lastname: Renaudineau
authors.affiliation: Department of Nephrology, Yves Le Foll Hospital, Saint Brieuc, France;
authors.forename: Christophe
authors.initials: C
authors.lastname: Charasse
authors.affiliation: Department of Nephrology, University Hospital, Rennes, France;
authors.forename: Marie-Pascale
authors.initials: MP
authors.lastname: Morin
authors.affiliation: Department of Nephrology, University Hospital, Brest, France;
authors.forename: Marie-Christine
authors.initials: MC
authors.lastname: Moal
authors.affiliation: Department of Nephrology, University Hospital, Nantes, France;
authors.forename: Jacques
authors.initials: J
authors.lastname: Dantal
authors.affiliation: Department of Nephrology, Laennec Hospital, Quimper, France;
authors.forename: Bassem
authors.initials: B
authors.lastname: Wehbe
authors.affiliation: Department of Nephrology, Vannes Hospital, Vannes, France;
authors.forename: Régine
authors.initials: R
authors.lastname: Perrichot
authors.affiliation: Department of Nephrology, University Hospital, Rennes, France;
authors.forename: Thierry
authors.initials: T
authors.lastname: Frouget
authors.affiliation: Department of Nephrology, University Hospital, Rennes, France;
authors.forename: Cécile
authors.initials: C
authors.lastname: Vigneau
authors.affiliation: Department of Nephrology, Yves Le Foll Hospital, Saint Brieuc, France;
authors.forename: Jérôme
authors.initials: J
authors.lastname: Potier
authors.affiliation: Department of Nephrology, Pontivy Hospital, Pontivy, France;
authors.forename: Philippe
authors.initials: P
authors.lastname: Jousset
authors.affiliation: AUB Santé, Brest, France;
authors.forename: Marie-Paule
authors.initials: MP
authors.lastname: Guillodo
authors.affiliation: Department of Nephrology, Laennec Hospital, Quimper, France;
authors.forename: Pascale
authors.initials: P
authors.lastname: Siohan
authors.affiliation: SBRA, Hemodialysis Unit, Brest, France;
authors.forename: Nazim
authors.initials: N
authors.lastname: Terki
authors.affiliation: Department of Nephrology, Lorient Hospital, Lorient, France;
authors.forename: Théophile
authors.initials: T
authors.lastname: Sawadogo
authors.affiliation: AUB Santé, Lorient, France;
authors.forename: Didier
authors.initials: D
authors.lastname: Legrand
authors.affiliation: ECHO Dialysis Unit, Vannes, France;
authors.forename: Victorio
authors.initials: V
authors.lastname: Menoyo-Calonge
authors.affiliation: AUB Santé, Quimper, France;
authors.forename: Seddik
authors.initials: S
authors.lastname: Benarbia
authors.affiliation: Department of Nephrology, Saint Nazaire Hospital, Saint Nazaire, France;
authors.forename: Dominique
authors.initials: D
authors.lastname: Besnier
authors.affiliation: Department of Nephrology, University Hospital, Tours, France; and.
authors.forename: Hélène
authors.initials: H
authors.lastname: Longuet
authors.affiliation: European University of Brittany, Brest, France; National Institute of Health and Medical Sciences, INSERM U1078, Brest, France; Department of Molecular Genetics, University Hospital, Brest, France; EFS Bretagne, Brest, France.
authors.forename: Claude
authors.initials: C
authors.lastname: Férec
authors.affiliation: Department of Nephrology, University Hospital, Brest, France; European University of Brittany, Brest, France; yannick.lemeur@chu-brest.fr.
authors.forename: Yannick
authors.initials: Y
authors.lastname: Le Meur
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D000465:Algorithms; D019540:Area Under Curve; D003430:Cross-Sectional Studies; D018450:Disease Progression; D005260:Female; D005838:Genotype; D005919:Glomerular Filtration Rate; D006801:Humans; D006973:Hypertension / Q000150:complications*; D007676:Kidney Failure, Chronic / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D009154:Mutation; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications* / Q000235:genetics* / Q000503:physiopathology; D011237:Predictive Value of Tests; D011379:Prognosis; D016016:Proportional Hazards Models; D011507:Proteinuria / Q000209:etiology*; D012372:ROC Curve; D012307:Risk Factors; D012737:Sex Factors; D015996:Survival Rate; D050396:TRPP Cation Channels / Q000235:genetics; D055815:Young Adult
chemical_list: D050396:TRPP Cation Channels
keywords: ADPKD; end-stage renal disease; genetic renal disease; progression of renal failure; risk factors