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J Am Soc Nephrol. 2016 Mar;27(3):942-51. doi: 10.1681/ASN.2015010016. Epub 2015 Jul 6.

The PROPKD Score: A New Algorithm to Predict Renal Survival in Autosomal Dominant Polycystic Kidney Disease.

Author information

1
Department of Nephrology, University Hospital, Brest, France; European University of Brittany, Brest, France; National Institute of Health and Medical Sciences, INSERM U1078, Brest, France;
2
National Institute of Health and Medical Sciences, INSERM U1078, Brest, France; Department of Molecular Genetics, University Hospital, Brest, France;
3
Department of Pharmacology, INSERM U850, University Hospital, Limoges, France;
4
Department of Nephrology, University Hospital, Nantes, France;
5
Department of Nephrology, Broussais Hospital, Saint Malo, France;
6
Department of Nephrology, Yves Le Foll Hospital, Saint Brieuc, France;
7
Department of Nephrology, University Hospital, Rennes, France;
8
Department of Nephrology, University Hospital, Brest, France;
9
Department of Nephrology, Laennec Hospital, Quimper, France;
10
Department of Nephrology, Vannes Hospital, Vannes, France;
11
Department of Nephrology, Pontivy Hospital, Pontivy, France;
12
AUB Santé, Brest, France;
13
SBRA, Hemodialysis Unit, Brest, France;
14
Department of Nephrology, Lorient Hospital, Lorient, France;
15
AUB Santé, Lorient, France;
16
ECHO Dialysis Unit, Vannes, France;
17
AUB Santé, Quimper, France;
18
Department of Nephrology, Saint Nazaire Hospital, Saint Nazaire, France;
19
Department of Nephrology, University Hospital, Tours, France; and.
20
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.
21
Department of Nephrology, University Hospital, Brest, France; European University of Brittany, Brest, France; yannick.lemeur@chu-brest.fr.

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.

KEYWORDS:

ADPKD; end-stage renal disease; genetic renal disease; progression of renal failure; risk factors

PMID:
26150605
PMCID:
PMC4769200
[Available on 2017-03-01]
DOI:
10.1681/ASN.2015010016
[Indexed for MEDLINE]
Free PMC Article

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