PROPKD Score
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:1
PROPKD Calculator
Variable
Points
being male
1
hypertension before 35 years of age
2
first urologic event* before 35 years of age
2
mutation
PKD2 mutation
0
nontruncating PKD1 mutation
2
truncating PKD1 mutation
4
PROPKD Score =
SUM
Sample PROPKD Score Calculation
ADPKD patient info: 29 year old male with hypertension and a truncating PKD1 mutation
1 point for being male
2 points for hypertension before 35 years of age
4 points for a truncating PKD1 mutation
7 points PROPKD Score
HIGH Risk of Progression to ESRD
Total Points: PROPKD Score
Risk of Progression to ESRD
0
1
2
3
LOW
70.6 median age for ESRD onset
Eliminates evolution to ESRD before age 60†
†negative predictive value of 81.4%
4
5
6
INTERMEDIATE
56.9 median age for ESRD onset
7
8
9
HIGH
49 median age for ESRD onset
Forecasts ESRD onset before age 60‡
‡positivepredictive value of 90.9%
