Table 1.

Highlights of the observations obtained from the CRISP study program

Liver cystic disease: 95% prevalence of liver cystic disease by age 35, women more often than men (9)
Genotype: PKD1 individuals demonstrate larger kidneys with more cysts than age-matched PKD2 individuals; rate of change in cyst growth are similar between PKD1 and PKD2 individuals (11,12)
Hypertension: Hypertensive ADPKD individual demonstrate larger kidney volumes than age-matched normotensive ADPKD individuals that grow at a faster rate (5,47)
Kidney volume: Increases over time with growth due to cyst expansion; associated with proteinuria and reduction in GFR (8)
Renal blood flow: Is reduced early in ADPKD, before loss of renal function, and is associated with increased renal size (10,48)
Ultrasound versus MRI: Demonstrates reduced reliability and reproducibility with ultrasound and decreasing correlation with MRI-based estimates of renal volume as kidney size increases (49)
MDRD estimates of GFR in ADPKD individuals with intact renal function: are less reliable than other regression models using serum creatinine concentration (50)
  • MRI, magnetic resonance imaging.