While years ago it was felt that the course of polycystic kidney disease was totally unpredictable [16], more recent studies have shown that mutations in PKD1, male sex, early onset hypertension and macrohematuria, baseline GFR, albuminuria, renal cyst volume and uric acid are among the most important factors which predict more severe disease progression and an earlier onset of ESRD [8]–[11], [17]–[23]. Here, PKD1 is linked to polycystic kidney disease.