Although 2 pediatric patients carried the same PAX2 variant (c.76dupG) and presented with the same CAKUT phenotype (bilateral cystic KHD), their clinical course was quite different: at the age of 4 years, CKD stage G3b and A3 albuminuria of 84 g/mol (B005-II.02) or CKD stage G2 and A2 albuminuria of 14 g/mol (B061-II.01) (Table 2, and Figures 1 and 3). Here, PAX2 is linked to chronic kidney disease.