Among the reported cases of PAX2-related disorders, 147/234 (63.0%) individuals were diagnosed with RCS, 42/234 (18.0%) individuals were diagnosed with isolated CAKUT, 19/234 (8.4%) individuals were diagnosed with nephrosis, 2/234 (0.9%) individuals were clinically diagnosed with CAKUT and nephrosis, and 24/234 (10.3%) individuals were screened for genetic cause for CKD of unknown etiology. The gene discussed is PAX2; the disease is nephrosis.