Out of these cases, four of them presented PAX2 mutations with FSGS renal histopathology: a 15-year-old boy with FSGS (AD)/multicystic dysplastic kidney (c.419G>T, p.Arg140Leu), a 2-year-old boy with RCS (c.69delC, pVal26CysfsX2), a 20-year-old female with ESRD due to FSGS (c.70_71insG, p.Val26Glyfx*28) and an 11-year-old boy with IgA nephropathy or FSGS (c.1178G>C, pArg393Pro) (Table 1) [50]. Here, PAX2 is linked to IgA glomerulonephritis.