The first variant is a WT1 missense mutation: NM_024426.6; exon 9; c.1400G>A (p.Arg467Gln), affecting a known hotspot residue within the third zinc finger domain and previously associated with severe WT1-related nephropathy (formerly classified as DDS subtype) (Figure 1B) (2, 13). The gene discussed is WT1; the disease is Nephropathy.