NPHS4, previously called sporadic isolated steroid-resistant NS isolated diffuse mesangial sclerosis, is associated with rapid progression to end-stage renal disease (ESRD) early in life (Jeanpierre et al. 1998; Schumacher et al. 1998; Ito et al. 2001, 2010; Benetti et al. 2010; Yang et al. 2013; Lipska et al. 2014; Dome and Huff 2016). This evidence concerns the gene WT1 and Diffuse mesangial sclerosis.