Congenital nephrotic syndrome type 10
Congenital nephrotic syndrome type 10 is an autosomal recessive disorder caused by mutations of the EMP2 gene. The nephrotic syndrome manifests before 5 years of age. It may respond to immunosuppressive therapy but often relapses.
Proteinurie often develops into nephrotic range, it response to immunosuppressive therapy, but often relapses.
Gee HY et al. (2014) Mutations in EMP2 cause childhood-onset nephrotic syndrome.[^]