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.
Proteinuria | |
Proteinurie often develops into nephrotic range, it response to immunosuppressive therapy, but often relapses. |
1. |
Gee HY et al. (2014) Mutations in EMP2 cause childhood-onset nephrotic syndrome. |
2. |
OMIM.ORG article Omim 615861 |