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. 2009 Feb 27;24(11):2097–2108. doi: 10.1007/s00467-009-1137-6

Table 3.

Two groups of steroid-resistant nephrotic syndromes (SRNS) leading to end-stage renal disease in children (GBM glomerular basement membrane, Sd syndrome)

Primary idiopathic SRNS Inherited SRNS
A multifactorial disease A renal disease
T-cell dysregulation Abnormal podocyte–GBM complex
Podocyte as a target Podocyte as a disease
Idiopathic FSGS Genetic forms of NS Syndromic forms of NS
Finnish type NS (NPHS1) WT1-associated SRNS
Other recessive FSGS Pierson Sd, Schimke Sd
(NPHS2, NPHS3) Charcot–Marie–Tooth SRNS
Dominant FSGS
(ACTN4, TRPC6, CD2AP)
Circulating factor No circulating factor
Immunosuppression available No effect of immunosuppression