Table 5.

Characteristics of patients with NS classified with monogenic NS

SubjectAncestryHistologyGeneGenotypeAmino Acid ChangeHGMDPediatricRemissionImmunosuppressive AgentRAAS Agent
1AFRMCDNPHS1HOM_ALTp.A765VNoYesCompleteNoneYes
2AMRFSGSTRPC6HETp.N765SNoYesCompleteSteroidYes
3AFRMCDCD2APHETp.M496IYesYesCompleteSteroidNo
4EURIgM nephropathyWT1HETp.C350RNoYesCompleteSteroid, tacrolimus, MMFNo
5EURICGNINF2HETp.E220KYesYesNoneNoneYes
6EURFSGSNPHS2HOM_ALTp.A284VYesYesNoneNoneYes
7EURMCDINF2HETp.A13TYesNoCompleteSteroidYes
8EURFSGSTRPC6HETp.D798HNoNoPartialNoneYes
LMX1BHETp.D374NNo
9AMRFSGSINF2HETp.P35RNoNoPartialSteroid, CNIYes
  • AFR, African 1000G continental ancestry; AMR, American 1000G continental ancestry; EUR, European continental ancestry; MCD, minimal change disease; ICGN, immune complex GN; HOM_ALT, homozygous for the alternative allele; HET, heterozygous variant; MMF, mycophenolate mofetil; CNI, calcineurin inhibitor; RAAS, renin-angiotensin-aldosterone system.