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Idiopathic nephrotic syndrome and serum permeability factors: a molecular jigsaw puzzle

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Abstract

Nephrotic syndrome is traditionally defined using the triad of edema, hypoalbuminemia, and proteinuria, but this syndrome is very heterogeneous and difficult to clarify. Its idiopathic form (INS) is probably the most harmful and essentially comprises two entities: minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS). We will consider some hypotheses regarding the mechanisms underlying INS: (i) the presence of several glomerular permeability factors in the sera of patients that alter the morphology and function of podocytes leading to proteinuria, (ii) the putative role of immune cells. Thanks to recent data, our understanding of these disorders is evolving towards a more multifactorial origin. In this context, circulating factors may be associated according to sequential kinetic mechanisms or micro-environmental changes that need to be determined. In addition, the resulting proteinuria may trigger more proteinuria enhancing the glomerular destabilization.

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Fig. 1

Abbreviations

SCF:

Serum circulating factor

INS:

Idiopathic nephrotic syndrome

FSGS:

Focal segmental glomerulosclerosis

rFSGS:

Recurrent focal segmental glomerulosclerosis

MCD:

Minimal change disease

CLC-1:

Cardiotrophin-like cytokine-1

suPAR:

Soluble urokinase plasminogen activator receptor

APOL1:

Apolipoprotein A-I

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Correspondence to Hans-Kristian Lorenzo.

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Candelier, J., Lorenzo, H. Idiopathic nephrotic syndrome and serum permeability factors: a molecular jigsaw puzzle. Cell Tissue Res 379, 231–243 (2020) doi:10.1007/s00441-019-03147-y

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Keywords

  • Nephrotic syndrome
  • Permeability factors
  • Minimal change disease
  • Focal segmental glomerulosclerosis
  • Proteinuria