Idiopathic nephrotic syndrome and serum permeability factors: a molecular jigsaw puzzle
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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.
KeywordsNephrotic syndrome Permeability factors Minimal change disease Focal segmental glomerulosclerosis Proteinuria
Serum circulating factor
Idiopathic nephrotic syndrome
Focal segmental glomerulosclerosis
Recurrent focal segmental glomerulosclerosis
Minimal change disease
Soluble urokinase plasminogen activator receptor
Hans K. Lorenzo was supported by the “ Fondation du Rein”/“Fondation pour la Recherche Médicale”, grant number R16099LL.
Compliance with ethical statements
Conflict of interest
The authors declare that they have no competing interests.
This article does not contain any studies with human participants or animals performed by any of the authors.
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