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Pediatr Nephrol. 2012 Aug;27(8):1249-56. doi: 10.1007/s00467-011-1947-1. Epub 2011 Jun 28.

Physiopathology of idiopathic nephrotic syndrome: lessons from glucocorticoids and epigenetic perspectives.

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Department of Pediatric Pharmacology and Pharmacogenetics, Clinical Investigation Center CIC Inserm 9202, Hôpital Robert Debré, 48 Boulevard Sérurier, 75935 Paris Cedex 19, France.


Idiopathic nephrotic syndrome (INS) has been studied for decades in attempt to understand the physiopathological mechanisms explaining the disease. It is recognized as a multifactorial disease, with immunological components targeting kidney functions. Many hypotheses have been discussed or tested, including the role of a circulating factor, polymorphisms of genes implicated in lymphocyte maturation and differentiation, and DNA epigenetic modifications. In the present review, the data supporting these different (and probably combinatorial) hypotheses have been reviewed in order to identify and discuss the possible pathways implicated in the physiopathology of INS.

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