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Med Sci (Paris). 2019 Aug-Sep;35(8-9):659-666. doi: 10.1051/medsci/2019128. Epub 2019 Sep 18.

[Idiopathic nephrotic syndrome: une Arlésienne?]

[Article in French; Abstract available in French from the publisher]

Author information

1
Inserm U1197, Interactions cellules souches-niches-physiologie, tumeurs et réparations tissulaires, Hôpital Paul Brousse, Bâtiment Lavoisier, 14, avenue Paul-Vaillant Couturier, 94800 Villejuif, France. - Université Paris-Saclay, Campus universitaire d'Orsay, 91 405 Orsay, France. - Service de néphrologie, CHU Bicêtre, 94270 Le Kremlin Bicêtre, France.
2
Inserm U1197, Interactions cellules souches-niches-physiologie, tumeurs et réparations tissulaires, Hôpital Paul Brousse, Bâtiment Lavoisier, 14, avenue Paul-Vaillant Couturier, 94800 Villejuif, France. - Université Paris-Saclay, Campus universitaire d'Orsay, 91 405 Orsay, France.

Abstract

in English, French

The renal filtration is ensured by the kidney glomeruli selective for filtering the blood. The main actor of the glomerular filter is the podocyte whose interlaced pedicels bear protein complexes (nephrin, podocin, etc.) creating a molecular sieve (slit diaphragm) to achieve the filtration. Alterations of these podocytes lead to massive proteinuria, which characterizes the nephrotic syndrome. The idiopathic form is one of the most malignant and essentially comprises two entities: minimal change disease and focal segmental glomerulosclerosis. Many observations indicated that (1) immune cells are involved and that (2) there are several permeability factors in the blood that affect the morphology and function of podocytes (slit diaphragm with fractional foot processes fusion/effacement). Evidence for a permeability factor was chiefly derived from remission of proteinuria observed after implantation of a kidney with FSGS in healthy recipients or with other kidney diseases. Today, we are moving towards a multifactorial conception of the nephrotic syndrome where all these barely known factors could be associated according to a sequential kinetic mechanism that needs to be determined.

PMID:
31532378
DOI:
10.1051/medsci/2019128

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