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Médecine thérapeutique / Pédiatrie

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Physiopathology of idiopathic nephrotic syndrome Volume 18, issue 4, October-November-December 2015

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Authors
AP-HP
Hôpital universitaire Robert-Debré
Service de néphrologie pédiatrique
48, boulevard Sérurier
75935 Paris cedex 19
France
* Tirés à part

Idiopathic nephrotic syndrome is a worldwide disease, the only sign of which is massive proteinuria, subsequently marked by increased renal albumin clearance by a factor of between 1,000 and 10,000. The only lesion is an eradication of podocyte arborisation, which is visible exclusively under ultrastructural microscopy. These alterations are functional and regress completely after a few days of treatment with prednisone. The current hypothesis is that nephrotic syndrome is not a primary kidney disease but an immune disease for which the kidney is the functional and exclusive target. Immune system abnormalities are global and either affect humoral or cellular immunity, as well as bone marrow progenitor cells. The link between the immune system and the glomerular wall is likely to be a soluble circulating glomerular factor, although this has not yet been identified. The involvement of immunoglobulin, in reality, an auto-antibody, is plausible but so far unclear. The epidemiology of the disease in the Paris area suggests that environmental factors, in particular viral infections, also play a major role in triggering the immune mechanisms of disease.