Nephrotic Syndrome Clinical Trial
Official title:
Diagnostic and Prognostic Value of Miss-1 Study in Children and Adult With Nephrotic Syndrome MISSNEPHROTIQUE
The nephrotic syndrome is a rare disease defined by a proteinuria >3g/24h and a
hypoalbuminemia < 30g/L. Genetic and immune are the main causes. The acquired idiopathic
nephrotic syndrome presents histologically minimal glomerular lesions, sometimes associated
with segmental and focal hyalinosis. The idiopathic nephrotic syndrome (INS) represents 85%
of children's glomerular nephropathy and 25-30% of adult's.
Relapses are frequents, and can be pejorative up to 10% and lead to end-stage kidney failure.
Another immune cause is the extramembranous glomerulonephritis mediated by molecular targets
specific autoantibodies expressed at the podocytes surface.
Other immune causes include lupus nephropathy, ANCA vascularitis, Goodpasture disease, Berger
disease.
Easy diagnosis between these causes can be made with the renal biopsy.
Miss-1, a new protein activated during a inflammatory event, could be an actor in nephrotic
syndromes by modifying the podocyte's adhesion on the glomerular basal membrane. This would
modulate the structure and function of the slit diaphragm, as well as junctions between the
podocyte and the glomerular basal membrane, regulating podocytes' apoptosis.
This project is meant to propose and validate specific and non-invasive diagnostic and
prognostic tests for the acquired idiopathic nephrotic syndrome.
These tests rely on the measure of Miss-1 expression in circulating blood cells on flow
cytometry and its plasmatic concentration.
To date, no equivalent tests exist to diagnose idiopathic nephrotic syndrome (INS) from other
causes.
These simple tests would allow a quick diagnosis of acquired INS by avoiding an invasive
renal biopsy. It would also help anticipate the relapses of the disease and guide the
treatment modalities as do nowadays the PLA2R antibodies in idiopathic membranous
nephropathy.
We will propose the tests to every consent patient, hospitalized in the participating centers
(Néphrologie pédiatrique of Robert Debré hospital, Néphrologie adulte of Tenon hospital) with
a nephrotic syndrome.
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