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From Immune System Damage to Podocyte Cell Damage: Prospective Database and Biological Collection of Patients (Children and Adults) With MGLS and HSFP

Recruitingn/aAges 12 Months - anyAllCentre Hospitalier Universitaire de Nice
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In plain words

What the trial is, and whether it could suit you

Idiopathic Nephrotic Syndrome (INS) is a kidney disease characterized by massive proteinuria and hypoalbuminemia. It includes two anatomopathological entities: nephrotic syndrome with minimal glomerular lesions (SNLGM) and primary segmental and focal hyalinosis (PHF). Renal biopsy reveals a fusion of the feet of the podocytes without inflammatory lesions or deposits of immune complexes. Clinical and experimental observations strongly suggest that the immune system and podocyte dysfunction are the two facets of the disease. There are currently no clinical or biological markers to predict the diagnosis of corticosteroid sensitivity, corticosteroid dependence, or risk of recurrence of kidney disease after kidney transplantation. To our knowledge, no prospective studies have been designed to study both immune system alterations and podocyte damage as well as genetic predisposition variants i

Sign in to get the eligibility rules as simple questions to take to your doctor.

๐Ÿ“‹ Eligibility rules (as published)
Inclusion Criteria: * First episode of idiopathic nephrotic syndrome in a child aged 12 months to 18 years with a weight \> 10 kg and biologically defined as proteinuria \> 0.20 g / mmol creatinuria and hypoalbuminemia \<25 g / mmol * First episode of NIS defined in adults as albumin level \<30 g / L and a urinary protein/creatinine ratio (UPCR) โ‰ฅ 300 mg / mmol systematically associated with the performance of a renal anatomopathological examination and characterized by the absence of identifiable lesions by light microscopy (SNLGM) or the presence of HSF lesions. * For adults: signed informed consent to participate in the study * For children: patients will be informed and a written informed consent form will be signed by both parents of the children at inclusion * Patients affiliated to the French health system Exclusion Criteria: * Patients who have previously received corticosteroids and/or immunosuppressants * Patients with reduced CH50 and/or low C3 and/or low C4 and/or low C4 (in some cases increased sC5b9 and/or presence of a C3 nephritic Factor; an anti-C3b...) * SNLGM resulting from a secondary process (lymphoid hematopathies or neoplasia) or occurring following the administration of a treatment known to be associated with an SNLGM (lithium, interferon, non-steroidal anti-inflammatory drugs) * Non-primary FHH (absence of Nephrotic Syndrome, etiological assessment revealing FHH secondary to an identified cause (genetic or not), no introduction of corticosteroids or immunosuppressants as first-line treatment) * Positive serological screening test for HIV, hepatitis B or C * Positive immunological tests for antinuclear and anti-DNA antibodies or anti-PLA2R1 and anti-THSD7A * Patient under guardianship or curatorship
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What it tests

  • blood sample
  • tissue sample
  • excreta
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Where

2 sites
Nice Hospital
Nice, France
APHP
Paris, France
๐Ÿค Taking part is your choice, and you can leave at any time. Only the trial team can confirm you are eligible โ€” always decide together with your own doctor.
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