From Immune System Damage to Podocyte Cell Damage: Prospective Database and Biological Collection of Patients (Children and Adults) With MGLS and HSFP
BiobankSNI
1 other identifier
observational
144
1 country
2
Brief Summary
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 in NIS. Therefore, the use of steroids/immunosuppressive agents is purely empirical with a multitude of side effects. The objective is to identify and test new therapeutic targets rather than conducting new trials with existing treatments, using either drug candidates or molecules selected by high throughput screening of libraries of repositioning molecules using an appropriate read-out. The biobank may also be used to analyze the effects of conventional treatments on identified new biomarkers. We expect the project to produce original and patentable results with subsequent valuation. Patentability will be anticipated before any publication on the subject. The patent and valorization cells of hospitals, INSERM and Universities will be involved in the results as soon as they are obtained.
Trial Health
Trial Health Score
Automated assessment based on enrollment pace, timeline, and geographic reach
participants targeted
Target at P50-P75 for all trials
Started May 2020
Longer than P75 for all trials
2 active sites
Health score is calculated from publicly available data and should be used for screening purposes only.
Trial Relationships
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Study Timeline
Key milestones and dates
First Submitted
Initial submission to the registry
November 19, 2019
CompletedFirst Posted
Study publicly available on registry
November 22, 2019
CompletedStudy Start
First participant enrolled
May 20, 2020
CompletedPrimary Completion
Last participant's last visit for primary outcome
May 20, 2021
CompletedStudy Completion
Last participant's last visit for all outcomes
May 20, 2025
CompletedOctober 19, 2020
November 1, 2019
1 year
November 19, 2019
October 15, 2020
Conditions
Outcome Measures
Primary Outcomes (1)
number of biological samples
establish in each centre a prospective collection of blood, urine and kidney biopsy samples sampled and stored in accordance with standard operating procedures, supported by a harmonized clinical and biological database
5 years
Study Arms (2)
SNLGM
70 patients with an SNLGM
primary FSH
74 with a primary FSH
Interventions
Eligibility Criteria
patient with Idiopathic Nephrotic Syndrome
You may qualify if:
- 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) ≥ 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
- Patients affiliated to the French health system
You may not qualify if:
- 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
Contact the study team to confirm eligibility.
Sponsors & Collaborators
Study Sites (2)
Nice Hospital
Nice, 06000, France
APHP
Paris, 75000, France
Biospecimen
Blood: whole blood / plasma / serum collected specifically for the Biobank but during a blood test planned in the follow-up of patients. Tissue sample: slide of biopsies taken during the treatment Excreta: urine
MeSH Terms
Conditions
Interventions
Condition Hierarchy (Ancestors)
Intervention Hierarchy (Ancestors)
Central Study Contacts
Study Design
- Study Type
- observational
- Observational Model
- COHORT
- Time Perspective
- PROSPECTIVE
- Sponsor Type
- OTHER
- Responsible Party
- SPONSOR
Study Record Dates
First Submitted
November 19, 2019
First Posted
November 22, 2019
Study Start
May 20, 2020
Primary Completion
May 20, 2021
Study Completion
May 20, 2025
Last Updated
October 19, 2020
Record last verified: 2019-11
Data Sharing
- IPD Sharing
- Will not share