This is a multicentric retrospective observational cohort study.
As primary objective, the study aims to evaluate the factors associated with nephrotic syndrome remission in patient with nephrotic syndrome, biopsy-prove minimal change disease or focal segmental glomerulosclerosis, and an at-risk variant of the APOL1 gene.
As secondary objectives, this study aims:
* To evaluate the benefit of corticosteroids in obtaining the remission of nephrotic syndrome * To identify the predictors of complete renal remission of nephrotic syndrome * To evaluate the benefit of corticosteroids in reducing the incidence of end-stage renal disease * To assess the adverse events of corticosteroids in patients treated with corticosteroids.
Adult patients followed in 6 nephrology centers between 01/01/2016 and 01/06/2024.
With characterization of APOL1 gene risk status
Proteinuria/creatinuria ratio > 3 g/g at diagnosis of renal disease (within 48 hours of the diagnostic renal biopsy)
Hypoalbuminemia < 30 g/L at diagnosis of renal disease (within 48 h of diagnostic renal biopsy)
Presence of diffuse deposits of immunoglobulins or complement fractions on immunofluorescence study
Presence of endo- or extracapillary hypercellular lesions on light microscopy
Opposition to the use of medical data
Patients APOL1 gene status will be obtained from the Hôpital Européen Georges Pompidou genetic laboratory (performing all APOL1 gene analysis in Paris greater area). Patients with a biopsy-proven focal segmental glomerulosclerosis or minimal change disease and nephrotic syndrome will be screened in each center.
Cohort
Retrospective
Not listed
nephrotic syndrome remission
Description: remission of nephrotic syndrome is defined as a complete or partial remission of nephrotic syndrome as follows: * Complete remission: * Urine protein / creatinine ratio \< 0.3 g/g AND * Albuminemia \> 35 g/L * Partial remission: * Urine protein / creatinine ratio \> 0.3 g/g AND * Urine protein / creatinine ratio \< 3.5 g/g AND * \> 50% reduction of urine protein / creatinine ratio from baseline (at the time of biopsy)
end stage renal disease
estimated glomerular filtration rate (eGFR) below 15 ml/min/1.73m2 repeatedly over a period \> 3 months OR dialysis \> 3 months OR kidney transplantation.
complete remission of nephrotic syndrome Time Frame : between kidney biopsy and last follow-up
Urine protein / creatinine ratio \< 0.3 g/g AND Albuminemia \> 35 g/L
adverse effects of corticosteroids
The number of serious adverse events will be measured in patients treated with corticosteroids according to the CTCAE toxicity grading system for the following adverse events combined: death (all causes), grade 3 or higher infections, sickle cell disease vaso-occlusive crisis, and new-onset or hospitalization-requiring diabetes mellitus.
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