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Differential risk of remission and ESRD in childhood FSGS

Debbie S Gipson1, Hyunsook Chin, Trevor P Presler

  • 1UNC Kidney Center, University of North Carolina, Chapel Hill, USA. Debbie_Gipson@med.unc.edu

Insights

Achieving remission of proteinuria in children with Focal Segmental Glomerulosclerosis (FSGS) significantly improves long-term kidney survival. Complete or partial remission dramatically reduces the risk of end-stage renal disease (ESRD).

Area of Science:

  • Pediatric Nephrology
  • Glomerular Diseases
  • Renal Disease Progression

Background:

  • Focal segmental glomerulosclerosis (FSGS) is a primary cause of steroid-resistant nephrotic syndrome in children.
  • FSGS is a leading cause of end-stage renal disease (ESRD) stemming from glomerular disease in pediatric populations.
  • Understanding factors influencing FSGS progression is crucial for improving patient outcomes.

Purpose of the Study:

  • To assess the risk of disease progression in children with primary FSGS.
  • To evaluate the impact of proteinuria remission status on disease progression in pediatric FSGS.
  • To identify predictors of proteinuria remission and renal survival in this cohort.

Main Methods:

  • Retrospective cohort study of 60 children and adolescents with primary FSGS from the Glomerular Disease Collaborative Network.
  • Analysis of patient demographics, clinical characteristics (proteinuria, hypertension, eGFR), and treatment outcomes.
  • Multivariate analysis to determine predictors of proteinuria remission and renal survival.

Main Results:

  • Complete remission was achieved in 20%, partial remission in 33%, and 47% of patients.
  • Angiotensin-converting enzyme inhibitor (ACE-I)/angiotensin II receptor blocker (ARB) therapy was predictive of proteinuria remission (HR 3.35).
  • Complete remission was associated with a 90% decreased risk of ESRD (HR 0.10) in multivariate analysis.

Conclusions:

  • Proteinuria remission status is a significant predictor of long-term renal survival in children with FSGS.
  • Achieving complete or partial remission of proteinuria substantially improves renal survival.
  • ACE-I/ARB therapy may play a role in achieving proteinuria remission in pediatric FSGS.

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