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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.
Abstract:
Focal segmental glomerulosclerosis (FSGS) is the leading cause of steroid-resistant nephrotic syndrome in childhood and the most common form of end stage renal disease (ESRD) from glomerular disease. In order to assess the risk of progression of children with primary FSGS and the impact of proteinuria remission status on disease progression, we undertook this study to describe a cohort of 60 children and adolescents from the Glomerular Disease Collaborative Network. Of the 60 patients included in the cohort, 58% were African American. Median age was 16 years. Proteinuria ranged from 1.0-24.0 g/day/1.73 m(2); 57% were hypertensive, and the median estimated glomerular filtration rate (eGFR) was 90.2 ml/min/1.73 m(2). Complete remission was achieved in 20%, partial remission in 33%, and 47% have not achieved remission during follow-up with all prescribed therapy. Only ACE-I/ARB therapy was predictive of proteinuria remission in multivariate analysis (hazard ratio [HR] 3.35; 95% confidence interval [CI] 1.42-7.92). Renal survival was much improved in patients with complete or partial remission compared with no remission in univariate analysis. In multivariate analysis comparing no remission status, complete remission was associated with a 90% decreased risk of ESRD (HR 0.10, 95% CI 0.01-0.79, p =0.03). In summary, proteinuria remission status is a valid predictor of long-term renal survival in children with FSGS.
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