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Background <p>Steroid-resistant nephrotic syndrome (SRNS) is the second leading cause of chronic kidney disease (CKD) in childhood. It represents a heterogeneous group of diseases with variable kidney outcomes that are still challenging to predict. In this study, our main objective is to describe predictive factors of remission states and kidney survival comparing genetic and non-genetic SRNS.</p> Methods <p>We conducted a retrospective analysis of 65 pediatric patients with SRNS treated at the pediatric outpatient clinic in Berlin between 2000 and 2023. Clinical characteristics, laboratory findings, and treatment strategies were systematically collected at multiple time points. Outcomes were defined by remission status, kidney survival (CKD stage I–IV), or progression to CKD stage V. Statistical analyses included univariate and multivariate logistic and Cox regression models adjusted for monogenic SRNS to identify predictors of remission and kidney survival.</p> Results <p>The median age of onset was 4.0&#xa0;years, with a male predominance of 57%. Patients were followed for a median of 5.9&#xa0;years. At the last follow-up, 26 patients achieved complete remission, 12 achieved partial remission, and 27 showed no remission. Kidney survival rates at 5 and 10&#xa0;years were 71% and 56%, respectively. High initial nephrotic-range proteinuria, confirmed genetic diagnoses, reduced eGFR, and hypoalbuminemia at 3-month and 1-year follow-ups were identified as negative predictive factors for complete or partial remission. These factors also correlated strongly with an elevated risk of progression to CKD stage V.</p> Conclusion <p>Our findings highlight additional prognostic factors influencing remission status and long-term kidney survival in pediatric SRNS, emphasizing the value of detailed early time-point analyses.</p> Graphical abstract <p></p>

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Outcomes and prognostic factors in childhood-onset steroid-resistant nephrotic syndrome: a retrospective single-center study

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年3月1日

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  • Emil Zerkowitz,
  • Jutta Gellermann,
  • Juliane Beckus,
  • Johannes Holle,
  • Caroline Kempf,
  • Philip Bufler,
  • Dominik Müller,
  • Julia Thumfart,
  • Verena Klämbt

摘要

Background

Steroid-resistant nephrotic syndrome (SRNS) is the second leading cause of chronic kidney disease (CKD) in childhood. It represents a heterogeneous group of diseases with variable kidney outcomes that are still challenging to predict. In this study, our main objective is to describe predictive factors of remission states and kidney survival comparing genetic and non-genetic SRNS.

Methods

We conducted a retrospective analysis of 65 pediatric patients with SRNS treated at the pediatric outpatient clinic in Berlin between 2000 and 2023. Clinical characteristics, laboratory findings, and treatment strategies were systematically collected at multiple time points. Outcomes were defined by remission status, kidney survival (CKD stage I–IV), or progression to CKD stage V. Statistical analyses included univariate and multivariate logistic and Cox regression models adjusted for monogenic SRNS to identify predictors of remission and kidney survival.

Results

The median age of onset was 4.0 years, with a male predominance of 57%. Patients were followed for a median of 5.9 years. At the last follow-up, 26 patients achieved complete remission, 12 achieved partial remission, and 27 showed no remission. Kidney survival rates at 5 and 10 years were 71% and 56%, respectively. High initial nephrotic-range proteinuria, confirmed genetic diagnoses, reduced eGFR, and hypoalbuminemia at 3-month and 1-year follow-ups were identified as negative predictive factors for complete or partial remission. These factors also correlated strongly with an elevated risk of progression to CKD stage V.

Conclusion

Our findings highlight additional prognostic factors influencing remission status and long-term kidney survival in pediatric SRNS, emphasizing the value of detailed early time-point analyses.

Graphical abstract