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Folly Beach Library
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Outcomes of steroid-resistant nephrotic syndrome in children not treated with intensified immunosuppression.
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- Author(s): Trautmann, Agnes; Seide, Svenja; Lipska-Ziętkiewicz, Beata S.; Ozaltin, Fatih; Szczepanska, Maria; Azocar, Marta; Jankauskiene, Augustina; Zurowska, Alexandra; Caliskan, Salim; Saeed, Bassam; Morello, William; Emma, Francesco; Litwin, Mieczyslaw; Tsygin, Alexey; Fomina, Svitlana; Wasilewska, Anna; Melk, Anette; Benetti, Elisa; Gellermann, Jutta; Stajic, Natasa
- Source:
Pediatric Nephrology. May2023, Vol. 38 Issue 5, p1499-1511. 13p. 2 Diagrams, 3 Charts, 2 Graphs. - Source:
- Additional Information
- Subject Terms:
- Abstract: Background: The aim of the current PodoNet registry analysis was to evaluate the outcome of steroid-resistant nephrotic syndrome (SRNS) in children who were not treated with intensified immunosuppression (IIS), focusing on the potential for spontaneous remission and the role of angiotensin blockade on proteinuria reduction. Methods: Ninety-five pediatric patients who did not receive any IIS were identified in the PodoNet Registry. Competing risk analyses were performed on 67 patients with nephrotic-range proteinuria at disease onset to explore the cumulative rates of complete or partial remission or progression to kidney failure, stratified by underlying etiology (genetic vs. non-genetic SRNS). In addition, Cox proportional hazard analysis was performed to identify factors predicting proteinuria remission. Results: Eighteen of 31 (58.1%) patients with non-genetic SRNS achieved complete remission without IIS, with a cumulative likelihood of 46.2% at 1 year and 57.7% at 2 years. Remission was sustained in 11 children, and only two progressed to kidney failure. In the genetic subgroup (n = 27), complete resolution of proteinuria occurred very rarely and was never sustained; 6 (21.7%) children progressed to kidney failure at 3 years. Almost all children (96.8%) received proteinuria-lowering renin–angiotensin–aldosterone system (RAAS) antagonist treatment. On antiproteinuric treatment, partial remission was achieved in 7 of 31 (22.6%) children with non-genetic SRNS and 9 of 27 children (33.3%) with genetic SRNS. Conclusion: Our results demonstrate that spontaneous complete remission can occur in a substantial fraction of children with non-genetic SRNS and milder clinical phenotype. RAAS blockade increases the likelihood of partial remission of proteinuria in all forms of SRNS. [ABSTRACT FROM AUTHOR]
- Abstract: Copyright of Pediatric Nephrology is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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