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Published on: September 20, 2019
Childhood Idiopathic Nephrotic Syndrome: Does the Initial Steroid Treatment Modify the Outcome? A Multicentre,
Andrea Pasini1, Cristina Bertulli1, Luca Casadio2
1Nephrology and Dialysis Unit, Department of Pediatrics, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.
Insights
Adjusting initial steroid dosing for time to remission (TTR) did not reduce relapse rates in childhood idiopathic nephrotic syndrome. Younger age and low total serum protein predict relapse risk, independent of steroid regimens.
Area of Science:
- Pediatric Nephrology
- Clinical Pharmacology
Background:
- Idiopathic nephrotic syndrome (INS) frequently recurs in children after initial treatment.
- Investigating steroid dosing strategies adjusted for risk factors may reduce relapse rates.
Purpose of the Study:
- To assess if initial steroid regimens, tailored by time to remission (TTR), impact relapse frequency and steroid dependence in pediatric INS.
- To identify prognostic factors influencing the disease course of childhood INS.
Main Methods:
- A prospective, multicenter cohort study involving 143 children with INS.
- Children were assigned steroid regimens based on TTR: ≤10 days (Group A, 20-week prednisone) or >10 days (Group B, 22-week prednisone).
- Retrospective data from the same centers were analyzed for comparison.
Main Results:
- No significant differences in relapse rates or frequent relapser/steroid-dependent rates were observed between Group A and Group B.
- Time to remission (TTR) did not correlate with relapse risk.
- Younger age at onset and lower total serum protein levels were identified as significant predictors of relapse.
Conclusions:
- Initial steroid regimens adjusted for TTR did not alter relapse rates in childhood INS.
- Younger age and low total serum protein are independent predictors of relapse risk.
- Intensified prednisone regimens did not improve relapse outcomes in this cohort.
Abstract:
Background: A great majority of children with idiopathic nephrotic syndrome will relapse after successful treatment of the initial episode. The possibility that different steroid dosing regimens at onset, adjusted for risk factors, can reduce the rate of relapse represents an interesting option to investigate. Objectives: To evaluate the effect of the initial steroid regimen, adjusted for time to remission (TTR), on the frequency of relapses and steroid dependence, and to verify the influence of prognostic factors on disease course. Methods: A multicentre, prospective, cohort study. Children with nephrotic syndrome, with TTR ≤ 10 days (Group A), were given a 20-week prednisone regimen (2,828 mg/m2) and those with a TTR >10 days, a 22-week regimen (3,668 mg/m2) (Group B). Previously published retrospective data from the same centers were also evaluated. Main outcomes were: relapse rate, number of frequent relapsers + steroid dependent children and total prednisone dose after induction. Results: 143 children were enrolled. Rate of relapsed subjects (77 vs. 79%) and frequent relapsers + steroid dependent subjects (40 vs. 53%) did not differ between Groups A and B, or between the retrospective and prospective cohorts. The cumulative prednisone dose taken after the induction treatment was similar in both groups and in the retrospective and prospective cohorts. TTR was not associated with relapse risk. Age at onset and total serum protein were significantly lower in relapsing patients. At ROC analysis, the best cut-off was 5.3 years for age at onset and 4.2 g/dL for total serum protein. According to these cut-offs, older children with higher total serum protein had a higher relapse free survival rate (58%) than younger children with lower total serum protein (17%). Conclusions: TTR was not found to be a prognostic factor of relapse; because of this, different steroid regimens, adjusted for TTR, did not modify the relapse rate in any relevant measure. Conversely, younger age and low total serum protein were independent predictors of relapse risk, however this outcome was not modified by higher prednisone regimens. Clinical Trial Registration:https://www.ClinicalTrials.gov/, identifier: NCT01386957 (www.nefrokid.it).
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