TY - JOUR
T1 - Childhood Idiopathic Nephrotic Syndrome
T2 - Does the Initial Steroid Treatment Modify the Outcome? A Multicentre, Prospective Cohort Study
AU - the NefroKid Study Group
AU - Pasini, Andrea
AU - Bertulli, Cristina
AU - Casadio, Luca
AU - Corrado, Ciro
AU - Edefonti, Alberto
AU - Ghiggeri, Gian Marco
AU - Ghio, Luciana
AU - Giordano, Mario
AU - La Scola, Claudio
AU - Malaventura, Cristina
AU - Maringhini, Silvio
AU - Mastrangelo, Antonio P.
AU - Materassi, Marco
AU - Mencarelli, Francesca
AU - Messina, Giovanni
AU - Monti, Elena
AU - Morello, William
AU - Puccio, Giuseppe
AU - Romagnani, Paola
AU - Montini, Giovanni
N1 - Funding Information:
All phases of this study were supported by the nonprofit association Il Sogno di Stefano and The Nando Peretti Foundation.
Publisher Copyright:
© Copyright © 2021 Pasini, Bertulli, Casadio, Corrado, Edefonti, Ghiggeri, Ghio, Giordano, La Scola, Malaventura, Maringhini, Mastrangelo, Materassi, Mencarelli, Messina, Monti, Morello, Puccio, Romagnani, Montini and the NefroKid Study Group.
PY - 2021/7/8
Y1 - 2021/7/8
N2 - 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).
AB - 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).
KW - age at onset
KW - childhood idiopathic nephrotic syndrome
KW - frequent relapsers
KW - prognostic factors
KW - steroid dependency
KW - steroid treatment
KW - total serum protein
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U2 - 10.3389/fped.2021.627636
DO - 10.3389/fped.2021.627636
M3 - Article
AN - SCOPUS:85111021685
VL - 9
JO - Frontiers in Pediatrics
JF - Frontiers in Pediatrics
SN - 2296-2360
M1 - 627636
ER -