Childhood Idiopathic Nephrotic Syndrome: Does the Initial Steroid Treatment Modify the Outcome? A Multicentre, Prospective Cohort Study

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).

Incidence of relapse and frequently relapsing/steroid‐dependent nephrotic syndrome in Chinese children with steroid‐sensitive nephrotic syndrome: A cohort study

“…And according to the Kaplan–Meier curve, the first relapse mainly occurred in the first 2 years after the first onset, which was also indicated by other studies 4,5,20 . Our results suggest that the incidence of relapse in Chinese children with SSNS might not be as high as previously reported in cohort studies or randomized controlled trials of other countries (range: 67–85%) 4–6,11,20–23 . The follow‐up time in this study was quite long, and it was less likely that the incidence was underestimated.…”

“…4,5,20 Our results suggest that the incidence of relapse in Chinese children with SSNS might not be as high as previously reported in cohort studies or randomized controlled trials of other countries (range: 67-85%). [4][5][6]11,[20][21][22][23] The follow-up time in this study was quite long, and it was less likely that the incidence was underestimated. According to the Chinese guideline for SSNS, our patients took prolonged prednisone therapy.…”

“…However, the incidence data of FR/SDNS from cohort studies of other countries and regions that follow up for more than 2 years ranges from 22.1% to 53.2%. 6,11,23,29 Besides, Banh et al…”

“…Currently, studies assessing the incidence of FR/SDNS in Chinese children with SSNS are lacking. However, the incidence data of FR/SDNS from cohort studies of other countries and regions that follow up for more than 2 years ranges from 22.1% to 53.2% 6,11,23,29 . Besides, Banh et al found that the incidence of FR/SDNS in East/Southeast Asian populations was significantly lower than in Europeans by conducting a large multiethnic cohort study (30% vs. 45% at 5 years), which suggests the ethnic differences in the incidence of FR/SDNS 4 .…”

“…At present, the data about the incidence of relapse and FR/SDNS in Chinese children with SSNS is lacking. Some studies reported risk factors for relapse and FR/SDNS, such as age at onset, 5,6 sex, 7 time to first remission, 8,9 but these factors are not consistent in all studies 10–12 . Moreover, few studies evaluate the discrimination and calibration of their risk prediction models.…”