Steroid-sensitive nephrotic syndrome: From childhood to adulthood

Fakhouri, Fádi; Bocquet, N.; Taupin, Pierre; Presne, Claire; Gagnadoux, Marie‐France; Landais, Paul; Lesavre, Philippe; Chauveau, Dominique; Knebelmann, Bertrand; Broyer, M.; Grünfeld, Jean‐Pierre; Niaudet, Patrick

doi:10.1053/ajkd.2003.50116

Cited by 164 publications

(125 citation statements)

References 18 publications

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“…In particular, the association between young age and recurrent SSNS has previously been reported by [3,5,[7][8][9]. In our study, the two youngest ages (from 1.5 to <3 and from three to <6 years) were stably significant relapse prognostic variables in both Cox and multinomial regression models.…”

Section: Discussionsupporting

confidence: 80%

“…The hypertension was reported in approximately 11-37.5% of patients in the international literature [8,10,13,30] but only in 0-9.0% in our study. It is important that increase in blood pressure was detected after 4-5 weeks of treatment; it was controllable and limited in time to tapering PRED.…”

Section: Discussioncontrasting

confidence: 63%

“…Unlike our approach, international studies reported these cases as steroid related AEs, even though they are also rarely seen [4,8,10].…”

Section: Discussionmentioning

confidence: 69%

“…Other unfavorable consequences of the therapy, such as growth retardation, overweight or obesity, being causes by limitations of the prolonged using PRED and alkylating agents [8,13], were minimized in the cohort under study, as it is reported in [2,3,31]. This in turn can be explained by reducing the number of re-treatments associated with reducing of total cumulative doses.…”

Section: Discussionmentioning

confidence: 99%

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Clinical Course of Steroid Sensitive Nephrotic Syndrome in Children: Outcome and Outlook

Fomina¹

2011

TOPEDJ

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Abstract:Introduction: The aim of our study was to investigate the relative efficiency and adverse effects of various treatments of steroid sensitive nephrotic syndrome (SSNS) in children, and to determine factors associated with relapse risk in these patients. Materials and Method:We retrospectively studied the data from 690 SSNS children treated in referral center over 25 years. The analyzed treatment protocols were: Prednisolone (PRED, eight weeks in a dose 1.5-2.0 mg/kg, then it tapering and given for 9-12 months), Chlorambucil (CHL, cumulative dose 28.5-30 mg/kg), Cyclophosphamide intravenously (CYC I.V., cumulative dose of 30-36 mg/kg, then supporting dose of CHL, cumulative dose of 20-25 mg/kg) and intramuscular (CYC I.M., cumulative dose of 120-150 mg/kg). The alkylating agents were used after remission induction by PRED and under its protection.Results: Cumulative relapse-free survival was 81.9%, 69.0% and 64.5% after 12, 36 and 60 months, respectively. In multivariate analyses, relapse risk was associated with age of treatment (<6 years), and both PRED and CYC I.V. The only predictive factor for early relapse was PRED, unlike two and more relapses group where PRED and CYC I.V. as well as age from 3 to 6 years was highly prognostic. The high probability of sustained remission in combination with relatively mild adverse effects was observed for PRED used at first episode and CHL used at relapse. Conclusion:To summarize, our protocols characterized by the prolonged PRED and CHL demonstrated promising results and should be considered as an efficient alternative strategy in SSNS management.

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Section: Discussionsupporting

confidence: 80%

Section: Discussioncontrasting

confidence: 63%

“…Unlike our approach, international studies reported these cases as steroid related AEs, even though they are also rarely seen [4,8,10].…”

Section: Discussionmentioning

confidence: 69%

Section: Discussionmentioning

confidence: 99%

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Clinical Course of Steroid Sensitive Nephrotic Syndrome in Children: Outcome and Outlook

Fomina¹

2011

TOPEDJ

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“…Several factors have been linked to a prolonged disease course in children with FRNS, including age at the onset of nephrotic syndrome (13,14) and number of relapses or steroid dependency (13,15,16). However, factors associated with relapse after cyclosporine have not been clearly established.…”

Section: Introductionmentioning

confidence: 99%

Two-Year Follow-Up of a Prospective Clinical Trial of Cyclosporine for Frequently Relapsing Nephrotic Syndrome in Children

Ishikura¹,

Yoshikawa

Nakazato

et al. 2012

Clinical Journal of the American Society of Nephrology

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SummaryBackground and objectives Although the safety and efficacy of cyclosporine in children with frequently relapsing nephrotic syndrome (FRNS) have been confirmed, no prospective follow-up data on relapse after cyclosporine have appeared. This study is a prospective follow-up trial after 2-year treatment with cyclosporine to investigate cyclosporine dependency after its discontinuation.Design, setting, participants, & measurements Participants who had undergone 2-year protocol treatment with microemulsified cyclosporine for FRNS between January 2000 and December 2005 were followed for an additional 2 years. The primary end point was relapse-free survival after the complete discontinuation of cyclosporine, and the secondary end point was regression-free survival (time to regression to FRNS).Results After exclusion of 7 patients who showed regression to FRNS during the 2-year treatment period, 49 children (median age, 6.5 years) were followed, and classified as children without (n=32; group A) and with (n=17; group B) relapse during the initial cyclosporine treatment. Overall, relapse-free survival probability at 24 months after cyclosporine discontinuation was 15.3% and regression to FRNS-free survival probability was 40.8%. By group, the probability of relapse-free survival was significantly higher in group A (17.9%) than in group B (8.3%) (P,0.001).Conclusions Children with FRNS who receive cyclosporine are at high risk of relapse after discontinuation, particularly those who experience relapse during cyclosporine treatment.

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Minimal Change Disease in Adults

Hogan

2013

Core Concepts in Parenchymal Kidney Disease

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Steroid-sensitive nephrotic syndrome: From childhood to adulthood

Cited by 164 publications

References 18 publications

Clinical Course of Steroid Sensitive Nephrotic Syndrome in Children: Outcome and Outlook

Clinical Course of Steroid Sensitive Nephrotic Syndrome in Children: Outcome and Outlook

Two-Year Follow-Up of a Prospective Clinical Trial of Cyclosporine for Frequently Relapsing Nephrotic Syndrome in Children

Minimal Change Disease in Adults

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