Corticosteroid therapy for nephrotic syndrome in children

Hahn, Deirdré; Hodson, Elisabeth M; Willis, Narelle S; Craig, Jonathan C.

doi:10.1002/14651858.cd001533.pub5

Cited by 136 publications

(113 citation statements)

References 88 publications

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“…Over the past 3 years, however, the results of three prospective controlled studies have, to some extent, modified our point of view on the optimal therapeutic approach at onset. In summary, as evidenced in the most recent Cochrane review (44), extending the treatment regimen for .12 weeks total or giving higher doses does not significantly modify the onset of frequent relapses.…”

Section: Childrenmentioning

confidence: 71%

Minimal Change Disease

Vivarelli

Massella

Ruggiero

et al. 2016

CJASN

374

383

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Minimal change disease (MCD) is a major cause of idiopathic nephrotic syndrome (NS), characterized by intense proteinuria leading to edema and intravascular volume depletion. In adults, it accounts for approximately 15% of patients with idiopathic NS, reaching a much higher percentage at younger ages, up to 70%-90% in children >1 year of age. In the pediatric setting, a renal biopsy is usually not performed if presentation is typical and the patient responds to therapy with oral prednisone at conventional doses. Therefore, in this setting steroid-sensitive NS can be considered synonymous with MCD. The pathologic hallmark of disease is absence of visible alterations by light microscopy and effacement of foot processes by electron microscopy. Although the cause is unknown and it is likely that different subgroups of disease recognize a different pathogenesis, immunologic dysregulation and modifications of the podocyte are thought to synergize in altering the integrity of the glomerular basement membrane and therefore determining proteinuria. The mainstay of therapy is prednisone, but steroid-sensitive forms frequently relapse and this leads to a percentage of patients requiring second-line steroid-sparing immunosuppression. The outcome is variable, but forms of MCD that respond to steroids usually do not lead to chronic renal damage, whereas forms that are unresponsive to steroids may subsequently reveal themselves as FSGS. However, in a substantial number of patients the disease is recurrent and requires long-term immunosuppression, with significant morbidity because of side effects. Recent therapeutic advances, such as the use of anti-CD20 antibodies, have provided long-term remission off-therapy and suggest new hypotheses for disease pathogenesis.

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Section: Childrenmentioning

confidence: 71%

Minimal Change Disease

Vivarelli

Massella

Ruggiero

et al. 2016

CJASN

374

383

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“…Results are divided into subgroups according to risk of bias for blinding (performance and detection bias) and are ordered by trial weights. Reproduced with permission from Hahn et al [9] corticosteroids in the initial episode of SSNS has changed the conclusions of the review. Prolonging prednisolone therapy for 6 months does not reduce the risk for relapse compared with 2 or 3 months of therapy in children aged 1-17 years at presentation.…”

Section: Discussionmentioning

confidence: 99%

“…We have updated the 2007 systematic review to include these three recently published trials and another small trial published in 2012 [9]. When all studies evaluating the duration of corticosteroid therapy in the first episode of SSNS were combined, the number of children relapsing by 12-24 months was significantly reduced in children treated for ≥3 months compared with 2 months (8 studies, 741 children: RR 0.80; 95 % CI 0.64-1.00) and in children treated for 5-6 months Reproduced with permission from Hahn et al [9] compared with 3 months (7 studies, 763 children: RR 0.62; 95 % CI 0.45-0.85).…”

Section: Update Of the Systematic Reviewmentioning

confidence: 99%

“…When all studies evaluating the duration of corticosteroid therapy in the first episode of SSNS were combined, the number of children relapsing by 12-24 months was significantly reduced in children treated for ≥3 months compared with 2 months (8 studies, 741 children: RR 0.80; 95 % CI 0.64-1.00) and in children treated for 5-6 months Reproduced with permission from Hahn et al [9] compared with 3 months (7 studies, 763 children: RR 0.62; 95 % CI 0.45-0.85). The number of children developing FRNS was significantly reduced in children treated for ≥3 months compared with 2 months (6 studies, 582 children: RR 0.68; 95 % CI 0.47-1.00), but there was no significant difference in the number of children developing FRNS between those treated for 5-6 months and and those treated for 3 months (5 studies, 591 children: RR 0.78; 95 % CI 0.50 -1.22).…”

Section: Update Of the Systematic Reviewmentioning

confidence: 99%

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Corticosteroids for the initial episode of steroid-sensitive nephrotic syndrome

2015

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“…The Cochrane Database Systematic Review of 2015 suggests that there are many factors that determine the risk of relapse viz. ethnic background, living standards, nutrition, immunisation and infection 23 .…”

Section: Methodsmentioning

confidence: 99%

Long-term tapering regimen of prednisolone for the initial episode of nephrotic syndrome

Thalgahagoda

Karunadasa

Kudagammana

et al. 2017

Sri Lanka J Child Health

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Background: Childhood nephrotic syndrome (NS) has a relapsing-remitting course resulting in a significant corticosteroid burden or a prescription of cytotoxic immunosuppressive therapy. The ideal steroid regimen for the initial episode remains elusive.Objective: This study was conducted at a single centre to assess the outcome of a longer regimen of prednisolone for the initial episode of childhood NS in comparison to the shorter regimen used earlier. Methods:The clinical records of children with NS were stored manually since 1994 and electronically since 2002. From 1994 to 2002 all patients attending with the initial episode of NS were prescribed 60mg/m 2 prednisolone for 4 weeks followed by 40mg/m 2 on alternate days for 4 weeks (short term therapy / ST). From 2002 the initial episode was treated with 60mg/m 2 prednisolone for 4 weeks, followed by 60mg/m 2 on alternate days for 2 weeks tapering by 10mg/m 2 every 2 weeks over a period of 12 weeks (long term therapy / LT). The outcome of patients treated with ST over a period of 8 years was compared with patients treated with LT for 8 years.Results: Fifty eight children were treated with ST and 112 with LT. The relapse rate with LT was 12.4% at 6 months, 30.5% at 12 months and 48% at 24 months of the study compared to the 31.5% at 6 months 61% at 12 months and 81% at 24 months for ST. The mean time period for the first relapse to occur was 22.8 months for LT, whereas this was 12.5 months for ST. Conclusion:A long term tapering regimen is superior in maintaining sustained remission compared to short term therapy for the first episode of childhood NS. _________________________________________

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Corticosteroid therapy for nephrotic syndrome in children

Abstract: Analysis 3.1. Comparison 3 Steroid therapy in the first episode of nephrotic syndrome: 1 month versus 2 months of therapy, Outcome

Cited by 136 publications

References 88 publications

Minimal Change Disease

Minimal Change Disease

Corticosteroids for the initial episode of steroid-sensitive nephrotic syndrome

Long-term tapering regimen of prednisolone for the initial episode of nephrotic syndrome

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