Rituximab in children with steroid sensitive nephrotic syndrome: in quest of the optimal regimen

Chan, Eugene Yu-hin; Tullus, Kjell

doi:10.1007/s00467-020-04609-0

Cited by 26 publications

(24 citation statements)

References 53 publications

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“…Nowadays, both MMF and rituximab are valuable agents Fig. 3 Calcium and vitamin D diagnostics and supplementation at presentation in the treatment of SDNS [21,23]. Variation was present among our respondents on the rituximab regimen used.…”

Section: Discussionmentioning

confidence: 92%

“…Similarly, substantial variations in rituximab dose are present worldwide, ranging from 375 to 1500 mg/m 2 per treatment course [24,25]. Recently, Chan et al [23] reviewed the effect of patient factors, rituximab dose, and use of maintenance immunosuppression on treatment outcomes. Debate exists on the indications for rituximab use.…”

Section: Discussionmentioning

confidence: 99%

“…Strict monitoring is essential, especially in young children, to timely identify severe adverse events including persistent hypogammaglobulinemia. Finally, large prospective studies are needed to identify the exact indications and optimal rituximab regimen in patients with nephrotic syndrome [23]. After this questionnaire was sent, new evidence regarding the added value of levamisole in steroid-sensitive nephrotic syndrome became available [26], which consequently was not reflected in this survey.…”

Section: Discussionmentioning

confidence: 99%

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Practice variations in the management of childhood nephrotic syndrome in the Netherlands

et al. 2021

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Nephrotic syndrome in childhood is a common entity in the field of pediatric nephrology. The optimal treatment of children with nephrotic syndrome is often debated. Previously conducted studies have shown significant variability in nephrotic syndrome management, especially in the choice of steroid-sparing drugs. In the Netherlands, a practice guideline on the management of childhood nephrotic syndrome has been available since 2010. The aim of this study was to identify practice variations and opportunities to improve clinical practice of childhood nephrotic syndrome in the Netherlands. A digital structured survey among Dutch pediatricians and pediatric nephrologists was performed, including questions regarding the initial treatment, relapse treatment, kidney biopsy, additional immunosuppressive treatment, and supportive care. Among the 51 responses, uniformity was seen in the management of a first presentation and first relapse. Wide variation was found in the tapering of steroids after alternate day dosing. Most pediatricians and pediatric nephrologists (83%) would perform a kidney biopsy in case of steroid-resistant nephrotic syndrome, whereas for frequent relapsing and steroid-dependent nephrotic syndrome this was 22% and 41%, respectively. Variation was reported in the steroid-sparing treatment. Finally, significant differences were present in the supportive treatment of nephrotic syndrome.Conclusion: Substantial variation was present in the management of nephrotic syndrome in the Netherlands. Differences were identified in steroid tapering, use of steroid coverage during stress, choice of steroid-sparing agents, and biopsy practice. To promote guideline adherence and reduce practice variation, factors driving this variation should be assessed and resolved. What is Known:• National and international guidelines are available to guide the management of childhood nephrotic syndrome.• Several aspects of the management of childhood nephrotic syndrome, including the choice of steroid-sparing drugs and biopsy practice, are controversial and often debated among physicians.What is New:• Significant practice variation is present in the management of childhood nephrotic syndrome in the Netherlands, especially in the treatment of FRNS, SDNS, and SRNS.• The recommendation on the steroid treatment of a first episode of nephrotic syndrome in the KDIGO guideline leaves room for interpretation and is likely the cause of substantial differences in steroid-tapering practices among Dutch pediatricians and pediatric nephrologists.

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Section: Discussionmentioning

confidence: 92%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Practice variations in the management of childhood nephrotic syndrome in the Netherlands

et al. 2021

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“…These children are at high risk of significant medication side effects such as short stature, Cushing syndrome, infection, and calcineurin inhibitor-associated nephrotoxicity. Rituximab, a chimeric anti-CD monoclonal antibody, has emerged as an effective therapy in children with FRSDNS to induce long-term disease remission and avoid steroid toxicities [3,4].…”

Section: Introductionmentioning

confidence: 99%

“…The safety profiles of rituximab appear to be safe in children, but unusual significant complications can occur [3][4][5]. Most infusion reactions are self-limiting, but there are worrying side effects such as agranulocytosis, lung injury, fatal hepatitis reactivation, and multifocal leukoencephalopathy [6][7][8][9].…”

Section: Introductionmentioning

confidence: 99%