Three year outcome of childhood idiopathic nephrotic syndrome under a unified immunosuppressive protocol

Hibino, Satoshi; Uemura, Osamu; Nagai, Takuhito; Yamakawa, Satoshi; Iwata, Naoyuki; Ito, Hidekazu; Nakano, Masaru; Tanaka, Kazuki

doi:10.1111/ped.12498

Cited by 15 publications

(8 citation statements)

References 14 publications

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“…In our study, the histopathological diagnosis of one child was FSGS and another was MCNS. Despite renal pathology, genetic factors and ethnicity seem to modulate the response to treatment and progression of ESRD (31,32). Another study also found that increased episodes of AKI were associated with increased risk of ESRD, but the causality was not established (18).…”

Section: Discussionmentioning

confidence: 96%

Immunosuppressive Therapy in Children With Steroid-Resistant Nephrotic Syndrome

et al. 2018

Iran J Pediatr

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Background: Treatment of steroid-resistant nephrotic syndrome (SRNS) remains a huge challenge in pediatric patients. Immunosuppressive agents including cyclosporine A (CsA), tacrolimus (TAC) and mycophenolate mofetil (MMF) are recommended for the management of children with SRNS. The aim of this study was to compare the efficacy of CsA, TAC and MMF in children with SRNS and provide guidance for clinical practice. Methods: This is a retrospective analysis of the records of 70 children with SRNS recruited from a children hospital over a period of seven years. They were treated with CsA, TAC and MMF as initial immunosuppressive therapy in addition to steroids. Complete or partial remission was considered a good response. Results: Five (41.7%) of 12 children who were on CsA therapy achieved remission at 6 months and 5 (41.7%) at 12 months. Nine (19.1%) of 47 patients treated with TAC achieved remission at 6 months, 20 (42.6%) at 12 months and 6 (12.8%) within or over 24 months. The remission achieved at 6 months and 12 months was 4 (36.4%) and 2 (18.2%) respectively in MMF group. The relapse rates in children who had achieved remission were 30.0%, 45.7% and 50.0% in CsA, TAC and MMF group respectively. Conclusions: Based on similar baseline characteristics, CsA and TAC as initial therapy for SRNS have a better remission and relapse rates whereas MMF shows a rapid remission effect.

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Section: Discussionmentioning

confidence: 96%

Immunosuppressive Therapy in Children With Steroid-Resistant Nephrotic Syndrome

et al. 2018

Iran J Pediatr

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“…Outcome with CsA has been shown to be improved when combined with other immune-suppressants such as MMF [13, 16]. In Iran [13], among 23 children who were CsA resistant, complete remission was observed in 11 cases (47.82%) and partial remission in 2 cases (8.7%) after combined MMF and CsA therapy thus increasing the overall remission rate.…”

Section: Discussionmentioning

confidence: 99%

Cyclosporine in the treatment of childhood idiopathic steroid resistant nephrotic syndrome: a single centre experience in Nigeria

Ladapo¹,

Esezobor²,

Lesi³

2016

Pan Afr Med J

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IntroductionChildren with steroid resistant nephrotic syndrome usually require treatment with second-line agents and calcineurin inhibitors such as cyclosporine are now recommended as initial therapy. These agents only recently become available in our environment and their impact on care is unknown. We reviewed the short-term treatment outcomes of their use in comparison with previous outcomes.MethodsMedical records of children managed for idiopathic steroid resistant nephrotic syndrome over a 5 year period were reviewed. Remission rates and improvement in renal function following use of various agents were compared.ResultsOf 103 children with idiopathic nephrotic syndrome, 25(24.3%) were steroid resistant, of whom 17 received additional medications. Full remission rate for cyclosporine was 70% (7/10). Remission rates prior to the availability of cyclosporine were 40% (2/5) for cyclophosphamide and 66% (2/3), (partial remission only) with enalapril, an angiotensin converting enzyme inhibitor used in combination with alternate day prednisolone. One child with cyclophosphamide resistance subsequently achieved remission with cyclosporine. Remission was not related to sex (p=0.96), age (p=0.54), serum albumin (p=0.37) or hypertension (p=0.43) but to serum cholesterol (p= 0.02). The estimated glomerular filteration rate (eGFR) among children treated with cyclosporine ranged from 30-167 ml/min/1.73m2 as follows: >90 (5); 60-89 (3); 30-59 (2) while the mean pre and post treatment eGFR in those with eGFR <90 were 60 and 104ml/min/1.73m2 respectively (p=0.03). Mortality rate was 10% (1/10) in children treated with cyclosporine compared with 28.6% (2/7) in those treated with other medications (p=0.54).ConclusionCyclosporine resulted in improved treatment outcomes in children with idiopathic steroid resistant nephrotic syndrome.

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“…Former triple therapy approaches comprised CNI plus prednisolone and a third agent, such as MMF [102,103] or mizoribine [104] (Table 2). Of these, MMF may be an effective third agent, supported by additional reports which show that remission was achieved in 20 to 50% of patients refractory to treatment with CNIs and corticosteroids [32, 105,106]. Some retrospective case series have also showed that MMF, as a maintenance agent, enabled CNI-and steroid-free remission in some patients with CNI dependence and/or toxicity [32, 107,108].…”

Section: Therapy For Cni-dependent or Cni-resistant Srnsmentioning

confidence: 95%

Current understandings in treating children with steroid-resistant nephrotic syndrome

et al. 2020

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Steroid-resistant nephrotic syndrome (SRNS) remains a challenge for paediatric nephrologists. SRNS is viewed as a heterogeneous disease entity including immune-based and monogenic aetiologies. Because SRNS is rare, treatment strategies are individualized and vary among centres of expertise. Calcineurin inhibitors (CNI) have been effectively used to induce remission in patients with immune-based SRNS; however, there is still no consensus on treating children who become either CNI-dependent or CNI-resistant. Rituximab is a steroid-sparing agent for patients with steroid-sensitive nephrotic syndrome, but its efficacy in SRNS is controversial. Recently, several novel monoclonal antibodies are emerging as treatment option, but their efficacy remains to be seen. Non-immune therapies, such as angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers, have been proven efficacious in children with SRNS and are recommended as adjuvant agents. This review summarizes and discusses our current understandings in treating children with idiopathic SRNS.

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Three year outcome of childhood idiopathic nephrotic syndrome under a unified immunosuppressive protocol

Abstract: Treatment with CsA and combination of CsA plus MMF was useful for SDNS and for remission induction in SRNS.

Cited by 15 publications

References 14 publications

Immunosuppressive Therapy in Children With Steroid-Resistant Nephrotic Syndrome

Immunosuppressive Therapy in Children With Steroid-Resistant Nephrotic Syndrome

Cyclosporine in the treatment of childhood idiopathic steroid resistant nephrotic syndrome: a single centre experience in Nigeria

Current understandings in treating children with steroid-resistant nephrotic syndrome

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