Steroid-resistant nephrotic syndrome: long-term evolution after sequential therapy

Pena, A De La; Bravo, Jorge Castro; Melgosa, Marta; Fernández, C.; Meseguer, C. García; Román, Laura Espinosa; Alonso, A.; Picazo, M.L.; Navarro, Mercedes

doi:10.1007/s00467-007-0567-2

Cited by 22 publications

(15 citation statements)

References 44 publications

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“…CsA treated children achieved a 41.7% remission rate at 6 months, this was 36.4% and 19.1% in MMF and TAC group. This was much lower than reported in previous studies (12,13,(20)(21)(22), they all reported a 6 months' remission rate of over 50%. Whether the significant difference and doses of concomitant steroids led to this result deserves further study.…”

Section: Discussioncontrasting

confidence: 68%

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: Discussioncontrasting

confidence: 68%

Immunosuppressive Therapy in Children With Steroid-Resistant Nephrotic Syndrome

et al. 2018

Iran J Pediatr

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“…Studies shown glucocorticoid remains the mainstay of childhood NS treatment. There are many factors induced to modulate NS response to drug intervention such as the expression of P-gp, a product of MDR-1 [18,19]. In the kidney, the P-gp is expressed in the brush border membrane of proximal tubular epithelial cells [20].…”

Section: Discussionmentioning

confidence: 99%

IDENTIFICATION OF FUNCTIONAL SNPs OF MDR1 GENE AMONG NEPHROTIC SYNDROME CHILDREN IN SOUTH INDIA

Arumugam

2017

Asian J Pharm Clin Res

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Objective: This study was conducted to determine the frequency of C3435T and G2677T/C single nucleotide polymorphisms of multi drug resistance gene -1 (MDR1) in nephrotic syndrome (NS) children in relation to healthy subjects. The role and association of these SNPs were also determined, whether response and/or resistance to steroid treatment in children with NS in south India. Methods:Genomic DNA was isolated from 371 blood samples collected from children with NS and controls. Among 173 cases, categorized into steroid-resistant NS (SRNS) were 90 and steroid-sensitive NS (SSNS) were 83 and 198 blood samples were included as controls. All samples were subjected to DNA extraction, and polymerase chain reaction followed by restriction fragment length polymorphism for identification of C3435T and G2677T/C genomic variations. Results:The frequencies of MDR-1 C3435T, CT, TT, and CC genotypes and SNP G2677T/C GG and G allele genotypes were observed in this study group. In SRNS, children showed significantly higher frequencies of MDR-1 C3435T, CT, TT, and TT+CC genotypes were observed than SRNS and controls. The allele frequencies of SRNS children showed CC -4%, CT -32% and TT -12% and in SSNS children, CC -10.98%, CT -27.2% and TT -13.9% were observed. Furthermore, increased frequencies of MDR-1 C3435T CT, TT, TT+CC genotypes, or T allele were observed in children aged <9 years old. There were no different genotype and allele frequency observed in G2677T/C genotypes NS children and controls. Conclusion:Based on these data, we are suggesting that MDR-1 C3435T gene polymorphisms are risk factors of increased susceptibility, earlier onset of NS as well as leads to steroid resistance. Whereas SNP G2677T/C gene polymorphisms do not have significant role observed in this study population.

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“…Therefore, MMF has little effect on other cells and is unlikely to cause severe adverse reactions such as bone marrow suppression. This drug has recently been used to treat nephrotic syndrome in adults and children [6][7][8][9][10][11][12][13][14][15][16][17][18][19]. However, in reviewing the literature, we were unable to determine the benefits of MMF treatment in children with SRINS, especially those <2 years.…”

Section: Introductionmentioning

confidence: 85%

Mycophenolate mofetil therapy for children with steroid-resistant nephrotic syndrome

Duan

et al. 2009

Pediatr Nephrol

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Treating children with steroid-resistant nephrotic syndrome (SRNS) has been a clinical challenge for pediatricians. We recruited 24 children (18 boys and six girls) with steroid-resistant idiopathic nephrotic syndrome (SRINS) who were <2 years. All patients were administered prednisone 2 mg/kg per day prior to mycophenolate mofetil (MMF). By the end of the eighth week, MMF was initiated at 25-30 mg/kg daily for 6- 12 months. Prednisone dose was reduced stepwise. Biochemical assays were performed every 2 months. Complete remission was achieved in 15 patients, partial remission in six, and no response to MMF was noted in three. With MMF treatment, the levels of urinary protein and serum cholesterol decreased and that of serum albumin increased in a time-dependant manner. We demonstrated the MMF could reduce proteinuria in SRINS children <2 years. Our study suggests that MMF therapy might be an effective strategy for treating SRINS in children <2 years.

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Steroid-resistant nephrotic syndrome: long-term evolution after sequential therapy

Cited by 22 publications

References 44 publications

Immunosuppressive Therapy in Children With Steroid-Resistant Nephrotic Syndrome

Immunosuppressive Therapy in Children With Steroid-Resistant Nephrotic Syndrome

IDENTIFICATION OF FUNCTIONAL SNPs OF MDR1 GENE AMONG NEPHROTIC SYNDROME CHILDREN IN SOUTH INDIA

Mycophenolate mofetil therapy for children with steroid-resistant nephrotic syndrome

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