Cyclophosphamide versus cyclosporine A therapy in steroid-resistant nephrotic syndrome: a retrospective study with a mean 5-year follow-up

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

Abstract

Objective

To compare the clinical efficacy of cyclophosphamide (CTX) and cyclosporine A (CSA) in initial treatment of children with steroid-resistant nephrotic syndrome (SRNS).

Methods

Prospectively maintained databases were reviewed to retrospectively compare two cohorts with SRNS that received peroral administration of 2 to 2.5 mg/kg/d CTX for 3 to 6 months or 1 to 5 mg/kg/d CSA for 2 years until the primary analysis cut-off date during 2007 to 2011. The time to first on-study relapse of SRNS was the primary endpoint. The effective rate was the second endpoint.

Results

A total of 127 children with SRNS were included (CTX-treated cohort: n = 62; CSA-treated cohort: n = 65), with a mean 5-year follow-up. CTX-treated children showed a significantly delayed time to first on-study relapse of SRNS compared with CSA-treated children (hazard ratio 0.66, 95% confidence interval 0.32–1.75). The relapse rate (rate/year) in CTX-treated children (1.1 ± 0.1) at the 24-month follow-up was significantly higher than that with CSA (0.4 ± 0.2). This difference persisted until the final follow-up.

Conclusions

CSA is associated with a significantly lower relapse rate and significantly higher effective rate compared with CTX, especially in children with minimal change disease.

Related collections

Most cited references 27

Record: found
Abstract: found
Article: not found

Treatment of steroid-sensitive nephrotic syndrome: new guidelines from KDIGO.

Rebecca Lombel, Debbie S. Gipson, Elisabeth Hodson (2013)

The 2012 Kidney Disease: Improving Global Outcomes (KDIGO) clinical practice guideline on glomerulonephritis (GN) is intended to assist the practitioner caring for patients with GN. Two chapters of this guideline focus specifically on nephrotic syndrome in children. Guideline development followed a thorough evidence review, and management recommendations and suggestions were based on the best available evidence. Critical appraisal of the quality of evidence and strength of recommendations followed the Grades of Recommendation Assessment, Development and Evaluation (GRADE) approach. Chapters 3 and 4 of the guideline focus on the management of nephrotic syndrome in children aged 1-18 years. Guideline recommendations for children who have steroid-sensitive nephrotic syndrome (SNSS), defined by their response to corticosteroid therapy with complete remission, are addressed here. Recommendations for those with steroid-resistant nephrotic syndrome (SRNS) (i.e., do not achieve complete remission) are discussed in the companion article. Limitations of the evidence, including the paucity of large-scale randomized controlled trials, are discussed. This article provides a short description of the KDIGO process, the guideline recommendations for treatment of SSNS in children and a brief review of relevant treatment trials related to each recommendation.

0 comments Cited 86 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children.

Agnes Trautmann, Sven Schnaidt, Beata S. Lipska-Ziętkiewicz … (2017)

We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and <20 years of age): 612 had documented responsiveness to intensified immunosuppression (IIS), 1155 had kidney biopsy results, and 212 had an established genetic diagnosis. We assessed risk factors for ESRD using multivariate Cox regression models. Complete and partial remission of proteinuria within 12 months of disease onset occurred in 24.5% and 16.5% of children, respectively, with the highest remission rates achieved with calcineurin inhibitor-based protocols. Ten-year ESRD-free survival rates were 43%, 94%, and 72% in children with IIS resistance, complete remission, and partial remission, respectively; 27% in children with a genetic diagnosis; and 79% and 52% in children with histopathologic findings of minimal change glomerulopathy and FSGS, respectively. Five-year ESRD-free survival rate was 21% for diffuse mesangial sclerosis. IIS responsiveness, presence of a genetic diagnosis, and FSGS or diffuse mesangial sclerosis on initial biopsy as well as age, serum albumin concentration, and CKD stage at onset affected ESRD risk. Our findings suggest that responsiveness to initial IIS and detection of a hereditary podocytopathy are prognostic indicators of favorable and poor long-term outcome, respectively, in children with steroid-resistant nephrotic syndrome. Children with multidrug-resistant sporadic disease show better renal survival than those with genetic disease. Furthermore, histopathologic findings may retain prognostic relevance when a genetic diagnosis is established.

0 comments Cited 80 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study.

François Nobili, Aurélia Liutkus, Lucie Bessenay … (2009)

Long-term outcome of idiopathic steroid-resistant nephrotic syndrome was retrospectively studied in 78 children in eight centers for the past 20 years. Median age at onset was 4.4 years (1.1-15.0 years) and the gender ratio was 1.4. Median follow-up period was 7.7 years (1.0-19.7 years). The disease in 45 patients (58%) was initially not steroid-responsive and in 33 (42%) it was later non-responsive. The main therapeutic strategies included administration of ciclosporine (CsA) alone (n = 29; 37%) and CsA + mycophenolate mofetil (n = 18; 23%). Actuarial patient survival rate after 15 years was 97%. Renal survival rate after 5 years, 10 years and 15 years was 75%, 58% and 53%, respectively. An age at onset of nephrotic syndrome (NS) > 10 years was the only independent predictor of end-stage renal disease (ESRD) in a multivariate analysis using a Cox regression model (P < 0.001). Twenty patients (26%) received transplants; ten showed recurrence of the NS: seven within 2 days, one within 2 weeks, and two within 3-5 months. Seven patients lost their grafts, four from recurrence. Owing to better management, kidney survival in idiopathic steroid-resistant nephrotic syndrome (SRNS) has improved during the past 20 years. Further prospective controlled trials will delineate the potential benefit of new immunosuppressive treatment.

0 comments Cited 62 times – based on 0 reviews      Review now

Bookmark

All references

Author and article information

Journal

Journal ID (nlm-ta): J Int Med Res

Journal ID (iso-abbrev): J. Int. Med. Res

Journal ID (publisher-id): IMR

Journal ID (hwp): spimr

Title: The Journal of International Medical Research

Publisher: SAGE Publications (Sage UK: London, England )

ISSN (Print): 0300-0605

ISSN (Electronic): 1473-2300

Publication date (Electronic): 05 September 2018

Publication date (Print): November 2018

Volume: 46

Issue: 11

Pages: 4506-4517

Affiliations

[1 ]Department of Pediatrics, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, 510700, China

[2 ]Department of Physical Examination, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, 510700, China

[3 ]Department of Radiology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, 510700, China

[4 ]Department of Orthopaedics, Jinshan Hospital, Fudan University, Shanghai, 201508, China

[5 ]Department of Orthopaedics and Central Laboratory, The Third Hospital Affiliated to Nantong University, Wuxi, Jiangsu, 214041, China

Author notes

[#]

These authors contributed equally to this work

[*]Mingdong Zhao, Jinshan Hospital, Fudan University, No. 1508 Longhang Road, Shanghai 201508, China. Email: zhaonissan@ 123456163.com

[*]Fenglai Yuan, The Third Hospital Affiliated to Nantong University, Wuxi, Jiangsu 214041, China. Email: bjjq88@ 123456163.com

[*]Keke Gui, Jinshan Hospital, Fudan University, No. 1508 Longhang Road, Shanghai 201508, China. Email: gilbird@ 123456163.com

Article

Publisher ID: 10.1177_0300060518782017

DOI: 10.1177/0300060518782017

PMC ID: 6259365

PubMed ID: 30185089

SO-VID: b771cb8b-f292-4151-9d60-ad5bd7c6467d

License:

Creative Commons Non Commercial CC BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License ( http://www.creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages ( https://us.sagepub.com/en-us/nam/open-access-at-sage).

Cyclophosphamide versus cyclosporine A therapy in steroid-resistant nephrotic syndrome: a retrospective study with a mean 5-year follow-up

Read this article at

Abstract

Objective

Methods

Results

Conclusions

Related collections

International Journal of Medicine and Medical Research

Most cited references 27

Treatment of steroid-sensitive nephrotic syndrome: new guidelines from KDIGO.

Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children.

Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study.

Author and article information

Journal

Affiliations

Author notes

Article

History

Categories

Comments

Comment on this article

Similar content 277

Cited by 4

Most referenced authors 429