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Oral cyclophosphamide therapy in 100 children with steroid-sensitive nephrotic syndrome: experience from a developing country

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Abstract

Background

Oral cyclophosphamide (CYP) is an important therapeutic agent in treatment of steroid-sensitive nephrotic syndrome having a steroid-dependent (SD) or frequent relapsing (FR) course. This retrospective observational study aimed to determine response to oral CYP and factors associated with positive response in these patients.

Methods

We studied 100 children (male; 75) with FR (19%) and SD (81%) NS treated with CYP in the Pediatric Nephrology clinic. Responders were defined as children in whom steroids were stopped for at least 6 months following CYP and factors affecting response were analysed. Relapse-free survival was estimated by Kaplan–Meier method.

Results

Median age at onset of NS was 3 years (IQR 2–5.2). Median age at CYP was 5.7 years (IQR 3.7–7.9). Fifty percent of patients were in the responder group at 6 months of CYP. Relapse-free survival post CYP therapy was 31% at 1 year, 11% at 2 years. Factors predicting good response were age at onset of NS > 3 years with 61.2% response at 6 months (p = 0.028) and older age at CYP initiation (> 5 years) with 61% response (p = 0.008). Multivariate regression analysis showed age at start of CYP > 5 years was an independent factor for good response (p = 0.044, OR = 2.903, CI −1.03 to 8.18).

Conclusions

Judicious selection of patients, especially with age of onset of NS more than 3 years and initiation of CYP after age of 5 years, can predict better response in this group of patents.

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Acknowledgements

The authors acknowledge all of the parents round the world who are constantly involved in the care of children with nephrotic syndrome.

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Authors and Affiliations

  1. Department of Pediatrics, Dayanand Medical College and Hospital, Ludhiana, Punjab, India

    Jasjeet Sandhu, Deepak Bhat, Gurdeep Singh Dhooria, Puneet A. Pooni, Siddharth Bhargava, Shruti Kakkar & Karambir S. Gill

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  1. Jasjeet Sandhu
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  2. Deepak Bhat
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Contributions

JS, DB: recruited the subjects, collected the data, carried out the literature review and prepared the initial draft of the manuscript; PAP, SB, SK: contributed to manuscript writing; GSD: conceived and designed the study, analysed the data and finalized the manuscript. All authors have approved the manuscript submitted.

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Correspondence to Gurdeep Singh Dhooria.

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Sandhu, J., Bhat, D., Dhooria, G.S. et al. Oral cyclophosphamide therapy in 100 children with steroid-sensitive nephrotic syndrome: experience from a developing country. Pediatr Nephrol 36, 2759–2767 (2021). https://doi.org/10.1007/s00467-021-05052-5

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