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Year : 2022  |  Volume : 32  |  Issue : 4  |  Page : 320-326

Childhood steroid-resistant nephrotic syndrome: Long-term outcomes from a Tertiary Care Center

1 Department of Pediatrics, Army College of Medical Sciences, New Delhi, India
2 Department of Pediatrics, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi, India
3 Department of Pathology, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, India

Correspondence Address:
Mukta Mantan
Department of Pediatrics, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijn.ijn_258_21

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Introduction: Steroid-resistant nephrotic syndrome (SRNS) is a rare condition that accounts for about 10% to 20% of all nephrotic syndromes in children. While calcineurin inhibitors induce remission in the majority, the data on long-term outcomes are limited. This retrospective study aimed to look at the clinical profile, biopsy findings, and long-term treatment outcomes in children with SRNS. Methods: The records of all children (1–18 years) with SRNS with biopsy findings of minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), or mesangioproliferative glomerulonephritis, who received treatment for a minimum period of 12 months and were in follow-up during the years 2007–2018 at a tertiary care teaching hospital were retrieved. The clinical, histopathological, and biochemical factors and treatment outcomes were recorded and analyzed. Results: Ninety-one (72 boys) children with a median (interquartile range [IQR]) age of onset of nephrotic syndrome as 48 (24–87) months were included. MCD and FSGS were the most common histopathological types (57.1% and 36.3%, respectively) and 62 (68.1%) patients had late steroid resistance. Calcineurin inhibitors (CNIs) were used in 86.8% of the children, and response rates with cyclosporine and tacrolimus for complete remission (CR) were 80% and 73.7%, respectively, with median (IQR) time to response being 3 (2–4) months. The presence of MCD on histology and the use of CNIs were significantly associated with CR (P < 0.01). At a median (IQR) follow-up of 5 (3–7) years, 76 (83.5%) children had either CR or partial remission, four (4.4%) developed chronic kidney disease and five (5.5%) died (three due to end-stage renal disease and two of infective complications). Conclusion: SRNS children with MCD on biopsy, late resistance, and response to CNIs have better long-term outcomes. Most patients respond to CNIs within the first 6 months of use and need therapy for at least 24 to 36 months.


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Indian Journal of Nephrology
Published by Wolters Kluwer - Medknow
Online since 20th Sept '07