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

Abstract

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.

Hypomagnesemia and Fractional Excretion of Magnesium (FeMg) in Children with Nephrotic Syndrome

“…[16] A Retrospective study from our centre in 92 SRNS patients showed that 31.9% had initial resistance and 68.1% late resistance. [17] A higher proportion of late resistance in the present study could be due to the inclusion of patients in disease remission and remission rates are better in late compared to initial resistance. Most patients with SRNS course were treated with either of the CNI's; cyclosporine and tacrolimus were used for more than 2 years in 40% and 20% of SRNS patients respectively and 28% were administered rituximab additionally during the treatment course.…”

Treatment outcome of nephrotic syndrome and associated factors among Children at Public Specialized Hospitals of Bahir Dar City, northwest, Ethiopia: Retrospective study design

“…This might be due to patients with decreased urine output having decreased renal blood ow and reduced peri-renal capillaries, which in turn results in a poor outcome for NS. However, the nding was in contravention of the study conducted in India (43). This discrepancy might be due to differences in sample size, genetic, and ethnic differences, age variation (up to 18 years), and study design (follow-up study).…”

Mutation of NPHS1, NPHS2, WT1, LAMB2, COL4A5 and other genes in children with idiopathic steroid resistant nephrotic syndrome

“…Approximately 1020% of them have steroid resistant nephrotic syndrome (SRNS). [2][3][4] The patient who has no urinary remission within four weeks of prednisolone therapy 60 mg/m 2 /day followed by three intravenous pulses of methylprednisolone is termed SRNS. 5 Response to steroids varies due to various pathophysiological mechanisms.…”