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Outcomes of children with idiopathic steroid resistant nephrotic syndrome: a single centre observational study
Om P Mishra1, Minketan Sidar1, Vineeta V Batra2
1Banaras Hindu University, Institute of Medical Sciences, Department of Pediatrics, Division of Pediatric Nephrology, Varanasi, India.
Insights
Half of children with idiopathic steroid resistant nephrotic syndrome (SRNS) achieved remission. Factors like age, medication, and histology did not impact remission or kidney function survival in this 5-year study.
Area of Science:
- Pediatric Nephrology
- Immunology
- Clinical Medicine
Background:
- Idiopathic steroid-resistant nephrotic syndrome (SRNS) presents variable outcomes in pediatric populations.
- Understanding factors influencing remission and kidney function is crucial for managing SRNS.
Purpose of the Study:
- To determine the cumulative remission rate in children with SRNS.
- To identify factors affecting remission status, kidney function survival, and medication side effects.
Main Methods:
- A cohort of 114 pediatric patients with SRNS was analyzed over a 5-year follow-up period.
- Treatment involved a calcineurin inhibitor-based protocol, prednisolone, and angiotensin-converting enzyme inhibitors.
- Data collected included age of onset, eGFRcr, histopathology, and adverse events.
Main Results:
- 57.3% of patients achieved remission (30% complete, 27.3% partial).
- Kidney function survival was 87.3%, with 12.7% progressing to chronic kidney disease (CKD).
- Age of onset, calcineurin inhibitor use, and histopathology did not significantly influence remission or kidney function survival.
Conclusions:
- Approximately half of pediatric SRNS patients achieved remission.
- Early disease onset, specific medications (cyclosporine/tacrolimus), and histopathology did not predict remission or short-term kidney function outcomes.
- Further research may explore other therapeutic targets for SRNS.
Introduction:
Idiopathic steroid resistant nephrotic syndrome (SRNS) has variable outcomes in children. The primary objective of the present study was to assess the cumulative remission rate and the secondary objectives were to assess factors affecting the remission status, kidney function survival, and adverse effects of medications.
Methods:
One hundred fourteen patients with SRNS were included. Calcineurin inhibitor-based treatment protocol along with prednisolone and angiotensin-converting enzyme inhibitor were used, and patients were followed over 5 years.
Results:
Median age was 4.5 years; 53.5% of cases were between 1 to 5 years of age. Sixty-two patients (54.4%) were at initial stage and 52 (45.6%) were at a late SRNS stage. Median eGFRcr was 83.5 mL/min/1.73m2 at presentation. Of the 110 patients, 63 (57.3%) achieved remission [complete remission 30 (27.3%), partial remission 33 (30%)], and 47 (42.7%) had no remission. Kidney function survival was 87.3% and 14 cases (12.7%) had progression to CKD (G3-8, G4-3, G5-1, and G5D-2). Median duration of follow up was 36 months (IQR 24, 60). Age of onset, cyclosporine/tacrolimus, eGFRcr, and histopathology (MCD/FSGS) did not affect remission. Similarly, remission status in addition to age of onset, drug protocol, and histopathology did not significantly affect kidney function during a period of 5 years. Hypertension, cushingoid facies, short stature, cataract, and obesity were observed in 37.7, 29.8, 25.5, 17.5, and 0.7% of cases, respectively.
Conclusion:
About half of the cases achieved remission. Age of onset of disease, cyclosporine/tacrolimus use, and histopathological lesion neither affected remission status nor short-term kidney function survival in SRNS.
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