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Year : 2023  |  Volume : 8  |  Issue : 1  |  Page : 7-23

Steroid-resistant nephrotic syndrome in children: Clinicohistology and pattern of response to immunosuppressive

Department of Paediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh

Correspondence Address:
Prof. Ranjit Ranjan Roy
Department of Paediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Shahbag, Dhaka 1000
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/pnjb.pnjb_5_23

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Background: Nephrotic syndrome (NS) is the most common glomerular disease in childhood. With corticosteroids, 80%–85% of children achieve remission, although 10%–15% remain refractory or develop steroid resistance in NS. Steroid-resistant nephrotic syndrome (SRNS) management is difficult due to its varied etiology, frequent lack of remission induced by immunosuppressive treatment, and consequences including drug toxicity, infections, thrombosis, end-stage kidney disease, and recurrence following renal transplantation. Renal histology and genetic mutation play an important role in the management of SRNS. Calcineurin inhibitor (CNI) is the first-line drug of SRNS. In CNI-resistant and multidrug-resistant cases, rituximab and newer drugs are a good option. There is a 35%–50% chance of developing chronic kidney disease (CKD) in SRNS, and supportive measures are required in that case. Few studies have described clinical characteristics, renal biopsy patterns, and treatment outcomes in Bangladeshi children with SRNS. This study evaluated the clinical characteristics at diagnosis, histological pattern, immunosuppressive therapy, and patient outcomes after immunosuppressive medication. Materials and Methods: All the records of children (1–18 years old) with idiopathic NS who had been followed up in the Pediatric Nephrology Department, Bangabandhu Sheikh Mujib Medical University (BSMMU), from January 2019 to December 2021 were reviewed retrospectively. Results: Among 606 NS cases during the study period, 90 cases (14.85%) were SRNS. Males comprised 70% of the population, whereas females made up 30%. The mean age at the time of diagnosis was 76.21 ± 51.55 months. Hematuria was found in 35.5% of cases, hypertension in 40.8%, and impaired renal function in 34.2% at the time of presentation. Children had primary resistance in 65.7% of cases. Biopsy report was found in 65 cases. The most common histopathological pattern were minimal change disease (MCD) (43.08%) and focal segmental glomerulosclerosis (FSGS) (38.46%). Complete remission was achieved in 66% of cases, and 14% developed CKD. Renal impairment and mesangioproliferative glomerulonephritis (MesPGN) increased the risk for CKD. Patients who received CNI or CNI and mycophenolate mofetil had a higher rate of complete remission. Conclusion: Hypertension and hematuria were prevalent in SRNS children. Most lesions were MCD. CNI or combination therapy remissioned more children. Renal impairment at presentation and non-MCD lesion increased CKD risk (FSGS and MesPGN). Long-term follow-up is needed to confirm poorer renal survival.

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