Steroid resistant nephrotic syndrome in children: Clinical presentation, renal histology, complications, treatment and outcome at Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh

Rr, Roy; Sms, Haque; Mamun, Abdullah Al; Muinuddin, Golam; Mh, Rahman

doi:10.9790/3013-040110107

iosrphr

2014

DOI: 10.9790/3013-040110107

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Steroid resistant nephrotic syndrome in children: Clinical presentation, renal histology, complications, treatment and outcome at Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh

Roy Rr¹,

Haque Sms²,

Abdullah Al Mamun³

et al.

Abstract: Steroid resistant nephrotic syndrome (SRNS) remains a challenge for pediatric nephrologists. The underlying histopathology usually affects the course of the disease and the response to treatment. This study was designed to determine clinical presentation, renal histology, complications, treatment and outcome in children presenting with SRNS. A prospective analysis was carried out among 32 steroid resistant nephrotic syndrome patients aged 1-18 year in the

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Cited by 2 publications

References 20 publications

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Study of steroid-resistant nephrotic syndrome: a single center experience

Elbeltagi,

Kersh,

Fathy

et al. 2024

Egypt Pediatric Association Gaz

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Background Nephrotic syndrome (NS) is a disease with favorable outcomes in most cases. Failure to respond to steroids is one of the most important predictors of clinical outcome. We performed a retrospective study on 51 primary idiopathic SRNS cases presenting between January 2011 and June 2021 at Alexandria University Children’s Hospital (AUCH) by retrieving data from their clinic files. Cases with secondary causes of NS or suspected genetic causes were excluded from our study. Patients’ demographic data, clinical and laboratory findings at the time of presentation, complications occurring during the course of treatment, immunosuppressants used along with steroids, and outcomes at the last follow-up were recorded. The mean duration of follow-up was 5.67 ± 3.07 years. Results Our results showed that there was male predominance (2.9:1), the mean age at first presentation was 4.53 ± 3.03 years, and persistent hematuria and systemic hypertension were found in 41.6% and 57.1%, respectively. The most common biopsy finding was MesPGN (37.3%). The most utilized immunosuppressant was cyclosporine (80.4%). Complete remission was fortunately the most prevalent outcome among our cases (52.2%). Conclusions Our study concluded that biopsy findings and thepresence of hematuria in SRNS cases are the most crucial factors in determining the final outcome. MCNS finding in biopsy is significantly correlated with complete remission (p value = 0.043). Persistent hematuria whether gross or microscopic was significantly correlated with unremitting disease (p value = 0.017).

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Study of steroid-resistant nephrotic syndrome: a single center experience

Elbeltagi,

Kersh,

Fathy

et al. 2024

Egypt Pediatric Association Gaz

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Steroid-Resistant Nephrotic Syndrome in Children

Roy,

Sultana,

Jesmin

et al. 2023

Paediatric Nephrology Journal of Bangladesh

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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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Steroid resistant nephrotic syndrome in children: Clinical presentation, renal histology, complications, treatment and outcome at Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh

Cited by 2 publications

References 20 publications

Study of steroid-resistant nephrotic syndrome: a single center experience

Study of steroid-resistant nephrotic syndrome: a single center experience

Steroid-Resistant Nephrotic Syndrome in Children

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