Abstract
BACKGROUND: Steroid-resistant nephrotic syndrome (SRNS) is one of the most challenging conditions to be managed in pediatric nephrology that often requires a multi-step and individualized treatment approach; otherwise, it may continue to progress to end-stage renal disease. Histopathological evaluation with a kidney biopsy remains a cornerstone in the diagnosis, guiding the management through the identification of patterns such as focal segmental glomerulosclerosis and minimal change disease. Epidemiological data from the Middle East remain limited and underreported, with regional cohorts notably underrepresented in both genetic and therapeutic outcome studies. This study aims to characterize the histopathological findings of pediatric SRNS cases managed at Queen Rania Children's Hospital, offering locally relevant insights to inform diagnostic and treatment strategies. METHOD: This study is a retrospective study of medical records of pediatric patients with steroid-resistant nephrotic syndrome at Queen Rania Children's Hospital in Jordan during the period from January 2020 to August 2024. It included demographics, early presentation, histopathological findings, and response to treatment. RESULTS: A total of 72 children were diagnosed with steroid-resistant nephrotic syndrome (SRNS), of whom 53% (n = 38) were female. The most frequent initial presentation was generalized edema. Minimal change disease (MCD) was the predominant histopathological finding, identified in 34.7% (n = 25) of cases across all age groups, while focal segmental glomerulosclerosis (FSGS) was more commonly observed in older children aged 8-14 years. A positive family history was reported in 18.1% (n = 13) of participants. Angiotensin-converting enzyme inhibitors (ACEIs) were the most frequently prescribed adjunct therapy with prednisolone, used in 51.4% (n = 37) of cases for both blood pressure management and antiproteinuric effect. Overall, 76.3% (n = 55) of the cohort exhibited a favorable prognosis, defined by sustained remission, stable renal function, and absence of progression to end-stage renal disease (ESRD) during the follow-up period. These outcomes were most commonly observed in patients with minimal change disease (MCD) and those who responded to adjunct therapy with ACE inhibitors, highlighting the potential impact of early intervention. CONCLUSION: Pediatric SRNS requires individualized evaluation, as age-related diagnostic patterns and variable clinical presentations influence outcomes. While most patients responded favorably to targeted therapies, the risk of progression to ESRD still existed, as the underlying mechanisms of steroid resistance remain insufficiently understood. These findings support the need for personalized treatment approaches and ongoing research into the underlying immunologic, genetic, and histopathologic mechanisms driving steroid resistance.