World journal of clinical pediatrics · 2025
Steroid response and outcomes in childhood nephrotic syndrome: A multicenter, cross-sectional study from Jordan.
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Ajarmeh Salma A, Akl Kamal, Al Shawabkeh Muna, Al Baramki Jumana
Аннотация
BACKGROUND: Childhood nephrotic syndrome (NS) outcomes vary widely based on steroid responsiveness and complications.
AIM: To evaluate steroid response, outcomes, and the use of steroid-sparing medications in children with nephrotic syndrome in Jordan.
METHODS: This retrospective study evaluated the demographics and outcomes of 122 children aged 1-18 years with NS between 2011 and 2021 across three centers in Jordan. The outcomes assessed included steroid sensitivity rates, dependence, frequent relapses, complications [chronic kidney disease (CKD), end-stage kidney disease (ESKD)], infections, and need for steroid-sparing treatment.
RESULTS: Of 64% were boys; median age of disease onset was 4 years. Steroid-sensitive and steroid-resistant NS (SRNS) were observed in 81.1% and 18.9% of patients, respectively; 28.7% and 9% had steroid-dependent and frequently relapsing NS, respectively. Kidney biopsies were conducted in 46.7%, the most common finding was minimal change disease in 56.1%; 82.6% of biopsied SRNS cases showed focal segmental glomerulosclerosis. The median time to first relapse was 9 months. 41.8% of patients required steroid-sparing medications, with cyclosporine and mycophenolate mofetil being the most frequently used. Despite these treatments, relapse occurred in 11.5% of cases. Infections, primarily urinary tract infections, affected 24.6% of patients, 7.4% progressed to high-grade CKD, and 6.6% required dialysis. SRNS was significantly associated with hematuria, hypertension, and ESKD.
CONCLUSION: Most patients were steroid sensitive, with minimal change being the most common. Focal segmental glomerulosclerosis was the predominant histopathology in the steroid-resistant cases. SRNS patients had worse outcomes, with more infections, CKD, and ESKD.
AIM: To evaluate steroid response, outcomes, and the use of steroid-sparing medications in children with nephrotic syndrome in Jordan.
METHODS: This retrospective study evaluated the demographics and outcomes of 122 children aged 1-18 years with NS between 2011 and 2021 across three centers in Jordan. The outcomes assessed included steroid sensitivity rates, dependence, frequent relapses, complications [chronic kidney disease (CKD), end-stage kidney disease (ESKD)], infections, and need for steroid-sparing treatment.
RESULTS: Of 64% were boys; median age of disease onset was 4 years. Steroid-sensitive and steroid-resistant NS (SRNS) were observed in 81.1% and 18.9% of patients, respectively; 28.7% and 9% had steroid-dependent and frequently relapsing NS, respectively. Kidney biopsies were conducted in 46.7%, the most common finding was minimal change disease in 56.1%; 82.6% of biopsied SRNS cases showed focal segmental glomerulosclerosis. The median time to first relapse was 9 months. 41.8% of patients required steroid-sparing medications, with cyclosporine and mycophenolate mofetil being the most frequently used. Despite these treatments, relapse occurred in 11.5% of cases. Infections, primarily urinary tract infections, affected 24.6% of patients, 7.4% progressed to high-grade CKD, and 6.6% required dialysis. SRNS was significantly associated with hematuria, hypertension, and ESKD.
CONCLUSION: Most patients were steroid sensitive, with minimal change being the most common. Focal segmental glomerulosclerosis was the predominant histopathology in the steroid-resistant cases. SRNS patients had worse outcomes, with more infections, CKD, and ESKD.