Steroid response and outcomes in childhood nephrotic syndrome: A multicenter, cross-sectional study from Jordan

Childhood nephrotic syndrome (NS) outcomes vary widely based on steroid responsiveness and complications.

To evaluate steroid response, outcomes, and the use of steroid-sparing medications in children with nephrotic syndrome in Jordan.

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.

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.

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.