Primary focal segmental glomerulosclerosis in children: epidemiology, clinical presentation and prognosis

Focal segmental glomerulosclerosis is a histopathological entity.

To analyze the epidemiological, clinical and histological profile of primary focal segmental glomerulosclerosis in children, as well as their prognostic factors.

This was a retrospective cross-sectional study over a period of 20 years (2001-2020), conducted in the Department of Pediatrics at Charles Nicolle Hospital in Tunis, which included children followed for primary focal segmental glomerulosclerosis.

There were 35 children, 19 boys and 16 girls. The median age was 4.5 years. Nephrotic syndrome was seen in 88% of patients. Macroscopic hematuria was found in 4 cases, hypertension in 8 cases and renal failure in 7 cases at presentation. The most common variant was the not otherwise specified variant (77.1%). Steroid-sensitive nephrotic syndrome was observed in 71% of cases, and steroid-resistance in 29% of cases. Treatment with cyclosporine was indicated in 23 patients with complete remission rate of 56.5%. 42,8% children had progressed to chronic kidney disease, including an end-stage renal disease in 11.5% of cases. Only the presence of a family history of kidney disease was found as a predictive factor of progression to chronic kidney disease and end-stage renal disease. Renal survival rates were estimated at 100% at 3 years, 85% at 5 years and 73% at 10 years.

Identification of patients at high risk for chronic kidney disease progression, such as those with a family history of kidney disease or those who have failed to respond to corticosteroids, would allow therapeutic adjustments.