Pediatric focal segmental glomerulosclerosis in Jordan: A tertiary hospital experience

Area of Science:

• Pediatric Nephrology
• Glomerular Diseases
• Renal Pathology

Background:

• Primary focal segmental glomerulosclerosis (FSGS) is a leading cause of nephrotic syndrome in children.
• Understanding the epidemiology and clinical course of FSGS is crucial for improving patient outcomes.
• Data on pediatric FSGS in the Middle East, particularly Jordan, is limited.

Purpose of the Study:

• To investigate the demographic characteristics, clinical presentation, and outcomes of children diagnosed with primary FSGS in Jordan.
• To identify specific risk factors and treatment responses within this cohort.
• To assess the prevalence of familial FSGS and its impact on disease progression.

Main Methods:

• Retrospective chart review of 99 pediatric patients diagnosed with FSGS.
• Data collected from July 2010 to July 2016 at a tertiary care hospital.
• Analysis included demographics, clinical course, treatment, and long-term outcomes.

Main Results:

• The mean age of presentation was 3.71 years, with 66% males.
• 66.6% of patients presented with steroid resistance; 20.2% had familial FSGS.
• Cyclosporine showed a 46.9% response rate. Long-term outcomes included 29.3% complete remission, 31.3% partial remission, 22.2% end-stage renal disease, and 11.1% mortality.

Conclusions:

• Jordanian children with FSGS exhibit a high prevalence of the familial form.
• A significant proportion of patients progress to end-stage renal disease, highlighting the aggressive nature of FSGS in this cohort.
• Early identification and tailored treatment strategies are essential for managing pediatric FSGS.