Evaluation of Children with Nephrotic Syndrome: A Single- Center Experience

Objective: Nephrotic syndrome (NS) is one of the most frequent occurring chronic kidney diseases in children. In this study, our aim was to assess the sociodemographic structure, determination of clinical data, diagnostic approaches, treatment methods applied and the factors effective on prognosis in patients followed with a diagnosis of nephrotic syndrome in our clinic. Method: The files of 256 patients diagnosed with primary nephrotic syndrome between the ages of 0 and 18 who were followed in Department of Pediatric Nephrology during a period of 15 years between 2004 and 2019 were examined retrospectively. 220 patients whose data were sufficient and who had a sufficient follow-up time were included in the study. Results: 137 of our patients were male, 83 were female and the ratio of male/female was 1.65. Average onset age of the cases was 4.8 ± 2.6 years (1 months-16 years). Renal disease history in the family was 16.4% and the rate of consanguineous marriage was 25.9%. 24% of our patients were found to have microscopic hematuria, 28.2% were found to have hypertension. Of the patients who received biopsy, 32 were determined as FSGS, 18 as MCD, 11 as MPGN, 3 as IgM nephropathy, 2 as hereditary nephrite, 2 as MezPGN, 1 as MGN, 1 as congenital NS and 2 as other reasons. While a total of 193 patients (87.7%) were sensitive to steroid, 27 patients (12.2%) were found to be resistant to steroid. While 78 (35.5%) of the patients were followed with no relapse, 120 (54.5%) patients were relapsing and 22 (10%) patients had frequent relapses. In their follow-up, mortality rate of our patients was found as 2.7%. Conclusion: With this study, we assessed children with primary nephrotic syndrome in our area comprehensively for the first time. Consanguineous marriage rate was higher in our patients. Our findings were also remarkable in terms of demonstrating that primary NS follows its own course in each child depending on the underlying genetic structure.