Özet:

Aim: Focal segmental glomerulosclerosis (FSGS) is one of the common causes of nephrotic syndrome (NS) in children. This study aims to determine the demographical data, clinical course, treatment and renal outcome of children with primary FSGS and report the experience of a single center. Material and Methods: A retrospective study of the long-term outcome of 38 patients with diagnosis of primary FSGS at a tertiary pediatric care hospital from the period July 2005 to July 2019 was conducted. Results: The study included 38 patients (23 female and 15 male) with FSGS, and the mean age at diagnosis was 8.5 ± 4.2 years. The mean follow-up duration was 4.8± 4.1 (1-14.6) years. Seventeen (44.7%) patients were steroid-resistant NS (SRNS) and 21 (55.3%) patients were steroid-sensitive NS (SSNS) [12 (31.6%) steroid-dependent NS (SDNS) and 9 (23.7%) frequently relapsing NS (FRNS)]. There was no significant difference between these groups in age, gender, hematuria, serum albumin and urine protein level at presentation (p > 0.05). Long-term follow-up showed that 47% of SRNS patients achieved complete remission, 23.5% partial remission and 29.4% resistant to all therapies. ESRD was developed 15.8% of the FSGS patients. Risk factors for poor prognosis were the presence of hypertension (HT) at admission, female gender, and unresponsiveness to initial treatment. Conclusion: Focal segmental glomerulosclerosis in childhood, shows changes in response to treatment and prognosis. In this study, we presented our data on risk factors affecting prognosis.

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