Focal segmental glomerulosclerosis in libyan children: tripoli children hospital experience (2000-2020)

Background: Focal Segmental Glomerulosclerosis (FSGS) is characterized by steroid resistant nephrotic syndrome. Pathologically it is consisted of glomerulosclerosis, which is both focal, involving a minority of glomeruli, and segmental, affecting a portion of the glomerular globe. The aims of this study are, to find out aLibyan experience on frequency, clinical presentations and outcomes among children with primary FSGS at Tripoli Children Hospital. Patients and methods: This is retrospective case series observational clinic-based study. Children who diagnosed as primary FSGS by renal biopsy were included in this study from 2000 to 2020 at Tripoli Children Hospital. The case sheet which used for data collection includes: Age at presentation, gender, Presence of hematuria, hypertension and renal impairment.  Family history of nephrotic syndrome and FSGS, response to steroid therapy, choice of treatment and clinical outcomes. Typical presentation included the extreme of age at presentation from 2-8 years, absent of persistent hematuria or absence of macroscopic hematuria, absence of persistent hypertension, renal impairment and low serum complement. Result: Were viewed files of 41 children diagnosed by renal biopsy as FSGS, male composed of 65.9% of children.  The age at diagnosis of nephrotic syndrome was 5.15 ± 3.29 [range 1 - 15 years]. 75.6% of children presented from 2-8 years of age. 15(36.6%) children had renal impairment, 9(22%) children had hematuria and 17(17.5%) had hypertension. 2 (4.9%) achieved remission initially, steroid resistant seen in 29 (70.7%) and 10 (24.3%) patients had steroid dependent and frequent relapse. Long-term follow-up showed complete remission in 24.4%. End-stage renal disease in 24.4%, and death in7.3%, 2.9%underwent successful renal transplantation, 7.3% transferred to adult for further follow up with adult nephrologist and 9.8% missed follow up. Conclusion: More than two third of the cases of FSGS in our population presented as steroid resistant nephrotic syndrome. 24.4% of FSGS progressed to ESRD.