Primary focal segmental glomerulosclerosis in Egyptian children: a 10-year single-centre experience

Abstract

Primary focal segmental glomerulosclerosis (FSGS) in children accounts for 7-20% of all cases of idiopathic nephrotic syndrome (NS). To evaluate the clinical course of children with primary FSGS, the records of 72 patients with biopsy-proven FSGS admitted between 1995 and 2008 were retrospectively analysed. Forty-eight patients were male (66.7%). The mean age at presentation was 76.5 +/- 54 (range 12-192) months. The mean duration of follow-up was 76.3 +/- 42 (range 9-156) months. Seventeen patients (23.6%) presented with gross haematuria at initial presentation, 15 (20.8%) presented with hypertension and 10 patients (14%) presented with chronic renal insufficiency (CRI). The initial response to oral prednisolone was steroid resistance in 63 patients (87.5%) and steroid dependence in 4 patients (5%), while 5 patients (6.9%) were biopsied from the start because of atypical presentation. According to pathological classification, there were the tip variant (2%), collapsing variant (6%), perihilar variant (7%) and NOS (not otherwise specified; 85%). At the last clinical visit, 12 patients (16.7%) were in complete remission and 11 (15.3%) had progressed to CRI. Renal survival rates at 5 and 10 years were 93% and 68%, respectively. In conclusion, in this study we had a low percentage of FSGS, and fewer patients presenting with gross haematuria and hypertension compared with previous reports. In addition, the short-term overall renal survival seems to be better in our cohort.

A predictive model of progressive chronic kidney disease in idiopathic nephrotic syndrome

“…We also observed that 61 % of 113 patients with FSGS were-steroid-resistant. These data are in agreement with those of other studies that show a preponderance of steroid resistance among children with FSGS [33,34,37]. In the International Study of Kidney Disease in Children study, two thirds of 37 patients with FSGS did not respond to steroids [36].…”

“…It should be pointed out, however, that other studies also reported that resistance to steroids is a much better predictor of CKD in children with INS than the pathological diagnosis of FSGS [34,36,41]. In line with these studies, our findings have clearly shown that initial response to steroids is a good predictor of long-term renal survival in children with INS.…”

“…Although MCNS accounts for the majority of cases of INS in children, particularly those younger than 6 years old [9,28], we also found a significant percentage of FSGS, as observed by other authors [3,4,29,[33][34][35]. Three aspects might at least in part explain our results: (1) many cases of nonbiopsied INS presented a clinical course compatible with MCNS [9,28,36]; (2) more complex patients were referred to our unit after initial assistance in primary/secondary services; and (3) the prevalence of FSGS has been increasing over the last two decades [3,4].…”

NPHS2 mutations

“…We have previously reported the unfavorable prognosis of our patients with SRNS-FSGS. 9 Although the number of patients reported in this study was small with short duration of follow up, it was observed that in patients bearing NPHS2 mutations the disease started earlier, all of them had FSGS and none of them developed ESRD. However, the other clinical characteristics were comparable in patients with NPHS2 mutations and those without.…”

Association of Histologic Variants in FSGS Clinical Trial with Presenting Features and Outcomes

“…The FSGS-CT findings support significant differences in demographic features between histologic subtypes. Tip and collapsing variants were more common in teenagers and adults than children (5,6,12,(14)(15)(16)(17)(18)(19). Only 29% of tip patients were children aged 2-12 years at onset of FSGS, whereas 43% were over 18 years.…”