Abstract: Objective · To explore the correlation between immunofluorescence (IMF) deposition and corticosteroid effect in childhood focal segmental glomerulosclerosis (FSGS) manifesting with nephrotic syndrome (NS).  Methods · Renal IMF deposition and clinical data of the children clinically diagnosed with NS and pathologically diagnosed with FSGS in the Department of Pediatric Nephrology in Xinhua Hospital, Shanghai Jiao Tong University from January 1990 to December 2015 were reviewed and analyzed retrospectively.  Results · The renal pathological types classified by IMF of 47 patients diagnosed with FSGS manifesting with NS showed that 2 cases (4.26%) were IgA type, 12 cases (14.89%) were IgM type, 4 cases (8.51%) were complement (C) type, 1 case (2.13%) was IgG+A+M type, 5 cases (10.64%) were IgG+A+M+C type, 1 case (2.13%) was IgA+C type, 12 cases (25.53%) were IgM+C type, and there was no immune complexes present in 15 cases (31.91%). The IMF deposition showed 9 cases with IgA+, 25 cases with IgM+, 8 cases with IgG+, 23 cases with C3+, 3 cases with C4+, 6 cases with C1q+, 5 cases with FN+, 12 cases with all negative. After 4 weeks of treatment with oral prednisone at full dose, complete remission was presented in 34 cases (72.34%), partial remission was presented in 7 cases (14.89%), and no remission was presented in 6 cases (12.77%). There was no statistically difference in the corticosteroid effect among the different types of IMF (H=1.792, P=0.408). The corticosteroid effect had statistical differences between C1q+ and C1q- patients (χ2=7.22, P=0.027), while it had no significant differences in other conditions.  Conclusion · In childhood FSGS manifesting with NS, C1q+ patients have relatively poor reaction to the corticosteroid therapy compared to C1q- ones.

Key words:  nephrotic syndrome, focal segmental glomerulosclerosis, immunofluorescence, corticosteroid, treatment effect, child