Clinical and Pathological Analysis of 48 Children with Focal Segmental Glomerulosclerosis / 实用儿科临床杂志

ABSTRACT

Objective To investigate the relationship between the clinical character and pathological variants as well as response to treatment of focal segmental glomerulosclerosis(FSGS) in children.Methods The clinical feature, laboratory data and histological variants of 48 children with idiopathic FSGS proven by renal biopsy were studied retrospectively. Among them,34 were boys;14 cases were girls. The age ranged from 1.75 to 16.0 years.Results Of 48 children with FSGS,nephrotic syndrome in 32(66.7%)was the predominant clinical manifestation. The pathological classification included FSGS not otherwise specified(NOS) in 29 cases(60.42%), perihilar variant in 14 cases(29.17%), tip variant 3 cases(6.25%), cellualr variant 2 cases(4.17%)and no collapsing variant. The different pathological variants had similar rate of segmental sclerosis glomeruli; perihilar variant had a higher global sclerosis glomeruli than FSGS NOS and other variants and there was a statistical significance(P 0.05).Vascular lesions were rarely seen in all kinds of histological variants of children.Conclusions Hypertension and renal insufficiency are less frequently seen in children FSGS, isolated hematuria as unique clinic presentation is common in FSGS. All pathological variants have tubular-interstitial lesions, but vascular lesions are rarely seen. Most FSGS children with nephrotic syndrome are sensitive to steroid at initial stage, and easy to develop frequent relapse gradually, immunosuppressive agent may be helpful to elevate remission rate.