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目的 探讨儿童局灶节段硬化性肾小球肾炎 (FSGS)临床及病理特点。方法 对 2 0例儿童FSGS患者的临床表现、病理改变以及对激素治疗的反应进行观察 ,并进一步分析三者间关系。结果 儿童FSGS的发生率为 5 %。临床表现以浮肿及蛋白尿为主 ,肾病综合征占 70 % ,高血压及肾功能不全分别占 1 0 %、5 %。半数患者对激素治疗敏感。激素治疗不敏感患者均伴有IgM的沉积 ,其中 90 %临床表现为肾病综合征。结论 1 .儿童FSGS患者临床及病理上都较成人轻 ,对激素治疗较成人敏感 ;2 .临床表现为肾病综合征或高血压或肾功能不全者对激素治疗不敏感 ;3.病理表现为IgM沉积或电子致密物沉积 ,或有间质纤维化者对激素治疗不敏感。
Objective To investigate the clinical and pathological features of focal segmental sclerosing glomerulonephritis (FSGS) in children. Methods The clinical manifestations, pathological changes and response to hormone therapy in 20 children with FSGS were observed and the relationship among them was further analyzed. Results The incidence of FSGS in children was 5%. Clinical manifestations of edema and proteinuria, nephrotic syndrome accounted for 70%, hypertension and renal insufficiency accounted for 10%, 5%. Half of patients are sensitive to hormone therapy. Hormone-insensitive patients are accompanied by IgM deposition, of which 90% of clinical manifestations of nephrotic syndrome. Children with FSGS are clinically and pathologically lighter than adults, more sensitive to hormone therapy than adults; 2. Clinical manifestations of nephrotic syndrome or hypertension or renal insufficiency of hormone therapy are not sensitive; 3 pathological manifestations of IgM Deposition or deposition of electron dense material, or interstitial fibrosis are not sensitive to hormone therapy.