小儿肾小球薄基膜病的临床病理研究

Thin basement membrane nephropathy in children:A clinicopathological study

目的 :探讨小儿肾小球薄基膜病 (TBMN)的临床病理特征。方法 :对 11例TBMN患儿进行了临床、病理及超微结构的系统观察 ,测量了肾小球基膜 (GBM)及致密层的厚度 ,对小儿薄基膜病的临床病理特征进行了分析。结果 :11例小儿TBMN临床主要表现为单纯血尿 ,无其它明显的阳性体征 ,光镜下肾小球无明显改变或轻微异常 ,未见蛋白管型 ,包曼囊内及肾小管腔内可见渗出的红细胞 ,电镜下GBM广泛变薄 ,平均厚度 <2 0 0nm ,致密层厚度 <10 0nm。结论 :小儿肾小球薄基膜病的诊断主要依靠电镜 ,同时必须强调与临床病史、生化检查及病理组织学、免疫组化紧密结合方可确诊。

Purpose To study the clinicopathological features of thin basement membrane nephropathy (TBMN) in children. Methods Eleven cases of TBMN in children were observed clinically and pathologically with HE, special stainings (PAS,PASM and Masson),immunohistochemistry(IgA, IgG, IgM and C 3)and ultrastructural examination. Results Eleven cases of TBMN in children were showed as isolated hematuria,glomerule with normal appearance or slight change and characterized by diffuse thin basement membrane in ultrastructural observation( the thickness of GBM<200 nm, and dense layer<100 nm). Conclusion The diagnosis of TBMN in children depends on the ultrastructural observation and the measurement of thickness of GBM, incorporated with the history and full laboratory examination.