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目的探讨原发性局灶节段性肾小球硬化(FSGS)的病理诊断和病理特点,提高对本病的诊断水平。方法对2005年6月至2013年6月病理确诊的72例原发性FSGS患儿的临床资料进行回顾性分析。依据2004年原发性FSGS组织病理学分型标准,分为非特异型、顶部型、细胞型、门部型、塌陷型5个亚型,比较不同病理分型的病理和临床特点。结果 72例原发性FSGS患儿中,非特殊型占56.9%,顶端型占22.2%,细胞型占16.7%,门部型占2.8%,塌陷型占1.4%。本病临床表现以肾病综合征为主,顶部型以单纯型肾病综合征为主(87.5%),非特殊型以肾炎型肾病综合征为主(78.0%)。结论 FSGS临床及病理表现不均一,不同病理类型之间存在差异。顶部型FSGS诊断的确立依赖标本中肾小球的数目,细胞型FSGS诊断不依赖标本中肾小球的数目。电镜下足细胞空泡变性易见于非特殊型局灶节段性肾小球硬化患儿。全面评价各型FSGS临床病理特征,有助于提高诊断的准确性。
Objective To investigate the pathological features and pathological features of primary focal segmental glomerulosclerosis (FSGS) and to improve the diagnosis of this disease. Methods The clinical data of 72 patients with primary FSGS diagnosed from June 2005 to June 2013 were analyzed retrospectively. According to the 2004 primary FSGS histopathological classification criteria, divided into non-specific type, top type, cell type, portal type, collapsed type 5 subtypes, pathological and clinical features of different pathological types. Results 72 cases of children with primary FSGS accounted for 56.9% of non-special type, 22.2% of the top type, cell type accounted for 16.7%, portal type accounted for 2.8%, collapse accounted for 1.4%. The clinical manifestations of the disease mainly nephrotic syndrome, the top type of simple nephrotic syndrome (87.5%), non-special nephritic nephrotic syndrome (78.0%). Conclusion The clinical and pathological findings of FSGS are not uniform, and there are differences between different pathological types. Top FSGS diagnosis depends on the number of glomeruli in the specimen, and the diagnosis of cell type FSGS does not depend on the number of glomeruli in the specimen. Electron microscopic degeneration of podocytes easily seen in non-special focal segmental glomerulosclerosis in children. A comprehensive evaluation of various types of FSGS clinicopathological features, help to improve the diagnostic accuracy.