儿童原发性局灶节段性肾小球硬化的临床与病理特点

Clinical and pathological characteristics of focal segmental glomerulosclerosis in children

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 clinical and pathological characteristics of focal segmental glomerulosclerosis （FSGS） in children, and to improve the diagnosis of the disease. Methods The data of 72 children who were diagnosed as FSGS were studied retrospectively. Results The children were divided into 5 subtypes based on the Columbia classification： non other specific subtype （56.9%）, tip variant （22.2%）, cellular variant （16.7%）, peri-hilar variant （2.8%）, and collapsing variant （1.4%）. The predominant presenting feature was nephritic syndrome. Tip variant always performed as simple nephritic syndrome, while the common subtype almost presented as nephritis-type syndrome. Conclusion The presenting features of the 5 variants are various. The renal biopsy is the golden standard to determine the different variants. The diagnosis of tip varianis is based on the number of the glomerular, while the diagnosis of cellular variant isn＇t. The improvement of the diagnosis depends on the comprehensive evaluation of the various types of FSGS.