脂蛋白肾病1例报告及文献复习

A case of lipoprotein glomerulopathy and a literature review of the subject

回顾性分析2011年6月在中南大学湘雅二医院诊断的1例脂蛋白肾病的临床表现、实验室以及肾活检病理检查结果。本病例以肾病综合征为主要临床表现,实验室检查有血浆载脂蛋白(apo)E和apoB升高,肾活检光镜显示肾小球毛细血管呈血管瘤样扩张,管腔内充满淡染的板层状空泡样物质;免疫组织化学测定显示毛细血管腔内apoE染色阳性;透射电镜显示毛细血管腔内充满大小不一的空泡样脂肪凝聚物,呈簇状或层状排列。脂蛋白肾病无特异性临床表现,诊断脂蛋白肾病除根据临床表现外,还应结合肾脏病理检查,特征性的病理表现在诊断脂蛋白肾病中起重要作用。

To improve the understanding of lipoprotein glomerulopathy, the clinical data of a single case of lipoprotein glomerulopathy, diagnosed at the Second Xiangya Hospital, Central South University in June 2011, were retrospectively analyzed. The analysis included clinical manifestation, laboratory results, and pathological information on kidney biopsy. Nephrotic syndrome was seen as the major clinical feature in this patient. Levels of serum apoE and apoB were increased. Obvious expansion of glomerular capillary cavities, filled with a weakly stained thrombus-like substance, were observed. ApoE immunohistochemical staining showed positive staining in capillary luminal contents. Transmission electron microscopy revealed capillary lurnina occluded by various foaming lipid depositions in clustered or layered arrangement. There were no special symptoms nor obvious abnormal laboratory results of the LPG patient. Diagnosis of LPG should be based on clinical manifestations and renal biopsy. Gharacteristic pathological performance plays an important role in the diagnosis oflipoprotein glomerulopathy.