Zou G M, Zhuo L, Tan M, Li W G
Division of Nephrology, China-Japan Friendship Hospital, Beijing 100029, China.
Zhonghua Yi Xue Za Zhi. 2018 Sep 25;98(36):2910-2913. doi: 10.3760/cma.j.issn.0376-2491.2018.36.007.
To investigate the clinicopathologic features of lipoprotein glomerulopathy (LPG). A total of 6 cases (5 males and 1 female, with a mean age of 27.5 years and age range of 11-53 years) of lipoprotein glomerulopathy with complete clinicopathologic data were enrolled. Except for light microscope, immunofluorescence and electron microscopic examination, renal biopsy tissues were checked by oil red O staining. The gene map of apolipoprotein E (ApoE) of 2 cases were analyzed. All 6 cases presented with heavy proteinuria or nephrotic syndrome, and high level of low-density lipoprotein (LDL), very low-density lipoprotein (vLDL), ApoE. Family history of LPG was found in 3 cases, and 2 patients progressed to uremia, or even to death. Pathologic features showed that lipoprotein deposited in glomerulus capillary lumen and renal tubular epithelial cells. Gene analysis demonstrated that 2 cases expressed abnormal ApoE gene (162G>C and 455G>C). Lipoprotein glomerulopathy is autosomal-recessive disease with mutation of ApoE. Common clinical manifestations of LPG are heavy proteinuria or nephrotic syndrome, with a poor prognosis. Renal biopsy pathologic diagnosis can confirm this kidney disease. Emboli of lipoprotein being observed in glomerulus capillary lumen is the pathological feature of LPG.
探讨脂蛋白肾小球病（LPG）的临床病理特征。纳入6例具有完整临床病理资料的脂蛋白肾小球病患者（5例男性，1例女性，平均年龄27.5岁，年龄范围11 - 53岁）。除进行光镜、免疫荧光及电镜检查外，肾活检组织行油红O染色检查。分析2例患者载脂蛋白E（ApoE）的基因图谱。6例患者均表现为大量蛋白尿或肾病综合征，低密度脂蛋白（LDL）、极低密度脂蛋白（vLDL）、ApoE水平升高。3例有LPG家族史，2例患者进展至尿毒症甚至死亡。病理特征显示脂蛋白沉积于肾小球毛细血管腔及肾小管上皮细胞。基因分析表明2例存在ApoE基因异常（162G>C和455G>C）。脂蛋白肾小球病是一种伴有ApoE突变的常染色体隐性疾病。LPG常见临床表现为大量蛋白尿或肾病综合征，预后较差。肾活检病理诊断可确诊本病。肾小球毛细血管腔内可见脂蛋白栓子是LPG的病理特征。
