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  • Title: [Clinicopathologic features of lipoprotein glomerulopathy: observation of 6 cases].
    Author: Zou GM, Zhuo L, Tan M, Li WG.
    Journal: Zhonghua Yi Xue Za Zhi; 2018 Sep 25; 98(36):2910-2913. PubMed ID: 30293348.
    Abstract:
    Objective: To investigate the clinicopathologic features of lipoprotein glomerulopathy (LPG). Methods: 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. Results: 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). Conclusions: 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. 目的： 观察和探讨脂蛋白肾病的临床和病理特点。 方法： 回顾性分析2008年1月至2018年1月中日友好医院肾病科6例临床和病理资料完整的脂蛋白肾病患者资料，其中男5例，女1例，年龄11～53岁，平均27.5岁。肾活检标本经光学显微镜、特殊染色、透射电子显微镜观察，通过基因分析及追踪随访，总结和分析该病的诊断要点和预后。 结果： 6例脂蛋白肾病均表现为大量蛋白尿和肾病综合征，血低密度和极低密度脂蛋白升高，载脂蛋白E升高，其中3例有家族史，2例发展为尿毒症或死亡。肾活检显示肾小球毛细血管腔和肾小管上皮细胞内脂蛋白沉积，脂蛋白栓子样物质形成。其中2例载脂蛋白E基因检测发现162G>C及455G>C基因突变。 结论： 脂蛋白肾病属于常染色体隐性遗传载脂蛋白E基因突变导致的肾脏疾病。临床表现为大量蛋白尿和肾病综合征，预后差，肾活检病理检查是确诊的方法，肾小球脂蛋白栓子样物质是其特殊的病变特征。.
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