脂蛋白肾病2例临床病理分析  

Lipoprotein glomerulopathy: a clinicopathological analysis of 2 cases

作　　者：张静[1] 刘彦仿[1] 刘健[1] 陈威[2] 王汉民[2] 崔继红[1] 任丽君 

机构地区：[1]第四军医大学西京医院病理科,西安710033 [2]第四军医大学西京医院肾内科,西安710033 [3]澄城县医院,陕西澄城715200

出　　处：《诊断病理学杂志》2005年第3期188-191,i007,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的阐明脂蛋白肾病的临床病理特点。方法回顾性分析2例脂蛋白肾病患者的临床表现,并对其肾活检组织进行光镜、免疫组化免疫荧光及超微结构观察。结果2例脂蛋白肾病患者均存在大量蛋白尿、肾病综合征,有程度不同的贫血及镜下血尿,血清总胆固醇及甘油三酯升高。B超检查双肾体积增大。镜检可见肾小球体积明显增大,毛细血管襻高度扩张,襻内可见层状改变的大小不同、多少不等的栓子。免疫组化染色证实“栓子”apoE染色(+),超微结构观察证实“栓子”内含颗粒状嗜锇酸脂质空泡。结论脂蛋白肾病属少见肾病,患者临床常表现为蛋白尿、肾病综合征,肾小球毛细血管襻内apoE染色阳性的巨大脂蛋白栓子是脂蛋白肾病特征性的形态学改变。Objective To investigate the clinical and pathological characteristics of lipoprotein glomerulopathy (LPG). Methods Clinical manifestations of 2 LPG patients were analyzed retrospectively. Light, electron microscopy and immunofluorescence/immunohistochemical stain were performed with renal tissues from biopsy. Results All patients had mass proteinuria, nephritic syndrome, anaemia and microscopic hematuria. The levels of total cholesterin and triglyceride in serum were elevated. The sizes of kidneys and glomerule were enlarged. Prominent laminated thrombi were found extensively in dilated, balloon-like capillary lumina. Immunohistochemical staining showed that the thrombi were strongly positive for apoE. Granules and various sizes of lipidic vacuoles in the thrombi were observed under electronic microscope. Conclusion LPG is a unique renal disease characterized by extensively intraglomerular lipoprotein thrombi with apoE positive reaction, and its clinical characteristics are mass proteinuria and nephritic syndrome.

关 键 词：脂蛋白肾病 临床病理 载脂蛋白E 

分 类 号：R692.6[医药卫生—泌尿科学]