脂蛋白肾病临床与病理分析

Clinical and Pathological Analyses of Lipoprotein Glomerulopathy

目的 探讨脂蛋白肾病的临床病理特点。方法 回顾性分析 3例经肾活检病理证实为脂蛋白肾病患者的临床病理特点。结果 男 1例 ,女 2例 ,汉族 ,均因浮肿就诊。 1例有肾脏病家族史。尿蛋白 0 .8～ 1.5 g/ 2 4 h,血清白蛋白降低 ,伴贫血。 1例血清肌酐轻度升高 ,血压升高 ,血清甘油三脂和胆固醇升高。 1例肾脏体积增大。肾活检发现肾小球体积增大 ,毛细血管袢扩张 ,袢内见淡染栓子 ,呈网眼样不嗜银的结构。免疫荧光示免疫球蛋白和补体阴性或少量非特异沉积 ,袢内物质 apo E染色阳性。结论 脂蛋白肾病患者多表现浮肿、蛋白尿 ,伴低白蛋白血症与贫血 ,肾活检发现毛细血管袢内脂蛋白栓子 ,apo E染色阳性是确诊的关键。

Objective To investigate the clinical and pathological characteristics of lipoprotein glomerulopathy. Methods We retrospectively analyzed 3 cases of lipoprotein glomerulopathy. Results The 3 patients, 1 male and 2 females, were young Hans. They were admitted to our hospital because of edema. Patient 1 had a positive family history. Her proteinuria ranged between 0.8-1.5 g/d, her serum albumin levels were below the normal lower limit, and she was afflicted with anemia. Patient 2 was found having slightly increased serum creatinine, hypertension, and increased total cholesterol and triglyceride level. The kidneys of patient 3 were enlarged. Increments of glomerular size and capillary lumen space were observed under microscope. Bioptic specimens of the patients' kidneys displayed extensive prominent lucent casts in the capillary lumen, which were stained as pale mesh-like substance and were not stained by silver impregnation. Immunofluorescence microscopy revealed faint immunoglobulin deposit. These casts were stained positive for apoE. Conclusion Lipoprotein glomerulopathy is pathologically characterized by extensive glomerular capillary casts which are stained positive for apoE, and clinically it is characterized by edema, proteinuria, hypoalbuminaemia and anemia.