常染色体显性多囊肾病Han:SPRD大鼠肾脏病理观察及其意义  

Morphology of renal tissue in Han:SPRD,a rat model of autosomal dominant polycystic kidney disease

作　　者：李林[1] 戴兵[1] 孙田美[1] 颜永碧[2] 梅长林[1] 

机构地区：[1]第二军医大学长征医院肾内科,上海200003 [2]基础医学部细胞生物学教研室,上海200433

出　　处：《第二军医大学学报》2005年第1期83-86,共4页Academic Journal of Second Military Medical University

基　　金：上海市科委重大基础项目基金(02JC14029);国家自然科学基金 ( 30170901 );国家科技部重大科技专项基金(2002AA2Z3130)

摘　　要：目的:观察常染色体显性多囊肾病(autosomal dominant polycystic kidney disease, ADPKD)的Han:SPRD大鼠模型的肾脏病理变化,为揭示ADPKD发病机制提供证据与线索。方法:选取雄性2 周龄Han:SPRD纯合大鼠(cy/cy)和3 个月龄杂合大鼠(cy/+),取其肾组织进行H E染色、PAS染色、Masson染色以及透射电镜观察。结果:光镜下发现Han:SPRD大鼠肾脏体积增大;囊肿衬里上皮细胞增殖显著;小管基膜增厚,可见灶性小管萎缩和较多蛋白管型;肾小球数目减少、体积缩小,渐趋废弃,呈明显缺血样改变,血管壁增厚。透射电镜可见肾小球毛细血管腔阻塞,基底膜薄厚不均,部分断裂;小管上皮细胞胞质脱落,微绒毛紊乱、部分缺如;细胞外间质成分稀少;囊肿衬里上皮细胞内可见大量线粒体,高尔基体、核糖体丰富。结论:Han:SPRD大鼠肾脏组织学和超微结构的改变可解释多囊肾病的部分临床表现;并观察到Han:SPRD大鼠肾小管上皮细胞微绒毛异常,为揭示疾病的发病机制提供了线索。Objective:To observe the renal histology and ultrastructural morphology of Han:SPRD rat model of autosomal dominant polycystic kidney disease(ADPKD).Methods: Two-week-old male homozygotes rats and 3-month-old male heterozygotes were examined in this study. The renal sections were stained with H-E, PAS and Masson and observed by light microscope and electron microscope. Results: Light micrographs of the kidneys showed that the kidney volume enlarged moderately. The cyst-lining epithelia were significantly hyperplastic and the basement of the tubule was thickened;focal tubular atrophy and protein casts could be observed frequently;the number and volume of glomerulus decreased;and the wall of blood vessels were thickened. All the manifestations indicated ischemic state.On transmission electron microscope examination, the capillary lumen was obstructive, and the glomerular basement membrane had various thickness with segmental disruption. The microvilli on the tubule brush borders were in disorder and vanished occasionally. The components of the extracellular matrix were decreased while the mitochondria, Golgi apparatus and ribosome in the cyst-lining epithelia were increased. Conclusion: The changes of renal histology and ultrastructural morphology of the Han:SPRD rat model can interpret the clinical presentations.The microvilli obnormalities of the tubule epithelial cells is observed for the first time,which provides clues to the pathogenetic mechanisms of the polycystic kidney disease.

关 键 词：多囊肾病 常染色体显性 动物模型 Han:SPRD大鼠 

分 类 号：R692.1[医药卫生—泌尿科学]