血管壁淀粉样蛋白沉积型淀粉样变性肾病六例临床和病理分析[J].pdfVIP

·576· ．论著． 血管壁淀粉样蛋白沉积型淀粉样变性肾病 六例临床和病理分析 邹古明 董葆李文歌 【摘要】 目的通过报告6例血管壁淀粉样蛋白沉积型淀粉样变性肾病(VADAN)的临床和病 理特性，以提高对VADAN的诊治水平。方法收集6例VADAN患者行免疫病理、光镜及电镜检查， 并进行淀粉样蛋白的分型。结果6例患者均为中老年(52～73岁)患者，男女各3例。其中2例为 多发性骨髓瘤。多数病例仅呈轻度的蛋白尿和血尿。l例因合并微小病变型肾病，呈现肾病综合征 表现；1例因合并IgA肾病，呈现蛋白尿和血尿；l例因骨髓瘤管型肾病，呈现急性。肾衰竭。经肾活检 证实，淀粉样蛋白仅沉积于小叶间动脉。6例患者均为原发性系统性淀粉样变性肾病，2例多发性骨 髓瘤继发者，为K型，其余为入型。结论VADAN属于少见的淀粉样变性。肾病类型，临床表现不同于 常见的淀粉样变性肾病，肾活检有助于其鉴别诊断。 【关键词】淀粉样变性； 肾疾病；病理学，临床；淀粉样蛋白 Theclinicaland fbatllresofvascular of in6 pathologic amyloiddepositsamyloidnephropathy ZOU patients Gu—ruing+，DONGBao，LIWen—ge．‘CenterofNephrology，China—JapanFriendship 100029，China Hospital，Beijing Corre印o，以i，曙author：ZOUGu—ming，Email：cliffzou@口咖．sina．corn To clinicaland featuresofararevascular 【Abstract】Objective analyze pathologic amyloid of 6 asto its andtreatment． depositsamyloidnephropathy(VADAN)inpatients，SOimprovediagnosis MethodsAll received andelectron patients immunopathology，microscopymicroscopyexamination，and were Therewere3 malesand3femaleswith from52to73 types ages amyloid analyzed．Results ranging sufferedfrom had albuminuriaandhematuria． years．Twopatients multiplemyeloma．Majoritypatientsslight One combinedwithminimal disease patient changegtomerularpresentednephroticsyndrome．Onepatient combinedwith hadalbuminuriaandhematuria．Andone had east IgAnephropathy patientmyeloma withacuterenal interlobulararterialwall nephmpathy failure．Kidneybiopsyprovedamyloiddepositsalong inall6 cases from wereK therests