肾淀粉样变性病32例临床病理特点及误漏诊分析  

The clinical-pathologic characteristics and misdiagnose causes in 32 patients with renal amyloidosis

作　　者：曲贞[1] 郑欣[1] 鄂洁[1] 周福德[1] 王素霞[2] 刘刚[1] 

机构地区：[1]北京大学第一医院肾内科(北京大学肾病研究所),北京100034 [2]北京大学第一医院电镜室,北京100034

出　　处：《中国实用内科杂志》2009年第1期75-77,共3页Chinese Journal of Practical Internal Medicine

基　　金：北京大学"985"工程二期建设项目(985-2-033-39);北京大学第一医院归国人员科研基金

摘　　要：目的从多系统受累临床表现和病理分型方面了解肾淀粉样变性病特点,分析临床误漏诊情况及原因。方法回顾性分析北京大学第一医院1999年1月至2006年1月肾内科确诊32例肾淀粉样变性患者的临床资料,应用免疫组织化学方法对进行淀粉样蛋白A重新染色分型。结果90.6%患者发病年龄大于40岁。87.5%的患者有超过2个系统受累。65.6%的患者曾经被误诊为原发性肾病综合征。免疫组织化学方法示仅1例患者AA蛋白阳性,19例行免疫荧光检查,κ轻链阳性10.5%,λ轻链阳性15.8%,两者均阳性占10.5%,两者均阴性占63.2%。结论肾淀粉样变性病常累及多系统,临床表现多样,误诊率高。中老年肾病综合征患者出现体重下降、巨舌、肝、脾肿大和血压下降是可靠的线索,应常规行血、尿免疫固定电泳检查。确诊AA型需依靠免疫组织化学AA蛋白染色。Objective To depict the systemic manifestations and the pathologic classifications in renal amyloidosis and reveal the causes of misdiagnose and increase the diagnostic accuracy. Methods We retrospectively analyze the clinical data 32 patients who were diagnosed as having renal amyloidosis ,by immunohistochemical method on renal biopsy sections for immunoglobulin light chains λ, κ and amyloid A protein. Results 90. 6% of patients were over 40 years old. 87. 5% of patients had two or more systems involvement. 65.6% of patients were misdiagnosed as having primary nephrotie syndrome. 19 patients took the immunofluorescence examination of light chains on the biopsies. κ chains were present in 10. 5% of cases,h 15.8% ,both 10. 5% ,none 63.2%. Conclusion Renal amyloidosis often involve multiple systems, with diverse clinical presentation, leading to misdiagnose. Although weigh loss, macorglossia, hepatomegaly, splenomegaly and hypotension might be useful clue, definite diagnosis usually rests on immunohistochemical staining of AA protein.

关 键 词：肾淀粉样变性 病理分型 

分 类 号：R5[医药卫生—内科学]