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国际病理科学与临床杂志 2010, 30(1) 43- DOI:
10.3969/j.issn.16732588.2010. ISSN: 1673-2588 CN: 43-1458/R |
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| 论著 |
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20例肾淀粉样变的临床与病理特点分析 |
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肖红波， 刘瑞洪， 袁曙光， 凌光辉， 夏运成， 许向青， 王娟， 毕慧欣， 谌南览 |
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中南大学湘雅二医院肾病内科， 肾病研究所， 长沙 410011 |
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摘要：
目的：分析肾淀粉样变的临床与病理特点，以减少误诊和漏诊，提高临床诊治水平。方法：回顾性分析2005年1月至2009年8月在中南大学湘雅二医院肾内科经活检诊断为肾淀粉样变的20例患者的临床和病理资料。结果：20例中95% 的患者年龄在40岁以上，55％的患者有2个以上系统损害，80%表现为典型的肾病综合征。肾活检HE染色下肾小球系膜及毛细血管袢周边可见均一的淀粉样物质呈块状沉积，亦可见于间质、血管及肾小管壁。高锰酸钾氧化刚果红染色示AL型18例，AA型2例。AL型与AA型病理改变类似，但前者可见免疫球蛋白轻链κ和λ沉积。结论：肾淀粉样变在中老年蛋白尿患者中并非少见，临床表现有时缺乏特异性，肾活检光镜下的典型病理学改变结合刚果红染色可确诊。对于疑似患者应尽快行肾活检及刚果红染色，以免误诊与漏诊。 |
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关键词：
肾淀粉样变
肾病综合征
刚果红染色
病理分型
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Analysis of clinical and pathological characteristics
of 20 patients with renal amyloidosis |
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XIAO Hongbo, LIU Ruihong, YUANG Shuguang, LING Guanghui, XIA Yuncheng,
XU Xiangqing, WANG Juan, BI Huixin, CHEN Nanlan |
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Department of Nephrology，Second Xiangya Hospital， Central South University；Institute of Nephrology，Changsha 410011，China |
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Abstract:
Objective To analyze the clinical and pathological characteristics of renal amyloidosis, to decrease the chance of misdiagnosis and missed diagnosis, and to improve the diagnosis accuracy and the therapeutical effect.MethodsWe retrospectively analyzed the clinical and pathological data of 20 patients with renal amyloidosis，who were hospitalized in the Second Xiangya Hospital from Jan. 2005 to Aug. 2009 and were diagnosed as renal amyloidosis by renal biopsy. ResultsOf the 20 patients， 19（95%） were over 40 years old, 55% patients suffered more than 2 systems injuries, and 80% patients displayed the typical nephrotic syndrome. Brickred homogeneous amyloid deposition could be seen in glomerular mesangial cell and capillary membrane loop after HE staining as well as in the interstitials, the blood vessels, and the tubular wall. Eighteen patients were AL type and potassium permanganate test was positive. Another 2 were AA type and potassium permanganate test was negative. The pathological characteristics of AL type was similar to that of AA type. Immunoglobulin light chain κ and λ could be detected in AL type but not in AA type.Conclusion Renal amyloidosis is common in elderly patients. The clinical manifestations of renal amyloidosis are not always specific. It could be made the final diagnosis through examining the typical pathophysiolgical changes by renal biopsy combined with Congo red staining. Suspected patients should be performed with renal biopsy and Congo red staining as soon as possible to avoid misdiagnosis and missed diagnosis. |
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Keywords:
renal amyloidosis；nephrotic syndrome；Congo red staining；pathological type
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收稿日期 2009-12-22 修回日期 2010-01-09 网络版发布日期 |
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DOI: 10.3969/j.issn.16732588.2010. |
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基金项目:
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通讯作者: 袁曙光 |
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作者简介: |
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作者Email: shuguangy@hotmail.com |
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| 参考文献： |
| ［1］ 陈楠.肾淀粉样变性诊断治疗的临床体会［J］.肾脏病与透析肾移植杂志， 2005, 14(5):432433.
CHEN Nan. The clinical experience of diagnosis and treatment of renal amyloidosis［J］. Chinese Journal of Nephrology, Dialysis & Transplantaion, 2005, 14(5):432433.
［2］Nishi S,Alchi B,Imai N，et al. New advances in renal amyloidosis［J］. Clin Exp Nephrol，2008, 12(2):93101.(Review)
［3］Picken MM. Immunoglobulin light and heavy chain amyloidosis AL/AH: renal pathology and differential diagnosis［J］.Contrib Nephrol，2007,153:135155.(Review)
［4］尹广, 黎磊石, 刘志红. 肾淀粉样变性的临床、病理及免疫病理［J］.肾脏病与透析肾移植杂志, 1994, 3(1):2124.
YIN Guang, LI Leishi, LIU Zhihong. The clinical， pathological and immunopathological characteristic of renal amyloidosis ［J］. Chinese Journal of Nephrology, Dialysis & Transplantaion, 1994, 3(1):2124.
［5］曲贞, 刘刚. 肾淀粉样变病诊断的新认识及治疗进展［J］. 诊断学理论与实践, 2007, 6(6):502504.
QU Zhen,LIU Gang.The new insignt of diagnosis and treatment of renal amyloidosis［J］. The Theory and Practice of Diagnostics，2007, 6(6):502504.
［6］Picken MM. New insights into systemic amyloidosis: the importance of diagnosis of specific type［J］. Curr Opin Nephrol Hypertens, 2007, 16 (3):196203.
［7］Dember LM. Amyloidosisassociated kidney disease［J］. J Am Soc Nephrol, 2006, 17(12):34583471. |
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