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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。
Objective: To analyze the clinical and pathological characteristics of renal amyloidosis to reduce the misdiagnosis and missed diagnosis, and to improve the clinical diagnosis and treatment. Methods: The clinical and pathological data of 20 patients with renal amyloidosis diagnosed by biopsy in Department of Nephrology, Second Xiangya Hospital of Central South University from January 2005 to August 2009 were retrospectively analyzed. RESULTS: Ninety-five percent of 20 patients were over 40 years old, 55% had more than two systemic lesions, and 80% showed typical nephrotic syndrome. Renal biopsy under the glomerular mesangium and capillaries 袢 around the homozygous visible amyloid massive deposition, also found in the stroma, blood vessels and renal tubular wall. Potassium permanganate oxidized Congo red staining showed AL type in 18 cases, AA type in 2 cases. AL and AA pathological changes similar, but the former visible immunoglobulin light chain κ and λ deposition. Conclusion: Renal amyloidosis is not uncommon in patients with albuminuria in middle-aged and elderly patients. The clinical manifestations are sometimes not specific. The typical histopathological changes under renal biopsy can be diagnosed by Congo red staining. For suspected patients as soon as possible renal biopsy and Congo red staining, so as to avoid misdiagnosis and missed diagnosis.