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目的:分析我国汉族人群系统性轻链淀粉样变性(AL型淀粉样变性)的临床特征,比较不同部位活检对确诊的意义。方法:回顾性分析2009年1月至2014年10月在南京军区南京总医院肾脏科住院确诊的186例AL型淀粉样变性患者临床资料和病理诊断,以肾活检为诊断的金标准,比较皮肤脂肪活检和直肠黏膜活检对诊断AL型淀粉样变性的敏感性。结果:186例患者的平均年龄为(54.8±9.1)岁,男女比例为2.44∶1,143例(76.9%)的患者血清中检测出单克隆免疫球蛋白轻链。皮肤脂肪活检的诊断敏感性为92.5%,95%CI为88%~97%,直肠黏膜活检的诊断敏感性为94.9%,95%CI为92%~98%,结合皮肤脂肪和直肠黏膜两种活检方式,诊断阳性率达100%。结论:活检仍然是确诊AL型淀粉样变性的金标准,皮肤脂肪和直肠黏膜活检操作简单、花费低、并发症少且敏感性高,两种方法结合能够提高诊断的阳性率,对于难以进行肾活检的患者,皮肤脂肪和直肠黏膜是合适的活检取材部位。
Objective: To analyze the clinical features of systemic light chain amyloidosis (ALA) in Han Chinese and to compare the significance of biopsy in different sites. Methods: A retrospective analysis of clinical data and pathological diagnosis of 186 patients with AL-amyloidosis admitted to Department of Nephrology, Nanjing General Hospital of Nanjing Military Command from January 2009 to October 2014 was performed. The renal biopsy was used as the gold standard for diagnosis. Sensitivity of Fat Biopsy and Rectal Mucosal Biopsy to Diagnosing AL Amyloidosis. Results: The average age of 186 patients was (54.8 ± 9.1) years and the ratio of male to female was 2.44:1. The monoclonal immunoglobulin light chain was detected in sera of 143 patients (76.9%). The diagnostic sensitivity of the skin fat biopsy was 92.5% with a 95% CI of 88% -97%. The diagnostic sensitivity of the rectal mucosal biopsy was 94.9% and the 95% CI was 92% -98%. The combined skin fat and rectal mucosa Biopsy, the diagnosis of positive rate of 100%. Conclusions: Biopsy is still the gold standard for the diagnosis of AL amyloidosis. The biopsy of skin fat and rectal mucosa is simple, low cost, less complications and high sensitivity. Combination of the two methods can improve the positive rate of diagnosis, Biopsy patients, skin fat and rectal mucosa are the appropriate biopsy sites.