全切片扫描在肾组织轻链型淀粉样物质沉积患者研究中的应用

发布时间：2018-02-26 10:41

  本文关键词： 轻链型淀粉样变性 全切片扫描分析 临床特点 预后　出处：《肾脏病与透析肾移植杂志》2017年02期 　论文类型：期刊论文

【摘要】：目的:探讨轻链型淀粉样变性(AL型)淀粉样物质在肾组织沉积部位及程度与临床表现及预后的关系。方法:回顾分析经肾活检确诊的AL型淀粉样变性患者的临床病理资料,利用全切片扫描技术计算淀粉样物质占组织的百分比,分析其与临床及预后的关系。结果:AL型淀粉样变性患者259例,男性147例,中位年龄57岁,肾脏均受累,肠道受累71.06%,心脏受累43.22%。中位病程7月,蛋白尿4.80±3.15g/24h,19.31%的患者肾功能不全。中位随访时间22.3月,患者平均生存时间24.24月,1、2、3和5年人生存率分别为81.8%、64.1%、52.4%和29.6%;肾脏生存率分别为90.8%、82.3%、76.8%和47.8%。淀粉样物质累及肾小球(99.61%),肾血管(92.66%)和肾间质(50.19%)。淀粉样物质占肾小球、肾血管、肾间质及整个肾组织的百分比分别为11.81%±11.38%、14.14%±14.05%、3.34%±5.36%和4.25%±5.77%,且与多项临床指标具有相关性。淀粉样物质占肾小球百分比、年龄、尿N-乙酰-β-D-氨基葡萄糖苷酶(NAG)及尿视黄醇结合蛋白(RBP)为患者预后的独立危险因素。淀粉样物质占肾血管、肾间质及整个肾组织百分比,血清肌酐,尿RBP为肾脏预后的独立危险因素。结论:全切片扫描定量分析是观察淀粉样物质在肾脏不同部位沉积及其程度的有效方法,并证实蛋白尿、血清肌酐等与淀粉样物质肾脏不同部位沉积有一定相关性。肾脏不同部位淀粉样物质沉积与患者预后和肾脏预后相关。
[Abstract]:Objective: to investigate the relationship between the location and degree of amyloid deposition in renal tissue and the clinical manifestation and prognosis. Methods: the clinicopathological data of patients with AL type amyloidosis diagnosed by renal biopsy were retrospectively analyzed. The percentage of amyloid in tissue was calculated by whole section scanning technique, and the relationship between amyloid content and clinical and prognosis was analyzed. Results 259 cases of amyloidosis of type A were male 147 cases, median age 57 years old, all of them were involved in kidney. The median course of disease on July was 4.80 卤3.15g / 24 h proteinuria (4.80 卤3.15g / 24 h, 19.31%), and the median follow-up time was 22.3 months. The average survival time of the patients was 24.24 months and 52.4% and 29.664%, respectively, while the renal survival rates were 90.8% and 82.3% and 47.8%, respectively. The amyloid involvement of the glomeruli was 99.61and 92.666.66) and the interrenal interstitial mass was 50.190.The amyloid substances occupied the glomeruli, the renal blood vessels, the renal vessels, and the renal vessels, and the renal vessels, the renal vessels, the renal vessels, the renal vessels, the renal vessels, the renal vessels, the renal vessels, the renal vessels, the renal vessels, the renal vessels, and the amyloid substances. The percentage of renal interstitial tissue and the whole renal tissue were 11.81% 卤11.38% 卤14.14% 卤14.05% 卤3.34% 卤5.36% and 4.25% 卤5.77%, respectively. Urinary N-acetyl- 尾 -Daminoglycosidase (NAG) and urinary retinol binding protein (RBP) were independent prognostic factors. The percentage of amyloid in renal vessels, renal mesenchyme and the whole renal tissue, serum creatinine, serum creatinine, serum creatinine, serum creatinine, serum creatinine, and serum creatinine were determined. Conclusion: full slice scanning quantitative analysis is an effective method to observe the deposition and degree of amyloid in different parts of kidney, and to confirm proteinuria. Serum creatinine was correlated with the deposition of amyloid in different parts of the kidney, which was related to the prognosis of the patients and the prognosis of the kidney.
【作者单位】： 南京大学医学院附属金陵医院(南京军区南京总医院)肾脏科国家肾脏疾病临床医学研究中心全军肾脏病研究所;无锡市人民医院;
【基金】：国家重点研发计划课题(2016YFC0904103,2016YFC0901202) 国家科技支撑计划课题(2015BAI12B02,2015BAI12B05)
【分类号】：R597.2
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