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学科主题: 临床医学
题名:
A clinicopathological analysis in a large cohort of Chinese patients with renal amyloid light-chain amyloidosis
作者: Yao, Ying; Wang, Su-Xia1; Zhang, You-Kang; Qu, Zhen; Liu, Gang; Zou, Wan-Zhong
关键词: amyloidosis ; immunoglobulin light chains ; proteinuria ; renal biopsy ; renal insufficiency
刊名: NEPHROLOGY DIALYSIS TRANSPLANTATION
发表日期: 2013-03-01
DOI: 10.1093/ndt/gfs501
卷: 28, 期:3, 页:689-697
收录类别: SCI
文章类型: Article
WOS标题词: Science & Technology
类目[WOS]: Transplantation ; Urology & Nephrology
研究领域[WOS]: Transplantation ; Urology & Nephrology
关键词[WOS]: SYSTEMIC AMYLOIDOSIS ; CLINICAL-FEATURES ; KIDNEY ; AL ; INVOLVEMENT ; PREVALENCE ; DEPOSITION ; DIAGNOSIS ; BIOPSIES
英文摘要:

The aim of the study is to investigate the association between clinical and pathological features in a large cohort of Chinese patients with renal immunoglobulin light-chain amyloidosis (AL).

A series of 186 patients with renal AL amyloidosis diagnosed between 1990 and 2011 were retrospectively reviewed. The extent of amyloid deposition in glomeruli, blood vessels and tubulointerstitium were evaluated semiquantitatively. The renal amyloid load was defined by the sum of glomerular, vascular and interstitial deposits. The associations between the clinical manifestations and pathological features were analyzed.

The extent of glomerular amyloid deposition was positively correlated with the level of proteinuria. Patients with codeposition of amyloid and immune complexes (ICs) in glomeruli had higher levels of proteinuria than those without ICs. Advanced glomerular amyloid deposition was an independent pathological factor associated with renal insufficiency at diagnosis. The degree of vascular amyloid (VA) deposition was positively correlated with cardiac involvement and hepatic involvement. Patients with AL- showed a higher prevalence of hepatic involvement and more severe VA deposition than patients with AL-. High renal amyloid load independently predicted the increased risk for overall death after adjusting for recognized confounders.

The degree and localization of amyloid deposits in the kidney of AL patients were associated with the degree of proteinuria and renal insufficiency, as well as extrarenal organs involvement. There were some differences between AL- and AL - in clinical and pathological characteristics. The renal amyloid load was an independent predictor for overall mortality.

语种: 英语
WOS记录号: WOS:000316120000026
Citation statistics:
内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/55272
Appears in Collections:北京大学第一临床医学院_肾脏内科_期刊论文

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作者单位: 1.Peking Univ, Inst Nephrol, Beijing 100871, Peoples R China
2.Minist Hlth China, Key Lab Renal Dis, Beijing, Peoples R China
3.Peking Univ, Hosp 1, Dept Med, Div Renal, Beijing 100871, Peoples R China

Recommended Citation:
Yao, Ying,Wang, Su-Xia,Zhang, You-Kang,et al. A clinicopathological analysis in a large cohort of Chinese patients with renal amyloid light-chain amyloidosis[J]. NEPHROLOGY DIALYSIS TRANSPLANTATION,2013,28(3):689-697.
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