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肾淀粉样变性的临床病理特征:一项对47例患者的单中心研究

Clinicopathological features of renal amyloidosis: a single-center study on 47 cases.

作者信息

Luo Chang-Qing, Zhang Yu-An, Li Zhen-Qiong, Wang Yu-Mei

机构信息

Department of Nephrology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China.

Department of Nephrology, The Third People's Hospital of Yunnan Province, Kunming, 650011, China.

出版信息

J Huazhong Univ Sci Technolog Med Sci. 2015 Feb;35(1):48-53. doi: 10.1007/s11596-015-1387-4. Epub 2015 Feb 12.

Abstract

The correlations between the clinicopathological features and the long-term outcomes of renal amyloidosis (RA) were analyzed with a view to develop strategies for improving diagnosis and prognosis of RA. We retrospectively reviewed the clinicopathological characteristics of 47 patients diagnosed with RA between 2004 and 2014 at the Wuhan Union Hospital. The data on the renal histology, clinical manifestations, and prognosis of RA patients were retrieved from the hospital records and characteristic patterns were identified. The histological changes in the kidneys were correlated with the clinical manifestations of RA. Additionally, most RA patients in this study had decreased serum levels of κ light chain and increased urine levels of κ and λ light chains as well as presence of M-protein in the urine and serum. Patients with early RA showed no specific pathognomonic symptoms. Bleeding associated with diagnostic renal biopsy was rare. We recommend that the routine work-up of patients aged over 40 years and presenting with non-diabetic nephropathy includes the non-invasive tests for the measurement of serum and urine levels of κ and λ light chains as well as protein electrophoresis tests for the presence of urinary and serum M-protein. Additionally, such patients should undergo renal biopsy screening with Cong-red staining to ensure early diagnosis of RA and improve their survival, since the risk of hemorrhage related to renal biopsy screening is low at early stages of RA.

摘要

分析肾淀粉样变性(RA)的临床病理特征与长期预后之间的相关性,以期制定改善RA诊断和预后的策略。我们回顾性分析了2004年至2014年期间在武汉协和医院确诊为RA的47例患者的临床病理特征。从医院记录中检索RA患者的肾脏组织学、临床表现和预后数据,并确定特征模式。肾脏的组织学变化与RA的临床表现相关。此外,本研究中的大多数RA患者血清κ轻链水平降低,尿κ和λ轻链水平升高,且尿和血清中存在M蛋白。早期RA患者无特异性诊断症状。诊断性肾活检相关出血罕见。我们建议,对于40岁以上且患有非糖尿病性肾病的患者,常规检查应包括测量血清和尿κ和λ轻链水平的非侵入性检测,以及检测尿和血清中M蛋白的蛋白电泳检测。此外,此类患者应接受刚果红染色的肾活检筛查,以确保RA早期诊断并提高其生存率,因为在RA早期,与肾活检筛查相关的出血风险较低。

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