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伴有神经病变的轻链型心脏淀粉样变性:一例报告

Light-chain cardiac amyloidosis with neuropathy: a case report.

作者信息

Xu Zhan-Wen, Li Ya-Qin, Liu Li-xia, Zhou Bing-Juan

机构信息

Department of Cardiology, Affiliated Hospital of Hebei University, Baoding, People's Republic of China.

Department of Ultrasound, Affiliated Hospital of Hebei University, Baoding, People's Republic of China.

出版信息

Clin Interv Aging. 2015 Jul 31;10:1219-22. doi: 10.2147/CIA.S87540. eCollection 2015.

Abstract

Light-chain amyloidosis is a relatively rare multisystem disorder. The disease often is normally difficult to diagnose due to its broad range of characters without specific symptoms. A 62-year-old male patient presented with heart failure after experiencing a long period of unexplained and untreated gastrointestinal symptoms. Clinical examination and laboratory findings indicated a systemic process with cardiac involvement. Echocardiography revealed concentric left ventricular hypertrophy with enhanced echogenicity and preserved ejection fraction. Rectum biopsy confirmed amyloid deposition. The side effect of delayed diagnosis on prognosis and the appropriate diagnostic strategy has been discussed.

摘要

轻链型淀粉样变性是一种相对罕见的多系统疾病。由于其具有广泛的特征且无特异性症状,该疾病通常难以诊断。一名62岁男性患者在经历了长时间无法解释且未得到治疗的胃肠道症状后出现心力衰竭。临床检查和实验室检查结果表明存在累及心脏的全身性病变。超声心动图显示左心室向心性肥厚,回声增强,射血分数正常。直肠活检证实有淀粉样蛋白沉积。文中讨论了延迟诊断对预后的影响以及合适的诊断策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/74a5/4527338/2c75f304afc9/cia-10-1219Fig1.jpg

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