Chronic diarrhea as the initial clinical manifestation of light-chain amyloidosis with cardiac involvement despite negative duodenal and rectal biopsies.

Experimental & Clinical Cardiology Pub Date : 2013-01-01
Christian Pfluecke, Stefan Ulbrich, Karim Ibrahim, Kathrin D Geiger, Ruth H Strasser, Carsten Wunderlich
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引用次数: 0

Abstract

Early and accurate diagnosis and a prompt initiation of treatment are critical for the prognosis of light-chain amyloidosis. The present article describes a case involving a 62-year-old patient who experienced unexplained, chronic diarrhea with negative duodenal and rectal biopsies. Serum immunofixation, a free light-chain assay, electrocardiography and echocardiography were performed after the patient developed syncope. The results of these diagnostic investigations showed characteristic signs of systemic amyloidosis. Cardiac and bone marrow biopsies confirmed the diagnosis of systemic light-chain amyloidosis. The chronic diarrhea was found to be due to an autonomic neuropathy of the enteric nervous system.

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慢性腹泻作为轻链淀粉样变的最初临床表现,并累及心脏,尽管十二指肠和直肠活检呈阴性。
早期准确诊断和及时开始治疗对轻链淀粉样变的预后至关重要。这篇文章描述了一个涉及62岁患者的病例,他经历了不明原因的慢性腹泻,十二指肠和直肠活检阴性。患者发生晕厥后进行血清免疫固定、游离轻链试验、心电图和超声心动图检查。这些诊断调查的结果显示系统性淀粉样变性的特征性征象。心脏和骨髓活检证实系统性轻链淀粉样变的诊断。慢性腹泻被发现是由于肠道神经系统的自主神经病变。
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来源期刊
Experimental & Clinical Cardiology
Experimental & Clinical Cardiology CARDIAC & CARDIOVASCULAR SYSTEMS-
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6-12 weeks
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