尽管十二指肠和直肠活检结果为阴性，但慢性腹泻仍作为心脏受累的轻链淀粉样变性的初始临床表现。
Chronic diarrhea as the initial clinical manifestation of light-chain amyloidosis with cardiac involvement despite negative duodenal and rectal biopsies.
作者信息
Pfluecke Christian, Ulbrich Stefan, Ibrahim Karim, Geiger Kathrin D, Strasser Ruth H, Wunderlich Carsten
机构信息
University of Technology Dresden, Department of Internal Medicine and Cardiology, Heart Center Dresden, University Hospital, Dresden, Germany.
出版信息
Exp Clin Cardiol. 2013 Spring;18(2):148-50.
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.
早期准确诊断并及时开始治疗对于轻链淀粉样变性的预后至关重要。本文描述了一例涉及一名62岁患者的病例，该患者出现不明原因的慢性腹泻，十二指肠和直肠活检结果为阴性。患者出现晕厥后进行了血清免疫固定电泳、游离轻链检测、心电图和超声心动图检查。这些诊断性检查结果显示出系统性淀粉样变性的特征性体征。心脏和骨髓活检确诊为系统性轻链淀粉样变性。发现慢性腹泻是由于肠神经系统的自主神经病变所致。
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