胃肠道淀粉样变的临床和内镜表现:一个病例系列。

Clujul medical (1957) Pub Date : 2018-10-01 Epub Date: 2018-10-30 DOI:10.15386/cjmed-951
Andree Hermes Koop, Omar Y Mousa, Ming-Hsi Wang
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引用次数: 11

摘要

胃肠(GI)淀粉样变是罕见的,有不同的临床和内镜表现。在这个病例系列中,我们报告了3例原发性系统性淀粉样蛋白轻链(AL)淀粉样变性伴胃肠道病变并伴有消化道出血的患者。我们也提供了一个简短的文献回顾,包括临床表现,内窥镜的发现,病理,和处理的胃肠道淀粉样变。胃肠道淀粉样变的内镜表现各不相同,包括易碎的粘膜糜烂、溃疡、粘膜下血肿或粘膜增厚伴息肉样突起。内窥镜检查结果可能与淀粉样原纤维的病理沉积有关。胃肠道淀粉样变的治疗通常侧重于基础疾病的管理和支持性护理。胃肠病学家应该熟悉内窥镜检查结果,因为它们可能是疾病的第一个提示,并允许明确的诊断。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Clinical and endoscopic manifestations of gastrointestinal amyloidosis: a case series.

Gastrointestinal (GI) amyloidosis is rare and has varying clinical and endoscopic presentations. In this case series, we present three patients with primary systemic amyloid-light chain (AL) amyloidosis with GI involvement and complications of GI bleeding. We also provide a brief review of the literature, including clinical presentation, endoscopic findings, pathology, and management of GI amyloidosis. The endoscopic findings of GI amyloidosis can vary, including friable mucosa with erosions, ulcers, and submucosal hematomas or mucosal thickening with polypoid protrusions. The endoscopic findings may correlate with the pathologic deposition of amyloid fibrils. Treatment of GI amyloidosis is generally focused on management of the underlying condition and supportive care. Gastroenterologists should be familiar with the endoscopic findings as they may be the first suggestion of disease and allow for definitive diagnosis.

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