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一例罕见的胃肠道轻链淀粉样变性病例。

A Rare Case of Light Chain Amyloidosis of the Gastrointestinal Tract.

作者信息

Dvir Kathrin, Galarza-Fortuna Gliceida M, Willet Anna, Febres-Aldana Christopher, Cortez Nathaly, Rapaka Samuel, Coombs Andre, Goldberg Robert, Schwartz Michael, Ben-David Kfir

机构信息

Department of Internal Medicine, Mount Sinai Medical Center, USA.

The Arkadi M. Rywlin M.D. Department of Pathology, Mount Sinai Medical Center, USA.

出版信息

Case Rep Surg. 2020 Jun 30;2020:1921805. doi: 10.1155/2020/1921805. eCollection 2020.

Abstract

A 65-year-old Hispanic female presented with a one-year history of anorexia, nausea, early satiety, epigastric discomfort, and a 20 kg weight loss. Computed tomography (CT) demonstrated heterogeneous liver parenchyma. Upper endoscopy revealed large, fungating, infiltrative mass at the lesser gastric curvature incisura, highly suspicious of gastric tumor; however, initial biopsy of the gastric mass was equivocal and an exploratory laparoscopy was performed. Repeated intraoperative biopsies of the gastric mass and of liver parenchyma demonstrated diffuse hyalinized stroma consistent with amyloid deposition, and a bone marrow biopsy confirmed the diagnosis of primary light chain (AL) amyloidosis.

摘要

一名65岁的西班牙裔女性,有一年的厌食、恶心、早饱、上腹部不适病史,体重减轻了20公斤。计算机断层扫描(CT)显示肝脏实质不均匀。上消化道内镜检查发现胃小弯切迹处有巨大、蕈状、浸润性肿块,高度怀疑为胃肿瘤;然而,胃肿块的初次活检结果不明确,遂进行了 exploratory laparoscopy(此处原文可能有误,推测为“ exploratory laparotomy”,即剖腹探查术)。术中对胃肿块和肝脏实质进行多次活检,结果显示弥漫性透明化间质,符合淀粉样蛋白沉积,骨髓活检确诊为原发性轻链(AL)淀粉样变性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c4c9/7345595/67ec38753e49/CRIS2020-1921805.001.jpg

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