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肠道原发性局限性淀粉样变性:病理学家观点

Primary Localized Amyloidosis of the Intestine: A Pathologist Viewpoint.

作者信息

Alshehri Saeed Ali, Hussein Mahmoud Rezk Abdelwahed

机构信息

Department of Pathology, Armed Forces Hospitals, Southern Region, King Fahd Hospital, Saudi Arabia.

Department of Pathology, Faculty of Medicine, Assuit University Hospitals, Assuit, Egypt.

出版信息

Gastroenterology Res. 2020 Aug;13(4):129-137. doi: 10.14740/gr1303. Epub 2020 Aug 14.

Abstract

BACKGROUND

Localized amyloidosis of the intestine is a rare entity, which can clinically masquerade several conditions such as colitis, polyps, and malignant tumors. This study aims to evaluate the clinicopathological features of this entity.

METHODS

To evaluate the clinicopathological features of this entity, a comprehensive search of the literature (1960 to 2019) was done using the following keywords: "amyloidosis" and "small intestine" or "duodenum" or "ileum" or "jejunum" or "colon". We identified 756 studies about gastrointestinal amyloidosis. Data were examined for 27 studies about localized intestinal amyloidosis. The clinicopathological features were described.

RESULTS

The age at presentation ranged from 29 to 88 years. The male to female ratio was 3:1. The jejunum and sigmoid colon were the most commonly involved sites. Abdominal pain and intestinal obstruction (small intestine), or rectal bleeding (sigmoid region) were the most common clinical presentations. Colonoscopic findings included wall thickening, mucosal ulcerations (small intestine), and tumor-like masses (colon).

CONCLUSIONS

The clinical presentations of localized intestinal amyloidosis depend on the site of the deposition of the amyloid. In most cases, amyloid deposits consisted of light chain protein.

摘要

背景

局限性肠道淀粉样变性是一种罕见疾病,在临床上可能伪装成多种病症,如结肠炎、息肉和恶性肿瘤。本研究旨在评估该疾病的临床病理特征。

方法

为评估该疾病的临床病理特征,我们使用以下关键词对文献(1960年至2019年)进行了全面检索:“淀粉样变性”以及“小肠”或“十二指肠”或“回肠”或“空肠”或“结肠”。我们共识别出756项关于胃肠道淀粉样变性的研究。对其中27项关于局限性肠道淀粉样变性的研究数据进行了审查,并描述了其临床病理特征。

结果

发病年龄在29岁至88岁之间。男女比例为3:1。空肠和乙状结肠是最常受累的部位。腹痛和肠梗阻(小肠)或直肠出血(乙状结肠区域)是最常见的临床表现。结肠镜检查结果包括肠壁增厚、黏膜溃疡(小肠)和肿瘤样肿块(结肠)。

结论

局限性肠道淀粉样变性的临床表现取决于淀粉样蛋白的沉积部位。在大多数情况下,淀粉样沉积物由轻链蛋白组成。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0457/7433369/70f40b730628/gr-13-129-g001.jpg

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