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表现为孤立性十二指肠肿瘤的Al型全身性淀粉样变性。

Al-type generalized amyloidosis showing a solitary duodenal tumor.

作者信息

Yousuf M, Akamatsu T, Matsuzawa K, Katsuyama T, Sugiyama A, Ikeda S, Kiyosawa K, Furuta S

机构信息

2nd Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan.

出版信息

Hepatogastroenterology. 1992 Jun;39(3):267-9.

PMID:1505902
Abstract

An amyloid tumor of the duodenum is a rare occurrence. A patient who presented with epigastric discomfort, in whom a barium meal study revealed a tumor of the duodenum is described. Upper gastrointestinal endoscopy showed a submucosal location of the tumor, and examination of a biopsy revealed amyloid deposition of the AL type. Diffuse tumor involvement of adjacent tissues of the duodenum and also deposits of amyloid fibrils in the liver and skin with no evidence of a chronic pre- existing disease in the patient led to the diagnosis of primary systemic amyloidosis. The distinguishing feature of this case was the formation of a solitary and relatively large tumor in the duodenum mimicking a submucosal tumor, which is in contrast to most reported cases of multiple and smaller amyloid tumors in other parts of the gastrointestinal tract.

摘要

十二指肠淀粉样瘤较为罕见。本文描述了一名出现上腹部不适的患者,其钡餐检查显示十二指肠有肿瘤。上消化道内镜检查显示肿瘤位于黏膜下层,活检检查发现为AL型淀粉样沉积。十二指肠相邻组织出现弥漫性肿瘤累及,肝脏和皮肤也有淀粉样纤维沉积,且患者无慢性基础疾病史,据此诊断为原发性系统性淀粉样变性。该病例的独特之处在于十二指肠形成了一个孤立且相对较大的肿瘤,类似黏膜下肿瘤,这与大多数报道的胃肠道其他部位多发且较小的淀粉样瘤病例不同。

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