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一名成年患者出现新发蛋白丢失性肠病,诊断为隐匿性水肿性原发性肠淋巴管扩张症。

Elusive Edematous Primary Intestinal Lymphangiectasia in an Adult Patient Presenting With New Onset of Protein-Losing Enteropathy.

作者信息

Noble Oscar, Minjares Ramon O, Yorke Rebecca, Barroso Alberto

机构信息

Lynda K. and David M. Underwood Center for Digestive Disorders, Houston Methodist Hospital, Houston, TX.

Houston Methodist Research Institute and Weil Cornell College of Medicine, Houston, TX.

出版信息

ACG Case Rep J. 2025 Jul 7;12(7):e01764. doi: 10.14309/crj.0000000000001764. eCollection 2025 Jul.

Abstract

Primary intestinal lymphangiectasia is a rare disorder characterized by the dilation of lymphatic vessels in the small intestine, resulting in the loss of proteins, particularly albumin, into the gastrointestinal tract which leads to a protein-losing enteropathy. We report the case of a 66-year-old African American man who presented with insidious abdominal pain, chronic diarrhea, hypoalbuminemia, leukopenia, and anemia. An elusive diagnosis of edematous subtype primary intestinal lymphangiectasia with protein-losing enteropathy was confirmed through a multimodal diagnostic approach, including video capsule endoscopy and small bowel endoscopy with biopsies. Treatment was focused on optimizing the patient's nutritional status.

摘要

原发性肠淋巴管扩张症是一种罕见的疾病,其特征是小肠淋巴管扩张,导致蛋白质,尤其是白蛋白,流失到胃肠道,从而引发蛋白丢失性肠病。我们报告了一例66岁的非裔美国男性患者,他表现为隐匿性腹痛、慢性腹泻、低白蛋白血症、白细胞减少和贫血。通过多模式诊断方法,包括视频胶囊内镜检查和小肠内镜活检,确诊为水肿型原发性肠淋巴管扩张症伴蛋白丢失性肠病。治疗重点是优化患者的营养状况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3da9/12233920/f045f9f53128/ac9-12-e01764-g001.jpg

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