Elderly Onset Primary Intestinal Lymphangiectasia—A Rare Case

IF 1.7 Q3 GASTROENTEROLOGY & HEPATOLOGY
JGH Open Pub Date : 2025-01-22 DOI:10.1002/jgh3.70102
Li-Han Goh, Madhavan Manoharan, Khean-Lee Goh
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引用次数: 0

Abstract

Primary intestinal lymphangiectasia (PIL) is a rare protein-losing gastroenteropathy characterized by diffuse or localized ectasia of the enteric lymphatics, which can be accompanied by lymphatic abnormalities in other parts of the body. This condition results in hypoalbuminemia, hypogammaglobulinemia, and lymphopenia due to the abnormal leakage of lymphatic fluid into the gastrointestinal tract. As there are no specific serological or radiological tests available, the gold standard for diagnosing intestinal lymphangiectasia is endoscopic examination with histopathological examination of intestinal biopsy specimens. We present a rare case of primary intestinal lymphangiectasia in a 62-year-old Chinese woman who presented with a six-month history of lethargy, persistent diarrhea, and progressive weight loss. Gastroscopy and colonoscopy were performed, and biopsies revealed dilated intestinal lymphatics with broadened villi in the small intestine. Secondary causes of intestinal lymphangiectasia were ruled out, confirming the diagnosis of PIL. The patient was treated with a high-protein, low-fat diet supplemented with medium-chain triglycerides, resulting in significant clinical improvement.

Abstract Image

老年发病原发性肠淋巴管扩张1例。
原发性肠淋巴管扩张症(PIL)是一种罕见的蛋白质丢失性肠胃病,其特征是肠淋巴管的弥漫性或局限性扩张,可伴有身体其他部位的淋巴管异常。这种情况导致低白蛋白血症、低丙种球蛋白血症和淋巴细胞减少,这是由于淋巴液异常渗漏到胃肠道所致。由于没有特定的血清学或放射学检查,诊断肠淋巴管扩张的金标准是内镜检查和肠活检标本的组织病理学检查。我们报告一例罕见的原发性肠淋巴管扩张病例,患者为62岁的中国女性,表现为六个月的嗜睡、持续性腹泻和进行性体重减轻。进行胃镜和结肠镜检查,活检显示小肠淋巴扩张,绒毛变宽。排除肠淋巴管扩张的继发原因,确认PIL的诊断。患者采用高蛋白、低脂饮食,并辅以中链甘油三酯治疗,临床明显改善。
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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来源期刊
JGH Open
JGH Open GASTROENTEROLOGY & HEPATOLOGY-
CiteScore
3.40
自引率
0.00%
发文量
143
审稿时长
7 weeks
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