肠淋巴管扩张症(IL)的特征是肠淋巴管扩张,这可能会破裂并导致淋巴流失到肠道中。由于蛋白质含量高,脂蛋白,和肠淋巴中的淋巴细胞,淋巴丢失可能导致低蛋白血症,低蛋白血症,低球蛋白血症,和淋巴细胞减少症.此外,可能会耗尽矿物质,脂质,和脂溶性维生素。IL可以是原发性的,由于淋巴系统固有的功能障碍,或者其次,可能直接或间接阻碍淋巴引流的各种因素的结果。这种情况已成为具有重要临床兴趣的主题。鉴于肠道淋巴系统在人体体液稳态中起着重要作用,适应性免疫,营养和药物吸收,肠道运输,和全身新陈代谢,它的功能障碍可能有更广泛的影响。虽然原发性IL是罕见的,具有不同的临床特征,并发症,治疗反应,和结果,继发性IL比以前认为的更常见。IL的明确诊断需要内窥镜检查发白的绒毛(通常类似于雪花)和小肠粘膜中扩张的乳的组织学确认。IL的治疗具有挑战性,涉及饮食调整,管理潜在的医疗条件,使用西罗莫司和奥曲肽等药物。认识到其患病率和不同的病因对于有针对性地管理这种具有挑战性的医疗状况至关重要。本文提供了与IL相关的临床意义的全面探索。此外,它为现有诊断和管理环境中的关键知识差距提供了宝贵的见解。
Intestinal lymphangiectasia (IL) is characterized by the dilation of intestinal lymphatic vessels, which can rupture and cause loss of lymph into the intestine. Due to the high content of proteins, lipoproteins, and lymphocytes in the intestinal lymph, loss of lymph might result in hypoproteinemia, hypoalbuminemia, hypogammaglobulinemia, and lymphocytopenia. In addition, there may be a depletion of minerals, lipids, and fat-soluble vitamins. IL can be primary due to inherent malfunctioning of the lymphatic system, or secondly, a result of various factors that may hinder lymphatic drainage either directly or indirectly. This condition has emerged as a subject of significant clinical interest. Given that the intestinal lymphatic system plays an important role in the body\'s fluid homeostasis, adaptive immunity, nutrient and drug absorption, intestinal transport, and systemic metabolism, its dysfunction may have wider implications. Although primary IL is rare, with varied clinical features, complications, treatment response, and outcomes, secondary IL is more common than previously believed. The definitive diagnosis of IL requires endoscopic demonstration of whitish villi (which frequently resemble snowflakes) and histological confirmation of dilated lacteals in the small intestinal mucosa. Treatment of IL is challenging and involves dietary modifications, managing underlying medical conditions, and using medications such as sirolimus and octreotide. Recognizing its prevalence and diverse etiology is crucial for targeted management of this challenging medical condition. This article provides a comprehensive exploration of the clinical implications associated with IL. In addition, it offers valuable insights into critical knowledge gaps in the existing diagnostic and management landscape.