原发性小肠淋巴管扩张症(InL)是一种少见的蛋白丢失性肠病,它以小肠淋巴引流受阻、乳糜管扩张以及绒毛结构扭曲为特征,淋巴管的阻塞以及小肠淋巴压力的升高导致淋巴液漏出至小肠管腔,最终导致吸收不良和蛋白的丢失。该病自1961年Waldmann等[1]首次报道,我国由蒋木平[2]1990年首 InL is a rare form of protein-losing enteropathy that is characterized by obstruction of small bowel lymphatic drainage, dilatation of the chylothorax and distortion of the villus structure, obstruction of the lymphatic vessels, and increased lymphatic pressure in the small intestine As a result, lymph leaks into the lumen of the small intestine, eventually leading to malabsorption and protein loss. The disease was first reported by Waldmann et al [1] in 1961, and was first reported by Jiang Muping [2] in 1990