小肠淋巴管扩张症的研究进展

小肠淋巴管扩张症(IL)是一种罕见的蛋白丢失性肠病,小肠淋巴管和(或)乳糜管扩张、破裂,淋巴液漏出,特征性表现为低蛋白血症、低γ球蛋白血症、低脂血症、水肿/淋巴水肿、浆膜腔积液和淋巴细胞绝对计数减少。病因不明。内镜发现小肠淋巴管扩张并见相应组织学改变可确诊。淋巴管造影现已不作为诊断IL的常规方法。99Tcm标记的人血清白蛋白核素显像和粪便α1抗胰蛋白酶测定可证实肠道丢失蛋白。低脂、富含中链三酰甘油饮食是目前最主要、基础的治疗方法。

Research Advances in Intestinal Lymphangiectasia

Intestinal lymphangiectasia(IL)is a rare disorder characterized by dilated intestinal vessles and/or lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to hypoalbuminemia,hypogammaglobulinemia,hypolipidemia,edma/lymphedema,pleural effusions and lymphopenia.Etiology remains unknown.Diagnosis is confirmed by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of intestinal biopsy specimens.Lymphoscintigraphy is not a routine and useful methodology for IL diagnosis.Protein-losing enteropathy is confirmed by 99Technetium-labeled human serum albumin(99Tcm-HSA)scintigraphy and the elevated 24-h stool α1-antitrypsin clearance.A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of IL medical management.