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.