Primary intestinal lymphangiectasia

Abstract

Primary intestinal lymphangiectasia is a rare disease, which is characterized by the obstruction of lymphatic return, dilated lymphatics and distorted structure of villi. Lymph fluid leaks into the small bowel lumen as a result of the obstruction of lymphatics and elevated pressure of lymphatics, and leads to malabsorption and loss of proteins. The causes and pathogenesis of PIL are not clear, hut the abnormal immunologic mechanism has got people's attention. Recently, the endoscopic technique has been improved, and the diagnosis rate of PIL has increased obviously. Antisecosis is the basic and effective treatment, especially for children. Key words: Primary intestinal lymphangiectasia; Child; Diarrhea; Edema