Abstract

FIGUEIREDO, Margarida et al. Primary Intestinal Lymphangiectasia - A Cause of Exsudative Enteropathy. J Port Gastrenterol. [online]. 2010, vol.17, n.1, pp.28-31. ISSN 0872-8178.

Primary Intestinal Lymphangiectasia (PIL) is a rare form of lymphatic disorder, of unknown aetiology. It is characterized by dilated lymphatic vessels of the bowel leading to lymph leakage and exsudative enteropathy. Presentation is variable and symptoms are related to low serum protein, lymphocytes, immunoglobulin and fat malabsorption. The authors report a case of PIL diagnosed in a 3 years old boy. The diagnosis was based on clinical, endoscopic and histological findings. Despite the large involvement of the intestinal mucosa (small bowel X-ray), the child had good clinical response to high protein and decreased long-chain fat diet. The boy is now 16 years old and has a life similar to the other healthy adolescents despite maintaining oedema of the face and limbs.

Keywords : oedema; Primary Intestinal Lymphangiectasia; protein-losing enteropathy.

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