Catalog

Primary intestinal lymphangiectasia

No MMS code

Link to WHO Definition

Primary intestinal lymphangiectasia – http://id.who.int/icd/entity/52162548

Primary intestinal lymphangiectasia (PIL) is a rare disorder characterised by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. The main manifestation is moderate-to-severe bilateral lower limb oedema. Oedema can progress to anasarca and includes pleural effusion, pericarditis or chylous ascites.