title: Primary intestinal lymphangiectasia: is it always bad? Two cases with different outcome.
abstract: Primary intestinal lymphangiectasia (PIL) or Waldmann's disease is a rare protein-losing gastroenteropathy of unknown etiology. Less than 200 cases have been reported globally. Patients may be asymptomatic or present edema, lymphedema, diarrhea, ascites and other manifestations. We report two pediatric cases with PIL with extremely different outcome in a 3-year follow-up period. The first patient presented with persistent diarrhea, hypoalbuminemia and failure to thrive, while the second patient presented with an abrupt eyelid edema. Hypoproteinemia was the common laboratory finding for the two patients and upper gastrointestinal endoscopy established the diagnosis. The first patient relapsed five times during the follow-up period after the diagnosis had been made and required intravenous albumin administration and micronutrient supplementation. The second patient revealed normal gastrointestinal endoscopy 4 months after the diagnosis had been established; he followed an unrestricted diet and remained asymptomatic throughout the follow-up period. PIL can be either severe, affecting the entire small bowel, leading to lifetime disease, or sometimes affects part of the small bowel, leading to transient disorder.
authors.affiliation: Third Pediatric Department, Papageorgiou Hospital, Thessaloniki, Greece.
authors.forename: Ioannis
authors.initials: I
authors.lastname: Xinias
authors.forename: Antigoni
authors.initials: A
authors.lastname: Mavroudi
authors.forename: Evi
authors.initials: E
authors.lastname: Sapountzi
authors.forename: Agathi
authors.initials: A
authors.lastname: Thomaidou
authors.forename: Maria
authors.initials: M
authors.lastname: Fotoulaki
authors.forename: Athanasios
authors.initials: A
authors.lastname: Kalambakas
authors.forename: Elina
authors.initials: E
authors.lastname: Karypidou
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Kollios
authors.forename: Grigorios
authors.initials: G
authors.lastname: Pardalos
authors.forename: George
authors.initials: G
authors.lastname: Imvrios
keywords: Children; Outcome; Primary intestinal lymphangiectasia; Protein-losing enteropathy; Wireless capsule endoscopy