Primary intestinal lymphangiectasia in a 23-month- old girl

Primary intestinal lymphangiectasia (PIL) is a rare protein-losing gastroenteropathy which is defined as dilation of existing mucosal, submucosal, or subserosal lymphatics within the gastrointestinal tract. That causes loss of lymph fluid into the gastrointestinal tract, leading to the development o...

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Bibliographic Details
Published inOxford Medical Case Reports Vol. 2019; no. 7; p. omz065
Main Authors Mohammad, Lava, Omran, Razan, Ibrahim, Alexandr, Hourieh, Mohammad Adib, Ibrahim, Ali
Format Journal Article
LanguageEnglish
Published England Oxford University Press 01.07.2019
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Case Report
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Summary:Primary intestinal lymphangiectasia (PIL) is a rare protein-losing gastroenteropathy which is defined as dilation of existing mucosal, submucosal, or subserosal lymphatics within the gastrointestinal tract. That causes loss of lymph fluid into the gastrointestinal tract, leading to the development of hypoproteinemia, edema, lymphocytopenia, hypogammaglobinemia, and immunologic anomalies. It is usually diagnosed in patients younger than 3 years old and is rarely first diagnosed in adulthood. Here we have a case report in a 23-month- old female presented with the complaint of peripheral edema and diarrhea. The diagnosis of PIL was made through upper gastrointestinal endoscopy and pathology histologic analysis. Patient placed on oral supplements of medium-chain triglycerides, a high protein diet, supplements of fat-soluble vitamins and responded well.
ISSN:2053-8855
2053-8855
DOI:10.1093/omcr/omz065