Synonyms
Primary intestinal lymphangiectasia (PIL); Waldmann’s disease
Definition
Intestinal lymphangiectasia is defined as the presence of dilated lymphatics within the intestinal mucosa. The form, known as secondary intestinal lymphangiectasia, is associated with a local neoplastic or inflammatory condition causing lymphatic obstruction. Conditions associated with secondary intestinal lymphangiectasia include lymphoma, carcinoma, Crohn’s disease, systemic lupus erythematosus, Behçet’s disease, radiation therapy, trauma, heart disease, and liver transplantation. Primary intestinal lymphangiectasia, on the other hand, is a rare congenital disorder characterized by severe protein-losing enteropathy, peripheral edema, steatorrhea, and lymphocytopenia. Effusions may develop in pleural, pericardial, and peritoneal cavities with gross chylous ascites. There is a major structural abnormality of the lymphatic system consisting of dilatation and tortuosity of lymphatic vessels resulting in...
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Braamskamp, M. J., Dolman, K. M., & Tabbers, M. M. (2010). Clinical practice. Protein-losing enteropathy in children. European Journal of Pediatrics, 169(10), 1179–1185.
Vignes, S., & Bellanger, J. (2008). Primary intestinal lymphangiectasia (Waldmann’s disease). Orphanet Journal of Rare Diseases, 3, 5.
Wen, J., Tang, Q., Wu, J., Wang, Y., & Cai, W. (2010). Primary intestinal lymphangiectasia: Four case reports and a review of the literature. Digestive Diseases and Sciences, 55(12), 3466–3472.
Xinias, I., Mavroudi, A., Sapountzi, E., Thomaidou, A., Fotoulaki, M., Kalambakas, A., Karypidou, E., Kollios, K., Pardalos, G., & Imvrios, G. (2013). Primary intestinal lymphangiectasia: Is it always bad? Two cases with different outcome. Case Reports in Gastroenterology, 20, 153–163.
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Ensari, A. (2017). Intestinal Lymphangiectasia. In: Carneiro, F., Chaves, P., Ensari, A. (eds) Pathology of the Gastrointestinal Tract. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-40560-5_1497
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DOI: https://doi.org/10.1007/978-3-319-40560-5_1497
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Publisher Name: Springer, Cham
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Online ISBN: 978-3-319-40560-5
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