BACKGROUND Congenital intestinal lymphangiectasia is a disease which leads to protein losing enteropathy. Tortuous, dilated lymphatic vessels in the intestinal wall and mesenterium are typical features of the disease. Clinical manifestations include malabsorption, diarrhea, steatorrhea, edema and effusions. Specific diet and medication are required for disease control. CASE REPORT A 19-year o...

An immunocompetent 8-year-old boy with cytomegalovirus (CMV)-associated transient protein-losing enteropathy (PLE) is described. Colonoscopic examination revealed lymphoid hyperplasia of the terminal ileum. Histological examination of the biopsied specimens showed marked dilation of the lymphatic vessels. Primary CMV infection was demonstrated by serological test and polymerase chain reaction. ...

SUMMARY In the case of the 19-year-old girl reported, lymphopericardium was associated with congenital abnormalities of the lymphatic system and with gastrointestinal loss of protein secondary to intestinal lymphangiectasia. No communication with the thoracic duct was found. Additional In(lexing Words: Intestinal lymphangiectasia Chylopericardium Pericardiocentesis CI1111RONIC pericardial effus...

Primary intestinal lymphangiectasia (PIL) is a rare disorder, especially in adults. It causes a local disruption of chylus transport and is part of the exudative gastroenteropathies. Conservative therapy includes dietary measures or somatostatin medication. Taking the differential diagnosis of PIL into consideration is a major challenge, since patients suffering from PIL may present with diarrh...

Primary intestinal lymphangiectasia (PIL), also known as Waldmann's disease, is an exudative enteropathy resulting from morphologic abnormalities in the intestinal lymphatics. In this article, we describe a 12-year-old boy with PIL that led to protein-losing enteropathy characterized by diarrhea, hypoalbuminemia associated with edema (serum albumin level: 1.0 g/dL), and hypogammaglobulinemia (s...

Primary intestinal lymphangiectasia (PIL) is rare disorder characterized by congenital malformation or obstruction of intestinal lymphatic drainage; it is responsible for protein losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL management. The administration of...