Primary Intestinal Lymphangiectasia (Waldmann’s Disease) Presenting with Chylous Effusions in a 15-Year-Old

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Primary intestinal lymphangiectasia (Waldmann's disease)

Primary intestinal lymphangiectasia (PIL), Waldmann's disease, is a rare disorder of unknown etiology characterized by dilated intestinal lacteals leading to 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 patie...

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Primary Intestinal Lymphangiectasia Manifested as Unusual Edemas and Effusions

Primary intestinal lymphangiectasia (PIL) is a rare disorder of unknown etiology characterized by diffuse or localized dilation and eventual rupture of the enteric lymphatic vessels in mucosa, submucosa, and/or subserosa. Lymph, rich in all kinds of proteins and lymphocytes, leaks into the gastrointestinal tract via the affected lymphatic vessels causing hypoproteinemia and lymphopenia. The mai...

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Primary intestinal lymphangiectasia.

Primary intestinal lymphangiectasia (PIL) is a rare disease of intestinal lymphatics presenting with hypoproteinemia, bilateral lower limb edema, ascites, and protein losing enteropathy. We report a series of 4 children from Chennai, India presenting with anasarca, recurrent diarrhea, hypoproteinemia and confirmatory features of PIL on endoscopy and histopathology.

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ژورنال

عنوان ژورنال: JOURNAL OF CLINICAL AND DIAGNOSTIC RESEARCH

سال: 2017

ISSN: 2249-782X

DOI: 10.7860/jcdr/2017/29055.10522