Primary Intestinal Lymphangiectasia and its Association With Generalized Lymphatic Anomaly

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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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Association of nephrotic syndrome with intestinal lymphangiectasia.

The occurrence of exudative enteropathy in children with the nephrotic syndrome was first reported by Nussle et al. in 1961, but no intestinal pathology has previously been described in nephrotic children, whether with or without protein-losing enteropathy. The finding in small bowel biopsy specimens of intestinal lymphangiectasia, in 4 of 7 cases of nephrotic syndrome, suggests that this assoc...

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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 case of pil in a 22 year old patient from tabriz, iran who presented with anasarca edema, recurrent diarrhea, hypoproteinemia and confirmatory features of pil on endoscopy and histopathology. therefo...

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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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ژورنال

عنوان ژورنال: Journal of Pediatrics Review

سال: 2016

ISSN: 2322-4401,2322-4398

DOI: 10.17795/jpr-4790