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KMID : 0359020060330050307
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Korean Journal of Gastrointestinal Endoscopy
2006 Volume.33 No. 5 p.307 ~ p.312
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A Case of Protein Losing Enteropathy Caused by Primary Intestinal Lymphangiectasia
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Lee Se-Young
Kim Sae-Rom
Kwon Young-Lan
Youn Young-Deuk
Yeo Ju-Chun
Ryu Hyon-Uk
Kim Jun-Chul
Lee Myung-Kwon
Park Chang-Keun
Lee Sang-Mun
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Abstract
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Primary intestinal lymphangiectasia is a rare congenital cause of protein losing enteropathy that is characterized by chronic diarrhea, generalized edema, ascites, hypoproteinemia, hypoalbuminemia, and lymphopenia. We encountered an 18-year-old woman who suffered from longstanding diarrhea and progressive leg edema. The laboratory findings showed the typical features of this disorder. The presence of enteric protein loss was documented with the 24 hour fecal clearance of alpha(1)-antitrypsin and (99m)Tc human serum albumin scintigraphy. A duodenoscopy and biopsy showed scattered white spots and markedly dilated lymphatics in the tips of the villi, respectively. The patient’s clinical symptoms improved after placing her on a high protein and low fat diet with medium chain triglyceride supplements.
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KEYWORD
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Protein Losing enteropathy, Primary intestinal Lymphangiectasia, (99m)Tc human serum albumin scintigraphy, α1-antitrypsin clearance
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