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KMID : 1038820210240050413
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Pediatric Gastroenterology, Hepatology & Nutrition
2021 Volume.24 No. 5 p.413 ~ p.422
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The Update of Treatment for Primary Intestinal Lymphangiectasia
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Kwon Yi-Young
Kim Mi-Jin
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Abstract
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Intestinal lymphangiectasia is a rare disease which is causing protein-losing enteropathy. Treatment of intestinal lymphangiectasia can be a challenge for clinicians because of the lack of specific guidelines regarding pharmacological indications. We sought to introduce a diagnostic approach and suggest guidelines for treatment. After exclusion of secondary intestinal lymphangiectasia, magnetic resonance lymphangiography is a promising tool for the assessment of abnormal lymphatic lesions in primary intestinal lymphangiectasia. Determining the extent of the lesion provides direction for treatment options. Focal short-segment intestinal lymphangiectasia can be treated via intestinal resection or radiologic embolization after dietary therapy failure. Diffuse intestinal lymphangiectasia and extensive lymphangiectasia should be treated with several drugs with a full understanding of their mechanisms.
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KEYWORD
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Lymphangiectasis, Therapeutic embolization, Octreotide, Sirolimus, Everolimus
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