The Update of Treatment for Primary Intestinal Lymphangiectasia

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 sug...

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Bibliographic Details
Published inPediatric gastroenterology, hepatology & nutrition Vol. 24; no. 5; pp. 413 - 422
Main Authors Kwon, Yiyoung, Kim, Mi Jin
Format Journal Article
LanguageKorean
Published 2021
Subjects
Therapeutic embolization
Lymphangiectasis
Sirolimus
Octreotide
Everolimus
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Summary: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.
Bibliography:KISTI1.1003/JNL.JAKO202127137844418
ISSN:2234-8646
2234-8840