INTESTINE Vol.21 No.6(2-6)


特集名 小腸の炎症性病変を見直す
題名 各論(6)小腸リンパ管拡張症の臨床像と治療
発刊年月 2017年 11月
著者 中村 正直 名古屋大学大学院医学系研究科消化器内科学
著者 大宮 直木 藤田保健衛生大学消化管内科
著者 廣岡 芳樹 名古屋大学医学部附属病院光学医療診療部
著者 後藤 秀実 名古屋大学大学院医学系研究科消化器内科学
【 要旨 】 腸リンパ管拡張症はリンパ管の狭窄閉塞もしくはリンパ管内圧の上昇に伴い蛋白や脂肪などが末梢のリンパ管から腸管内腔へ漏出する蛋白漏出性胃腸症の代表的疾患である.粘膜,粘膜下層におけるリンパ管の拡張の証明とそれによる臨床症状,臨床所見を認めた際に診断に至る.蛋白漏出性腸症としての診断も重要である.小腸粘膜所見が白色絨毛,散布性白点を有する白色絨毛群と,異常なしもしくは軽度絨毛腫大,ケルクリング襞腫大を認める非白色絨毛群の二つのタイプに分けられ,それらは治療の反応性が異なる.治療は栄養療法,薬剤療法,外科手術があるが著効する治療法は現在まで認めない.予後では日和見感染に注意が必要である.
Theme Reviewing inflammatory diseases of small bowel
Title Clinical features of intestinal lymphangiectasia and treatment
Author Masanao Nakamura Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine
Author Naoki Ohmiya Department of Gastroenterology, Fujita Health University School of Medicine
Author Yoshiki Hirooka Department of Endoscopy, Nagoya University Hospital
Author Hidemi Goto Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine
[ Summary ] Intestinal lymphangiectasia (IL) is the primary etiology of protein-losing enteropathy, which is a rare and occasionally life-threatening syndrome characterized by enteric loss of plasma proteins in abnormal amounts. IL can be diagnosed through histopathological findings, which exhibit dilated lymphatic vessels in the small bowel mucosa and submucosa. Double-balloon enteroscopic findings are classified into two categories : the white villi type and the non-white villi type. The white villi type is defined as having white plaques. The white-tipped villi are scattered in the small bowel (termed typical lymphangiectasia). The non-white villi type is often defined as apparently being normal. However under more detailed observation, low and round villi of normal color are diffused in the small bowel. Corticosteroid response is better in the non-white villi type. Treatment for IL includes diet therapy, medication, and surgical treatment, although there is not a good standardized therapy. We should be aware that opportunistic infections occur in IL patients with weakened immune systems.
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