3) 腸リンパ管拡張症

Intestinal Lymphangiectasia

要旨　腸リンパ管拡張症は，リンパ流障害によるリンパ管の著明な拡張とともに，蛋白を含むリンパ液が腸管内へ漏出する疾患である．臨床的には浮腫，下痢，脂肪便，腹痛，乳糜腹水・胸水，低アルブミン血症，低Ca血症，末梢血リンパ球数の減少，低γ-グロブリン血症，易感染性を示す．診断には，十二指腸・小腸内視鏡検査で特徴的所見（白色絨毛，散布性白点，白色小隆起，粘膜下腫瘤様隆起）を認めること，およびα₁アンチトリプシン・クリアランス試験や^99mTc-human serum albuminシンチグラフィーで蛋白漏出性腸症を確認することが有用である．また，リンパ管造影やリンパ管シンチグラフィーでのリンパ管異常も参考になる．治療の原則は経口脂肪摂取の制限であるが，二次性では基礎疾患の治療を行う．

　Intestinal lymphangiectasia is a disease characterized by a dilatation of lymphatics, which leads to the loss of lymph to the intestine. Clinical symptoms and signs include edema, diarrhea, steatorrhea, abdominal pain, chylous ascites and pleural effusion, hypoalbuminemia, hypocalcemia, peripheral lymphocytopenia, and an increased susceptibility to infection. Diagnosis can be established by endoscopic findings such as white villi, white spots, white nodules, and submucosal elevations, and the evidence of protein-losing enteropathy, which is confirmed by the alpha 1-antitrypsin clearance test and ^99mTc-human serum albumin scintigraphy. Lymphangiography and lymphoscintigraphy are also of value. The principle for the treatment of this disease is the restriction of oral fat intake, although underlying problems should also be treated in secondary disorders.