Intestinal Lymphangiectasia Secondary to an Inflammatory Process: Case Report and Review of Literature

小腸淋巴管擴張症次發於一發炎病程：一病例報告及文獻回顧

彭世明(Shyn-Ming Peng) ； 吳正雄(Cheng-Shyong Wu) ； 黃啟誌(Chi-Chih Huang) ； 簡榮南(Rong-Nan Chien) ； 陳邦基(Pang-Chi Chen) ； 薛綏(Swei Hsueh)

《中華民國消化系醫學會雜誌》 9卷1期 (1992/03) Pp. 29-33

https://doi.org/10.6557/GJTa.199203_9(1).0005

摘要

小腸淋巴管擴張症幷發腸蛋白質漏失症，爲極少見之疾病，好發于小孩子。其特微爲全身水腫，血清中蛋白質極低，淋巴球流失及小腸粘膜或粘膜下淋巴管擴張。我們報告－38歲成年男性，具典型小腸淋巴管擴張症及腸蛋白質漏失症之臨床表徵之病例，並經由小腸病理切片證實爲小腸淋巴管擴張症。
其與原發性小腸淋巴管擴張症有下列不同點：較晚發病年齡，極高之紅血球沈降率，及其他發炎之表徵。系列之檢查排除其他可確認的次發性小腸淋巴管擴張症。文獻上報告此類病人有些對於類固醇反應極佳，然而此病例對低劑量類固醇反應並不理想。

關鍵字

小腸淋巴管擴張症 ； 腸蛋白質漏失症

並列摘要

A 38 year-old male presented with a one-year history of generalized edema and diarrhea. Laboratory tests revealed severe hypoproteinemia, lymphocytopenia, no proteinuria or steatorrhea, and a normal intestinal D-xylose absorption test. Intestinal lymphangiectasia with protein-losing enteropathy was documented by a high alpha-1-antitrypsin clearance rate in stool and an intestinal histopathlogic study. However, there were differences from primary lymphangiectasia, such as later age of onset, high erythrocyte sedimentation rate, leukocytosis, and decreased C3 level. Intestinal lymphangiectasia secondary to an inflammatory process was considered likely in this patient. The response to low dose steroid was unsatisfactory.

並列關鍵字

Intestinal lymphangiectasia ； protein-losing enteropathy