Korean J Gastrointest Endosc > Volume 33(5); 2006 > Article
Korean Journal of Gastrointestinal Endoscopy 2006;33(5): 307-312.
원발성 장림프관확장증에 의한 단백상실성 장질환 1예
이세영·여주천·윤영득·김새롬·권영란·류현욱·김준철·이명권·박창근·이상문
대구파티마병원 내과
A Case of Protein Losing Enteropathy Caused by Primary Intestinal Lymphangiectasia
Se Young Lee, M.D., Ju Chun Yeo, M.D., Young Deuk Youn, M.D., Sae Rom Kim, M.D., Young Lan Kwon, M.D., Hyon Uk Ryu, M.D., Jun Chul Kim, M.D., Myung Kwon Lee, M.D., Chang Keun Park, M.D. and Sang Mun Lee, M.D.
Department of Internal Medicine, Daegu Fatima Hospital, Daegu, Korea
Abstract
Primary intestinal lymphangiectasia is a rare congenital cause of protein losing enteropathy that is characterized by chronic diarrhea, generalized edema, ascites, hypoproteinemia, hypoalbuminemia, and lymphopenia. We encountered an 18-year-old woman who suffered from longstanding diarrhea and progressive leg edema. The laboratory findings showed the typical features of this disorder. The presence of enteric protein loss was documented with the 24 hour fecal clearance of α1-antitrypsin and 99mTc human serum albumin scintigraphy. A duodenoscopy and biopsy showed scattered white spots and markedly dilated lymphatics in the tips of the villi, respectively. The patient's clinical symptoms improved after placing her on a high protein and low fat diet with medium chain triglyceride supplements. (Korean J Gastrointest Endosc 2006;33:307⁣312)
Key Words: Protein losing enteropathy, Primary intestinal lymphangiectasia, 99mTc human serum albumin scintigraphy, α1-antitrypsin clearance
주요어: 단백상실성 장질환, 원발성 장림프관확장증, 99mTc 인혈청알부민스캔, α1-antitrypsin 청소율
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