원발성 장림프관확장증에 의한 단백상실성 장질환 1예
A Case of Protein Losing Enteropathy Caused by Primary Intestinal Lymphangiectasia
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원발성 장림프관확장증은 림프관 발생 과정에서의 기형으로 인해 비정상적으로 확장된 림프관 및 이를 통해 혈장 단백, 림프구, 유미미립 등이 풍부한 림프액이 장관 내로 누출되어 설사, 부종, 복수, 저단백혈증, 유미흉, 림프 부종, 림프구 감소증 등을 일으키는 드문 질환이다. 저자들은 전신 부종 및 설사를 주소로 내원한 환자에서 24시간 대변 α1-antitrypsin 청소율 및 99mTc 인혈청알부민 스캔을 통해 위장관을 통한 단백 소실을 증명하였고, 내시경 및 조직 검사에서 비정상적으로 확장된 림프관을 확인하였으며, 영상 진단 등으로 이차성 장림프관확장증을 배제할 수 있어서, 원발성 장림프관확장증으로 진단하였기에 문헌 고찰과 함께 보고하는 바이다.
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)
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