A 19-year-old female was admitted to our hospital because of fever attacks, intermittent whitish stools and pretibial pitting edema. Laboratory examinations on admission demonstrated the following abnormal findings ; T. P. 3.4 g/dl, absolute lymphocyte count 377/mm3, and 125I-PVP test 5.2%. Duodenal and jejunal endoscopic examinations showed scattered white spots and white villi, from which the biopsy specimens revealed dilated lymphatics in the mucosa. On lymphangiogram, marked tortuosity and irregular dilatation of para-aortic lymphatics were seen. Since underlying causes of lymphangiectasia were not evident, she was diagnosed as having primary intestinal lymphangiectasia, and was treated successfully with a defined formula diet containing medium chain triglyceride, namely, the presenting symptoms, laboratory data and endoscopic findings were markedly improved about 1.5 months after the treatment.
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