A case of lymphangioleiomyomatosis associated with protein-losing gastroenteropathy lymphangiectasia

A case of lymphangioleiomyomatosis associated with protein-losing gastroenteropathy - lymphangiectasia

Nippon Shokakibyo Gakkai Zasshi. 2008 Dec

Kinoshita A, Odagi I, Aoki Y, Hirohama K, Ishiguro H, Nikami T, Tamaki S, Searashi Y, Sudou S, Hokari A, Ishikawa T, Negishi M, Nishino H, Tajiri H, Ikegami M.
Division of Gastroenterology and Hepatology, Department of Internal Medicine, The Jikei University School of Medicine.

A 29-year-old woman was referred to our hospital for the intensive examination of leg edema and hypoproteinemia. CT scan of showed multiple thin-walled cysts in both lungs, suggesting lymphangioleiomyomatosis. CT scan of the abdomen, lymphoscintigraphy showed enlarged abdominal lymph nodes. Protein loss from the gastrointestinal tract was documented by measurement of the clearance of alpha-1 antitrypsin from the plasma using a 72h stool collection and (99m)Tc human serum albumin scintigraphy. We thought that secondary lymphangiectasia with lymphangioleiomyomatosis caused protein-losing gastroenteropathy. Dietary therapy resulted in symptomatic improvement.

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