How to treat an extensive form of primary intestinal lymphangiectasia?  

作　　者：Rosana Troskot Dragan Jurcic Ante Bilic Marija Gomercic Palcic Stanko Tezak Ivana Brajkovic 

机构地区：[1]Faculty of Medicine,J.J.Strossmayer University of Osijek,Division of Gastroenterology and Hepatology,Department of Internal Medicine,Clinical Hospital“Sveti Duh” [2]Division of Gastroenterology and Hepatology,Department of Internal Medicine,“Sestre milosrdnice”University Hospital Center [3]University of Zagreb School of Medicine [4]Clinical Department of Nuclear Medicine and Radiation Protection,University Hospital Centre Zagreb [5]Dialysis clinic,Polyclinic“Sveti Duh”II

出　　处：《World Journal of Gastroenterology》2015年第23期7320-7325,共6页世界胃肠病学杂志（英文版）

摘　　要：We report a case of a 42-year-old man with a rare disorder known as primary intestinal lymphangiectasia,which is characterized by dilated intestinal lymphatics that lead to the development of protein-losing enteropathy. The patient presented with a grand mal seizure caused by malabsorption-derived electrolytes and a protein disorder. Signs of the disease, including chronic diarrhea and peripheral edema, manifested10 years ago, but a diagnosis was never made. The diagnosis was suspected because of the clinical manifestations, laboratory tests, imaging and endoscopic findings. Hyperemic and edematous mucosa of the small intestine corresponded to scattered white spots with dilated intestinal lymphatics and whitish villi in the histological specimen of the biopsied jejunal mucosa.Although numerous therapeutic strategies are available,only octreotide therapy proved to be an effective means of therapeutic resolution in this patient. Although the patient had a partial remission following the use of a slow release formula of octreotide, his prognosis, clinical course, and future treatment challenges are yet to be determined.We report a case of a 42-year-old man with a rare disorder known as primary intestinal lymphangiectasia,which is characterized by dilated intestinal lymphatics that lead to the development of protein-losing enteropathy. The patient presented with a grand mal seizure caused by malabsorption-derived electrolytes and a protein disorder. Signs of the disease, including chronic diarrhea and peripheral edema, manifested10 years ago, but a diagnosis was never made. The diagnosis was suspected because of the clinical manifestations, laboratory tests, imaging and endoscopic findings. Hyperemic and edematous mucosa of the small intestine corresponded to scattered white spots with dilated intestinal lymphatics and whitish villi in the histological specimen of the biopsied jejunal mucosa.Although numerous therapeutic strategies are available,only octreotide therapy proved to be an effective means of therapeutic resolution in this patient. Although the patient had a partial remission following the use of a slow release formula of octreotide, his prognosis, clinical course, and future treatment challenges are yet to be determined.

关 键 词：Primary intestinal lymphangiectasia MALABSORPTION SEIZURE Treatment DIET OCTREOTIDE 

分 类 号：R574.5[医药卫生—消化系统]