Journal List > Korean J Pediatr Gastroenterol Nutr > v.12(1) > 1110169

Korean J Pediatr Gastroenterol Nutr. 2009 Mar;12(1):79-83. Korean.
Published online Mar 31, 2009.
Copyright © 2009 The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition
A Case of Lipoprotein Lipase Deficiency in an Infant with Recurrent Pancreatitis
Hae Jin Park, Byeong Sam Choi, Hye Ran Yang, Ju Young Chang, Jae Sung Ko, Choong Ho Shin, Sei Won Yang and Jeong Kee Seo
Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.

Familial chylomicronemia syndrome is a rare disorder characterized by severe hypertriglyceridemia and fasting chylomicronemia. Causes of the syndrome include lipoprotein lipase (LPL) deficiency, apolipoprotein C-II deficiency, or the presence of inhibitors to LPL. We managed a 3-month-old girl who had recurrent acute pancreatitis caused by chylomicronemia. We report the first case of familial chylomicronemia in Korea caused by LPL deficiency in an infant with recurrent acute pancreatitis.

Keywords: Chylomicronemia; Lipid; Lipoprotein lipase; Pancreatitis; Infant; Hypertriglyceridemia