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KMID : 0816120090120010079
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Korean Journal of Pediatric Gastroenterolology and Nutrition
2009 Volume.12 No. 1 p.79 ~ p.83
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A Case of Lipoprotein Lipase Deficiency in an Infant with Recurrent Pancreatitis
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Park Hae-Jin
Shin Choong-Ho
Yang Sei-Won
Seo Jeong-Kee
Ko Jae-Sung
Yang Hye-Ran
Jang Ju-Young
Choi Byeong-Sam
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Abstract
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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.
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KEYWORD
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Chylomicronemia, Lipid, Lipoprotein lipase, Pancreatitis, Infant, Hypertriglyceridemia
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