A Case of Lipoprotein Lipase Deficiency inan Infant with Recurrent Pancreatitis.
- Author:
Hae Jin PARK
1
;
Byeong Sam CHOI
;
Hye Ran YANG
;
Ju Young CHANG
;
Jae Sung KO
;
Choong Ho SHIN
;
Sei Won YANG
;
Jeong Kee SEO
Author Information
1. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. jkseo@snu.ac.kr
- Publication Type:Case Report
- Keywords:
Chylomicronemia;
Lipid;
Lipoprotein lipase;
Pancreatitis;
Infant;
Hypertriglyceridemia
- MeSH:
Fasting;
Humans;
Hyperlipoproteinemia Type I;
Hypertriglyceridemia;
Infant;
Korea;
Lipoprotein Lipase;
Lipoproteins;
Pancreatitis
- From:Korean Journal of Pediatric Gastroenterology and Nutrition
2009;12(1):79-83
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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.