A Case of Lipoprotein Lipase Deficiency in an Infant with Recurrent Pancreatitis

H. Park, Byeong Sam Choi, H. Yang, J. Chang, J. Ko, C. Shin, S. Yang, J. Seo
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引用次数: 0

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. (Korean J Pediatr Gastroenterol Nutr 2009; 12: 79∼83)
复发性胰腺炎婴儿脂蛋白脂肪酶缺乏1例
家族性乳糜微粒血症综合征是一种罕见的疾病,其特征是严重的高甘油三酯血症和空腹乳糜微粒血症。该综合征的原因包括脂蛋白脂肪酶(LPL)缺乏,载脂蛋白C-II缺乏或LPL抑制剂的存在。我们处理一个3个月大的女孩谁有复发急性胰腺炎引起的乳糜微粒血症。我们报告的第一例家族性乳糜微粒血症在韩国引起的LPL缺乏症的婴儿与复发性急性胰腺炎。韩国儿科胃肠病学杂志2009;12: 79∼83)
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