A case of adolescent hyperlipoproteinemia with xanthoma and acute pancreatitis, associated with decreased activities of lipoprotein lipase and hepatic triglyceride lipase.
A case of adolescent hyperlipoproteinemia with xanthoma and acute pancreatitis, associated with decreased activities of lipoprotein lipase and hepatic triglyceride lipase.
复制标题
青少年高脂蛋白血症伴黄瘤和急性胰腺炎一例，与脂蛋白脂肪酶和肝甘油三酯脂肪酶活性降低有关。
DOI：
10.2169/internalmedicine.49.4058
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发表时间：
2010
影响因子：
1.2
通讯作者：
I. Shimomura
中科院分区：
文献类型：
--
作者：
Koichi Fujita;N. Maeda;J. Kozawa;Kakeyoshi Murano;K. Okita;H. Iwahashi;S. Kihara;M. Ishigami;M. Omura;Tadashi Nakamura;K. Shirai;T. Yamamura;T. Funahashi;I. Shimomura
脂蛋白脂肪酶 (LPL) 和肝甘油三酯脂肪酶 (HTGL) 增强乳糜微粒 (CM) 和极低密度脂蛋白 (VLDL) 转运的甘油三酯 (TG) 的水解。我们报告了一名 15 岁男孩患有严重高乳糜微粒血症的病例，其中残余脂蛋白和总胆固醇 (T-Chol) 水平较高。脂质谱的精确检查显示，尽管 LPL 和 HTGL 的蛋白质质量保持不变，但 LPL 和 HTGL 的活性均下降。此外，苯扎贝特治疗有效改善了该病例的高甘油三酯血症。这是第一例高乳糜微粒血症，LPL 和 HTGL 活性降低且蛋白质量未受影响，但没有明显的免疫功能障碍。
Lipoprotein lipase (LPL) and hepatic triglyceride lipase (HTGL) enhance the hydrolysis of triglycerides (TG) transported by chylomicron (CM) and very-low-density lipoprotein (VLDL). We report a case of severe hyperchylomicronemia with high levels of remnant lipoprotein and total cholesterol (T-Chol) in a 15-year-old boy. Precise examination of the lipid profile showed decreased activities of both LPL and HTGL, although the protein mass for LPL and HTGL were maintained. In addition, bezafibrate treatment effectively ameliorated hypertriglyceridemia in this case. This is the first case of hyperchylomicronemia with decreased activities and unaffected protein masses for both LPL and HTGL, without overt immuno-dysfunction.
影响因子：
--
作者：
Reichlin, M;Fesmire, J;Wolfson-Reichlin, M
通讯作者：
Wolfson-Reichlin, M