A Case of Adolescent Hyperlipoproteinemia with Xanthoma and Acute Pancreatitis, Associated with Decreased Activities of Lipoprotein Lipase and Hepatic Triglyceride Lipase

被引：2

作者：

Fujita, Koichi ^{[1
]}

Maeda, Norikazu ^{[1
]}

Kozawa, Junji ^{[1
]}

Murano, Kakeyoshi ^{[2
]}

Okita, Kohei ^{[1
]}

Iwahashi, Hiromi ^{[1
]}

Kihara, Shinji ^{[1
]}

Ishigami, Masato ^{[3
]}

Omura, Motoko ^{[4
]}

Nakamura, Tadashi ^{[1
]}

Shirai, Kohji ^{[5
]}

Yamamura, Taku ^{[3
]}

Funahashi, Tohru ^{[1
]}

Shimomura, Iichiro ^{[1
]}

机构：

[1] Osaka Univ, Grad Sch Med, Dept Metab Med, Suita, Osaka, Japan

[2] Toho Univ, Med Ctr, Sakura Hosp, Res Dev Ctr, Sakura, Japan

[3] Osaka Univ, Grad Sch Med, Div Hlth Sci, Suita, Osaka, Japan

[4] Omura Internal Med & Cardiol Clin, Himeji, Hyogo, Japan

[5] Toho Univ, Med Ctr, Sakura Hosp, Dept Internal Med, Sakura, Japan

来源：

INTERNAL MEDICINE | 2010年 / 49卷 / 22期

关键词：

lipoprotein lipase; hepatic triglyceride lipase; chylomicron; remnant lipoprotein; pancreatitis; triglyceride; SYSTEMIC-LUPUS-ERYTHEMATOSUS; I HYPERLIPOPROTEINEMIA; AUTOIMMUNE HYPERCHYLOMICRONEMIA; APOLIPOPROTEIN-E; INHIBITION; HYPERTRIGLYCERIDEMIA; HYPERLIPIDEMIA; DEFICIENCY;

D O I：

10.2169/internalmedicine.49.4058

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：2467 / 2472

页数：6

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