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

Koichi Fujita; Norikazu Maeda; Junji Kozawa; Kakeyoshi Murano; Kohei Okita; Hiromi Iwahashi; Shinji Kihara; Masato Ishigami; Motoko Omura; Tadashi Nakamura; Kohji Shirai; Taku Yamamura; Tohru Funahashi; Iichiro Shimomura

doi:10.2169/internalmedicine.49.4058

CASE REPORTS

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

Koichi Fujita, Norikazu Maeda, Junji Kozawa, Kakeyoshi Murano, Kohei Okita, Hiromi Iwahashi, Shinji Kihara, Masato Ishigami, Motoko Omura, Tadashi Nakamura, Kohji Shirai, Taku Yamamura, Tohru Funahashi, Iichiro Shimomura

Author information

Koichi Fujita
Department of Metabolic Medicine, Graduate School of Medicine, Osaka University
Norikazu Maeda
Department of Metabolic Medicine, Graduate School of Medicine, Osaka University
Junji Kozawa
Department of Metabolic Medicine, Graduate School of Medicine, Osaka University
Kakeyoshi Murano
Research Development Center, Sakura Hospital, Medical Center, Toho University
Kohei Okita
Department of Metabolic Medicine, Graduate School of Medicine, Osaka University
Hiromi Iwahashi
Department of Metabolic Medicine, Graduate School of Medicine, Osaka University
Shinji Kihara
Department of Metabolic Medicine, Graduate School of Medicine, Osaka University
Masato Ishigami
Division of Health Sciences, Graduate School of Medicine, Osaka University
Motoko Omura
Omura Internal Medicine and Cardiology Clinic
Tadashi Nakamura
Department of Metabolic Medicine, Graduate School of Medicine, Osaka University
Kohji Shirai
Department of Internal Medicine, Sakura Hospital, Medical Center, Toho University
Taku Yamamura
Division of Health Sciences, Graduate School of Medicine, Osaka University
Tohru Funahashi
Department of Metabolic Medicine, Graduate School of Medicine, Osaka University
Iichiro Shimomura
Department of Metabolic Medicine, Graduate School of Medicine, Osaka University

Keywords: lipoprotein lipase, hepatic triglyceride lipase, chylomicron, remnant lipoprotein, pancreatitis, triglyceride

JOURNAL OPEN ACCESS

2010 Volume 49 Issue 22 Pages 2467-2472

DOI https://doi.org/10.2169/internalmedicine.49.4058

Details

Abstract

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.

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