Specific Cardiomyopathy Caused by Multisystemic Lipid Storage in Jordans' Anomaly

Hiroyuki NAGAI; Yuichi OSHIMA; Hisao HIROTA; Masahiro IZUMI; Shoko SUGlYAMA; Yoshikazu NAKAOKA; Kazuo TERAI; Shinji HASEGAWA; Hitone TATEYAMA; Masanori KlKUl; Keiko YAMAUCHI-TAKIHARA; Ichiro KAWASE

doi:10.2169/internalmedicine.42.587

Hiroyuki NAGAI, Yuichi OSHIMA, Hisao HIROTA, Masahiro IZUMI, Shoko SUGlYAMA, Yoshikazu NAKAOKA, Kazuo TERAI, Shinji HASEGAWA, Hitone TATEYAMA, Masanori KlKUl, Keiko YAMAUCHI-TAKIHARA, Ichiro KAWASE

Author information

Hiroyuki NAGAI
Department of Internal Medicine, Sakai Municipal Hospital
Yuichi OSHIMA
Department of Molecular Medicine, Osaka University Graduate School of Medicine
Hisao HIROTA
Department of Molecular Medicine, Osaka University Graduate School of Medicine
Masahiro IZUMI
Department of Molecular Medicine, Osaka University Graduate School of Medicine
Shoko SUGlYAMA
Department of Molecular Medicine, Osaka University Graduate School of Medicine
Yoshikazu NAKAOKA
Department of Molecular Medicine, Osaka University Graduate School of Medicine
Kazuo TERAI
Department of Molecular Medicine, Osaka University Graduate School of Medicine
Shinji HASEGAWA
Division of Tracer Kinetics, Biomedical Research Center, Osaka University Graduate School of Medicine
Hitone TATEYAMA
Internal Medicine, Tominaga Hospital
Masanori KlKUl
Department of Pathology, Osaka Prefectural Habikino Hospital
Keiko YAMAUCHI-TAKIHARA
Department of Molecular Medicine, Osaka University Graduate School of Medicine
Ichiro KAWASE
Department of Molecular Medicine, Osaka University Graduate School of Medicine

Keywords: lipid metabolic disorder, FDG-PET, BMIPP

JOURNAL FREE ACCESS

2003 Volume 42 Issue 7 Pages 587-590

DOI https://doi.org/10.2169/internalmedicine.42.587

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Abstract

Multisystemic lipid storage disease is a rare disorder of lipid metabolism. We report one case of a Japanese man with systemic lipid storage in skeletal muscle and heart as well as in leukocytes (Jordans' anomaly). Positron emission tomography (PET) using ¹⁸F-fluoro-2-deoxyglucose (FDG) clearly revealed an abnormal increase of uptake during fasting in the left ventricle, suggesting changes in the energy metabolism in the heart.
(Internal Medicine 42: 587-590, 2003)

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