Specific cardiomyopathy caused by multisystemic lipid storage in Jordans' anomaly.

Authors :: Nagai H
Oshima Y
Hirota H
Izumi M
Sugiyama S
Nakaoka Y
Terai K
Hasegawa S
Tateyama H
Kikui M
Yamauchi-Takihara K
Kawase I
Source :: Internal medicine (Tokyo, Japan) [Intern Med] 2003 Jul; Vol. 42 (7), pp. 587-90.
Publication Year :: 2003
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.

Subjects :: Adult
Cardiomyopathies diagnostic imaging
Cardiomyopathies pathology
Humans
Leukocytes metabolism
Leukocytes pathology
Lipid Metabolism, Inborn Errors diagnosis
Lipid Metabolism, Inborn Errors pathology
Male
Tomography, Emission-Computed
Vacuoles pathology
Cardiomyopathies etiology
Lipid Metabolism, Inborn Errors complications
Muscular Diseases complications

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