Accumulation of acidic phospholipids in a case of hyperlipidemia with hepatosplenomegaly |
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| Authors: | Akira Yamamoto Susumu Adachi Takeshi Ishibe Yoshitake Shinji Yoshiyuki Kaki-Uchi Ko-Ichi Seki Teruo Kitani |
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| Affiliation: | (1) The Second Department of Internal Medicine, Osaka University Medical School, Fukushima-ku, 553 Osaka, Japan |
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| Abstract: | The results of studies made on an adult patient with hepatosplenomegaly, hyperlipidemia and accumulation of acidic phospholipids
in the liver are presented. Storage of a lipoid substance was demonstrated histologically in lymph nodes, the liver and the
bone marrow. Lipid analysis of the biopsy specimen from the liver revealed marked elevations of free cholesterol and two classes
of acidic phospholipids: phosphatidyl inositol and another one which was tentatively identified as lysobisphosphatidic acid.
Electron microscopic examination showed cytoplasmic inclusions with concentrically laminated structure. |
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