Cholesterol Ester Storage Diseaseと思われる一症例

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A case of cholesterol-ester storage disease, the first reported case in Japan, was described. <BR>A 57 year-old male, operated for cholecystectomy showed enlarged liver with orangereddish and granular surface. Liver function tests were fairly normal and serum cholesterol and low density lipoprotein were slightly elevated. However, LCAT was within normal limits. Gas chromatography of blie acids in the bile showed a marked increase in chenodeoxycholic acid. Cholesterol ester content in the liver tissue was more than 20 times the normal. Microscopic study of the liver specimen revealed swollen hepatocytes with fibrosis and electron microscopy showed numerous samll fat droplets and crystalline inclusions in hepatocytes and Kupffer cells. These crystalline inclusions are probably cholesterol ester deposits.
社団法人日本肝臓学会の論文