{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T05:42:29Z","timestamp":1784698949566,"version":"3.55.0"},"reference-count":31,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":9254,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[1990,5]]},"abstract":"<jats:sec>\n            <jats:title\/>\n            <jats:p>We describe three patients with cholesteryl ester storage disease. Diagnosis was confirmed by demonstrating a deficiency in lysosomal acid cholesteryl hydrolase activity in cultured skin fibroblasts from each of these patients. All had hepatomegaly, elevated serum aminotransferase activities and hyperlipoproteinemia. Histological examination of liver biopsy specimens before treatment revealed accumulation of fat within hepatocytes, bile duct epithelium and endothelial and Kupffer cells. Cholesterol crystals were recognized by their birefringence in frozen sections. A striking feature was the presence of markedly hypertrophied Kupffer cells and portal macrophages with foamy, tan\u2013colored cytoplasm that stained readily with the periodic acid\u2013Schiff reagent and aldehyde fuchsin. Periportal fibrosis was noted in all cases; incomplete cirrhosis was present in one case. Distinctive and hitherto undescribed lysosomal accumulations of triglyceride and cholesterol crystals were noted.<\/jats:p>\n            <jats:p>The patients were treated with lovastatin, a cholesterol\u2013lowering agent, for at least 12 mo. No significant changes were seen in serum lipoprotein concentrations or liver histopathology after therapy. Thus lovastatin did not have an obviously beneficial effect on abnormal lipid metabolism in these patients.(HEPATOLOGY 1990; 11:764\u2013772.)<\/jats:p>\n          <\/jats:sec>","DOI":"10.1002\/hep.1840110509","type":"journal-article","created":{"date-parts":[[2007,3,3]],"date-time":"2007-03-03T23:27:36Z","timestamp":1172964456000},"page":"764-772","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":46,"title":["Cholesteryl Ester Storage Disease: Hepatopathology and Effects of Therapy With Lovastatin"],"prefix":"10.1097","volume":"11","author":[{"given":"Adrian M.","family":"Di Bisceglie","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kamal G.","family":"Ishak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lionel","family":"Rabin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey M.","family":"Hoeg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"10.1002\/hep.1840110509-BIB1","first-page":"803","volume-title":"Metabolic basis of inherited disease","volume":"5","author":"Assmann","year":"1983","unstructured":". Acid lipase deficiency: Wolman's disease and cholesteryl ester storage disease. In: , , , , eds. Metabolic basis of inherited disease. Vol. 5. New York: McGraw-Hill, 1983: 803\u2013819."},{"key":"10.1002\/hep.1840110509-BIB2","first-page":"1190","volume":"36","author":"Hoeg","year":"1984","journal-title":"Am J Hum Genet"},{"key":"10.1002\/hep.1840110509-BIB3","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1016\/0006-2944(85)90123-1","volume":"33","author":"Kelly","year":"1985","journal-title":"Biochem Med"},{"key":"10.1002\/hep.1840110509-BIB4","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/0005-2760(82)90009-1","volume":"711","author":"Hoeg","year":"1982","journal-title":"Biochim Biophys Acta"},{"key":"10.1002\/hep.1840110509-BIB5","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1038\/clpt.1984.219","volume":"36","author":"Hoeg","year":"1984","journal-title":"Clin Pharmacol Ther"},{"key":"10.1002\/hep.1840110509-BIB6","series-title":"National Inst","volume-title":"Lipid research clinics population studies data book: Vol I. 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Edinburgh: Churchill Livingstone, 1987: 99\u2013180."},{"key":"10.1002\/hep.1840110509-BIB12","first-page":"239","volume-title":"The liver: an atlas and text of ultrastructural pathology","author":"Phillips","year":"1987","unstructured":", , . The liver: an atlas and text of ultrastructural pathology. New York: Raven Press, 1987: 239\u2013391."},{"key":"10.1002\/hep.1840110509-BIB13","first-page":"2785","volume":"75","author":"Lageron","year":"1967","journal-title":"La Presse M\u00e9dicale"},{"key":"10.1002\/hep.1840110509-BIB14","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1016\/0002-9343(68)90054-5","volume":"44","author":"Schiff","year":"1968","journal-title":"Am J Med"},{"key":"10.1002\/hep.1840110509-BIB15","first-page":"245","volume-title":"Bile salt metabolism","author":"O'Donnell","year":"1969","unstructured":". Serum bile acid patterns in hepatic cholesterol-ester storage disease. In: , , eds. Bile salt metabolism. 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