{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,13]],"date-time":"2026-01-13T07:25:55Z","timestamp":1768289155922,"version":"3.49.0"},"reference-count":33,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2013,9,1]],"date-time":"2013-09-01T00:00:00Z","timestamp":1377993600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2013,7,16]],"date-time":"2013-07-16T00:00:00Z","timestamp":1373932800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Hepatology"],"published-print":{"date-parts":[[2013,9]]},"DOI":"10.1016\/j.jhep.2013.04.016","type":"journal-article","created":{"date-parts":[[2013,4,26]],"date-time":"2013-04-26T02:44:31Z","timestamp":1366944271000},"page":"543-549","source":"Crossref","is-referenced-by-count":36,"title":["Hepatic cholesteryl ester accumulation in lysosomal acid lipase deficiency: Non-invasive identification and treatment monitoring by magnetic resonance"],"prefix":"10.1016","volume":"59","author":[{"given":"Peter E.","family":"Thelwall","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fiona E.","family":"Smith","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mark C.","family":"Leavitt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Canty","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wei","family":"Hu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kieren G.","family":"Hollingsworth","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christian","family":"Thoma","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael I.","family":"Trenell","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roy","family":"Taylor","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joseph V.","family":"Rutkowski","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrew M.","family":"Blamire","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anthony G.","family":"Quinn","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.jhep.2013.04.016_b0005","series-title":"The metabolic and molecular bases of inherited disease","first-page":"3551","article-title":"Acid lipase deficiency: Wolman disease and cholesterol ester storage disease","author":"Assmann","year":"2001"},{"key":"10.1016\/j.jhep.2013.04.016_b0010","doi-asserted-by":"crossref","first-page":"1230","DOI":"10.1016\/j.jhep.2013.02.014","article-title":"Cholesteryl ester storage disease: review of the findings in 135 reported patients with an under-diagnosed disease","volume":"58","author":"Bernstein","year":"2013","journal-title":"J Hepatol"},{"key":"10.1016\/j.jhep.2013.04.016_b0015","doi-asserted-by":"crossref","first-page":"910","DOI":"10.1016\/S0022-3476(77)80557-X","article-title":"Cholesterol ester storage disease: clinical, biochemical, and pathological studies","volume":"90","author":"Beaudet","year":"1977","journal-title":"J Pediatr"},{"key":"10.1016\/j.jhep.2013.04.016_b0020","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1006\/mgme.1999.2904","article-title":"Lysosomal acid lipase mutations that determine phenotype in Wolman and cholesterol ester storage disease","volume":"68","author":"Anderson","year":"1999","journal-title":"Mol Genet Metab"},{"key":"10.1016\/j.jhep.2013.04.016_b0025","doi-asserted-by":"crossref","first-page":"528","DOI":"10.1016\/S0168-8278(00)80407-9","article-title":"Subclinical course of cholesteryl ester storage disease in an adult with hypercholesterolemia, accelerated atherosclerosis, and liver cancer","volume":"32","author":"Elleder","year":"2000","journal-title":"J Hepatol"},{"key":"10.1016\/j.jhep.2013.04.016_b0030","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1111\/j.1749-6632.1987.tb32915.x","article-title":"Spatial localization in NMR spectroscopy in vivo","volume":"508","author":"Bottomley","year":"1987","journal-title":"Ann N Y Acad Sci"},{"key":"10.1016\/j.jhep.2013.04.016_b0035","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1002\/jmri.21492","article-title":"Dixon techniques for water and fat imaging","volume":"28","author":"Ma","year":"2008","journal-title":"J Magn Reson Imaging"},{"key":"10.1016\/j.jhep.2013.04.016_b0040","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1002\/jmri.1880050311","article-title":"Proton MR spectroscopy in quantitative in vivo determination of fat content in human liver steatosis","volume":"5","author":"Longo","year":"1995","journal-title":"J Magn Reson Imaging"},{"key":"10.1016\/j.jhep.2013.04.016_b0045","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1111\/j.1365-2036.2012.05121.x","article-title":"Correlation between liver histology and novel magnetic resonance imaging in adult patients with non-alcoholic fatty liver disease \u2013 MRI accurately quantifies hepatic steatosis in NAFLD","volume":"36","author":"Permutt","year":"2012","journal-title":"Aliment Pharmacol Ther"},{"key":"10.1016\/j.jhep.2013.04.016_b0050","doi-asserted-by":"crossref","first-page":"4974","DOI":"10.1021\/bi00290a015","article-title":"Natural abundance carbon-13 nuclear magnetic resonance spectroscopy of liver and adipose tissue of the living rat","volume":"22","author":"Canioni","year":"1983","journal-title":"Biochemistry"},{"key":"10.1016\/j.jhep.2013.04.016_b0055","first-page":"486","article-title":"Genetic lipid storage disease with lysosomal acid lipase deficiency in rats","volume":"40","author":"Yoshida","year":"1990","journal-title":"Lab Anim Sci"},{"key":"10.1016\/j.jhep.2013.04.016_b0060","doi-asserted-by":"crossref","DOI":"10.1002\/hep.26289","article-title":"Clinical effect and safety profile of recombinant human lysosomal acid lipase in patients with cholesteryl ester storage disease","author":"Balwani","year":"2013","journal-title":"Hepatology"},{"key":"10.1016\/j.jhep.2013.04.016_b0065","unstructured":"Safety, tolerability and pharmacokinetics of SBC-102 in adult patients with lysosomal acid lipase deficiency. Available from: http:\/\/www.clinicaltrials.gov\/ct2\/show\/NCT01307098."},{"key":"10.1016\/j.jhep.2013.04.016_b0070","unstructured":"Extension study to evaluate the long-term safety, tolerability, and efficacy of SBC-102 in adult subjects with lysosomal acid lipase deficiency. Available from: http:\/\/www.clinicaltrials.gov\/ct2\/show\/NCT01488097."},{"key":"10.1016\/j.jhep.2013.04.016_b0075","unstructured":"An observational study of patients with lysosomal acid lipase deficiency\/cholesteryl ester storage disease phenotype. Available from: http:\/\/clinicaltrials.gov\/show\/NCT01528917."},{"key":"10.1016\/j.jhep.2013.04.016_b0080","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1016\/S0010-4825(01)00006-3","article-title":"ScienceDirect.com \u2013 computers in biology and medicine \u2013 java-based graphical user interface for MRUI, a software package for quantitation of in vivo\/medical magnetic resonance spectroscopy signals","volume":"31","author":"Naressi","year":"2001","journal-title":"Comput Biol Med"},{"key":"10.1016\/j.jhep.2013.04.016_b0085","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1007\/BF02668096","article-title":"Java-based graphical user interface for the MRUI quantitation package","volume":"12","author":"Naressi","year":"2001","journal-title":"MAGMA"},{"key":"10.1016\/j.jhep.2013.04.016_b0090","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1006\/jmre.1999.1835","article-title":"Time-domain quantification of series of biomedical magnetic resonance spectroscopy signals","volume":"140","author":"Vanhamme","year":"1999","journal-title":"J Magn Reson"},{"issue":"7","key":"10.1016\/j.jhep.2013.04.016_b0095","doi-asserted-by":"crossref","first-page":"784","DOI":"10.1002\/nbm.1622","article-title":"In vivo characterization of the liver fat (1)HMR spectrum","volume":"24","author":"Hamilton","year":"2011","journal-title":"NMR Biomed"},{"key":"10.1016\/j.jhep.2013.04.016_b0100","unstructured":"Spectral database for organic compounds, SDBSWeb. National Institute of Advanced Industrial Science and Technology. Available from: http:\/\/sdbs.riodb.aist.go.jp."},{"key":"10.1016\/j.jhep.2013.04.016_b0105","doi-asserted-by":"crossref","first-page":"2212","DOI":"10.1016\/S0022-2275(20)39205-1","article-title":"Cloning of rat lysosomal acid lipase cDNA and identification of the mutation in the rat model of Wolman\u2019s disease","volume":"36","author":"Nakagawa","year":"1995","journal-title":"J Lipid Res"},{"key":"10.1016\/j.jhep.2013.04.016_b0110","first-page":"506","article-title":"Chelex 100 as a medium for simple extraction of DNA for PCR-based typing from forensic material","volume":"10","author":"Walsh","year":"1991","journal-title":"BioTechniques"},{"key":"10.1016\/j.jhep.2013.04.016_b0115","doi-asserted-by":"crossref","first-page":"934","DOI":"10.1002\/hep.23797","article-title":"High-fructose, medium chain trans fat diet induces liver fibrosis and elevates plasma coenzyme Q9 in a novel murine model of obesity and nonalcoholic steatohepatitis","volume":"52","author":"Kohli","year":"2010","journal-title":"Hepatology"},{"key":"10.1016\/j.jhep.2013.04.016_b0120","unstructured":"den Boogaard van A, Van Hecke A, Van Huffel P, Graveron-Demilly S, van Ormondt D, de Beer R. MRUI: a graphical user interface for accurate routine MRS data analysis, Proceedings of the ESMRMB 13th Annual Meeting, Prague; 1996, p. 318"},{"key":"10.1016\/j.jhep.2013.04.016_b0125","first-page":"1190","article-title":"Cholesteryl ester storage disease and Wolman disease: phenotypic variants of lysosomal acid cholesteryl ester hydrolase deficiency","volume":"36","author":"Hoeg","year":"1984","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.jhep.2013.04.016_b0130","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1258\/0004563001899195","article-title":"Accumulated lipids, aberrant fatty acid composition and defective cholesterol ester hydrolase activity in cholesterol ester storage disease","volume":"37","author":"Todoroki","year":"2000","journal-title":"Ann Clin Biochem"},{"issue":"Suppl. 2","key":"10.1016\/j.jhep.2013.04.016_b0170","first-page":"E20","article-title":"Efficacy of SBC-102, a recombinant enzyme replacement therapy, across a broad range of doses in an in vivo model of lysosomal acid lipase deficiency","volume":"52","author":"Leavitt","year":"2011","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"10.1016\/j.jhep.2013.04.016_b0140","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1111\/j.1749-6632.2000.tb06417.x","article-title":"Observation of intramyocellular lipids by 1H-magnetic resonance spectroscopy","volume":"904","author":"Boesch","year":"2000","journal-title":"Ann N Y Acad Sci"},{"key":"10.1016\/j.jhep.2013.04.016_b0145","doi-asserted-by":"crossref","first-page":"658","DOI":"10.2214\/ajr.184.2.01840658","article-title":"MRI of fat distribution in a mouse model of lysosomal acid lipase deficiency","volume":"184","author":"Du","year":"2005","journal-title":"AJR Am J Roentgenol"},{"key":"10.1016\/j.jhep.2013.04.016_b0150","doi-asserted-by":"crossref","first-page":"1278","DOI":"10.1136\/gut.2011.242073","article-title":"Resistance exercise reduces liver fat and its mediators in non-alcoholic fatty liver disease independent of weight loss","volume":"60","author":"Hallsworth","year":"2011","journal-title":"Gut"},{"key":"10.1016\/j.jhep.2013.04.016_b0155","doi-asserted-by":"crossref","first-page":"S358","DOI":"10.1016\/S0168-8278(11)60902-1","article-title":"Recombinant lysosomal acid lipase normalizes liver weight, transaminases and histopathological abnormalities in an in vivo model of cholesterol ester storage disease","volume":"54","author":"Leavitt","year":"2011","journal-title":"J Hepatol"},{"key":"10.1016\/j.jhep.2013.04.016_b0160","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1002\/hep.22363","article-title":"Dietary cholesterol, rather than liver steatosis, leads to hepatic inflammation in hyperlipidemic mouse models of nonalcoholic steatohepatitis","volume":"48","author":"Wouters","year":"2008","journal-title":"Hepatology"},{"key":"10.1016\/j.jhep.2013.04.016_b0165","doi-asserted-by":"crossref","first-page":"e10","DOI":"10.1053\/j.gastro.2011.09.049","article-title":"A high-cholesterol diet exacerbates liver fibrosis in mice via accumulation of free cholesterol in hepatic stellate cells","volume":"142","author":"Teratani","year":"2012","journal-title":"Gastroenterology"}],"container-title":["Journal of Hepatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0168827813002705?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0168827813002705?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2022,2,14]],"date-time":"2022-02-14T20:55:26Z","timestamp":1644872126000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0168827813002705"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,9]]},"references-count":33,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2013,9]]}},"alternative-id":["S0168827813002705"],"URL":"https:\/\/doi.org\/10.1016\/j.jhep.2013.04.016","relation":{},"ISSN":["0168-8278"],"issn-type":[{"value":"0168-8278","type":"print"}],"subject":[],"published":{"date-parts":[[2013,9]]}}}