Hepatic cholesteryl ester accumulation in lysosomal acid lipase deficiency: Non-invasive identification and treatment monitoring by magnetic resonance

Journal of Hepatology

Volumn 59, Issue 3, 2013, Pages 543-549

  Thelwall, Peter E ^a   Smith, Fiona E ^a   Leavitt, Mark C ^b   Canty, David ^b   Hu, Wei ^b   Hollingsworth, Kieren G ^a   Thoma, Christian ^a   Trenell, Michael I ^a   Taylor, Roy ^a   Rutkowski, Joseph V ^b   Blamire, Andrew M ^a   Quinn, Anthony G ^b  

^a NEWCASTLE UNIVERSITY   (United Kingdom)

^b Synageva BioPharma Corp   (United States)

Author keywords

13C MR spectroscopy; 1H MR spectroscopy; Cholesteryl ester storage disease; Enzyme replacement therapy; LAL deficiency; LIPA; Liver fat; Lysosomal acid lipase; Sebelipase alfa; Wolman disease

Indexed keywords

ACID LIPASE; CHOLESTEROL; CHOLESTEROL ESTER; SEBELIPASE ALFA; TRIACYLGLYCEROL;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CASE REPORT; CHOLESTEROL ESTER STORAGE DISEASE; CHOLESTEROL LIVER LEVEL; CONTROLLED STUDY; DRUG MONITORING; ENZYME REPLACEMENT; HUMAN; LIPID COMPOSITION; LIPID LIVER LEVEL; LIPID STORAGE; LIVER; LYSOSOMAL ACID LIPASE DEFICIENCY; LYSOSOME; MALE; NON INVASIVE PROCEDURE; NONALCOHOLIC FATTY LIVER; NONHUMAN; NUCLEAR MAGNETIC RESONANCE SCANNER; PRIORITY JOURNAL; PROTON NUCLEAR MAGNETIC RESONANCE; RAT; SPECTROMETER;

(1)H MR SPECTROSCOPY; (13)C MR SPECTROSCOPY; CE; CESD; CHOLESTERYL ESTER; CHOLESTERYL ESTER STORAGE DISEASE; ENZYME REPLACEMENT THERAPY; ERT; LAL; LAL DEFICIENCY; LIPA; LIVER FAT; LYSOSOMAL ACID LIPASE; NAFLD; NON-ALCOHOLIC FATTY LIVER DISEASE; SEBELIPASE ALFA; TG; TRIGLYCERIDE; WOLMAN DISEASE;

ANIMALS; CHOLESTEROL ESTERS; DISEASE MODELS, ANIMAL; FATTY ACIDS; FATTY LIVER; HUMANS; LIVER; MAGNETIC RESONANCE SPECTROSCOPY; MALE; PROSPECTIVE STUDIES; RATS; RATS, SPRAGUE-DAWLEY; RATS, TRANSGENIC; RECOMBINANT PROTEINS; STEROL ESTERASE; TRIGLYCERIDES; WOLMAN DISEASE;

EID: 84882862828     PISSN: 01688278     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.jhep.2013.04.016     Document Type: Article

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