Liver and intrahepatic bile ducts-nontumor
Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 30 April 2012, last major update April 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.
● Deficient activity of lysosomal acid lipase (LAL) results in massive accumulation of cholesteryl esters and triglycerides in most tissues of the body
● Occurs in infancy and is nearly always fatal before the age of 1 year
● Heterozygous frequency of 3% in those of Iranian-Jewish ancestry in Los Angeles, California (Genet Test Mol Biomarkers 2011;15:395)
● Foamy Kupffer cells and hepatocytes
Fibrosis and Kuppfer cells
Electron microscopy description
● Lipid droplets in hepatocytes and Kupffer cells
● Cholesterol clefts in Kupffer cells
End of Liver and intrahepatic bile ducts-nontumor > Metabolic diseases > Wolman disease
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