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Liver and intrahepatic bile ducts-nontumor

Metabolic diseases

Wolman disease


Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 30 April 2012, last major update April 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.

General
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● 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)

Micro description
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● Foamy Kupffer cells and hepatocytes

Micro images
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Fibrosis and Kuppfer cells

Electron microscopy description
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● 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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