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Liver and intrahepatic bile ducts-nontumor

Metabolic diseases

Hereditary fructose intolerance


Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 24 April 2012, last major update April 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.

General
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● Caused by a deficiency of aldolase B activity in the liver and kidney (J Inherit Metab Dis 2010;33:715)

Diagrams
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Fructose metabolism

Micro description
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● Hepatocellular degeneration with focal fatty change, particularly in regenerative nodules; pseudorosettes, cholestasis, fibrosis, cirrhosis

Micro images
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Macrovesicular fatty changes with periportal and perivenular fibrosis, suggestive of metabolic liver disease

Electron microscopy description
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● Lucent partially membrane bound areas of cytoplasm (“fructose holes”), concentric arrays of endoplasmic reticulum and glycogen

Electron microscopy images
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Microvesicular fat droplets in hepatocyte, focal cytoplasmic necrosis

End of Liver and intrahepatic bile ducts-nontumor > Metabolic diseases > Hereditary fructose intolerance


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