Liver and intrahepatic bile ducts-nontumor
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.
● Caused by a deficiency of aldolase B activity in the liver and kidney (J Inherit Metab Dis 2010;33:715)
● Hepatocellular degeneration with focal fatty change, particularly in regenerative nodules; pseudorosettes, cholestasis, fibrosis, cirrhosis
Macrovesicular fatty changes with periportal and perivenular fibrosis, suggestive of metabolic liver disease
Electron microscopy description
● Lucent partially membrane bound areas of cytoplasm (“fructose holes”), concentric arrays of endoplasmic reticulum and glycogen
Electron microscopy images
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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