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.
● Defect peroxisomes, causes accumulation of very long chain fatty acids (VLCFA)
● Rare, occurs in 1 per 50-100,000 births
● Nonspecific changes of hepatocyte degeneration
Electron microscopy description
● No peroxisomes
Electron microscopy images
Severe parenchymatic destruction and cholestasis; no peroxisomes present
● J Inherit Metab Dis 1991;14:853, GeneReviews™ [Internet], updated 2011 Jan 18
End of Liver and intrahepatic bile ducts-nontumor > Metabolic diseases > Zellweger syndrome
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