Liver and intrahepatic bile ducts-nontumor

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Liver and intrahepatic bile ducts-nontumor

Metabolic diseases

Zellweger syndrome

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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● Defect peroxisomes, causes accumulation of very long chain fatty acids (VLCFA)
● Rare, occurs in 1 per 50-100,000 births

Micro description
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● Nonspecific changes of hepatocyte degeneration

Electron microscopy description
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● No peroxisomes

Electron microscopy images
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Severe parenchymatic destruction and cholestasis; no peroxisomes present

Additional references
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● 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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