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Liver and intrahepatic bile ducts - nontumor
Metabolic diseases
Zellweger syndrome
Revised: 23 October 2019, last major update April 2012
Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.
PubMed Search: Liver Zellweger syndrome[TI]
Metabolic diseases
Zellweger syndrome
Author: Komal Arora, M.D.
Topic Completed: 1 April 2012
Revised: 23 October 2019, last major update April 2012
Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.
PubMed Search: Liver Zellweger syndrome[TI]
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Table of Contents
Definition / general | Microscopic (histologic) description | Electron microscopy description | Electron microscopy images | Additional referencesCite this page: Arora K. Zellweger syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/liverzellweger.html. Accessed September 22nd, 2019.
Definition / general
- Defect peroxisomes, causes accumulation of very long chain fatty acids (VLCFA)
- Rare, occurs in 1 per 50,000 - 100,000 births
Microscopic (histologic) description
- Nonspecific changes of hepatocyte degeneration
Electron microscopy description
- No peroxisomes
Electron microscopy images
Images hosted on other servers:
Severe parenchymatic
destruction and
cholestasis; no
peroxisomes present
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