Liver and intrahepatic bile ducts - nontumor
Metabolic diseases
Zellweger syndrome



Topic Completed: 1 April 2012

Revised: 23 October 2019, last major update April 2012

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PubMed Search: Liver Zellweger syndrome[TI]


Komal Arora, M.D.
Page views in 2018: 135
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Cite this page: Arora K. Zellweger syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/liverzellweger.html. Accessed July 17th, 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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