Liver and intrahepatic bile ducts - nontumor
Metabolic diseases
Zellweger syndrome

Topic Completed: 1 April 2012

Revised: 9 March 2020

Copyright: 2002-2020,, Inc.

PubMed Search: Liver Zellweger syndrome[TI]

Komal Arora, M.D.
Page views in 2019: 155
Page views in 2020 to date: 30
Cite this page: Arora K. Zellweger syndrome. website. Accessed March 31st, 2020.
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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