Liver and intrahepatic bile ducts - nontumor
Metabolic diseases
Zellweger syndrome

Author: Komal Arora, M.D. (see Authors page)

Revised: 27 October 2017, last major update April 2012

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Liver Zellweger syndrome[TI]

Cite this page: Arora, K. Zellweger syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/liverzellweger.html. Accessed December 14th, 2017.
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