Virchows Archiv A

, Volume 416, Issue 3, pp 255–264

Pathology of hepatic peroxisomes and mitochondria in patients with peroxisomal disorders

Authors

  • J. L. Hughes
    • Departments of Histopathology and Chemical PathologyAdelaide Children's Hospital
  • A. Poulos
    • Departments of Histopathology and Chemical PathologyAdelaide Children's Hospital
  • E. Robertson
    • Departments of Histopathology and Chemical PathologyAdelaide Children's Hospital
  • C. W. Chow
    • Department of Anatomical Pathology and Murdoch InstituteRoyal Children's Hospital
  • L. J. Sheffield
    • Department of Anatomical Pathology and Murdoch InstituteRoyal Children's Hospital
  • J. Christodoulou
    • Department of Anatomical Pathology and Murdoch InstituteRoyal Children's Hospital
  • R. F. Carter
    • Departments of Histopathology and Chemical PathologyAdelaide Children's Hospital
Case Report

DOI: 10.1007/BF01678985

Cite this article as:
Hughes, J.L., Poulos, A., Robertson, E. et al. Vichows Archiv A Pathol Anat (1990) 416: 255. doi:10.1007/BF01678985

Summary

The morphology of hepatic peroxisomes in five patients with metabolic disorders believed to be due to inherited defects of peroxisomal function or biogenesis is described. Electron microscopy and cytochemical staining for catalase were used to identify peroxisomes in two boys with infantile Refsum's disease (IRD), a girl with autopsy confirmed neonatal adrenoleukodystrophy (NALD), and two boys with pseudo-Zellweger syndrome (PZS). In the patients with IRD and NALD hepatic peroxisomes were significantly reduced in size and number and contained electron dense centres. In the liver of the patients with PZS the peroxisomes were enlarged. Morphologically abnormal peroxisomes were also detected in autopsy tissue from one boy with PZS using electron microscopy. Lamellar-lipid inclusions and mitochondria with crystalline inclusions and/or abnormal cristae are also described in two patients, one with IRD, the other with NALD.

Key words

PeroxisomesPeroxisomal disordersNeonatal adrenoleukodystrophyInfantile Refsum's diseasePseudo-Zellweger diseaseMitochondria

Copyright information

© Springer-Verlag 1990