Virchows Archiv A

, Volume 401, Issue 3, pp 355–361 | Cite as

Striated adrenocortical cells in cerebro-hepato-renal (Zellweger) syndrome

  • Sidney Goldfischer
  • James M. Powers
  • Anne B. Johnson
  • Steven Axe
  • Frank R. Brown
  • Hugo W. Moser
Article

Summary

Adrenal glands from eight patients with the cerebro-hepatorenal syndrome, a disease in which there are no morphologically demonstrable peroxisomes, were studied histologically; one of the eight was also examined ultrastructurally. Seven of the eight demonstrated striated adrenocortical cells in the inner portion of the adrenal cortex. Ultrastructural examination confirmed that the striated cells contained the lammellae and lamellar-lipid profiles of very long chain fatty acids-cholesterol esters that are characteristic of adreno-leukodystrophy. This morphologic observation further emphasizes the common pathogenetic features of the cerebro-hepato-renal (Zellweger) syndrome and adreno-leukodystrophy.

Key words

Cerebro-hepato-renal syndrome Peroxisomes Fatty Acids Adreno-leukodystrophy Adrenal Cortex Zellweger Schilder 

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Copyright information

© Springer-Verlag 1983

Authors and Affiliations

  • Sidney Goldfischer
    • 1
  • James M. Powers
    • 2
  • Anne B. Johnson
    • 1
  • Steven Axe
    • 3
  • Frank R. Brown
    • 3
  • Hugo W. Moser
    • 3
  1. 1.Department of Pathology and The Rose F. Kennedy Center for ResearchMental Retardation and Human Development Albert Einstein College of MedicineBronxUSA
  2. 2.Department of PathologyMedical University of South CarolinaCharleston
  3. 3.Departments of PathologyPediatrics and Neurology The John Hopkins Medical Institutions and John F. Kennedy InstituteBronxUSA

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