Striated adrenocortical cells in cerebro-hepato-renal (Zellweger) syndrome.
Adrenal glands from eight patients with the cerebro-hepato-renal 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.
D000302:Adrenal Cortex / Q000473:pathology* / Q000648:ultrastructure; D000326:Adrenoleukodystrophy / Q000473:pathology; D001921:Brain / Q000002:abnormalities*; D006801:Humans; D007223:Infant; D008107:Liver Diseases / Q000151:congenital*; D008854:Microscopy, Electron; D007690:Polycystic Kidney Diseases / Q000473:pathology*; D013577:Syndrome