par Tondeur, Marianne 
;Loeb, Helmut
Référence Pediatric research, 3, 1, page (19-26)
Publication Publié, 1969
;Loeb, Helmut Référence Pediatric research, 3, 1, page (19-26)
Publication Publié, 1969
Article révisé par les pairs
| Résumé : | Extract: Liver biopsies from two children with Morquio’s disease were studied by electron microscopy. The diagnosis was based upon typical clinical findings and x-ray examination. Urinary excretion of keratosulfate was abnormally high in both cases. The Kupffer cells contained large relatively electron-lucid inclusions (0.3 to 4.3 μ in diameter) bounded by a single membrane and containing a finely arranged protein-like precipitate. These inclusions occasionally appeared as compact bodies (figs. 2 and 3). Membranous 'myelin-like' round bodies were infrequently seen in the vacuoles. Hepatocytes were usually normal, but some contained a few electron-lucid inclusions that resembled those seen in the Kupffer cells, although they were smaller in size (0.4 to 1.9 μ) (fig. 5). Peribiliary dense bodies were numerous and rarely contained myelin-like figures (fig. 4). In some atypical groups of cells, discontinuous limiting membranes were observed. These cells also contained electron-lucid vacuoles bounded by single membranes of varying sizes (0.3 to 4.0 μ) (fig. 6). The inclusions were very similar to those previously described in different forms of gargoylism, although the site was different. The rupture of some Kupffer and/or parenchymal cell membranes, secondary to technical manipulations, apparently caused the peculiar aspect of the atypical groups of cells. Speculation: This study supplies new evidence linking Morquio’s disease and gargoylism. Some pathogenic mechanisms appear to be similar in these diseases, both of which affect mucopolysaccharides of the liver in a similar way. © International Pediatrics Research Foundation, Inc. 1969. All Rights Reserved. |
