| Autor: |
Silberberg, R, Kaufman, R L, Rosenthal, R, Geme, J W St |
| Zdroj: |
Pediatric Research; August 1971, Vol. 5 Issue: 8 p416-416, 1p |
| Abstrakt: |
The chondrodystrophies are a heterogeneous group of disorders which have been classified on the basis of clinical, radiographic and genetic criteria. Histopathological studies of costochondral junction and iliac crest biopsies have allowed for the further classification of these disorders on the basis of the type of derangement in endochondral ossification. Electron microscopic stuides of resting cartilage from these biopsy specimens demonstrate that the chondrodystrophies may be further characterized on the basis of ultrastructural abnormalities in the chondrocytes or intercellular matrix.For examples in achondroplasia, a disease associated with normal endochondral ossification, no ultrastructural abnormalities are present in either the chondrocyte or the matrix. In the mucopolysaccharidoses, the matix is ultrastructurally normal, but the chondrocytes are filled with large cytoplasmic vacuoles of possible lysosomal origin. These vacuoles are uniform in appearance in the Hurler syndrome whereas in the Sanfilippo syndrome, two distinct populations of vacuoles can be identified. These ultrastructural observations provide a further clue as to the specific pathogentic mechanisms operative in the bone dysplasias. |
| Databáze: |
Supplemental Index |
| Externí odkaz: |
|
