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Biomedical subjects

R Silberberg

Publications and source records attributed to R Silberberg.

75 records · Page 5Linked to original sources

Metatropic dwarfism, the Kniest syndrome and the pseudoachondroplastic dysplasias.

Metatropic dwarfism, the Kniest syndrome, and pseudoachondroplastic dysplasia are specific chondrodystrophic disorders that have in common dysplasia of the metaphyses, epiphyses, and vertebrae. Metatropic dwarfism and the Kniest syndrome have been confused with Morquio's disease and with each other in the past, but can be easily distinguished on the basis of radiographic features, clinical features, and cartilage pathology. The pseudoachondroplastic dysplasias are associated with short limbed dwarfism and have been confused with achondroplastic dwarfism, but can be readily distinguished on the basis of the normal head, delayed age of onset, the typical radiographic features and the cartilage pathology.

Cartilage↗

Chondro-osseous pathology in the chondrodystrophies.

The majority of chondrodystrophies are associated with distinct abnormalities in chondro-osseous histopathology. In some disorders, the pathological abnormalities are characteristic and can be used as diagnostic criteria, in other instances the morphological abnormalities are not specific, and in still others, no abnormality in chondro-osseous morphology is present. In this paper, the chondro-osseous histopathology and ultrastructure of a number of chondrodystrophies are reviewed to illustrate the different pathogenetic mechanisms involved in each of these disorders.

Achondroplasia↗