hereditary multiple exostoses

(redirected from Multiple Osteochondroma)

he·red·i·tar·y mul·ti·ple ex·os·to·ses

[MIM*133700]
a disturbance of endochondral bone growth in which multiple, generally benign osteochondromas of long bones appear during childhood, commonly with shortening of the radius and fibula; the skull is not involved; the ill effects are usually mechanical but malignant change is rare; autosomal dominant inheritance with genetic heterogeneity. Type I is caused by a mutation in the exostosis-1 gene (EXT1) on 8q, type II is due to mutation in the exostosis-2 gene (EXT2) and type III maps to 19p.

hereditary multiple exostoses

a rare familial dyschondroplastic disease in which bony protuberances form on the shafts of the long bones and eventually develop into caps of cartilage covering the ends of the bones. The affected joints lose their mobility, and the bones stop growing. The disease begins in childhood and has no cure. Very rarely a chondrosarcoma may develop from the cap of an exostosis. See also Ollier's dyschondroplasia.

he·red·i·tar·y mul·ti·ple ex·os·to·ses

(hĕr-edi-tar-ē mŭlti-pĕl eksos-tōsēz)
Disturbance of endochondral bone growth in which multiple, generally benign osteochondromas of long bones appear during childhood, commonly with shortening of the radius and fibula; the skull is not involved; the ill effects are usually mechanical but malignant change is rare.

he·red·i·tar·y mul·ti·ple ex·os·to·ses

(hĕr-edi-tar-ē mŭlti-pĕl eksos-tōsēz) [MIM*133700]
Disturbance of endochondral bone growth in which multiple, generally benign osteochondromas of long bones appear during childhood, commonly with shortening of the radius and fibula. Cranium is not involved.
References in periodicals archive ?
The meshes also have potential therapeutic implications in the treatment of diseases such as multiple osteochondroma (MO), a rare disease creating bony spurs or lumps caused by abnormal production of these sugar molecules.
7) Multiple hereditary exostosis is an autosomal dominant condition associated with multiple osteochondromas and has a higher risk of malignant transformation.
If these findings are confirmed, solitary and multiple osteochondromas may be considered true neoplasms.
Chondrosarcoma may arise in osteochondroma, more commonly in patients with multiple osteochondromas.
The neoplastic pathogenesis of solitary and multiple osteochondromas.
Peripheral chondrosarcomas are associated with exostosin (EXT) genes, which cause multiple osteochondromas.
Determination of the mutation spectrum of the EXT1/EXT2 genes in British Caucasian patients with multiple osteochondromas, and exclusion of six candidate genes in EXT negative cases.
There have only been 16 cases of the hereditary bone growth disorder, now known as multiple osteochondromas, identified in ancient remains worldwide.

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