Multiple Hereditory Exostoses (Subscribe)  

Synonym: Hereditary multiple exostoses An inherited autosomal dominant disorder in which multiple osteochondromas are seen throughout the skeleton.1 The long bones of arms and legs are most commonly affected. It is associated with short stature and asymmetrical growth at the knees and ankles, which may lead to deformities.

* autosomal dominant
* M> F
* multiple exostoses
* "snowflake" calcification of mature cartilage cap
* may --> chondrosarcoma
* short metacarpals (esp. 4th + 5th)

Definition Most common of osteochondrodysplasias characterized by formation of multiple osteochondromas. Diagnostic Radiology/Musculoskeletal Imaging/Tumors Basic/Hereditary Multiple Exostoses From Wikibooks, the open-content textbooks collection

Multiple heredity exostosis is characterized by palpable osseous projections from the cortex of the bone, joint restrictions, and secondary osseous deformities. Deformities that are frequently noted include widening of the proximal femoral metaphysis, bilateral coxa valga, deviation and shortening of the ulna, and forearm shortening. This disorder is autosomal dominant...

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Multiple Exostoses Topic 2757 - Created: 2001-09-12 12:33:02-04 - Modified: 2002-10-12 15:36:44-04 ACR Index: 42.1542 -------------------------------------------------------------------------------- Osteochondromas (exostoses) are common benign bone tumors in children, with many being asymptomatic. The majority arise in childhood, and have a predilection for the metaphyses of the long bones, as seen in this case. Typically...

Multiple hereditary exostoses aka/or Multiple cartilaginous exostoses, type 1 Definitions Hereditary Multiple Exostoses: "Hereditary disorder transmitted by an autosomal dominant gene and characterized by multiple exostoses (multiple osteochondromas) near the ends of long bones. The genetic abnormality results in a defect in the osteoclastic activity at the metaphyseal ends of the bone during the remodeling...

EXOSTOSES, MULTIPLE, TYPE I Alternative titles MULTIPLE CARTILAGINOUS EXOSTOSES DIAPHYSEAL ACLASIS MULTIPLE OSTEOCHONDROMATOSIS Gene map locus 8q24.11-q24.13 multiple exostoses type I is caused by mutation in the gene encoding exostosin-1 (EXT1; 608177), which maps to chromosome 8q24. Multiple exostoses type II (133701) is caused by mutation in the gene encoding exostosin-2 (EXT2; 608210), on chromosome...

We defined the characteristics of dysplasia and coxa valga in hereditary multiple exostoses (HME) by radiological analysis of 24 hips in 12 patients. The degree and effect of the ‘osteochondroma load’ around the hip were quantified.
The data suggests that HME may cause anomalies of the hip as a reflection of a...