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  • Title: Clinical and radiographic analysis of osteochondromas and growth disturbance in hereditary multiple exostoses.
    Author: Porter DE, Emerton ME, Villanueva-Lopez F, Simpson AH.
    Journal: J Pediatr Orthop; 2000; 20(2):246-50. PubMed ID: 10739291.
    Abstract:
    Hereditary multiple exostoses (HME) is traditionally described as a skeletal dysplasia. However, the discovery that the EXT family of tumour suppressor genes are responsible for HME suggests that it is more appropriate to classify HME as a familial neoplastic trait. In a clinical and radiographic analysis of paired bone length and exostoses number and dimensions in a HME cohort, the local presence of osteochondromas was consistently associated with growth disturbance. In particular, an inverse correlation between osteochondroma size and relative bone length (p<0.01) was found. These data suggest that the growth retardation in HME may result from the local effects of enlarging osteochondromas rather than a skeletal dysplasia effect. This study provides the first clinical rationale for ablation of rapidly enlarging exostoses to reduce growth disturbance.
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