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Endosteal Hyperostosis

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Sclerosing Bone Dysplasias

Abstract

The nosology of this condition is confusing, and the designations ‘van Buchem disease’ and ‘hyperostosis corticalis’ are better known than the term ‘endosteal hyperostosis (van Buchem and other forms)’ which was used in the 1970 Paris Nomenclature. In the 1977 version of the Nomenclature, the condition was subdivided into autosomal dominant (Worth) and autosomal recessive (van Buchem) types, as shown below:

Endosteal Hyperostosis

 

1. Autosomal dominant (Worth)

Autosomal dominant osteosclerosis

2. Autosomal recessive (van Buchem)

Hyperostosis corticalis generalisata familiaris, or generalised cortical hyperostosis

It must be emphasised that clinical and radiographic differentiation of these two genetic conditions may be impossible; for this reason they are considered together in this chapter.

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© 1980 Springer-Verlag Berlin Heidelberg

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Beighton, P., Cremin, B.J. (1980). Endosteal Hyperostosis. In: Sclerosing Bone Dysplasias. Springer, London. https://doi.org/10.1007/978-1-4471-1292-1_13

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  • DOI: https://doi.org/10.1007/978-1-4471-1292-1_13

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-1294-5

  • Online ISBN: 978-1-4471-1292-1

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