Cleidocranial dysplasia: a review of the dental, historical, and practical implications with an overview of the South African experience
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Date
2013
Journal Title
Journal ISSN
Volume Title
Publisher
Elsevier
Abstract
Cleidocranial dysplasia (CCD) is an uncommon but well-known genetic skeletal condition. Several hundred affected
persons are members of a large extended family in the Cape Town Mixed Ancestry community of South Africa. The clinical
manifestations are often innocuous, but hyperdontia and other developmental abnormalities of the teeth are a major feature
and may require special dental management.
Over the past 40 years, the authors have encountered more than 100 affected persons in Cape Town. Emphasis has
been on dental management, but medical, genetic, and social problems have also been addressed. In this article, we have
reviewed the manifestations of the disorder in the light of our own experience, and performed a literature search with
emphasis on the various approaches to dental management and treatment options in CCD. Advances in the understanding of
the biomolecular pathogenesis of CCD are outlined and the international and local history of the disorder is documented.
Description
Keywords
Cleidocranial dysplasia (CCD), Genetic skeletal condition, Hyperdontia, Disorder, Biomolecular pathogenesis
Citation
Roberts, T. et al. (2013). Cleidocranial dysplasia: a review of the dental, historical, and practical implications with an overview of the South African experience. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, 115(1): 46 – 55