Oral manifestations of patients with Kenny-Caffey Syndrome.

Abstract

Kenny-Caffey syndrome (KCS) is a rare osteosclerotic bone dysplasia characterized by hypocalcemia, short stature, ophthalmological features, and teeth anomalies. The TBCE gene coding for a tubulin-specific chaperone E, is located at chromosome 1q42-q43, and is responsible for the recessive form. After reviewing the literature, we found around 60 cases, however with limited dental data. In this article 5 new individuals with KCS, are described focusing on oral findings. All cases had short roots and showed dental anomalies as hypo/oligodontia, microdontia. Dental anomalies are a constant feature in KCS, further study is required to better delineate the syndrome.

• Authors: Youssra Moussaid, Didier Griffiths, Béatrice Richard, Anne Dieux, Martine Lemerrer, Juliane Léger, Didier Lacombe, Isabelle Bailleul-Forestier
• Journal: European journal of medical genetics (Eur J Med Genet) Vol. 55 Issue 8-9 Pg. 441-5 ( 2012) ISSN: 1878-0849 [Electronic] Netherlands
• PMID: 22522175 (Publication Type: Journal Article, Review)
• Copyright: Copyright © 2012 Elsevier Masson SAS. All rights reserved.

Topics

• Abnormalities, Multiple (diagnostic imaging, genetics)
• Dwarfism (diagnostic imaging, genetics)
• Foot Deformities, Congenital (diagnostic imaging, genetics)
• Humans
• Hyperostosis, Cortical, Congenital (diagnostic imaging, genetics)
• Hypocalcemia (diagnostic imaging, genetics)
• Phenotype
• Radiography
• Tooth Abnormalities (diagnostic imaging, genetics)