title: 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.affiliation: Paediatric Dentistry, Garancière Paris Diderot University, France.
authors.forename: Youssra
authors.initials: Y
authors.lastname: Moussaid
authors.forename: Didier
authors.initials: D
authors.lastname: Griffiths
authors.forename: Béatrice
authors.initials: B
authors.lastname: Richard
authors.forename: Anne
authors.initials: A
authors.lastname: Dieux
authors.forename: Martine
authors.initials: M
authors.lastname: Lemerrer
authors.forename: Juliane
authors.initials: J
authors.lastname: Léger
authors.forename: Didier
authors.initials: D
authors.lastname: Lacombe
authors.forename: Isabelle
authors.initials: I
authors.lastname: Bailleul-Forestier
mesh_terms: D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000235:genetics; D004392:Dwarfism / Q000000981:diagnostic imaging / Q000235:genetics; D005532:Foot Deformities, Congenital / Q000000981:diagnostic imaging / Q000235:genetics; D006801:Humans; D006958:Hyperostosis, Cortical, Congenital / Q000000981:diagnostic imaging / Q000235:genetics; D006996:Hypocalcemia / Q000000981:diagnostic imaging / Q000235:genetics; D010641:Phenotype; D011859:Radiography; D014071:Tooth Abnormalities / Q000000981:diagnostic imaging / Q000235:genetics