The 'chef's hat' appearance of the femoral head in cleidocranial dysplasia

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dc.authorscopusid7007157201
dc.authorscopusid57225327542
dc.authorscopusid7102731102
dc.contributor.authorAktas S.
dc.contributor.authorWheeler D.
dc.contributor.authorSussman M.D.
dc.date.accessioned2024-06-12T10:25:36Z
dc.date.available2024-06-12T10:25:36Z
dc.date.issued2000
dc.description.abstractCleidocranial dysplasia (CCD) is inherited as an autosomal dominant disorder characterised by failure of membranous ossification. The condition is due to a mutation of the cbfa1 gene on chromosome 6 which has a role in the development of osteoblasts from the mesenchymal cells. In their growing years, these patients have an unusual shape of the femoral head reminiscent of a 'chef's hat'. In order to confirm the consistency of this sign, we have reviewed the radiographs of 28 patients with CCD. All except three had this appearance. The sign was also seen in patients with coxa vara associated with a variety of other conditions. The chef's hat sign may occur secondary to the particular mechanical environment created by coxa vara as well as abnormal cellular function in patients with CCD. Although coxa vara has some influence on the shape of the femoral head, it is not entirely responsible for its morphology since it was present in only six of the 28 patients with CCD.en_US
dc.identifier.doi10.1302/0301-620X.82B3.9919
dc.identifier.endpage408en_US
dc.identifier.issn0301-620X
dc.identifier.issue3en_US
dc.identifier.pmid10813178en_US
dc.identifier.scopus2-s2.0-0034015321en_US
dc.identifier.scopusqualityN/Aen_US
dc.identifier.startpage404en_US
dc.identifier.urihttps://doi.org/10.1302/0301-620X.82B3.9919
dc.identifier.urihttps://hdl.handle.net/20.500.14551/16430
dc.identifier.volume82en_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherBritish Editorial Society of Bone and Joint Surgeryen_US
dc.relation.ispartofJournal of Bone and Joint Surgery - Series Ben_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectAdolescent; Article; Autosomal Dominant Disorder; Cell Function; Child; Chromosome 6; Cleidocranial Dysplasia; Clinical Article; Clinical Feature; Controlled Study; Coxa Vara; Female; Femur Epiphysis; Femur Head; Gene Mutation; Hip Radiography; Human; Male; Morphology; Priority Journalen_US
dc.titleThe 'chef's hat' appearance of the femoral head in cleidocranial dysplasiaen_US
dc.typeArticleen_US

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