Document Detail

Cleidocranial dysplasia: etiology and stomatognathic and craniofacial abnormalities.
MedLine Citation:
PMID:  20357738     Owner:  NLM     Status:  MEDLINE    
Cleidocranial dysplasia (CCD) is a rare disorder which is inherited as an autosomal genetic trait. It is characterized by defective ossification, delayed bone and tooth development, stomatognathic and craniofacial abnormalities, and it is caused by mutations in the RUNX2 gene that is responsible for osteoblast differentiation. The purpose of this review is to collect and analyze data in the literature on orofacial typical manifestations of the syndrome and to present knowledge of the eziopatogenics mechanisms of the CCD. Clinical, genetic, aetiopathogenetic studies on this syndrome were compliled through a systematic approach using Medline. This review reports the cranio-facial features and dental characteristics of the CCD on the basis of all data in the literature. This review pays particular attention on the eziopatogenics mechanisms of CCD and summarises the results of the most recent studies. Access to detailed review of the etiopathogenic mechanisms of CCD is a fundamental support for clinicians as it can allow to make an informed assessment regarding the most effective choice of therapy. The review shows how an interdisciplinary approach is necessary for an appropriate treatment since CCD patients suffer from a skeletal third class, transverse deficiency of the maxilla, supernumerary permanent teeth and deficient eruption of impacted permanent teeth.
G D'Alessandro; T Tagariello; G Piana
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Minerva stomatologica     Volume:  59     ISSN:  0026-4970     ISO Abbreviation:  Minerva Stomatol     Publication Date:  2010 Mar 
Date Detail:
Created Date:  2010-04-01     Completed Date:  2010-09-16     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0421071     Medline TA:  Minerva Stomatol     Country:  Italy    
Other Details:
Languages:  eng; ita     Pagination:  117-27     Citation Subset:  D; IM    
Department of Dental Science, Dental School, Bologna University, Bologna, Italy.
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MeSH Terms
Abnormalities, Multiple*
Cleidocranial Dysplasia / complications*,  etiology*
Craniofacial Abnormalities / complications*
Stomatognathic System Abnormalities / complications*

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