Document Detail


Craniofacial and dental characteristics of Kabuki syndrome: nine years cephalometric follow-up.
MedLine Citation:
PMID:  23019839     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
OBJECTIVE: Kabuki syndrome is a rare autosomal dominant trait with variable clinical expression. Common dental manifestations of Kabuki syndrome are high-arched palate, micrognathia, malocclusion, microdontia, small dental arches, hypodontia, severe maxillary recession and mid-facial hypoplasia.
STUDY DESIGN: This report includes the oral manifestations of a Turkish patient with Kabuki syndrome with consideration of the long-term craniofacial prognosis for this patient based on the physical, clinical and radiological findings in 9 years follow-up period. General appearance of the patient was characterized by postnatal growth retardation, moderate mental retardation, peculiar face characterized by long palpebral fissures with eversion of the lateral third of the lower eyelids, prominent and cup-shaped ears, broad and depressed nasal tips, short fifth fingers, psychomotor retardation and dermatologic abnormalities.
RESULTS AND CONCLUSION: Cephalometric analysis revealed skeletal open bite; periapical and panoramic radiographic examinations showed agenesis permanent teeth. A patient with Kabuki syndrome, who may lead to a better understanding of the abnormalities, playing an important role in clinical diagnosis, planning and dental management is presented.
Authors:
Elif Bahar Tuna; Gülnaz Marşan; Koray Gençay; Figen Seymen
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of clinical pediatric dentistry     Volume:  36     ISSN:  1053-4628     ISO Abbreviation:  J Clin Pediatr Dent     Publication Date:  2012  
Date Detail:
Created Date:  2012-10-01     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9100079     Medline TA:  J Clin Pediatr Dent     Country:  United States    
Other Details:
Languages:  eng     Pagination:  393-400     Citation Subset:  D    
Affiliation:
Department of Pedodontics, Istanbul University, Faculty of Dentistry, Istanbul, Turkey. ebtuna@istanbul.edu.tr
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