IADR Abstract Archives

Analysis on Phenotypes in Family with Cleidocranial Dysplasia

Objectives:Cleidocranial dysplasia (CCD) (also known as cleidocranial dysostosis or mutational dysostosis) is an autosomal dominant skeletal dysplasia associated with clavicle, skull and facial bones hypoplasia and dental abnormalities. The present report aims to investigate the clinical and genetic features of CCD in a Chinese kindred.

Methods:A Chinese CCD family was collected and investigated by ascertainment. Comprehensive examination was performed for all available affected members. Clinical phenotypes were recorded and analyzed for the above individuals and the hereditary mode was determined in the family.

Results:A total of 7 individuals involving three generations were affected with CCD among the 19 members of the investigated Chinese family. The 4 available affected individuals exhibit typical clinical manifestations of CCD, including variable degrees of hypoplasia or aplasia of the clavicles, overdevelopment of the transverse diameter of the cranium, retardation in fontanel ossification and unerupted supernumerary permanent teeth. The disease phenotypes were transmitted in an autosomal dominant hereditary pattern in generations.

Conclusion:The pedigree here shows characteristic phenotypes and genetic mode for CCD. Early examination and proper diagnosis are of great importance for the future treatment and prevention.


Division: Asia/Pacific Region Meeting
Meeting: 2009 Asia/Pacific Region Meeting (Wuhan, China)
Location: Wuhan, China
Year: 2009
Final Presentation ID: 74
Abstract Category|Abstract Category(s): Scientific Groups
Authors
  • Zhang, Chen Zheng  ( Key Laboratory for Oral Biomedical Engineering of Ministry of Education, School and Hospital of Stomatology, Wuhan University, Wuhan, N/A, China )
  • Song, Ya Ling  ( Key Laboratory for Oral Biomedical Engineering of Ministry of Education, School and Hospital of Stomatology, Wuhan University, Wuhan, N/A, China )
  • Bian, Zhuan  ( Key Laboratory for Oral Biomedical Engineering of Ministry of Education, School and Hospital of Stomatology, Wuhan University, Wuhan, N/A, China )
  • SESSION INFORMATION
    Poster Session
    Poster Session I
    09/23/2009