지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2013.10
- 수록면
- 248 - 260 (13page)
이용수
초록· 키워드
오류제보하기
Cleidocranial dysplasia (CCD), an autosomal dominant disorder with a prevalence of 1 in 1,000,000 individuals, is mainly caused by mutations in Runx2 , a gene required for osteoblastic differentiation. It is generally characterized by hypoplastic clavicles, narrow thorax, and delayed or absent fontanel closure. Importantly, its orofacial manifestations, including midfacial hypoplasia, retained primary teeth, and impacted permanent and supernumerary teeth, severely impede the well-being of affected individuals. Successful treatment of the orofacial problems requires the combined efforts of dental specialists. However, only a few successfully treated cases have been reported because of the rarity of CCD and complexity of the treatment. This article presents the University of California, San Francisco (UCSF) treatment protocol for the dentofacial manifestations of CCD based on two treated and 17 diagnosed cases. The records of two patients with CCD who had been treated at the UCSF School of Dentistry and the treatment options reported in the literature were reviewed. The UCSF treatment protocol produced a successful case and a partially successful one (inadequate oral hygiene in the retention stage resulted in decay and loss of teeth). It provides general guidelines for successfully treating the orofacial manifestations of CCD.
목차
INTRODUCTION
CASE REPORT
DISCUSSION
CONCLUSION
REFERENCES
참고문헌 (14)
참고문헌 신청
1. [학술지(정기간행물)] - Ishii K / 1998 / Characteristics of jaw growth in cleidocranial dysplasia / Cleft Palate Craniofac J 35 : 161 ~ 166
2. [학술지(정기간행물)] - Lou Y / 2009 / A Runx2 threshold for the cleidocranial dysplasia phenotype / Hum Mol Genet 18 : 556 ~ 568
3. [학술지(정기간행물)] - Mundlos S / 1997 / Mutations involving the transcription factor CBFA1 cause cleidocranial dysplasia / Cell 89 : 773 ~ 779
4. [학술지(정기간행물)] - Ducy P / 1997 / Osf2/Cbfa1: a transcriptional activator of osteoblast differentiation / Cell 89 : 747 ~ 754
5. [학술지(정기간행물)] - Baumert U / 2005 / Cleidocranial dysplasia: molecular genetic analysis and phenotypic-based description of a Middle European patient group / Am J Med Genet A 139A : 78 ~ 85
2. [학술지(정기간행물)] - Lou Y / 2009 / A Runx2 threshold for the cleidocranial dysplasia phenotype / Hum Mol Genet 18 : 556 ~ 568
3. [학술지(정기간행물)] - Mundlos S / 1997 / Mutations involving the transcription factor CBFA1 cause cleidocranial dysplasia / Cell 89 : 773 ~ 779
4. [학술지(정기간행물)] - Ducy P / 1997 / Osf2/Cbfa1: a transcriptional activator of osteoblast differentiation / Cell 89 : 747 ~ 754
5. [학술지(정기간행물)] - Baumert U / 2005 / Cleidocranial dysplasia: molecular genetic analysis and phenotypic-based description of a Middle European patient group / Am J Med Genet A 139A : 78 ~ 85
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UCI(KEPA) : I410-ECN-0101-2014-510-002719493