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
- 저널정보
- Asia Association for Disability and Oral Health International Journal of Disability and Oral Health International Journal of Disability and Oral Health Volume 17 Number 2
- 발행연도
- 2021.12
- 수록면
- 27 - 32 (6page)
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초록· 키워드
오류제보하기
Schimke immuno-osseous Dysplasia (SIOD) is a rare multi-systemic disease caused by bi-allelic mutation of SMARCAL 1 gene encoding ATP dependent sucrose non-fermenting 2 family proteins involved in chromatin remodeling, which occurs during gene regulation, DNA replication and repair. Patients with SIOD commonly show spondyloepiphyseal dysplasia, progressive renal failure and T-cell immunodeficiency. Although the severity of the disease varies among individuals, patients with earlier onset only survive until the first decade of their life. SIOD patients exhibit unique dental features including hypodontia, microdontia and molar root hypoplasia, resulting in bell-shaped teeth. This case report describes a six year follow-up of a boy with SIOD throughout his mixed dentition stage. His dental manifestations as well as preventive dental management provided are presented. Antibiotic prophylaxis is necessary during invasive dental procedures considering the immunological status and platelet transfusion may be necessary in case of bone marrow failure.
목차
Introduction
Case report
Discussion
Conclusion
References
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