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 20 Number 1
- 발행연도
- 2024.6
- 수록면
- 31 - 39 (9page)
이용수
초록· 키워드
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Lesch-Nyhan syndrome (LNS) is a rare X-linked genetic condition resulting from an inherited disorder of purine metabolism. The absence of the hypoxanthine-guanine phosphoribosyl transferase enzyme leads to hyperuricemia, neurological symptoms, self-injury behavior, and hematological abnormalities. This case report presents a 31-month-old boy diagnosed with LNS who was referred owing to lower lip injuries. To mitigate self-injury behavior, a mouth guard was installed in the upper jaw, and a semi-fixed lip bumper was installed in the lower jaw. Digital dentistry techniques including intraoral scanning, 3D-printing of models, and undercut formation, were employed. Every six months, a new appliance was fitted to enhance retention. Maxillary mouth guard was worn for 24 hours, and a mandibular semi-fixed lip bumper was worn continuously except for 3 to 4 hours of eating time. Over the 4 year retention period, a conservative approach preserved the primary teeth and protected oral tissues. However, prolonged use of the mandibular lip bumper resulted in buccal and distal movement of the second mandibular primary molar, leading to left mandibular first molar locking. After gingivectomy, the appliance was replaced with a removable lip bumper that does not involve secondary primary molars. In summary, it should be borne in mind that the use of a lip bumper can result in secondary primary molar distalization, resulting in inevitable permanent molar impaction. So, appropriate device changes during the transition to mixed dentition appear to be a favorable treatment option. Treatment should vary depending on the LNS phenotype.
목차
Introduction
Case Report
Discussion
Summary
References
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