Semi-Fixed Lip Bumper in Lesch-Nyhan Syndrome: An Interim Treatment Modality

Junhee Lee; Eungyung Lee; Shin, Jong-Hyun; Shin Kim; JEONG,TAE-SUNG

doi:10.5933/JKAPD.2020.47.1.93

KJC Korea
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Journal of the Korean academy of pediatric dentistry 2021 KCI Impact Factor : 0.3

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pISSN : 1226-8496 / eISSN : 2288-3819

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Semi-Fixed Lip Bumper in Lesch-Nyhan Syndrome: An Interim Treatment Modality

Journal of the Korean academy of pediatric dentistry
Abbr : J Korean Acad Pediatr Dent
2020, 47(1), pp.93-98
DOI : 10.5933/JKAPD.2020.47.1.93

Publisher : The Korean Academy Of Pediatric Dentistry
Research Area : Dentistry

Received : September 16, 2019
Accepted : November 4, 2019
Published : February 29, 2020

Junhee Lee ¹, Eungyung Lee ¹, Shin, Jong-Hyun ², Shin Kim ², JEONG,TAE-SUNG ²

¹부산대학교 치의학전문대학원 소아치과학교실
²부산대학교

ABSTRACT
열림/닫힘 버튼

Lesch-Nyhan syndrome is a rare X-linked, recessively inherited disorder of purine metabolism, caused by complete absence of the enzyme hypoxanthine-guanine phosphoribosyl transferase. This syndrome is characterized by 3 major features: neurological dysfunction, hyperuricemia, and cognitive and behavioral disturbances (e.g., self-mutilation, which begins at 2 to 3 years of age). Uncontrollable self-mutilation begins with biting of the perioral tissues and extends into patterns such as finger biting and head hitting. This report describes the case of a 31-month-old boy who was diagnosed with Lesch-Nyhan syndrome with severe lip injuries caused by self-mutilative behaviors. The behaviors were blocked with a semi-fixed lip bumper for a short period. The device was applied to the patient on the day of the visit without the requirement for an oral impression. It was easy to manage oral hygiene and adjust the device because it was detachable by clinicians and guardians. Therefore, a semi-fixed lip bumper may be useful as an interim appliance to block selfmutilative behaviors in children with Lesch-Nyhan syndrome.
KEYWORDS
열림/닫힘 버튼

Lesch-Nyhan syndrome, Self-mutilation, Lip bumper, Oral hygiene

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* 2021년 이후 발행 논문의 참고문헌은 현재 구축 중입니다.

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