반 고정식 립 범퍼를 이용한 Lesch-Nyhan 증후군 환아의 자해습관 차단
Semi-Fixed Lip Bumper in Lesch-Nyhan Syndrome: An Interim Treatment Modality
이준희, 이은경, 신종현, 김신, 정태성,
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이준희 ( Lee Jun-Hee ) - Pusan National University School of Dentistry Department of Pediatric Dentistry
이은경 ( Lee Eun-Gyung ) - Pusan National University School of Dentistry Department of Pediatric Dentistry
신종현 ( Shin Jong-Hyun ) - Pusan National University School of Dentistry Department of Pediatric Dentistry
김신 ( Kim Shin ) - Pusan National University School of Dentistry Department of Pediatric Dentistry
정태성 ( Jeong Tae-Sung ) - Pusan National University School of Dentistry Department of Pediatric Dentistry
Abstract
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.
키워드
Lesch-Nyhan syndrome; Self-mutilation; Lip bumper; Oral hygiene
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