Journal of the korean academy of Pediatric Dentistry (대한소아치과학회지)

Korean Academy of Pediatric Dentistry (대한소아치과학회)
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Semi-Fixed Lip Bumper in Lesch-Nyhan Syndrome: An Interim Treatment Modality

반 고정식 립 범퍼를 이용한 Lesch-Nyhan 증후군 환아의 자해습관 차단

  • Lee, Junhee (Department of Pediatric Dentistry, School of Dentistry, Pusan National University) ;
  • Lee, Eungyung (Department of Pediatric Dentistry, School of Dentistry, Pusan National University) ;
  • Shin, Jonghyun (Department of Pediatric Dentistry, School of Dentistry, Pusan National University) ;
  • Kim, Shin (Department of Pediatric Dentistry, School of Dentistry, Pusan National University) ;
  • Jeong, Taesung (Department of Pediatric Dentistry, School of Dentistry, Pusan National University)
  • 이준희 (부산대학교 치의학전문대학원 소아치과학교실) ;
  • 이은경 (부산대학교 치의학전문대학원 소아치과학교실) ;
  • 신종현 (부산대학교 치의학전문대학원 소아치과학교실) ;
  • 김신 (부산대학교 치의학전문대학원 소아치과학교실) ;
  • 정태성 (부산대학교 치의학전문대학원 소아치과학교실)
  • Received : 2019.09.16
  • Accepted : 2019.11.04
  • Published : 2020.02.29
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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.

Lesch-Nyhan 증후군은 hypoxanthine-guanine phosphoribosyl transferase 결핍으로 인해 발생한 purine 대사 장애의 열성 유전 질환이다. 이 증후군은 신경 장애, 행동 장애 및 과요산혈증(hyperuricemia) 등의 증상이 나타난다. 특징적으로 자해 습관은 2 - 3세경 시작되는데, 구강 주위 조직이나 손, 발을 물어 뜯는 양상이 나타난다. 이 증례는 2세 7개월의 Lesch-Nyhan 증후군 환아의 자해습관을 반 고정식 립 범퍼를 이용하여 차단한 증례를 보고하는 바이다. 반 고정식 립 범퍼는 전통적인 인상채득 방식을 필요로 하지 않고 내원 당일 치료해 줄 수 있는 장점이 있으며, 보호자에 의해 탈, 부착이 가능하여 환아의 구강위생 관리에 용이하였다. 장치 장착 2 개월 후, 치아 주위 조직이 회복되고 연속성이 유지되는 양호한 결과를 보였다. 장치 장착 11개월 후, 양호한 회복양상이 유지되었으며 추가적인 외상이나 자해행동의 악화는 관찰되지 않았다. 따라서, semi-fixed lip bumper는 Lesch-Nyhan syndrome 환아의 자해습관을 차단하기 위한 interim appliance로 사용하는데 유용할 것으로 사료된다.

Keywords

References

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