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.
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초록· 키워드
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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.
목차
등록된 정보가 없습니다.
참고문헌 (16)
참고문헌 신청
Harris JC / 2018 / Lesch-Nyhan syndrome and its variants : examining the behavioral and neurocognitive phenotype / Curr Opin Psychiatry 31:96~102
Mak BS / 2000 / New mutations of the HPRT gene in Lesch-Nyhan syndrome / Pediatr Neurol 23:332~335
Jeong TS / 2006 / A preventive approach to oral self-mutilation in Lesch-Nyhan syndrome : a case report / Pediatr Dent 28:341~344
Kállay K / 2012 / Successful unrelated umbilical cord blood transplantation in Lesch-Nyhan syndrome / Metab Brain Dis 27:193~196
Fardi K / 2003 / Lesch-Nyhan syndrome : a preventive approach to self-mutilation / Int J Paediatr Dent 13:51~56
Mak BS / 2000 / New mutations of the HPRT gene in Lesch-Nyhan syndrome / Pediatr Neurol 23:332~335
Jeong TS / 2006 / A preventive approach to oral self-mutilation in Lesch-Nyhan syndrome : a case report / Pediatr Dent 28:341~344
Kállay K / 2012 / Successful unrelated umbilical cord blood transplantation in Lesch-Nyhan syndrome / Metab Brain Dis 27:193~196
Fardi K / 2003 / Lesch-Nyhan syndrome : a preventive approach to self-mutilation / Int J Paediatr Dent 13:51~56
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