Browse > Article
http://dx.doi.org/10.5933/JKAPD.2020.47.1.93

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

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)
Publication Information
Journal of the korean academy of Pediatric Dentistry / v.47, no.1, 2020 , pp. 93-98 More about this Journal
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.
Keywords
Lesch-Nyhan syndrome; Self-mutilation; Lip bumper; Oral hygiene;
Citations & Related Records
연도 인용수 순위
  • Reference
1 Harris JC : Lesch-Nyhan syndrome and its variants: examining the behavioral and neurocognitive phenotype. Curr Opin Psychiatry , 31:96-102, 2018.   DOI
2 Mak BS, Chi CS, Lin HY, et al.: New mutations of the HPRT gene in Lesch-Nyhan syndrome. Pediatr Neurol , 23:332-335, 2000.   DOI
3 Jeong TS, Lee JH, Tootla RG, et al.: A preventive approach to oral self-mutilation in Lesch-Nyhan syndrome: a case report. Pediatr Dent , 28:341-344, 2006.
4 Kallay K, Liptai Z, Krivan G, et al.: Successful unrelated umbilical cord blood transplantation in Lesch-Nyhan syndrome. Metab Brain Dis , 27:193-196, 2012.   DOI
5 Fardi K, Topouzelis N, Kotsanos N : Lesch-Nyhan syndrome: a preventive approach to self-mutilation. Int J Paediatr Dent , 13:51-56, 2003.   DOI
6 Saemundsson SR, Roberts MW : Oral self-injurious behavior in the developmentally disabled: review and a case. ASDC J Dent Child , 64:205-209, 1997.
7 Davila JM, Aslani MB, Wentworth E : Oral appliance attached to a bubble helmet for prevention of self-inflicted injury. ASDC J Dent Child , 63:131-134, 1996.
8 Evans J, Sirikumara M, Gregory M : Lesch-Nyhan syndrome and the lower lip guard. Oral Surg Oral Med Oral Pathol , 76:437-440, 1993.   DOI
9 Chen LR, Liu JF : Successful treatment of self-inflicted oral multilation using an acrylic splint retained by a head gear. Pediatr Dent , 18:408-410, 1996.
10 Sugahara T, Mishima K, Mori Y : Lesch-Nyhan syndrome: successful prevention of lower lip ulceration caused by self-mutilation by use of mouth guard. Int J Oral Maxillofac Surg , 23:37-38, 1994.   DOI
11 Jun IG, Chin JH, Hwang JH, et al.: Anesthetic experience of percutaneous nephrolithotomy for renal calculi in a patient with Lesch-Nyhan syndrome: a case report. Korean J Anesthesiol, 53:520-523, 2007.   DOI
12 Lesch M, Nyhan WL : A familial disorder of uric acid metabolism and central nervous system function. Am J Med , 36:561-570, 1964.   DOI
13 Mahmood A, Dubey P, Moser HW, Moser A : X-linked adrenoleukodystrophy: therapeutic approaches to distinct phenotypes. Pediatr Transplant , 9:55-62, 2005.   DOI
14 Dicks JL : Lesch-Nyhan syndrome: a treatment planning dilemma. Pediatr Dent , 4:127-130, 1982.
15 Lee JH, Berkowitz RJ, Choi BJ : Oral self-mutilation in the Lesch-Nyhan syndrome. ASDC J Dent Child , 69:66-69, 2002.
16 Benz CM, Reeka-Bartschmid AM, Agostini FG : Case report: the Lesch-Nyhan syndrome. Eur J Paediatr Dent , 5:110-114, 2004.