EFFECTIVE STOMATOLOGICAL MAINTENANCE OF PEDIATRIC PATIENT WITH LESCH–NYHAN SYNDROME
https://doi.org/10.15690/vsp.v13i1.928
Abstract
A rare clinical case of stomatological maintenance of 11-year old boy with Lesch-Nyhan syndrome (primary x-linked hyperuricemia) is demonstrated in the article. In order to decrease trauma risk of oral cavity organs and soft tissues (due to autoagression) individual dentoalveolar occlusive mouthguards for the upper and lower jaws were used. As a result of this procedure pain syndrome was arrested, the child became quieter and the possibility to provide him with individual education according to the specialized correction school program appeared. Usage of such palliative measures in severe clinical cases is a method of choice in the absence of positive effect of alternative methods of treatment.
About the Authors
N. V. TarasovaRussian Federation
V. G. Galonskii
Russian Federation
References
1. Beljakov Ju. A. Nasledstvennye bolezni i sindromy v stomatologicheskoj praktike [Genetically determined diseases and syndromes occur in dental practice treatment]. Moscow, Medicina, 2008. 240 p.
2. Jones K. L. Smith's Recognizable Patterns of Human Malformation. Moscow, Praktika, 2011. 1024 p.
3. Rudenskaja G. E., Zaharova E.Ju., Bessonova L. A., Zaharova O. M., Bobylova M.Ju., Fedorov E. S.. Medicinskaja genetika — Medical Genetics. 2010; 9: 41–48.
4. Lopez J. Is ZMP the toxic metabolite in Lesch–Nyhan disease? Med. Hypotheses. 2008; 71: 657–663.
5. Usov I. N., Astahova L. N., Usova Ju. I. Zdravoohranenie Belorussii — Healthcare of Byelorussia. 1975; 2: 74–75.
6. Lesch M., Nyhan W. L. A familial disorder of uric acid metabolism and central nervous system function. Am. J. Med. 1964; 36: 561–570.
7. Ledashheva T. A., Isachenko L. G., Panova T. F. Sindrom Ljosha– Nihana v klinicheskoj praktike. Sovremennye tehnologii profilaktiki nasledstvennyh boleznej i detskoj invalidnosti [Lesch-Nyhan syndrome in clinical practice. Innovations in prevention of genetically determined diseases and child disability]. 2009. pp. 204–212.
8. Olson L., Houlihan D. A review of behavioral treatments used for Lesch–Nyhan syndrome. Behav. Modif. 2000; 24: 202–222.
9. Zilli E., Hasselmo M. A model of behavioral treatments for selfmutilation behavior in Lesch–Nyhan syndrome. Neuroreport. 2008; 19: 459–462.
10. Anderson L., Ernst M., Davis S. Cognitive abilities of patients with Lesch–Nyhan ditease. J. Autism. Dev. Disord. 1992; 22: 189–203.
11. De Gregorio L., Jinnah H. A., Harris J. C., Nyhan W. L., Schretlen D. J., Trombley L. M., O'Neill J. P. Lesch–Nyhan disease in a female with a clinically normal monozygotic twin. Mol. Genet. Metab. 2005; 85: 70–77.
12. Jeong T. S., Lee J. H., Kim S., Kim J. H., Ruwaida G. H. A preventive approach to oral self-mutilation in Lesch–Nyhan syndrome: A сase report. Pediatric Dentistry. 2006; 28 (4): 341–344.
13. Sugahara T., Mishima K., Mori Y. Lesch–Nyhan syndrome: Successful prevention of lower lip ulceration caused by selfmutilation by use of a mouthguard. Int. J. Oral. Maxillofac. Surg. 1994; 23: 37–38.
14. Rashid N., Yusuf H. Oral self-mutilation by a 17-month-old child with Lesch–Nyhan syndrome. Int. J. Pediatr. Dent. 1997; 21 (2): 205–211.
15. Mass E., Gadoth N. Oro-dental self-mutilation in familial dysautonomia. J. Oral Pathol. Med. 1994; 23: 273–276.
16. Frost F., Mukkamala S., Covington E. Self-inflicted finger injury in individuals with spinal cord injury: an analysis of 5 cases. J. Spinal. Cord. Med. 2008; 31: 109–116.
17. Chen L. R., Liu J. F. Successful treatment of self-inflicted oral mutilation using an acrylic splint retained by a head gear. Pediatr. Dent. 1996; 18: 408–410.
18. Pigno M. A., Funk J. J. Prevention of tongue biting with a removable oral device: А clinical report. J. Prosthet. Dent. 2000; 83: 508–510.
19. Lee J. H., Beerkowiz R. J., Choi B. J. Oral self-mutilation in the Lesch–Nyhan syndrome. J. Dent. Child. 2002; 69: 66–69.
20. Silva D. R., da Fonseca M. A. Self-injurious behavior as a challenge for the dental practice: А case report. Pediatr. Dent. 2003; 5 (1): 61–66.
Review
For citations:
Tarasova N.V., Galonskii V.G. EFFECTIVE STOMATOLOGICAL MAINTENANCE OF PEDIATRIC PATIENT WITH LESCH–NYHAN SYNDROME. Current Pediatrics. 2014;13(1):162-166. (In Russ.) https://doi.org/10.15690/vsp.v13i1.928