The Journal of Korea Assosiation for Disability and Oral Health (대한장애인치과학회지)

Korean Association for Disability and Oral Health (대한장애인치과학회)
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DENTAL TREATMENT IN A PRADER-WILLI SYNDROME PATIENT : A CASE REPORT

프라더윌리증후군 환자에서의 치과 치료 : 증례보고

  • Lee, Soo Jeong (Division of Pediatric Dentistry, Department of Dentistry, Ajou University School of Medicine) ;
  • Baek, Kwangwoo (Division of Pediatric Dentistry, Department of Dentistry, Ajou University School of Medicine)
  • 이수정 (아주대학교병원 소아치과학교실) ;
  • 백광우 (아주대학교병원 소아치과학교실)
  • Received : 2015.11.25
  • Accepted : 2015.12.11
  • Published : 2015.12.31
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Abstract

Prader-will syndrome (PWS) is a genetic disorder displayed with short stature and behavioral problems. The major manifestation is hyperphagia and obesity but there is no known effective drug that can solve this problem. This article presents a case report of a 3-year 9-month old girl affected with PWS syndrome treated under general anesthesia due to lack of cooperation and multiple caries. The dentist should work as a member of the multidisciplinary medical team. Careful long term follow up is required to prevent dental caries and periodontal diseases as well as maintain good oral hygiene. A strict diet control should also be encouraged.

본 논문에서 소개된 환자는 전형적인 PWS 증상인 섭식 장애, 타액분비감소, 지적장애, 비만과 같은 특성이 관찰되었다. 전신마취 하에서 적극적인 치과 치료를 시행하였고 보호자에게 비만 및 치아 우식을 예방하기 위해 탄수화물과 당분 섭취를 제한하는 식습관에 대한 교육을 시행하였다. 환자의 내원 횟수를 늘려 치과 치료를 위한 전신마취까지 발전하지 않도록 정기적인 관리가 필요하다.

Keywords

References

  1. Cataletto M, Angulo M, Hertz G, Whitman B : Prader-Willi syndrome: A primer for clinicians. Int J Pediatr Endocrinol, 1:12, 2011.
  2. Cassidy SB, Forsythe M, Heeger S et al. : Comparison of phenotype between patients with Prader-Willi syndrome due to deletion 15q and uniparental disomy 15. Am J Med Genet, 68: 433-440, 1997. https://doi.org/10.1002/(SICI)1096-8628(19970211)68:4<433::AID-AJMG12>3.0.CO;2-T
  3. Nicholls RD, Knepper JLn : Genome organization, function, and imprinting in Prader-Willi and Angelman syndromes. Annu Rev Genomics Hum Genet 2:153-175, 2001. https://doi.org/10.1146/annurev.genom.2.1.153
  4. Wattendorf DJ, Muenk M : Prader-Willi Syndrome. Am Fam Physician, 72:827-830, 2005.
  5. Scardina GA, Fuca G, Messina P : Oral diseases in a patient affected with Prader-Willi syndrome. Eur J Paediatr Dent, 8:96-99, 2007. https://doi.org/10.1007/BF03262576
  6. Cassidy SB, Driscoll DJ : Prader-Willi syndrome. Eur J Hum Genet, 17:3-13, 2009. https://doi.org/10.1038/ejhg.2008.165
  7. Miller J, Silverstein J, Shuster J, Driscoll DJ, Wagner M : Short-term effects of growth hormone on sleep abnormalities in Prader-Willi syndrome. J Clin Endocrinol Metab, 91:413-417, 2006. https://doi.org/10.1210/jc.2005-1279
  8. Saeves R, Espelid I, Nordgarden H, et al. : Severe tooth wear in Prader-Willi syndrome. A case-control study. BMC Oral Health, 12:12, 2012. https://doi.org/10.1186/1472-6831-12-12
  9. Olczak-Kowalczyk D, Witt A, Gozdowski D, Ginalska-Malinowska M : Oral mucosa in children with Prader-Willi syndrome. J Oral Pathol Med, 40:778-784, 2011. https://doi.org/10.1111/j.1600-0714.2011.01034.x
  10. Bailleul-Forestier I, Verhaehe V, Voels A, et al. : The oro-dental phenotype in Prader-Willi syndrome: a survey of 15 patients. Int J Paediatr Dent, 18:40-47, 2008.
  11. Bots CP, Schueler YT, Brand HS, van Nieuw AA : A patient with Prader-Willi syndrome. Characteristics, oral consequences and treatment options. Ned Tijdschr Tandheelkd, 111:55-58, 2004.
  12. Stephenson JB : Neonatal presentation of Prader-Willi syndrome. Am J Dis Child, 146: 151-152, 1992.
  13. Miller JL, Strong TV, Heinemann J : Medication Trials for Hyperphagia and Food-Related Behaviors in Prader-Willi Syndrome. Diseases, 3:78-85, 2015. https://doi.org/10.3390/diseases3020078
  14. Butler MG, Meaney FJ : Standards for selected anthropometric measurements in Prader-Willi syndrome. Pediatr, 88:853-860, 1991.
  15. Carrel AL, Myers SE, Whitman BY, Allen DB : Growth hormone improves body composition, fat utilization, physical strength and agility, and growth in Prader-Willi syndrome: A controlled study. J Pediatr, 134:215-21, 1999. https://doi.org/10.1016/S0022-3476(99)70418-X
  16. Swaab DF, Purba JS, Hofman MA : Alterations in the hypothalamic paraventricular nucleus and its oxytocin neurons (putative satiety cells) in Prader-Willi syndrome: a study of five cases. J Clin Endocrinol Metab, 80:573-579, 1995.
  17. Holm VA, Pipes PL : Food and children with Prader-Willi syndrome. Am J Dis Child, 130:1063-1067, 1976.
  18. Veltman MW, Craig EE, Bolton PF : Autism spectrum disorders in Prader-Willi and Angelman syndromes: a systematic review. Psychiatr Genet, 15:243-254, 2005. https://doi.org/10.1097/00041444-200512000-00006
  19. Vogels A, Van Den Ende J, Fryns JP, et al. : Minimum prevalence, birth incidence and cause of death for Prader-Willi syndrome in Flanders. Eur J Hum Genet, 12:238-40, 2004. https://doi.org/10.1038/sj.ejhg.5201135
  20. Stevenson DA, Heinemann, J, Scheimann AO, et al. : Gastric rupture and necrosis in Prader-Willi syndrome. J Pediatr Gastroenterol Nutr, 45:272-274, 2007. https://doi.org/10.1097/MPG.0b013e31805b82b5
  21. Stevenson DA, Heinemann J, Scheimann AO, et al. : Deaths due to choking in Prader-Willi syndrome. Am J Med Genet A, 143:484-7, 2007.
  22. Butler JV, Whittington JE, Webb T, et al. : Prevalence of, and risk factors for, physical illhealth in people with Prader-Willi syndrome: a population-based study. Dev Med Child Neurol, 44:248-255, 2002. https://doi.org/10.1017/S001216220100202X
  23. Davies PSW, Knight B : Need to know nutrition for children with Prader Willi syndrome : a guide for parents and carers, Brisbane University of Queensland, 2012.
  24. Goldstone AP : Prader-Willi syndrome: advances in genetics, pathophysiology, and treatment. Trends Endocrinol Metab, 15:12-20, 2004. https://doi.org/10.1016/j.tem.2003.11.003
  25. Saeves R, Nordgarden H, Espelid I, et al. : Salivary flow rate and oral findings in Prader- Willi syndrome: a case-control study. Int J Paediatr Dent, 22:27-36, 2012.
  26. Friedlander AH, Yagiela JA, Paterno VI, Mahler ME : The pathophysiology, medical management and dental implications of fragile X, Rett, and Prader-Willi syndromes. J Calif Dent Assoc, 31:693-702, 2003.
  27. Yanagita M, Hirano H, Murakami S, et al. : Periodontal disease in a patient with Prader-Willi syndrome: a case report. J Med Case Reports, 5:329, 2011. https://doi.org/10.1186/1752-1947-5-329
  28. Kong EK, Jung YJ, Baek KW : Dental Treatment for a Patient with Seizure History and Intellectual Disability under General Anesthesia: A Case Report. J Dent Anesth Pain Med, 8: 35-39, 2008.