JOURNAL BROWSE
Search
Advanced SearchSearch Tips
DENTAL TREATMENT IN A PRADER-WILLI SYNDROME PATIENT : A CASE REPORT
facebook(new window)  Pirnt(new window) E-mail(new window) Excel Download
 Title & Authors
DENTAL TREATMENT IN A PRADER-WILLI SYNDROME PATIENT : A CASE REPORT
Lee, Soo Jeong; Baek, Kwangwoo;
  PDF(new window)
 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.
 Keywords
Prader-willi syndrome;Dental caries;General anesthesia;
 Language
Korean
 Cited by
 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. crossref(new window)

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. crossref(new window)

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. crossref(new window)

6.
Cassidy SB, Driscoll DJ : Prader-Willi syndrome. Eur J Hum Genet, 17:3-13, 2009. crossref(new window)

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. crossref(new window)

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. crossref(new window)

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. crossref(new window)

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. crossref(new window)

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. crossref(new window)

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. crossref(new window)

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. crossref(new window)

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. crossref(new window)

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. crossref(new window)

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. crossref(new window)

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. crossref(new window)

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.