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BMC Oral Health
Published in: BMC Oral Health 1/2012

Open Access 01-12-2012 | Research article

Severe tooth wear in Prader-Willi syndrome. A case–control study

Authors: Ronnaug Saeves, Ivar Espelid, Kari Storhaug, Leiv Sandvik, Hilde Nordgarden

Published in: BMC Oral Health | Issue 1/2012

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Abstract

Background

Prader-Willi syndrome (PWS) is a rare complex multsystemic genetic disorder characterized by severe neonatal hypotonia, endocrine disturbances, hyperphagia and obesity, mild mental retardation, learning disabilities, facial dysmorphology and oral abnormalities. The purpose of the present study was to explore the prevalence of tooth wear and possible risk factors in individuals with Prader-Willi syndrome.

Methods

Forty-nine individuals (6-40 years) with PWS and an age- and sex-matched control group were included. Tooth wear was evaluated from dental casts and intraoral photographs and rated by four examiners using the Visual Erosion Dental Examination (VEDE) scoring system and the individual tooth wear index IA. In accordance with the VEDE scoring system, tooth wear was also evaluated clinically. Whole saliva was collected.

Results

Mean VEDE score was 1.70 ± 1.44 in the PWS group and 0.46 ± 0.36 in the control group (p < 0.001). Median IA was 7.50 (2.60-30.70) in the PWS group and 2.60 (0.90-4.70) among controls (p < 0.001). In the PWS group tooth wear correlated significantly with age (VEDE; r = 0.79, p < 0.001, IA; r = 0.82, p < 0.001) and saliva secretion (VEDE; r = 0.46, p = 0.001, IA; r = 0.43, p = 0.002). Tooth grinding was also associated with tooth wear in the PWS group, as indicated by the mean VEDE 2.67 ± 1.62 in grinders and 1.14 ± 0.97 in non-grinders (p = 0.001) and median IA values 25.70 (5.48-68.55) in grinders and 5.70 (1.60-9.10) in non-grinders (p = 0.003). Multivariate linear regression analysis was performed with tooth wear as the dependent variable and PWS (yes/no), age, tooth grinding and saliva secretion as independent variables. PWS (yes/no), age and tooth grinding retained a significant association with tooth wear, VEDE (p < 0.001) and log IA (p < 0.001). The only factor significantly associated with tooth wear in the control group was age.

Conclusions

Our study provides evidence that tooth wear, in terms of both erosion and attrition, is a severe problem in Prader-Willi syndrome. There is therefore considerable need for prosthodontic rehabilitation in young adults with PWS.
Appendix
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Literature
1.
Whittington JE, Holland AJ, Webb T, Butler J, Clarke D, Boer H: Population prevalence and estimated birth incidence and mortality rate for people with Prader-Willi syndrome in one UK Health Region. J Med Genet. 2001, 38: 792-798. 10.1136/jmg.38.11.792.CrossRefPubMedPubMedCentral
2.
Smith A, Egan J, Ridley G, Haan E, Montgomery P, Williams K, Elliott E: Birth prevalence of Prader-Willi syndrome in Australia. Arch Dis Child. 2003, 88: 263-264. 10.1136/adc.88.3.263.CrossRefPubMedPubMedCentral
3.
Vogels A, Van Den Ende J, Keymolen K, Mortier G, Devriendt K, Legius E, Fryns JP: Minimum prevalence, birth incidence and cause of death for Prader-Willi syndrome in Flanders. Eur J Hum Genet. 2004, 12: 238-240. 10.1038/sj.ejhg.5201135.CrossRefPubMed
4.
Thomson AK, Glasson EJ, Bittles AH: A long-term population-based clinical and morbidity review of Prader-Willi syndrome in Western Australia. J Intellect Disabil Res. 2006, 50: 69-78. 10.1111/j.1365-2788.2005.00770.x.CrossRefPubMed
5.
Burd L, Vesely B, Martsolf J, Kerbeshian J: Prevalence study of Prader-Willi syndrome in North Dakota. Am J Med Genet. 1990, 37: 97-99. 10.1002/ajmg.1320370122.CrossRefPubMed
6.
Akefeldt A, Gillberg C, Larsson C: Prader-Willi syndrome in a Swedish rural county: epidemiological aspects. Dev Med Child Neurol. 1991, 33: 715-721.CrossRefPubMed
7.
Cassidy SB, Forsythe M, Heeger S, Nicholls RD, Schork N, Benn P, Schwartz S: Comparison of phenotype between patients with Prader-Willi syndrome due to deletion 15q and uniparental disomy 15. Am J Med Genet. 1997, 68: 433-440. 10.1002/(SICI)1096-8628(19970211)68:4<433::AID-AJMG12>3.0.CO;2-T.CrossRefPubMed
8.
Prader A, Labhart A, Willi H: Ein syndrom von adipositas, Kleinwuchs, Kryptorchismus, und Oligophrenie nach myotonieartigem Zustand in Neugeborenenalter. Schweiz Med Wochenschr. 1956, 86: 1260-1261.
9.
Nicholls RD, Knoll JH, Butler MG, Karam S, Lalande M: Genetic imprinting suggested by maternal heterodisomy in nondeletion Prader-Willi syndrome. Nature. 1989, 342: 281-285. 10.1038/342281a0.CrossRefPubMed
10.
Driscoll DJ, Waters MF, Williams CA, Zori RT, Glenn CC, Avidano KM, Nicholls RD: A DNA methylation imprint, determined by the sex of the parent, distinguishes the Angelman and Prader-Willi syndromes. Genomics. 1992, 13: 917-924. 10.1016/0888-7543(92)90001-9.CrossRefPubMed
11.
Nicholls RD: Genomic imprinting and uniparental disomy in Angelman and Prader-Willi syndromes: a review. Am J Med Genet. 1993, 46: 16-25. 10.1002/ajmg.1320460106.CrossRefPubMed
12.
Butler MG: Prader-Willi syndrome: current understanding of cause and diagnosis. Am J Med Genet. 1990, 35: 319-332. 10.1002/ajmg.1320350306.CrossRefPubMed
13.
Nicholls RD, Knepper JL: Genome organization, function, and imprinting in Prader-Willi and Angelman syndromes. Annu Rev Genomics Hum Genet. 2001, 2: 153-175. 10.1146/annurev.genom.2.1.153.CrossRefPubMed
14.
Holm VA, Cassidy SB, Butler MG, Hanchett JM, Greenswag LR, Whitman BY, Greenberg F: Prader-Willi syndrome: consensus diagnostic criteria. Pediatrics. 1993, 91: 398-402.PubMed
15.
Gunay-Aygun M, Schwartz S, Heeger S, O'Riordan MA, Cassidy SB: The changing purpose of Prader-Willi syndrome clinical diagnostic criteria and proposed revised criteria. Pediatrics. 2001, 108: E92-10.1542/peds.108.5.e92.CrossRefPubMed
16.
Butler JV, Whittington JE, Holland AJ, Boer H, Clarke D, Webb T: Prevalence of, and risk factors for, physical ill-health in people with Prader-Willi syndrome: a population-based study. Dev Med Child Neurol. 2002, 44: 248-255. 10.1017/S001216220100202X.CrossRefPubMed
17.
Maas AP, Sinnema M, Didden R, Maaskant MA, Smits MG, Schrander-Stumpel CT, Curfs LM: Sleep disturbances and behavioural problems in adults with Prader-Willi syndrome. J Intellect Disabil Res. 2010, 54: 906-917. 10.1111/j.1365-2788.2010.01306.x.CrossRefPubMed
18.
Yee BJ, Buchanan PR, Mahadev S, Banerjee D, Liu PY, Phillips C, Loughnan G, Steinbeck K, Grunstein RR: Assessment of sleep and breathing in adults with prader-willi syndrome: a case control series. J Clin Sleep Med. 2007, 3: 713-718.PubMedPubMedCentral
19.
Saeves R, Asten P, Storhaug K, Bagesund M: Orofacial dysfunction in individuals with Prader-Willi syndrome assessed with NOT-S. Acta Odontol Scand. 2011, 69: 310-315. 10.3109/00016357.2011.568961.CrossRefPubMed
20.
Bray GA, Dahms WT, Swerdloff RS, Fiser RH, Atkinson RL, Carrel RE: The Prader-Willi syndrome: a study of 40 patients and a review of the literature. Medicine (Baltimore). 1983, 62: 59-80.CrossRef
21.
Hart PS: Salivary abnormalities in Prader-Willi syndrome. Ann N Y Acad Sci. 1998, 842: 125-131. 10.1111/j.1749-6632.1998.tb09640.x.CrossRefPubMed
22.
Bailleul-Forestier I, Verhaeghe V, Fryns JP, Vinckier F, Declerck D, Vogels A: The oro-dental phenotype in Prader-Willi syndrome: a survey of 15 patients. Int J Paediatr Dent. 2008, 18: 40-47.PubMed
23.
Stephenson JB: Neonatal presentation of Prader-Willi syndrome. Am J Dis Child. 1992, 146: 151-152.PubMed
24.
Saeves R, Nordgarden H, Storhaug K, Sandvik L, Espelid I: Salivary flow rate and oral findings in Prader-Willi syndrome. A case control study. Int J Paediatr Dent. 2012, 22: 27-36.CrossRefPubMed
25.
Bazopoulou-Kyrkanidou E, Papagiannoulis L: Prader-Willi syndrome: report of a case with special emphasis on oral problems. J Clin Pediatr Dent. 1992, 17: 37-40.PubMed
26.
Salako NO, Ghafouri HM: Oral findings in a child with Prader-Labhart-Willi syndrome. Quintessence Int. 1995, 26: 339-341.PubMed
27.
Bots CP, Schueler YT, Brand HS, van Nieuw AA: A patient with Prader-Willi syndrome. Characteristics, oral consequences and treatment options. Ned Tijdschr Tandheelkd. 2004, 111: 55-58.PubMed
28.
Scardina GA, Fuca G, Messina P: Oral diseases in a patient affected with Prader-Willi syndrome. Eur J Paediatr Dent. 2007, 8: 96-99.CrossRefPubMed
29.
Anavi Y, Mintz SM: Prader-Labhart-Willi syndrome. Ann Dent. 1990, 49: 26-29.PubMed
30.
Bassarelli V, Baccetti T, Bassarelli T, Franchi L: The dentomaxillofacial characteristics of the Prader-Labhart-Willi syndrome. A clinical case report. Minerva Stomatol. 1991, 40: 811-819.PubMed
31.
Banks PA, Bradley JC, Smith A: Prader-Willi syndrome–a case report of the multidisciplinary management of the orofacial problems. Br J Orthod. 1996, 23: 299-304.CrossRefPubMed
32.
Young W, Khan F, Brandt R, Savage N, Razek AA, Huang Q: Syndromes with salivary dysfunction predispose to tooth wear: Case reports of congenital dysfunction of major salivary glands, Prader-Willi, congenital rubella, and Sjogren's syndromes. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001, 92: 38-48. 10.1067/moe.2001.113549.CrossRefPubMed
33.
Mulic A, Tveit AB, Wang NJ, Hove LH, Espelid I, Skaare AB: Reliability of two clinical scoring systems for dental erosive wear. Caries Res. 2010, 44: 294-299. 10.1159/000314811.CrossRefPubMed
34.
Ekfeldt A, Hugoson A, Bergendal T, Helkimo M: An individual tooth wear index and an analysis of factors correlated to incisal and occlusal wear in an adult Swedish population. Acta Odontol Scand. 1990, 48: 343-349. 10.3109/00016359009033627.CrossRefPubMed
35.
Lussi A: Dental erosion clinical diagnosis and case history taking. Eur J Oral Sci. 1996, 104: 191-198. 10.1111/j.1600-0722.1996.tb00067.x.CrossRefPubMed
36.
Bulman JS, Osborn JF: Statistics in dentistry. 1989, British Dental Journal, London
37.
Cole TJ, Bellizzi MC, Flegal KM, Dietz WH: Establishing a standard definition for child overweight and obesity worldwide: international survey. BMJ. 2000, 320: 1240-1243. 10.1136/bmj.320.7244.1240.CrossRefPubMedPubMedCentral
38.
39.
Lussi A, Schaffner M, Hotz P, Suter P: Dental erosion in a population of Swiss adults. Community Dent Oral Epidemiol. 1991, 19: 286-290. 10.1111/j.1600-0528.1991.tb00169.x.CrossRefPubMed
40.
Arnadottir IB, Saemundsson SR, Holbrook WP: Dental erosion in Icelandic teenagers in relation to dietary and lifestyle factors. Acta Odontol Scand. 2003, 61: 25-28.CrossRefPubMed
41.
Dugmore CR, Rock WP: A multifactorial analysis of factors associated with dental erosion. Br Dent J. 2004, 196: 283-286. 10.1038/sj.bdj.4811041.CrossRefPubMed
42.
Holbrook WP, Furuholm J, Gudmundsson K, Theodors A, Meurman JH: Gastric reflux is a significant causative factor of tooth erosion. J Dent Res. 2009, 88: 422-426. 10.1177/0022034509336530.CrossRefPubMed
43.
Smith BG, Knight JK: An index for measuring the wear of teeth. Br Dent J. 1984, 156: 435-438. 10.1038/sj.bdj.4805394.CrossRefPubMed
44.
Ganss C: How valid are current diagnostic criteria for dental erosion?. Clin Oral Investig. 2008, 12 (Suppl 1): S41-S49.CrossRefPubMed
45.
Al-Majed I, Maguire A, Murray JJ: Risk factors for dental erosion in 5-6 year old and 12-14 year old boys in Saudi Arabia. Community Dent Oral Epidemiol. 2002, 30: 38-46. 10.1034/j.1600-0528.2002.300106.x.CrossRefPubMed
46.
El AH, Bronkhorst EM, Truin GJ: A longitudinal study of tooth erosion in adolescents. J Dent Res. 2008, 87: 731-735. 10.1177/154405910808700813.CrossRef
47.
Larsen MJ, Poulsen S, Hansen I: Erosion of the teeth: prevalence and distribution in a group of Danish school children. Eur J Paediatr Dent. 2005, 6: 44-47.PubMed
48.
Holbrook WP, Ganss C: Is diagnosing exposed dentine a suitable tool for grading erosive loss?. Clin Oral Investig. 2008, 12 (Suppl 1): S33-S39.CrossRefPubMed
49.
Van't Spijker A, Rodriguez JM, Kreulen CM, Bronkhorst EM, Bartlett DW, Creugers NH: Prevalence of tooth wear in adults. Int J Prosthodont. 2009, 22: 35-42.PubMed
50.
Dawes C: Salivary flow patterns and the health of hard and soft oral tissues. J Am Dent Assoc. 2008, 139 (Suppl): 18S-24S.CrossRefPubMed
51.
Akefeldt A: Water intake and risk of hyponatraemia in Prader-Willi syndrome. J Intellect Disabil Res. 2009, 53: 521-528. 10.1111/j.1365-2788.2009.01169.x.CrossRefPubMed
52.
Mahoney EK, Kilpatrick NM: Dental erosion: part 1. Aetiology and prevalence of dental erosion. N Z Dent J. 2003, 99: 33-41.PubMed
53.
Millward A, Shaw L, Smith AJ, Rippin JW, Harrington E: The distribution and severity of tooth wear and the relationship between erosion and dietary constituents in a group of children. Int J Paediatr Dent. 1994, 4: 151-157.CrossRefPubMed
54.
Jensdottir T, Arnadottir IB, Thorsdottir I, Bardow A, Gudmundsson K, Theodors A, Holbrook WP: Relationship between dental erosion, soft drink consumption, and gastroesophageal reflux among Icelanders. Clin Oral Investig. 2004, 8: 91-96.CrossRefPubMed
55.
McAllister CJ, Whittington JE, Holland AJ: Development of the eating behaviour in Prader-Willi Syndrome: advances in our understanding. Int J Obes (Lond). 2011, 35: 188-197. 10.1038/ijo.2010.139.CrossRef
56.
Khan F, Young WG, Daley TJ: Dental erosion and bruxism. A tooth wear analysis from south east Queensland. Aust Dent J. 1998, 43: 117-127.CrossRefPubMed
57.
Johansson A, Johansson AK, Omar R, Carlsson GE: Rehabilitation of the worn dentition. J Oral Rehabil. 2008, 35: 548-566. 10.1111/j.1365-2842.2008.01897.x.CrossRefPubMed
58.
Macaluso GM, Guerra P, Di GG, Boselli M, Parrino L, Terzano MG: Sleep bruxism is a disorder related to periodic arousals during sleep. J Dent Res. 1998, 77: 565-573. 10.1177/00220345980770040901.CrossRefPubMed
59.
Shetty S, Pitti V, Satish Babu CL, Surendra Kumar GP, Deepthi BC: Bruxism: a literature review. J Indian Prosthodont Soc. 2010, 10: 141-148. 10.1007/s13191-011-0041-5.CrossRefPubMed
60.
Hertz G, Cataletto M, Feinsilver SH, Angulo M: Sleep and breathing patterns in patients with Prader Willi syndrome (PWS): effects of age and gender. Sleep. 1993, 16: 366-371.PubMed
61.
Holland AJ, Whittington JE, Butler J, Webb T, Boer H, Clarke D: Behavioural phenotypes associated with specific genetic disorders: evidence from a population-based study of people with Prader-Willi syndrome. Psychol Med. 2003, 33: 141-153. 10.2190/4FM9-T915-L7E3-EFMX.CrossRefPubMed
62.
Bartlett DW, Evans DF, Anggiansah A, Smith BG: A study of the association between gastro-oesophageal reflux and palatal dental erosion. Br Dent J. 1996, 181: 125-131. 10.1038/sj.bdj.4809187.CrossRefPubMed
63.
Munoz JV, Herreros B, Sanchiz V, Amoros C, Hernandez V, Pascual I, Mora F, Minguez M, Bagan JV, Benages A: Dental and periodontal lesions in patients with gastro-oesophageal reflux disease. Dig Liver Dis. 2003, 35: 461-467. 10.1016/S1590-8658(03)00215-9.CrossRefPubMed
64.
Pace F, Pallotta S, Tonini M, Vakil N, Bianchi PG: Systematic review: gastro-oesophageal reflux disease and dental lesions. Aliment Pharmacol Ther. 2008, 27: 1179-1186. 10.1111/j.1365-2036.2008.03694.x.CrossRefPubMed
65.
Valipour A, Makker HK, Hardy R, Emegbo S, Toma T, Spiro SG: Symptomatic gastroesophageal reflux in subjects with a breathing sleep disorder. Chest. 2002, 121: 1748-1753. 10.1378/chest.121.6.1748.CrossRefPubMed
66.
Green BT, Broughton WA, O'Connor JB: Marked improvement in nocturnal gastroesophageal reflux in a large cohort of patients with obstructive sleep apnea treated with continuous positive airway pressure. Arch Intern Med. 2003, 163: 41-45. 10.1001/archinte.163.1.41.CrossRefPubMed
67.
Friedenberg FK, Xanthopoulos M, Foster GD, Richter JE: The association between gastroesophageal reflux disease and obesity. Am J Gastroenterol. 2008, 103: 2111-2122. 10.1111/j.1572-0241.2008.01946.x.CrossRefPubMed
68.
Brandt BR, Rosen I: Impaired peripheral somatosensory function in children with Prader-Willi syndrome. Neuropediatrics. 1998, 29: 124-126. 10.1055/s-2007-973547.CrossRefPubMed
69.
Priano L, Miscio G, Grugni G, Milano E, Baudo S, Sellitti L, Picconi R, Mauro A: On the origin of sensory impairment and altered pain perception in Prader-Willi syndrome: a neurophysiological study. Eur J Pain. 2009, 13: 829-835. 10.1016/j.ejpain.2008.09.011.CrossRefPubMed
Metadata
Title
Severe tooth wear in Prader-Willi syndrome. A case–control study
Authors
Ronnaug Saeves
Ivar Espelid
Kari Storhaug
Leiv Sandvik
Hilde Nordgarden
Publication date
01-12-2012
Publisher
BioMed Central
Published in
BMC Oral Health / Issue 1/2012
Electronic ISSN: 1472-6831
DOI
https://doi.org/10.1186/1472-6831-12-12

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