Skip to main content

Advertisement

SpringerLink
Log in

Oral health of Brazilian individuals with mucopolyssaccaridosis

  • Original Scientific Article
  • Published:
European Archives of Paediatric Dentistry Aims and scope Submit manuscript

Abstract

Purpose

This study described and compared the oral characteristics of Brazilian individuals with mucopolysaccharidosis (MPS) and without MPS.

Methods

A cross-sectional study was performed with 29 individuals with MPS and 29 without MPS and their parents/guardians. The individuals were aged between 3 and 21 years and attended at two hospitals in Belo Horizonte, southeastern Brazil. The dental characteristics were evaluated by clinical examination of dental caries, gingivitis, malocclusion, dental anomalies and developmental defects of enamel. The parents/guardians answered a questionnaire about the sociodemographic and behavioural aspects of their children. The study was approved by the Research Ethics Committee of the Federal University of Minas Gerais.

Results

The average age of the individuals was 13.9 years (± 7.2). The majority were male (58.6%), had black/brown skin (70.7%) and were from favored economic class (89.7%). Dental caries, gingivitis, malocclusion and dental anomalies were more prevalent in the MPS group (p < 0.05).

Conclusion

The individuals with MPS had a higher prevalence of oral diseases and dental anomalies than the group without MPS.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Graph 1

Similar content being viewed by others

References

  • A review of the developmental defects of enamel index (DDE index) Commission on Oral Health, Research & Epidemiology. Report of an FDI Working Group. Int Dent J. 1992;42:411–26.

  • Allison PJ, Lawrence HP. A paired comparison of dental care in Canadians with Down syndrome and their siblings without Down syndrome. Community Dent Oral Epidemiol. 2004;32:99–106.

    Article  PubMed  Google Scholar 

  • Almeida-Barros RQ, Medeiros PFS, Azevedo MQA, Ortega AOL, Yamamoto ATA, Dornelas SKL, Bento PM. Evaluation of oral manifestations of patients with mucopolysaccharidosis IV and VI: clinical and imaging study. Clin Oral Investig. 2017;22(1):201–8.

    Article  PubMed  Google Scholar 

  • Antunes LA, Nogueira AP, Castro GF, Ribeiro MG, de Souza IP. Dental findings and oral health status in patients with mucopolysaccharidosis: a case series. Acta Odontol Scand. 2013;7:157–67.

    Article  Google Scholar 

  • Associação Brasileira de Empresas de Pesquisa (ABEP). Critério de Classificação Econômica Brasil. São Paulo. 2015. https://www.abep.org/codigos-e-guias-da-abep. Accessed 16 Jul 2019.

  • Berger IK, Fagondes SC, Giugliani R, Hardy KA, Lee KS, McArdle C, Scarpa M, Tobin JM, Ward SA, Rapoport DM. Respiratory and sleep disorders in mucopolysaccharidosis. J Inherit Metab Dis. 2013;36(2):201–10.

    Article  PubMed  Google Scholar 

  • Brasil. Ministério da Saúde. Coordenação Nacional de Saúde Bucal, SB2010. Pesquisa Nacional de Saúde Bucal. Resultados principais, p. 92. Brasília, DF: MS. 2011. https://189.28.128.100/dab/docs/geral/projeto_sb2010_relatorio_final.pdf. Accessed 16 Jul 2019.

  • Braúna APVS, Abreu MHNG, Resende VLS, Castilho LS. Risk factors for dental caries in children with developmental disabilities. Braz Oral Res. 2016;30:e79.

    Article  Google Scholar 

  • Ballıkaya E, Eymirli PS, Yıldız Y, Avcu N, Sivri HS, Uzamış-Tekçiçek M. Oral health status in patients with mucopolysaccharidoses. Turk J Pediatr. 2018;60(4):400–6.

    Article  PubMed  Google Scholar 

  • Boustedt K, Dahlgren J, Twetman S, Roswall J. Tooth brushing habits and prevalence of early childhood caries: a prospective cohort study. Eur Arch Paediatr Dent. 2019. https://doi.org/10.1007/s40368-019-00463-3.

    Article  PubMed  Google Scholar 

  • Federhen A, Burin M, Leistner-Segal S, Matte U, Tirelli K, Facchin A, Pasqualim G, Bender F, Rafaelli C, Giugliani R. Minimal estimated incidence of MPS I, II, IV-A and VI in Brazil and comparison to the rest of the world. J Inborn Errors Metab Screen. 2016;4:9–10.

    Google Scholar 

  • Fonseca FR, de Santana Sarmento DJ, Vasconcelos Medeiros PF, Diniz DN, dos Santos MT. Patients with mucopolysaccharidosis have tendencies towards vertical facial growth. J Oral Maxillofac Surg. 2014;72:2539–46.

    Article  PubMed  Google Scholar 

  • Hendriksz CJ, Berger KI, Lampe C, Kircher SG, Orchard PJ, Southall R, et al. Health-related quality of life in mucopolysaccharidosis: looking beyond biomedical issues. Orphanet J Rare Dis. 2016;11:119.

    Article  PubMed  PubMed Central  Google Scholar 

  • Hingston EJ, Hunter ML, Hunter B. Drage N Hurler's syndrome: dental findings in a case treated with bone marrow transplantation in infancy. Int J Paediatr Dent. 2006;16:207–12.

    Article  PubMed  Google Scholar 

  • Instituto Brasileiro de Geografia e Estatística (IBGE). Características étnico-raciais da população. Rio de Janeiro. 2013. https://portal.mte.gov.br/data/files/8A7C816A316B688101318AB565C54F14/PCERP2008.pdf. Accessed 16 Jul 2019.

  • James A, Hendriksz CJ, Addison O. The oral health needs of children, adolescents and young adults affected by a mucopolysaccharide disorder. J Inherit Metab Dis. 2012;2:51–8.

    Google Scholar 

  • Kantaputra PN, Kayserili H, Güven Y, et al. Oral manifestations of 17 patients affected with mucopolysaccharidosis type VI. J Inherit Metab Dis. 2014;37:263–8.

    Article  PubMed  Google Scholar 

  • Khan SA, Peracha H, Ballhausen D, Wiesbauer A, Rohrbach M, Gautschi M, Mason RW, Giugliani R, Suzuki Y, Orii KE, Orii T, Tomatsu S. Epidemiology of mucopolysaccharidoses. Mol Genet Metab. 2017;121:227–40.

    Article  PubMed  PubMed Central  Google Scholar 

  • Kirkwood BR, Stern JEssentials of Medical Statistics. 2ed. London: Blackwell, p. 501. 2003. https://www.ufpe.br/ppgero/images/documentos/stata.pdf. Accessed 14 Jul 2019.

  • Kuratani T, Miyawaki S, Murakami T, Takano-Yamamoto T. Early orthodontic treatment and long-term observation in a patient with Morquio syndrome. Angle Orthod. 2005;75:881–7.

    PubMed  Google Scholar 

  • Leighton SEJ, Papsin B, Vellodi A, Dinwiddie R, Lane R. Disorders breathing during sleep in patients with mucopolisaccharidoses. Int J Pediatr Otorhinolaryngol. 2001;58:127–38.

    Article  PubMed  Google Scholar 

  • Lin HY, Chuang CK, Chen MR, et al. Natural history and clinical assessment of Taiwanese patients with mucopolysaccharidosis IVA. Orphanet J Rare Dis. 2014;9:1–10.

    Article  Google Scholar 

  • Lobene RR, Weatherford T, Ross NM, Lamm RA, Menaker L. A modified gingival index for use in clinical trials. Clin Prev Dent. 1986;8:3–6.

    PubMed  Google Scholar 

  • Martins AM, Dualibi AP, Norato D, Takata ET, Santos ES, Valadares ER, Porta G, de Luca G, Moreira G, Pimentel H, Coelho J, Brum JM, Semionato Filho J, Kerstenetzky MS, Guimarães MR, Rojas MV, Aranda PC, Pires RF, Faria RG, Mota RM, Matte U, Guedes ZC. Guidelines for the management of mucopolysaccharidosis type I. J Pediatr. 2009;155:32–46.

    Article  Google Scholar 

  • McGovern E, Owens L, Nunn J, Bolas A, Meara AO, Fleming P. Oral features and dental health in Hurler Syndrome following hematopoietic stem cell transplantation. Int J Paediatr Dent. 2010;20:322–9.

    Article  PubMed  Google Scholar 

  • Neufeld EF, Muenzer J. The mucopolisaccharidosis. In: Scriver CR, Beandet AL, Sly S, Valle D, Childs B, Kinzler KW, editors. The metabolic and molecular basis of inherited disease. New York: McGraw-Hill; 2001. p. 3421–3452.

    Google Scholar 

  • Oliveira AC, Paiva SM, Campos MR, Czeresnia D. Factors associated with malocclusions in children and adolescents with Down syndrome. Am J Orthod Dentofacial Orthop. 2008;133:489.

    Article  Google Scholar 

  • Oliveira JS, Prado RR Jr, Lima KRS, Amaral HO, Moita Neto JM, Mendes RF. Intellectual disability ans impact on oral health: a paired study. Spec Care Dentist. 2013;33:262–8.

    Article  PubMed  Google Scholar 

  • Onçag G, Ertan Erdinc AM, Cal E. Multidisciplinary treatment approach of Morquio syndrome (Mucopolysaccharidosis Type IVA). Angle Orthod. 2006;76:335–40.

    PubMed  Google Scholar 

  • Ribeiro EM, Fonteles CS, Freitas AB, da Silva Alves KS, Monteiro AJ, da Silva CA. A clinical multicenter study of orofacial features in 26 Brazilian patients with different types of Mucopolysaccharidosis. Cleft Palate Craniofac J. 2014;11:57–69.

    Google Scholar 

  • Rolling I, Clausen N, Nyvad B, Sindet-Pedersen S. Dental findings in three siblings with Morquio’s syndrome. Int J Paediatr Dent. 1999;9:219–24.

    Article  PubMed  Google Scholar 

  • Sarmento DJS, Araújo TK, Mesquita GQTB, Diniz DN, Alvesreis FR, Medeiros PFV, Santos MTBRD, Godoy GP. Relationship between occlusal features and enzyme replacement therapy in patients with Mucopolysaccharidoses. J Oral Maxillofac Surg. 2017;76:785–92.

    Article  PubMed  Google Scholar 

  • Savitha NS, Saurabh G, Krishnamoorthy SH, Nandan S, Ambili A. Hunter's syndrome: a case report. J Indian Soc Pedod Prev Dent. 2015;33:66–8.

    Article  PubMed  Google Scholar 

  • Torres RO, Pintor AVB, Guedes FR, Freitas-Fernandes LB, Barth AL, Horovitz DDG, de Souza IPR. Oral and craniofacial manifestations in a Hunter syndrome patient with hematopoietic stem cell transplantation: a case report. Spec Care Dentist. 2018;38:51–4.

    Article  PubMed  Google Scholar 

  • Turra GS, Schwartz VD. Evaluation of orofacial motricity in patients with mucopolysaccharidosis: a cross-sectional study. J Pediatr. 2009;85:254–60.

    Google Scholar 

  • World Health Organization (WHO). Oral health surveys. Geneva: World Health Organization; 2013. p 53. https://www.who.int/oral_health/publications/9789241548649/en/. Accessed 29 Apr 2019.

Download references

Acknowledgements

This study was supported by the Brazilian fostering agencies Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq [Council of Scientific and Technological Development]), Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES [Coordination for the Advancement of Higher Education Personnel]), Fundação de Amparo a Pesquisa do Estado de Minas Gerais (FAPEMIG [State of Minas Gerais Research Foundation]) and Pró-Reitoria de Pesquisa da Universidade Federal de Minas Gerais (PRPq/UFMG [Dean's Office for Research Projects of the Federal University of Minas Gerais]).

Author information

Authors and Affiliations

  1. Department of Pediatric Dentistry, Faculty of Dentistry, Universidade Federal de Minas Gerais (UFMG), Av. Antônio Carlos, 6627 Campus Pampulha, Belo Horizonte, MG, 31270-901, Brazil

    T. D. Deps

  2. Department of Restorative Dentistry, Faculty of Dentistry, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, Brazil

    E. C. França

  3. Department of Complementary Propaedeutic, Faculty of Medicine, Federal University of Minas Gerais (UFMG), Belo Horizonte, Brazil

    E. R. Valadares

  4. Oral Health and Society Research Unit, Faculty of Dentistry, McGill University, Montreal, Canada

    B. Nicolau

  5. Department of Pediatric Dentistry, Faculty of Dentistry, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, Brazil

    I. A. Pordeus

  6. Department of Dental Public Health, Faculty of Dentistry, Federal University of Minas Gerais (UFMG), Belo Horizonte, Brazil

    A. C. Borges-Oliveira

Authors
  1. T. D. Deps
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. E. C. França
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. E. R. Valadares
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. B. Nicolau
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. I. A. Pordeus
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. A. C. Borges-Oliveira
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to T. D. Deps.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Deps, T.D., França, E.C., Valadares, E.R. et al. Oral health of Brazilian individuals with mucopolyssaccaridosis. Eur Arch Paediatr Dent 22, 13–19 (2021). https://doi.org/10.1007/s40368-020-00508-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s40368-020-00508-y

Keywords

Search

Navigation