Dental findings and management in a mucopolysaccharidosis type IIIB patient

Journal of Dentistry for Children

Volumn 79, Issue 3, 2012, Pages 176-180

  De Siqueira Mellara, Talitha ^a   Azevedo, Danielle Torres ^a   Faria, Gisele ^a   Filho, Paulo Nelson ^a   De Queiroz, Alexandra Mussolino ^a   Brentegani, Luiz Guilherme ^a  

^a UNIVERSITY OF SÃO PAULO   (Brazil)

Author keywords

Dentistry; Mucopolysaccharidosis type iiib; Pulp obliteration; Sanfilippo syndrome type B

Indexed keywords

ARTICLE; CASE REPORT; CHILD; CONGENITAL MALFORMATION; DECIDUOUS TOOTH; DENTAL CARIES; DENTAL PROCEDURE; FOLLOW UP; HUMAN; INCISOR; JAW DISEASE; MALE; MOLAR TOOTH; PATHOLOGY; RADIOGRAPHY; SANFILIPPO SYNDROME; TERATOMA; TOOTH DISEASE; TOOTH FLORA; TOOTH PULP;

CHILD; DENTAL CARE FOR DISABLED; DENTAL CARIES; DENTAL PULP CAVITY; DENTIGEROUS CYST; FOLLOW-UP STUDIES; HUMANS; INCISOR; MALE; MANDIBULAR DISEASES; MOLAR; MOLAR, THIRD; MUCOPOLYSACCHARIDOSIS III; TOOTH DISEASES; TOOTH GERM; TOOTH, DECIDUOUS;

EID: 84872278883     PISSN: 15518949     EISSN: 19355068     Source Type: Journal    
DOI: None     Document Type: Article

References (23)

1. Valstar MJ, Ruijter GJ, van Diggelen OP, Poorthuis BJ, Wijburg FA. Sanfilippo syndrome: A minireview. J Inherit Metab Dis 2008;31:240-52.
2. Baehner F, Schmiedeskamp C, Krummenauer F, et al. Cumulative incidence rates of the mucopolysaccharidoses in Germany. J Inherit Metab Dis 2005; 28:1011-7.
3. Van de Kamp JJ, Niermeijer MF, von Figura K, Giesberts MA. Genetic heterogeneity and clinical variability in the Sanfilippo syndrome (types A, B, and C). Clin Genet 1981;20:152-60.
4. Poorthuis BJ, Wevers RA, Kleijer WJ, et al. The frequency of lysosomal storage diseases in The Netherlands. Hum Genet 1999;105:151-6.
5. Johns Hopkins University. OMIM database. Mucopolysaccharidosis type IIIA. OMIM no. 252900. Available at: "http://omim.org/entry/252900". Accessed on September 20, 2011.
6. Coelho JC, Wajner M, Burin MG, Vargas CR, Giugliani R. Selective screening of 10,000 high-risk Brazilian patients for the detection of inborn errors of metabolism. Eur J Pediatr 1997;156:650-4.
7. Emre S, Terzioǧlu M, Coşkun T, et al. Biochemical and molecular analysis of mucopolysaccharidoses in Turkey. Turk J Pediatr 2002;44:13-7.
8. Michelakakis H, Dimitriou E, Tsagaraki S, Giouroukos S, Schulpis K, Bartsocas CS. Lysosomal storage diseases in Greece. Genet Couns 1995;6:43-7.
9. Neufeld EF, Muenzer J. The mucopolysaccharidoses. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The Metabolic and Molecular Bases of Inherited Disease. 8th ed. New York, NY: McGraw-Hill; 2001:3421-52.
10. Tessitore A, Villani GR, Di Domenico C, Filocamo M, Gatti R, Di Natale P. Molecular defects in the alpha-N-acetylglucosaminidase gene in Italian Sanfilippo type B patients. Hum Genet 2000;107:568-76.
11. Lee-Chen GJ, Lin SP, Lin SZ, et al. Identification and characterization of mutations underlying Sanfilippo syndrome type B (mucopolysaccharidosis type IIIB). J Med Genet 2002;39:E3.
12. Beesley C, Moraitou M, Winchester B, Schulpis K, Dimitriou E, Michelakakis H. Sanfilippo B syndrome: Molecular defects in Greek patients. Clin Genet 2004;65:143-9.
13. Beesley CE, Jackson M, Young EP, Vellodi A, Winchester BG. Molecular defects in Sanfilippo syndrome type B (mucopolysaccharidosis IIIB). J Inherit Metab Dis 2005;28:759-67.
14. Muenzer J, Wraith JE, Beck M, et al. A phase II/III clinical study of enzyme replacement therapy with idursulfase in mucopolysaccharidosis II (Hunter syndrome). Genet Med 2006;8:465-73.
15. Husain AM, Escolar ML, Kurtzberg J. Neurophysiologic assessment of mucopolysaccharidosis III. Clin Neurophysiol 2006;117:2059-63.
16. Webman MS, Hirsch SA, Webman H, Stanley HR. Obliterated pulp cavities in the Sanfilippo syndrome (mucopolysaccharidosis III). Oral Surg Oral Med Oral Pathol 1977;43:734-8.
17. Tamgadge A, Tamgadge S, Bhatt D, Bhalerao S, Pereira T, Padhye M. Bilateral dentigerous cyst in a non-syndromic patient: Report of an unusual case with review of the literature. J Oral Maxillofac Pathol 2011;15:91-5.
18. Sun CX, Ririe C, Henkin JM. Hyperplastic dental follicle: Review of literature and report of two cases in one family. Chin J Res 2010;13:71-5.
19. Ruijter GJ, Valstar MJ, Van de Kamp JM, et al. Clinical and genetic spectrum of Sanfilippo type C (MPS IIIC) disease in The Netherlands. Mol Genet Metab 2008;93:104-11.
20. Bax MC, Colville GA. Behavior in mucopolysaccharide disorders. Arch Dis Child 1995;73:77-81.
21. Cleary MA, Wraith JE. Management of mucopolysaccharidosis type III. Arch Dis Child 1993;69: 403-6.
22. Nidiffer FD, Kelly TE. Developmental and degenerative patterns associated with cognitive, behavioral and motor difficulties in the Sanfilippo syndrome: An epidemiological study. J Ment Defic Res 1983; 27:185-203.
23. Hopwood JJ, Morris CP. The mucopolysaccharidoses: Diagnosis, molecular genetics and treatment. Mol Biol Med 1990;7:381-404.