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Quintessence Publishing: Journals: QI
Quintessence International

Edited by Eli Eliav

ISSN 0033-6572 (print) • ISSN 1936-7163 (online)

Publication:
March 2012
Volume 43 , Issue 3

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Oral and systemic manifestations of mucopolysaccharidosis type VI: A report of seven cases

Renata Quirino de Almeida-Barros, DMD/Salomao Cury-Rad Oka, DMD/Ana Carolina Barbosa Pordeus, MD Student/Paula Frassinetti Vasconcelos de Medeiros, MD, MSc, PhD/Patricia Meira Bento, DMD, MSc, PhD/Gustavo Pina Godoy, DMD, MSc, PhD

Pages: e32-e38
PMID: 22299127

Mucopolysaccharidosis (MPS) is a group of rare metabolic diseases characterized by intralysosomal accumulation of glycosaminoglycans. MPS type VI or Maroteaux-Lamy syndrome is an autosomal-recessive syndrome caused by mutations in the lysosomal enzyme arylsulfatase B. A defect in the gene leads to accumulation of nondegraded mucopolysaccharides, resulting in severe cellular dysfunction with multisystem expression. The oral manifestations of MPS VI are not well described in the literature. This paper presents a series of seven patients with MPS VI, with the description of the general clinical manifestations and focus on the still rarely studied oral manifestations of the syndrome. Among them were high palate, open bite, impacted and/or included teeth, thickening of the pericoronal follicle, and changes in the temporomandibular joint. (Quintessence Int 2012;43:e32e38)

Key words: arylsulfatase B, metabolic diseases, mucopolysaccharidosis, oral manfestations

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