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J Dent Child (Chic). 2012 Sep-Dec;79(3):176-80.

Dental Findings and Management in a Mucopolysaccharidosis Type IIIB Patient.

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  • 1School of Dentistry, University of São Paulo at Ribeirão Preto, Brazil.


Mucopolysaccharidosis type IIIB (MPS IIIB) is an autosomal recessive disorder caused by deficiency of the lysosomal enzyme a-N-acetylglucosaminidase. Affected subjects present developmental delay, attention deficit disorder, uncontrollable hyperactivity, and aggressive behavior, followed by progressive dementia and death in late adolescence. The purpose of this paper is to report the dental findings and treatment in a child with MPS IIIB. His primary molars and permanent mandibular incisors presented obliterated pulp chambers and root canals, which may be a clinical manifestation of this disorder.

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