Dental Findings and Management in a Mucopolysaccharidosis Type IIIB Patient.

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 progress...

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Bibliographic Details
Published in:	Journal of Dentistry for Children Vol. 79; no. 3; pp. 176 - 181
Main Authors:	de Siqueira Mellara, Talitha, Torres Azevedo, Danielle, Faria, Gisele, Nelson Filho, Paulo, de Queiroz, Alexandra Mussolino, Guilherme Brentegani, Luiz
Format:	case study diagnostic images pictorial Journal Article
Published:	American Academy of Pediatric Dentistry Sep-Dec2012
Online Access:	View this record in EBSCOhost

Description
Summary:	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.