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 (Chicago, Ill.) Vol. 79; no. 3; p. 176
Main Authors:	Mellara, Talitha de Siqueira, Azevedo, Danielle Torres, Faria, Gisele, Nelson Filho, Paulo, Queiroz, Alexandra Mussolino de, Brentegani, Luiz Guilherme
Format:	Journal Article
Language:	English
Published:	United States 01-09-2012
Subjects:	Child Dental Care for Disabled Dental Caries - diagnosis Dental Caries - therapy Dental Pulp Cavity - abnormalities Dentigerous Cyst - diagnostic imaging Follow-Up Studies Humans Incisor - abnormalities Male Mandibular Diseases - diagnostic imaging Molar - abnormalities Molar, Third - abnormalities Mucopolysaccharidosis III - pathology Radiography Tooth Diseases - diagnosis Tooth Diseases - therapy Tooth Germ - abnormalities Tooth, Deciduous - abnormalities
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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.
ISSN:	1935-5068