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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Published inJournal 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
LanguageEnglish
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