Abstract | BACKGROUND: AIM: To investigate the oral features and dental health of patients with Hurler Syndrome who have undergone successful HSCT. MATERIALS AND METHODS: Twenty-five patients (median age 8.6 years) post-HSCT (mean age 9.4 months) underwent oral assessment (mean of 7.5 years post-HSCT). RESULTS: Dental development was delayed. Numerous occlusal anomalies were noted including: open-bite, class III skeletal base, dental spacing, primary molar infra-occlusion and ectopic tooth eruption. Dental anomalies included hypodontia, microdontia, enamel defects, thin tapering canine crowns, pointed molar cusps, bulbous molar crowns and molar taurodontism. Tooth roots were usually short/blunted/spindle-like in permanent molars. The prevalence of dental caries was low in the permanent dentition (mean DMFT 0.7) but high in the primary dentition (mean dmft 2.4). Oral hygiene instruction with plaque and or calculus removal was indicated in 71% of those that were dentate. CONCLUSION:
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Authors | Eleanor McGovern, Laura Owens, June Nunn, Andrew Bolas, Anne O' Meara, Padraig Fleming |
Journal | International journal of paediatric dentistry
(Int J Paediatr Dent)
Vol. 20
Issue 5
Pg. 322-9
(Sep 01 2010)
ISSN: 1365-263X [Electronic] England |
PMID | 20545789
(Publication Type: Journal Article)
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Topics |
- Adolescent
- Child
- Child, Preschool
- DMF Index
- Dental Caries
(etiology)
- Face
(abnormalities)
- Female
- Hematopoietic Stem Cell Transplantation
- Humans
- Infant
- Male
- Malocclusion
(etiology)
- Mucopolysaccharidosis I
(complications, therapy)
- Surveys and Questionnaires
- Tooth Abnormalities
(etiology)
- Tooth Erosion
(etiology)
- Young Adult
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