Oral features and dental health in Hurler Syndrome following hematopoietic stem cell transplantation.

Hdl Handle:
http://hdl.handle.net/10147/207448
Title:
Oral features and dental health in Hurler Syndrome following hematopoietic stem cell transplantation.
Authors:
McGovern, Eleanor; Owens, Laura; Nunn, June; Bolas, Andrew; Meara, Anne O'; Fleming, Padraig
Affiliation:
Dental Department, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland., eleanor.mcgovern@olchc.ie
Citation:
Int J Paediatr Dent. 2010 Sep 1;20(5):322-9. Epub 2010 Jun 2.
Journal:
International journal of paediatric dentistry / the British Paedodontic Society, [and] the International Association of Dentistry for Children
Issue Date:
1-Feb-2012
URI:
http://hdl.handle.net/10147/207448
DOI:
10.1111/j.1365-263X.2010.01055.x
PubMed ID:
20545789
Abstract:
BACKGROUND: Hurler Syndrome is associated with a deficiency of a specific lysosomal enzyme involved in the degradation of glycosaminoglycans. Hematopoietic stem cell transplantation (HSCT) in early infancy is undertaken to help prevent the accumulation of glycosaminoglycans and improve organ function. 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: Patients with Hurler Syndrome post-HSCT are likely to have delayed dental development, a malocclusion, and dental anomalies, particularly hypodontia and microdontia.
Language:
eng
MeSH:
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; Questionnaires; Tooth Abnormalities/*etiology; Tooth Erosion/etiology; Young Adult
ISSN:
1365-263X (Electronic); 0960-7439 (Linking)

Full metadata record

DC FieldValue Language
dc.contributor.authorMcGovern, Eleanoren_GB
dc.contributor.authorOwens, Lauraen_GB
dc.contributor.authorNunn, Juneen_GB
dc.contributor.authorBolas, Andrewen_GB
dc.contributor.authorMeara, Anne O'en_GB
dc.contributor.authorFleming, Padraigen_GB
dc.date.accessioned2012-02-01T10:25:16Z-
dc.date.available2012-02-01T10:25:16Z-
dc.date.issued2012-02-01T10:25:16Z-
dc.identifier.citationInt J Paediatr Dent. 2010 Sep 1;20(5):322-9. Epub 2010 Jun 2.en_GB
dc.identifier.issn1365-263X (Electronic)en_GB
dc.identifier.issn0960-7439 (Linking)en_GB
dc.identifier.pmid20545789en_GB
dc.identifier.doi10.1111/j.1365-263X.2010.01055.xen_GB
dc.identifier.urihttp://hdl.handle.net/10147/207448-
dc.description.abstractBACKGROUND: Hurler Syndrome is associated with a deficiency of a specific lysosomal enzyme involved in the degradation of glycosaminoglycans. Hematopoietic stem cell transplantation (HSCT) in early infancy is undertaken to help prevent the accumulation of glycosaminoglycans and improve organ function. 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: Patients with Hurler Syndrome post-HSCT are likely to have delayed dental development, a malocclusion, and dental anomalies, particularly hypodontia and microdontia.en_GB
dc.language.isoengen_GB
dc.subject.meshAdolescenten_GB
dc.subject.meshChilden_GB
dc.subject.meshChild, Preschoolen_GB
dc.subject.meshDMF Indexen_GB
dc.subject.meshDental Caries/etiologyen_GB
dc.subject.meshFace/abnormalitiesen_GB
dc.subject.meshFemaleen_GB
dc.subject.meshHematopoietic Stem Cell Transplantationen_GB
dc.subject.meshHumansen_GB
dc.subject.meshInfanten_GB
dc.subject.meshMaleen_GB
dc.subject.meshMalocclusion/*etiologyen_GB
dc.subject.meshMucopolysaccharidosis I/*complications/therapyen_GB
dc.subject.meshQuestionnairesen_GB
dc.subject.meshTooth Abnormalities/*etiologyen_GB
dc.subject.meshTooth Erosion/etiologyen_GB
dc.subject.meshYoung Adulten_GB
dc.titleOral features and dental health in Hurler Syndrome following hematopoietic stem cell transplantation.en_GB
dc.contributor.departmentDental Department, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland., eleanor.mcgovern@olchc.ieen_GB
dc.identifier.journalInternational journal of paediatric dentistry / the British Paedodontic Society, [and] the International Association of Dentistry for Childrenen_GB
dc.description.provinceLeinster-

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