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

Hdl Handle:
http://hdl.handle.net/10147/124290
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:
Oral features and dental health in Hurler Syndrome following hematopoietic stem cell transplantation. 2010, 20 (5):322-9 Int J Paediatr Dent
Journal:
International journal of paediatric dentistry / the British Paedodontic Society [and] the International Association of Dentistry for Children
Issue Date:
1-Sep-2010
URI:
http://hdl.handle.net/10147/124290
DOI:
10.1111/j.1365-263X.2010.01055.x
PubMed ID:
20545789
Abstract:
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.; To investigate the oral features and dental health of patients with Hurler Syndrome who have undergone successful HSCT.; 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).; 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.; Patients with Hurler Syndrome post-HSCT are likely to have delayed dental development, a malocclusion, and dental anomalies, particularly hypodontia and microdontia.
Item Type:
Article
Language:
en
MeSH:
Adolescent; Child; Child, Preschool; DMF Index; Dental Caries; Face; Female; Hematopoietic Stem Cell Transplantation; Humans; Infant; Male; Malocclusion; Mucopolysaccharidosis I; Questionnaires; Tooth Abnormalities; Tooth Erosion; Young Adult
ISSN:
1365-263X

Full metadata record

DC FieldValue Language
dc.contributor.authorMcGovern, Eleanoren
dc.contributor.authorOwens, Lauraen
dc.contributor.authorNunn, Juneen
dc.contributor.authorBolas, Andrewen
dc.contributor.authorMeara, Anne O'en
dc.contributor.authorFleming, Padraigen
dc.date.accessioned2011-03-11T16:35:00Z-
dc.date.available2011-03-11T16:35:00Z-
dc.date.issued2010-09-01-
dc.identifier.citationOral features and dental health in Hurler Syndrome following hematopoietic stem cell transplantation. 2010, 20 (5):322-9 Int J Paediatr Denten
dc.identifier.issn1365-263X-
dc.identifier.pmid20545789-
dc.identifier.doi10.1111/j.1365-263X.2010.01055.x-
dc.identifier.urihttp://hdl.handle.net/10147/124290-
dc.description.abstractHurler 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.-
dc.description.abstractTo investigate the oral features and dental health of patients with Hurler Syndrome who have undergone successful HSCT.-
dc.description.abstractTwenty-five patients (median age 8.6 years) post-HSCT (mean age 9.4 months) underwent oral assessment (mean of 7.5 years post-HSCT).-
dc.description.abstractDental 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.-
dc.description.abstractPatients with Hurler Syndrome post-HSCT are likely to have delayed dental development, a malocclusion, and dental anomalies, particularly hypodontia and microdontia.-
dc.language.isoenen
dc.subject.meshAdolescent-
dc.subject.meshChild-
dc.subject.meshChild, Preschool-
dc.subject.meshDMF Index-
dc.subject.meshDental Caries-
dc.subject.meshFace-
dc.subject.meshFemale-
dc.subject.meshHematopoietic Stem Cell Transplantation-
dc.subject.meshHumans-
dc.subject.meshInfant-
dc.subject.meshMale-
dc.subject.meshMalocclusion-
dc.subject.meshMucopolysaccharidosis I-
dc.subject.meshQuestionnaires-
dc.subject.meshTooth Abnormalities-
dc.subject.meshTooth Erosion-
dc.subject.meshYoung Adult-
dc.titleOral features and dental health in Hurler Syndrome following hematopoietic stem cell transplantation.en
dc.typeArticleen
dc.contributor.departmentDental Department, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland. eleanor.mcgovern@olchc.ieen
dc.identifier.journalInternational journal of paediatric dentistry / the British Paedodontic Society [and] the International Association of Dentistry for Childrenen
dc.description.provinceLeinster-

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