title: Morphological appearance and chemical composition of enamel in primary teeth from patients with 22q11 deletion syndrome.
abstract: Patients with 22q11 deletion syndrome have many and complex medical problems, including hypocalcemia and/or hypoparathyroidism. Odontological findings include enamel aberrations in both dentitions. In order to describe enamel morphology, chemical composition in primary teeth, and to investigate the relationship between medical history and morphological appearance, dental enamel was investigated in 38 exfoliated primary teeth from 15 children and adolescents. Morphology was studied by the use of a polarized light microscope, microradiography, scanning electron microscopy, X-ray microanalysis, and secondary ion mass spectrometry. The morphological findings were compared with medical history. The teeth showed, in principle, a normal morphological appearance with regard to prism structure. A high frequency of aberrations, such as hypomineralization, hypoplasia and extra incremental lines, were found. The majority of the aberrations were found around the neonatal line. There was a relationship between high numbers of medical problems in the patients and enamel deviations. The result supports the hypothesis of under-reporting of both hypocalcemia and hypoparathyroidism in patients with 22q11 deletion syndrome.
authors.affiliation: Mun-H-Center, National Orofacial Resource Center for Rare Disorders, Göteborg, Sweden. gunilla.klingberg@vgregion.se
authors.forename: Gunilla
authors.initials: G
authors.lastname: Klingberg
authors.forename: Wolfram
authors.initials: W
authors.lastname: Dietz
authors.forename: Sólveig
authors.initials: S
authors.lastname: Oskarsdóttir
authors.forename: Hans
authors.initials: H
authors.lastname: Odelius
authors.forename: Lars
authors.initials: L
authors.lastname: Gelander
authors.forename: Jörgen G
authors.initials: JG
authors.lastname: Norén
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002872:Chromosome Deletion; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics*; D003743:Dental Enamel / Q000002:abnormalities* / Q000737:chemistry; D005260:Female; D006801:Humans; D006996:Hypocalcemia / Q000150:complications / Q000235:genetics; D007037:Hypothyroidism / Q000150:complications / Q000235:genetics; D008297:Male; D012720:Severity of Illness Index; D013577:Syndrome; D014071:Tooth Abnormalities / Q000150:complications / Q000235:genetics*; D014094:Tooth, Deciduous / Q000737:chemistry / Q000473:pathology*