title: Dental aspects in patients with DiGeorge syndrome.
abstract: DiGeorge syndrome, which is caused by a microdeletion of 1.5 to 3.0 megabases on the long arm of chromosome 22, has an incidence of approximately 1:4,000 to 1:5,000 live births. The phenotypic spectrum of this disorder includes congenital heart defects, immunodeficiency due to thymic hypoplasia or aplasia, transient or permanent hypocalcemia due to parathyroid hypoplasia or aplasia, developmental retardation, and psychiatric disorders. Dental aspects in these patients include skeletal malformations, velopharyngeal insufficiency with or without cleft palate, small mouth, and hypotonus orofacial musculature, as well as impaired salivary flow. Enamel aberrations related to hypocalcemia may result in a higher frequency of dental caries. Based on a series of five patients, the medical and dental aspects that have to be considered in the care of patients with DiGeorge syndrome are presented.
authors.affiliation: Department of Pediatric Cardiology, Children's Hospital University of Erlangen-Nuremberg, Germany.
authors.forename: Okan
authors.initials: O
authors.lastname: Toka
authors.forename: Matthias
authors.initials: M
authors.lastname: Karl
authors.forename: Sven
authors.initials: S
authors.lastname: Dittrich
authors.forename: Stefan
authors.initials: S
authors.lastname: Holst
authors.forename: Alexandra
authors.initials: A
authors.lastname: Holst
mesh_terms: D000293:Adolescent; D002648:Child; D004245:DMF Index; D004062:DiGeorge Syndrome / Q000150:complications* / Q000235:genetics; D004200:Diseases in Twins; D005146:Facial Asymmetry / Q000209:etiology; D005260:Female; D006801:Humans; D008297:Male; D008310:Malocclusion / Q000209:etiology; D009056:Mouth Abnormalities / Q000209:etiology*; D009911:Oral Hygiene Index; D010512:Periodontal Index; D018570:Risk Assessment; D014071:Tooth Abnormalities / Q000209:etiology*; D014430:Twins, Monozygotic