Dental anomalies in Axenfeld-Rieger syndrome.
The authors describe the case of a 10-year-old girl presenting with Axenfeld-Rieger syndrome (ARS), a rare autosomal dominant condition. The patient showed severe hypodontia, microdontia and short roots. Early diagnosis of the syndrome from its dento-facial and systemic features is important so that subsequent ocular complications may be prevented.
O'Dwyer
E M
EM
Department of Oral and Maxillofacial Surgery, Arrowe Park Hospital, Upton, UK.
Jones
D C
DC
D000015:Abnormalities, Multiple / Q000235:genetics; D000848:Anodontia / Q000235:genetics; D000869:Anterior Eye Segment / Q000002:abnormalities; D002648:Child; D002894:Chromosomes, Human, Pair 4 / Q000235:genetics; D002896:Chromosomes, Human, Pair 6 / Q000235:genetics; D017742:Dental Care for Chronically Ill; D005124:Eye Abnormalities / Q000235:genetics; D019066:Facies; D005260:Female; D051858:Forkhead Transcription Factors / Q000235:genetics; D005799:Genes, Dominant; D018398:Homeodomain Proteins / Q000235:genetics; D006801:Humans; D009154:Mutation; D013577:Syndrome; D014071:Tooth Abnormalities / Q000235:genetics; D014157:Transcription Factors / Q000235:genetics; D000097577:Homeobox Protein PITX2
D051858:Forkhead Transcription Factors; D018398:Homeodomain Proteins; D014157:Transcription Factors; D000097577:Homeobox Protein PITX2; C112743:FOXC1 protein, human