title: Coffin-Lowry syndrome: findings and dental treatment.
abstract: Coffin-Lowry Syndrome is characterized by mental retardation, skeletal abnormalities, delayed bone development, short stature, tapered fingers, large ears, orbital hypertelorism, anteverted nares, and a prominent frontal region. This inherited disorder is x-linked and is genetically mapped to the Xp22.2 chromosomal region. This report presents the dental findings of three male members of the same family: an older brother and younger identical twins; all three were first examined at age 10. The father's family came from North Africa and the mother's from Eastern Europe. In addition to the reported typical characteristics of Coffin-Lowry Syndrome, a number of specific dental findings were found in all of the brothers, namely, maxillary protrusion, open bite, two-year retardation in age of eruption, agenesis of the permanent mandibular central incisors in all of the brothers, and a marked oligodontia in the oldest. Despite difficulties in management, the dental treatment for caries and gingivitis could be completed on the brothers without sedation.
authors.affiliation: Department of Pediatric Dentistry, The Maurice and Gabriela Goldschleger School of Dental Medicine, Tel Aviv University, Tel Aviv, Israel. dodew@post.tau.ac.il
authors.forename: David
authors.initials: D
authors.lastname: Wasersprung
authors.forename: Haim
authors.initials: H
authors.lastname: Sarnat
mesh_terms: D000848:Anodontia / Q000150:complications* / Q000000981:diagnostic imaging; D002648:Child; D038921:Coffin-Lowry Syndrome / Q000150:complications* / Q000000981:diagnostic imaging; D019140:Dental Care for Children; D017742:Dental Care for Chronically Ill; D019066:Facies; D006801:Humans; D007180:Incisor / Q000000981:diagnostic imaging; D008297:Male; D008310:Malocclusion / Q000150:complications*; D008437:Maxilla / Q000000981:diagnostic imaging; D019767:Maxillofacial Abnormalities / Q000150:complications; D011378:Prognathism / Q000150:complications; D011859:Radiography; D014078:Tooth Eruption