Dental maturity is advanced in Fragile X syndrome.
Fragile X (FraX) syndrome is the most common cause of inherited mental retardation. The FraX gene (FMR1) has been cloned, and the mutation causing the disease is now known. We estimated the effect of FraX on dental development in 28 affected boys (aged 4.9-17.6 years) and three carrier girls (aged 5.8, 10.4, and 12.7 years). Dental maturity, assessed on the basis of formation [Demirjian A, Goldstein H. 1976: Ann Hum Biol 3:411-421] and of emergence [Hägg U, Taranger J. 1985: Angle Orthod 55:93-107], was compared with growth in stature and skeletal maturity. The mean relative dental age was advanced in FraX males, based on formation (+1.4 SD) and on emergence (+1.1 SD). More pronounced advancement was seen in younger children. Dental maturity was advanced in heterozygous carrier girls as well. Height and skeletal maturity did not show a similar trend toward advanced development.
Kotilainen
J
J
Department of Pedodontics and Orthodontics, Institute of Dentistry, University of Helsinki, Finland.
Pirinen
S
S
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005600:Fragile X Syndrome / Q000503:physiopathology*; D006801:Humans; D008297:Male; D014070:Tooth / Q000254:growth & development*