title: Seckel syndrome: a report of a case.
abstract: Seckel syndrome, first defined by Seckel in 1960, is a rare (incidence 1:10,000), genetically heterogeneous autosomal recessive disorder presenting at birth. This syndrome is characterized by a proportionate dwarfism of prenatal onset, a severe microcephaly with a "bird-headed" like appearance (beaked nose, receding forehead, prominent eyes, and micrognathia), and mental retardation. The significance of dental alterations in this syndrome resides in the defect, hypoplastic enamel, being limited to the primary dentition; in most instances the second primary molar tooth is not affected. A case of the Seckel syndrome is presented.
authors.affiliation: Department of Pedodontics and Preventive Dentistry, Rajah Muthiah Dental College and Hospital, Annamalai University, Chidambaram, Cuddalore, Tamil Nadu, India.
authors.forename: K
authors.initials: K
authors.lastname: Ramalingam
authors.forename: S D
authors.initials: SD
authors.lastname: Kaliyamurthy
authors.forename: M
authors.initials: M
authors.lastname: Govindarajan
authors.forename: S
authors.initials: S
authors.lastname: Swathi
mesh_terms: D002613:Cheilitis / Q000175:diagnosis; D002648:Child; D017742:Dental Care for Chronically Ill; D003730:Dental Care for Persons with Disabilities; D003731:Dental Caries / Q000175:diagnosis; D004392:Dwarfism / Q000175:diagnosis*; D019066:Facies; D005260:Female; D005891:Gingivitis / Q000175:diagnosis; D006801:Humans; D008607:Intellectual Disability / Q000175:diagnosis*; D008312:Malocclusion, Angle Class II / Q000175:diagnosis; D008831:Microcephaly / Q000175:diagnosis*; D009059:Mouth Diseases / Q000175:diagnosis*; D011671:Pulpitis / Q000175:diagnosis; D014076:Tooth Diseases / Q000175:diagnosis*; D014082:Tooth Fractures / Q000175:diagnosis