publication . Article . 2012

Seckel syndrome: a report of a case.

S Swathi; K Ramalingam; M Govindarajan; SD Kaliyamurthy;
Open Access
  • Published: 01 Jan 2012 Journal: Journal of Indian Society of Pedodontics and Preventive Dentistry, volume 30, page 258 (issn: 0970-4388, Copyright policy)
  • Publisher: Medknow
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.
Subjects
Medical Subject Headings: stomatognathic diseasesstomatognathic system
free text keywords: General Dentistry, "Bird-headed" dwarf, mental retardation, Dentistry, RK1-715, Prominent eyes, Seckel syndrome, medicine.disease, medicine, Audiology, medicine.medical_specialty, Second primary cancer, Forehead, medicine.anatomical_structure, Dominance (genetics), Dentition, Hypoplastic Enamel, Microcephaly, Pediatrics, business.industry, business
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