title: Evaluation of oral and systemic manifestations in an amelogenesis imperfecta population.
abstract: OBJECTIVE
  The aim of this investigation was to describe the dental and craniofacial characteristics of patients with amelogenesis imperfecta (AI).
  
  
  METHODS
  The study group included 43 patients(33 female and 10 male) with a mean age of 11.4+/-2.6 years. A panoramic and a cephalometric radiograph were obtained from each of these patients. Clinically AI cases were divided into four main groups according to Witkop. All patients were evaluated for chronological, bone and dental age. The patients who had severe retarded bone age were evaluated for plasma growth hormone(GH) concentrations.
  
  
  RESULTS
  Dental and bone ages were retarded with respect to chronological age in five patients. Dental maturity and tooth eruption were not age- appropriate in some of our patients. In type III AI patients a delay in skeletal age was observed. Severe late eruption was seen in 3 patients, severe delay in dental maturity was noted in patients with type IV AI. Dental age was clinically lower in GH-deficient subjects, and skeletal age was consistently more retarded than dental age when compared to chronological age. Anterior open bite was present in both primary and permanent dentitions of 50% of the patients with type I AI, 30.8% of the patients with type II AI, and 60% of type III AI.
  
  
  CONCLUSIONS
  It is concluded that the primary structure for the classification of AI be based on the mode of inheritance, with the clinical and radiographic appearances (and any other features such as systemic findings) being the secondary discriminators.
authors.affiliation: Department of Pedodontics, Faculty of Dentistry, University of Istanbul, Istanbul 34 390, Turkey. gamzearen@hotmail.com
authors.forename: Gamze
authors.initials: G
authors.lastname: Aren
authors.forename: Didem
authors.initials: D
authors.lastname: Ozdemir
authors.forename: Sonmez
authors.initials: S
authors.lastname: Firatli
authors.forename: Cem
authors.initials: C
authors.lastname: Uygur
authors.forename: Elif
authors.initials: E
authors.lastname: Sepet
authors.forename: Erhan
authors.initials: E
authors.lastname: Firatli
mesh_terms: D000365:Age Determination by Skeleton; D000366:Age Determination by Teeth; D000367:Age Factors; D000567:Amelogenesis Imperfecta / Q000145:classification* / Q000473:pathology; D002508:Cephalometry; D002648:Child; D005260:Female; D019382:Human Growth Hormone / Q000097:blood / Q000172:deficiency; D006801:Humans; D008297:Male; D009059:Mouth Diseases / Q000145:classification* / Q000473:pathology; D024343:Open Bite / Q000145:classification; D011862:Radiography, Panoramic; D014076:Tooth Diseases / Q000145:classification* / Q000473:pathology; D014078:Tooth Eruption / Q000502:physiology; D014094:Tooth, Deciduous / Q000473:pathology
chemical_list: D019382:Human Growth Hormone