title: Dentinogenesis imperfecta in children with osteogenesis imperfecta: a clinical and ultrastructural study.
abstract: OBJECTIVE
  The aim of this study was to assess the correlation between osteogenesis imperfecta (OI) and dentinogenesis imperfecta (DI) from both a clinical and histological point of view, particularly clarifying the structural and ultrastructural dentine changes.
  
  
  METHODS
  Sixteen children (6-12 years aged) with diagnosis of OI were examined for dental alterations referable to DI. For each patient, the OI type (I, III, or IV) was recorded. Extracted or normally exfoliated primary teeth were subjected to a histological examination (to both optical microscopy and confocal laser-scanning microscopy).
  
  
  RESULTS
  A total of ten patients had abnormal discolourations referable to DI: four patients were affected by OI type I, three patients by OI type III, and three patients by OI type IV. The discolourations, yellow/brown or opalescent grey, could not be related to the different types of OI. Histological exam of primary teeth showed severe pathological change in the dentin, structured into four different layers. A collagen defect due to odontoblast dysfunction was theorized to be on the base of the histological changes.
  
  
  CONCLUSIONS
  There is no correlation between the type of OI and the type of discolouration. The underlying dentinal defect seems to be related to an odontoblast dysfunction.
authors.affiliation: Department of Pediatric Dentistry, Dental School, University of Brescia, Brescia, Italy. majorana@med.unibs.it
authors.forename: Alessandra
authors.initials: A
authors.lastname: Majorana
authors.forename: Elena
authors.initials: E
authors.lastname: Bardellini
authors.forename: Pier Carlo
authors.initials: PC
authors.lastname: Brunelli
authors.forename: Mariagrazia
authors.initials: M
authors.lastname: Lacaita
authors.forename: Angela Pia
authors.initials: AP
authors.lastname: Cazzolla
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Favia
mesh_terms: D002648:Child; D003804:Dentin / Q000473:pathology* / Q000648:ultrastructure*; D003811:Dentinogenesis Imperfecta / Q000145:classification / Q000150:complications* / Q000473:pathology*; D019229:Dentition, Permanent; D005260:Female; D006801:Humans; D008297:Male; D018613:Microscopy, Confocal; D010013:Osteogenesis Imperfecta / Q000145:classification / Q000150:complications* / Q000473:pathology; D014094:Tooth, Deciduous