title: Malignant infantile osteopetrosis: dental effects in paediatric patients. Case reports.
abstract: OBJECTIVE
  Malignant Infantile Osteopetrosis is a hereditary pathology caused due to osteoclastic cells which are incapable of carrying out their functions and hence do not resorb osseous tissue where required. Thus the consequence is that during growth phase, the medullary cavities and nervous tissue cavities do not undergo sufficient growth and the corresponding organs do not develop adequately. The aim of this study is to outline the role of the pediatric dentist who has to carry out protocols of primary, secondary, tertiary prevention intervening at many levels. Clinical features and dental effects are described. Two case reports are presented in this study.
  
  
  CONCLUSIONS
  Oral problems of osteopetrosis are delayed tooth eruption, absence of some teeth, malformed teeth, enamel hypoplasia, disturbed dentinogenesis, hypomineralisation of enamel and dentin, propensity for tooth decay, defects of the periodontal membrane, thickened lamina dura, mandibular protrusion, and the presence of odontomas. Tooth removal should be limited as it may induce bone fractures and osteomyelitis. The role of the pediatric dentist is defined.
authors.affiliation: University of Rome La Sapienza, Department of Paediatric Dentistry.
authors.forename: V
authors.initials: V
authors.lastname: Luzzi
authors.forename: G
authors.initials: G
authors.lastname: Consoli
authors.forename: V
authors.initials: V
authors.lastname: Daryanani
authors.forename: G
authors.initials: G
authors.lastname: Santoro
authors.forename: G L
authors.initials: GL
authors.lastname: Sfasciotti
authors.forename: A
authors.initials: A
authors.lastname: Polimeni
mesh_terms: D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D017742:Dental Care for Chronically Ill; D003744:Dental Enamel Hypoplasia / Q000209:etiology*; D005094:Exophthalmos / Q000209:etiology; D005146:Facial Asymmetry / Q000209:etiology*; D005260:Female; D006801:Humans; D007569:Jaw Abnormalities / Q000209:etiology*; D010022:Osteopetrosis / Q000150:complications* / Q000151:congenital / Q000601:surgery; D014071:Tooth Abnormalities / Q000209:etiology*