title: Systemic disorders and their influence on the development of dental hard tissues: a literature review.
abstract: OBJECTIVE
  This report highlights the influence of a number of disorders with systemic physiological effects that impact on the development of dental hard tissues. It focuses in particular, on the pathological effects of systemic conditions with less well recognised, but no less important, impacts on dental development. Such conditions, include cystic fibrosis, HIV/AIDS, leukaemia, Alstrom syndrome, hypophosphatasia, Prader-Willi syndrome, Tricho-dento-osseous syndrome, tuberous sclerosis, familial steroid dehydrogenase deficiency and epidermolysis bullosa. These, along with developmental and environmental causes of enamel and dentine defects, are discussed and the possible aetiology of such effects are proposed. Furthermore, the dental management and long-term dental care of these patients is outlined.
  
  
  METHODS
  MEDLINE/PubMed.
  
  
  CONCLUSIONS
  Enamel and dentine defects can present with a wide spectrum of clinical features and may be caused by a variety of factors occurring throughout tooth development from before birth to adulthood. These may include host traits, genetic factors, immunological responses to cariogenic bacteria, saliva composition, environmental and behavioural factors and systemic diseases. These diseases and their spectrum of clinical manifestations on the organs affected (including the dentition) require an increased knowledge by dental practitioners of the disease processes, aetiology, relevant treatment strategies and prognosis, and must encompass more than simply the management of the dental requirements of the patient. It is important that the impact of the disease and its treatment, particularly in respect of immunosuppression where dental interventions may become life-threatening, is also taken into consideration.
authors.affiliation: New York University NYU, College of Dentistry, Department of Pediatric Dentistry, New York, USA. atardrs@yahoo.com
authors.forename: Michael
authors.initials: M
authors.lastname: Atar
authors.forename: Egbert J
authors.initials: EJ
authors.lastname: Körperich
mesh_terms: D015096:3-Hydroxysteroid Dehydrogenases / Q000172:deficiency; D000163:Acquired Immunodeficiency Syndrome / Q000503:physiopathology; D056769:Alstrom Syndrome / Q000503:physiopathology; D003550:Cystic Fibrosis / Q000503:physiopathology; D004194:Disease; D004476:Ectodermal Dysplasia / Q000503:physiopathology; D004820:Epidermolysis Bullosa / Q000503:physiopathology; D015658:HIV Infections / Q000503:physiopathology; D006801:Humans; D007014:Hypophosphatasia / Q000503:physiopathology; D007938:Leukemia / Q000503:physiopathology; D009805:Odontogenesis / Q000502:physiology*; D011218:Prader-Willi Syndrome / Q000503:physiopathology; D014402:Tuberous Sclerosis / Q000503:physiopathology
chemical_list: D015096:3-Hydroxysteroid Dehydrogenases