title: Periodontal status of patients with dentin dysplasia type I: report of three cases within a family.
abstract: BACKGROUND
  Dentin dysplasia type I (DDI) is a rare hereditary disturbance of dentin formation. It is characterized by clinically normal-appearing crowns; obliteration of pulp chambers; and short, blunted and malformed roots that are commonly associated with periodontal attachment loss (PAL). In this context, we report three cases within a family with similar clinical and radiographic features of DDI but with differing microbiologic and periodontal conditions.
  
  
  METHODS
  A 42-year-old white female and her two daughters (25 and 10 years of age) presented with a diagnosis of DDI. Probing depth (PD), clinical attachment level (CAL), visible plaque, and bleeding on probing (BOP) were recorded. Subgingival biofilm samples were randomly collected and analyzed by checkerboard DNA-DNA hybridization.
  
  
  RESULTS
  The mother presented 34.9% of sites with PD > or =4 mm, 41.3% of sites with CAL > or =4 mm, and 57% of sites with BOP; both daughters presented no sites with PD or CAL >3 mm and <10% of sites with BOP. Microbiologic analysis detected Gemella morbillorum, Neisseria mucosa, and Staphylococcus aureus in > or =50% of the mother's samples. The daughters showed high levels (>10(4) bacterial cells) of some periodontopathic bacteria, including members of the red (Porphyromonas gingivalis) and orange (Fusobacterium periodonticum and F. nucleatum polymorphum) complexes and beneficial species of the yellow (Streptococcus gordonii) and purple (Veillonella parvula) complexes. The mother presented high mean levels only for four tested species (N. mucosa, Prevotella melaninogenica, Treponema denticola, and V. parvula).
  
  
  CONCLUSIONS
  A combination of radiographs, microbiologic analysis, and preventive professional monitoring care is important to avoid PAL and to provide oral health in patients with DDI.
authors.affiliation: Division of Graduate Periodontics, School of Dentistry, Federal University of Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
authors.forename: Lorena
authors.initials: L
authors.lastname: Da Rós Gonçalves
authors.forename: Cristiana Aroeira G R
authors.initials: CA
authors.lastname: Oliveira
authors.forename: Rose
authors.initials: R
authors.lastname: Holanda
authors.forename: Carina M
authors.initials: CM
authors.lastname: Silva-Boghossian
authors.forename: Ana Paula Vieira
authors.initials: AP
authors.lastname: Colombo
authors.forename: Lucianne Cople
authors.initials: LC
authors.lastname: Maia
authors.forename: Eduardo Jorge
authors.initials: EJ
authors.lastname: Feres-Filho
mesh_terms: D000328:Adult; D018441:Biofilms; D002648:Child; D003773:Dental Plaque / Q000382:microbiology; D003774:Dental Plaque Index; D003805:Dentin Dysplasia / Q000145:classification / Q000235:genetics*; D005260:Female; D005673:Fusobacterium / Q000302:isolation & purification; D016967:Fusobacterium nucleatum / Q000302:isolation & purification; D005884:Gingival Hemorrhage / Q000235:genetics; D006801:Humans; D042807:Neisseria mucosa / Q000302:isolation & purification; D017622:Periodontal Attachment Loss / Q000235:genetics; D010510:Periodontal Diseases / Q000235:genetics* / Q000382:microbiology; D010514:Periodontal Pocket / Q000235:genetics; D016966:Porphyromonas gingivalis / Q000302:isolation & purification; D001443:Prevotella melaninogenica / Q000302:isolation & purification; D040141:Staphylococcaceae / Q000302:isolation & purification; D013211:Staphylococcus aureus / Q000302:isolation & purification; D054773:Streptococcus gordonii / Q000302:isolation & purification; D048373:Treponema denticola / Q000302:isolation & purification; D014678:Veillonella / Q000302:isolation & purification