title: Aggressive periodontitis associated with Fanconi's anemia. A case report.
abstract: BACKGROUND
  Fanconi's anemia is an autosomal recessive disease associated with chromosomal breakage as well as pancytopenia, skin pigmentation, renal hypoplasia, cardiac defects, microcephaly, congenital malformations of the skeleton, hypogonadism, and increased risk of leukemia. The present report describes the periodontal clinical and microbiological status of an 11-year old male having Fanconi's anemia.
  
  
  METHODS
  Polymerase chain reaction analysis to detect human cytomegalovirus (HCMV), Epstein-Barr type 1 virus, and herpes simplex virus (HSV) was performed on paper-point samples pooled from either 3 periodontal sites with advanced attachment loss or 3 gingivitis sites with no clinical attachment loss. Anaerobic bacterial culture examination was performed on the pooled periodontitis sample.
  
  
  RESULTS
  The patient suffered from pancytopenia, allergy, asthma, hearing impairment, and mental retardation. Dentition consisted of 7 primary teeth, 11 erupted permanent teeth, and 14 unerupted permanent teeth. Most erupted teeth showed severe gingival inflammation with some gingival overgrowth and various degrees of periodontal attachment loss. Genomes of HCMV and HSV were detected in the pooled periodontitis sample and HCMV in the pooled gingivitis sample. The periodontitis sample but not the gingivitis sample revealed HCMV mRNA of major capsid protein, suggestive of active viral infection. The periodontitis sample also yielded Actinobacillus actinomycetemcomitans (1.1% of total isolates), FusobActerium species (7.9%), Campylobacter species (2.2%), Peptostreptococcus micros (3.4%), and Candida albicans (0.3%).
  
  
  CONCLUSIONS
  Oral features of Fanconi's anemia may include increased susceptibility to periodontitis. It is likely that underlying host defense impairment coupled with periodontal infection by HCMV and A. actinomycetemcomitans contribute to the severe type of periodontitis associated with Fanconi's anemia.
authors.affiliation: University of Southern California School of Dentistry, Los Angeles, USA.
authors.forename: H
authors.initials: H
authors.lastname: Nowzari
authors.forename: M G
authors.initials: MG
authors.lastname: Jorgensen
authors.forename: T T
authors.initials: TT
authors.lastname: Ta
authors.forename: A
authors.initials: A
authors.lastname: Contreras
authors.forename: J
authors.initials: J
authors.lastname: Slots
mesh_terms: D000189:Actinobacillus Infections / Q000150:complications; D016976:Aggregatibacter actinomycetemcomitans / Q000254:growth & development; D002169:Campylobacter Infections / Q000150:complications; D002648:Child; D003586:Cytomegalovirus Infections / Q000150:complications; D020031:Epstein-Barr Virus Infections / Q000150:complications; D005199:Fanconi Anemia / Q000150:complications*; D005674:Fusobacterium Infections / Q000150:complications; D005891:Gingivitis / Q000821:virology; D016908:Gram-Positive Bacterial Infections / Q000150:complications; D006561:Herpes Simplex / Q000150:complications; D006801:Humans; D008297:Male; D010462:Peptostreptococcus; D017622:Periodontal Attachment Loss / Q000821:virology; D010518:Periodontitis / Q000209:etiology* / Q000401:mortality / Q000821:virology