title: Oral human papillomavirus is common in individuals with Fanconi anemia.
abstract: BACKGROUND
  Fanconi anemia is a rare genetic disorder resulting in a loss of function of the Fanconi anemia-related DNA repair pathway. Individuals with Fanconi anemia are predisposed to some cancers, including oropharyngeal and gynecologic cancers, with known associations with human papillomavirus (HPV) in the general population. As individuals with Fanconi anemia respond poorly to chemotherapy and radiation, prevention of cancer is critical.
  
  
  METHODS
  To determine whether individuals with Fanconi anemia are particularly susceptible to oral HPV infection, we analyzed survey-based risk factor data and tested DNA isolated from oral rinses from 126 individuals with Fanconi anemia and 162 unaffected first-degree family members for 37 HPV types.
  
  
  RESULTS
  Fourteen individuals (11.1%) with Fanconi anemia tested positive, significantly more (P = 0.003) than family members (2.5%). While HPV prevalence was even higher for sexually active individuals with Fanconi anemia (17.7% vs. 2.4% in family; P = 0.003), HPV positivity also tended to be higher in the sexually inactive (8.7% in Fanconi anemia vs. 2.9% in siblings). Indeed, having Fanconi anemia increased HPV positivity 4.9-fold (95% CI, 1.6-15.4) considering age and sexual experience, but did not differ by other potential risk factors.
  
  
  CONCLUSIONS
  Our studies suggest that oral HPV is more common in individuals with Fanconi anemia. It will be essential to continue to explore associations between risk factors and immune dysfunction on HPV incidence and persistence over time.
  
  
  CONCLUSIONS
  HPV vaccination should be emphasized in those with Fanconi anemia as a first step to prevent oropharyngeal cancers, although additional studies are needed to determine whether the level of protection it offers in this population is adequate.
authors: Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio.
authors: Sharon L
authors: SL
authors: Sauter
authors: Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio.
authors: Susanne I
authors: SI
authors: Wells
authors: Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio.
authors: Xue
authors: X
authors: Zhang
authors: Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio.
authors: Elizabeth E
authors: EE
authors: Hoskins
authors: Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio.
authors: Stella M
authors: SM
authors: Davies
authors: Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio.
authors: Kasiani C
authors: KC
authors: Myers
authors: Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio.
authors: Robin
authors: R
authors: Mueller
authors: Chronic Viral Diseases Branch, National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, Georgia.
authors: Gitika
authors: G
authors: Panicker
authors: Chronic Viral Diseases Branch, National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, Georgia.
authors: Elizabeth R
authors: ER
authors: Unger
authors: Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio.
authors: Umasundari
authors: U
authors: Sivaprasad
authors: Division of Infectious Diseases, Indiana University School of Medicine, Indianapolis, Indiana.
authors: Darron R
authors: DR
authors: Brown
authors: Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio.
authors: Parinda A
authors: PA
authors: Mehta
authors: Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio. melinda.butsch.kovacic@cchmc.org.
authors: Melinda
authors: M
authors: Butsch Kovacic
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005199:Fanconi Anemia / Q000821:virology*; D005260:Female; D006258:Head and Neck Neoplasms / Q000821:virology; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D008875:Middle Aged; D009059:Mouth Diseases / Q000821:virology*; D009061:Mouth Mucosa / Q000821:virology; D027383:Papillomaviridae / Q000302:isolation & purification*; D030361:Papillomavirus Infections / Q000821:virology*; D055815:Young Adult