title: Elevated hydroperoxide levels and antioxidant patterns in Papillon-Lefèvre syndrome.
abstract: BACKGROUND
  Since it has been found that reactive oxygen species seem to be involved in the pathogenesis of both periodontitis and hyperkeratotic syndromes, we studied a group of patients belonging to 3 generations of a family with different degrees of severity of Papillon-Lefèvre syndrome (PLS) to ascertain whether altered concentrations of the most important hydrophobic and hydrophilic plasma antioxidants as well as products of oxidative damage are present in PLS.
  
  
  METHODS
  Coenzyme Q (CoQ), vitamin E, glutathione (GSH), and uric acid were evaluated by high-performance liquid chromatography (HPLC) (supplied with electrochemical detector) techniques and hydroperoxides by a spectrophotometric method.
  
  
  RESULTS
  GSH and uric acid were in the range of reference values; CoQ was very low in both the child of the third generation and his mother, and these 2 subjects had the highest hydroperoxide levels. The child also had extremely low values of vitamin E. In general, all family members showed abnormally high hydroperoxide levels, with the exception of those members who are phenotypically healthy.
  
  
  CONCLUSIONS
  Since the subjects with the lowest hydroperoxide contents are phenotypically healthy, whereas the affected individuals presented lower antioxidant levels and very high hydroperoxide concentrations, it has been suggested that a specific antioxidant therapy could be a promising approach in treating some PLS subjects. Moreover, unexpected manifestations of heterozygosity in the child of the third generation were also detected.
authors.affiliation: Institute of Biochemistry, Faculty of Medicine, School of Medicine, University of Ancona, Italy. mbattino@popcsi.unian.it
authors.forename: M
authors.initials: M
authors.lastname: Battino
authors.forename: M S
authors.initials: MS
authors.lastname: Ferreiro
authors.forename: S
authors.initials: S
authors.lastname: Bompadre
authors.forename: L
authors.initials: L
authors.lastname: Leone
authors.forename: F
authors.initials: F
authors.lastname: Mosca
authors.forename: P
authors.initials: P
authors.lastname: Bullon
mesh_terms: D000328:Adult; D000975:Antioxidants / Q000032:analysis*; D002675:Child, Preschool; D005260:Female; D005978:Glutathione / Q000097:blood; D006801:Humans; D008054:Lipid Peroxides / Q000097:blood; D008297:Male; D018384:Oxidative Stress; D010214:Papillon-Lefevre Disease / Q000097:blood* / Q000378:metabolism; D010375:Pedigree; D010545:Peroxides / Q000097:blood*; D014451:Ubiquinone / Q000097:blood; D014527:Uric Acid / Q000097:blood; D014810:Vitamin E / Q000097:blood
chemical_list: D000975:Antioxidants; D008054:Lipid Peroxides; D010545:Peroxides; D014451:Ubiquinone; D014810:Vitamin E; D014527:Uric Acid; D005978:Glutathione