Papillon Le-Fèvre Syndrome : associations to clinical and laboratory factors and results of treatment

The aim of this thesis was to elucidate factors possibly contributing to the rapidly progressing periodontitis in a group of Papillon-Lefèvre syndrome (PLS) patients and to evaluate a combined mechanical and antimicrobial treatment model in these patients. The total patient sample consisted of 20 individuals referred to King Faisal Specialist Hospital and Research Centre in Saudi Arabia for treatment of PLS. Saliva samples were obtained from 16 patients with the diagnosis of PLS. A control group was selected from healthy siblings and healthy children and young adults from Saudi Arabia. Both the unstimulated and the stimulated whole saliva secretion rates were significantly lower in PLS-patients compared to control individuals (p<0.01 and p< 0.05). The buffer capacity was also significantly lower (p<0.01), whereas IgA and lactoferrin concentrations were significantly higher (p<0.01) in the PLS-group. A significantly lower output was detected for peroxidase when calculating the amount or activity per time among the PLS-patients compared to controls. Subgingival microbial samples were obtained from 36 sites in 12 PLS patients in order to assess the occurrence of 18 different microbial species. Actinobacillus actinomycetemcomitans and P. gingivalis were only infrequently encountered in high levels in the subgingival samples whereas B. forsythus, T.denticola, P. intermedia and C. rectus reached high levels (≥10 6cells) in more then 50% of all patients. The results of the study indicate that there is no specific profile of the subgingival microflora in PLS patients. Blood samples were obtained from all 20 PLS individuals and from an equal number of healthy volunteers. Natural Killer cells (NK cells) from the PLS subjects exhibited significantly reduced cytotoxicity against K562 cells compared to controls (p