| 1. |
- Ullbro, Christer, et al.
(författare)
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Dental treatment for young patients with Papillon-Lefèvre syndrome
- 2006
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Ingår i: Eur Arch Paediatr Dent. ; 7:228-235
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Tidskriftsartikel (refereegranskat)abstract
- The aim of this article is to present a review of the most current literature regarding the aetiology of the periodontal disease that occurs in patients with Papillon-Lefèvre syndrome (PLS). It is also to encourage clinicians to adopt atreatment regime intending to save permanent teeth and alveolar bone in young patients with PLS. Review: The phenotypic expression of PLS is heterogeneous as regards the severity of the dermatological as well as the periodontal symptoms. The genetic defect is lack-of-function mutations of the gene encoding for cathepsin C, a lysosomal cysteine proteinase. The defect is associated with a severe reduction in the levels and activities of neutrophil-derived serine proteases (cathepsin G, elastase, Proteinase 3) and in the activation of granzyme B in natural killer cells. About 20 % of patients with PLS experience infections besides the periodontal infection, although the patients are not known to be unusually susceptible to viral infections. The reason why many patients with PLS do develop periodontal inflammation is most likely due to the fact that the amount of aerobic and anaerobic pathogens in the periodontal pocket overpower a somewhat weakened innate immune response. Based on this literature review a standardised dental treatment protocol for patients with PLS is suggested. Conclusion: It is evident that by regularly reducing the periopathogenic flora in the oral cavity and by instigating a potent antibiotic therapy at an early stage of any potential infection, patients with PLS are not predestined to be edentulous in the permanent dentition.
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| 2. |
- Ullbro, Christer, et al.
(författare)
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Phenotypic Variation and Allelic Heterogeneity in Young Patients with Papillon-Lefèvre Syndrome.
- 2006
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Ingår i: Acta Dermato-Venereologica. ; 8, s. 3-7
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Tidskriftsartikel (refereegranskat)abstract
- Papillon-Lefèvre syndrome is an autosomal recessive disorder characterized by palmoplantar hyperkeratosis and aggressive periodontitis. The aim of the study was to identify underlying cathepsin C mutations in 39 subjects with Papillon-Lefèvre syndrome and to explore any phenotypic associations. Genotyping and mutation analyses were performed using standard molecular techniques, and dermatological and oral characteristics were assessed with a semiquantitative clinical score. Three genotypes were present at microsatellite marker D11S1780 and two underlying mutations were identified. The most common genotype (183/183) was associated with an 815G → C mutation in exon 6 resulting in an arginine to proline change at amino acid 272 (R272P). Patients with the 173/173 genotype revealed an exon 7 G300D mutation resulting in a glycine to aspartic acid change at amino acid 300. The mutation in a family with 189/189 genotype remained unknown. A significant difference in hyperkeratosis of the feet was found between the patients with mutations G300D and R272P (p <0.05), but not regarding hands or periodontal condition. Young girls displayed significantly less palmoplantar hyperkeratosis ( p <0.05) than young boys. In conclusion, considerable phenotypic heterogeneity was observed within the two cardinal mutations and in the 189/189 genotype.
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| 3. |
- Ullbro, Christer, et al.
(författare)
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Preventive periodontal regimen in Papillon-Lefèvre syndrome
- 2005
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Ingår i: The Journal of clinical pediatric dentistry. ; 27:3, s. 226-32
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Tidskriftsartikel (refereegranskat)abstract
- PURPOSE: The purpose of this study was to evaluate the effect of a comprehensive preventive program, based on mechanical plaque control and local and systemic antibacterial measures, on periodontal health and preservation of permanent teeth in patients with Papillon-Lefevre syndrome (PLS). METHODS: Thirty-five consecutive PLS patients (median age=7 years; range=3-19 years) were treated and followed every third month over 3 to 7 years. Visible plaque, bleeding on probing, periodontal pockets > or = 5 mm, and number of lost permanent teeth were registered at the first visit and during the follow-up period. Due to severe periodontal inflammation, all primary teeth were extracted prior to the eruption of the first permanent tooth. Tooth-brushing was supported by comprehensive periodontal care and local and systemic chemotherapeutics (chlorhexidine and amoxicillin/metronidazole) on individual indications. RESULTS: Subjects treated strictly according to the program from their early years showed significantly fewer signs of periodontal disease and lost fewer permanent teeth than patients who started the program at an older age (P<.05). This was especially true if signs of periodontal disease had emerged when the treatment started. Compliance with the treatment protocol had a significant impact on the presence of plaque, bleeding surfaces, periodontal pockets, and number of lost permanent teeth. CONCLUSIONS: Early treatment and compliance with the preventive program were the major determinants for preserving permanent teeth in young PLS patients.
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