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Tissue plasminogen activator (t-PA) and placental plasminogen activator inhibitor (PAI-2) in gingival crevicular fluid from patients with Papillon-Lefèvre syndrome
Umeå University, Faculty of Medicine, Department of Odontology.
2004 (English)In: Journal of Clinical Periodontology, ISSN 0303-6979, E-ISSN 1600-051X, Vol. 31, no 9, 708-712 p.Article in journal (Refereed) Published
Abstract [en]

Objectives: Numerous patients with Papillon–Lefèvre syndrome (PLS) express a severe periodontal inflammation that results in premature loss of deciduous and permanent teeth. The plasminogen activating (PA) system is involved in physiological and pathological processes including epithelial healing, extracellular proteolysis and local inflammatory reactions. The aim of the study was to explore a possible role of the PA system in patients with PLS.

Material and Methods: Samples of gingival crevicular fluid (GCF) were collected from areas with gingival infection in 20 patients with PLS and in 20 healthy controls. The concentration of tissue plasminogen activator (t-PA) and inhibitor (PAI-2) was measured with ELISA.

Results: The median level of PAI-2 was significantly higher (p<0.01) in PLS patients than in the controls, while the median value of t-PA did not differ between the groups. No difference in t-PA or PAI-2 levels was found regarding age, gender or presence of active periodontal disease.

Conclusion: The findings indicate an atypical activity of the PA system with a disturbed epithelial function in PLS patients, suggesting that the periodontal destruction seen in patients with PLS is secondary to a hereditary defect in the defense system.

Place, publisher, year, edition, pages
2004. Vol. 31, no 9, 708-712 p.
Keyword [en]
gingival crevicular fluid, gingival inflammation, Papillon–Lefèvre syndrome, plasminogen activating (PA) system
National Category
Dentistry Dentistry
URN: urn:nbn:se:umu:diva-4312DOI: 10.1111/j.1600-051X.2004.00551.xPubMedID: 15312091OAI: diva2:143341
Available from: 2004-12-09 Created: 2004-12-09 Last updated: 2010-08-30Bibliographically approved
In thesis
1. Studies on clinical expression, genotype, and gingival crevicular fluid characteristics in young patients with Papillon-Lefèvre syndrome
Open this publication in new window or tab >>Studies on clinical expression, genotype, and gingival crevicular fluid characteristics in young patients with Papillon-Lefèvre syndrome
2004 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

Papillon-Lefèvre syndrome (PLS) is an autosomal recessive condition with palmoplantar hyperkeratosis and aggressive periodontitis as cardinal features. The disorder is linked to mutations of the gene for cathepsin C, a lysosomal protease essential in activation of serine proteases in immune and inflammatory cells. The genetic background of the disorder has been identified, but its relation to phenotypic expression is obscure.

The aims of the project were to explore phenotypic expression in young patients with PLS, and to investigate any correlation between clinical expression and identified genotype. Additionally, biochemical properties of gingival crevicular fluid (GCF) were investigated, and the result of an oral treatment protocol based on plaque control was evaluated.

Major results and conclusions from the studies were:

The severity of the skin lesions showed no correlation to patient’s age or level of periodontal disease, supporting the concept that the two major components of PLS are independent of each other.

Genotyping revealed two cardinal genotypes, but no correlation between the identified genotypes and expression of phenotypes could be found, suggesting that it is the interaction with environmental factors and/or other genes that is important in shaping the phenotype.

Analyses of gingival crevicular fluid (GCF) from patients with PLS did not show any clear-cut pathognominic expressions with regard to content of cytokines, metalloproteinases or inhibitor of metallproteinase 1.

The level of plasminogen activator inhibitor in GCF was significantly higher in PLS patients than in controls, indicating atypical activity of the plasminogen activating system with, possibly, disturbed epithelial function. This may affect the epithelial barrier function and its role in the innate defence system.

Evaluation of a PLS oral treatment protocol showed treatment from an early age and compliance to the program to be important in preserving permanent teeth in PLS.

51 p.
Umeå University odontological dissertations, ISSN 0345-7532 ; 89
Research subject
urn:nbn:se:umu:diva-384 (URN)91-7191-882-5 (ISBN)
Public defence
2004-12-17, Sal B, Tandläkarhögskolan, 9 tr, Norrlands Universitetssjukhus, Umeå, 13:00
Available from: 2004-12-09 Created: 2004-12-09Bibliographically approved

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