Cytokines in Papillon-Lefèvre Syndrome
Papillon-Lefèvre syndrome (PLS) is characterised by aggressively progressive periodontitis combined with palmo-plantar hyperkeratosis. It is caused by "loss of function" mutations in the cathepsin C gene. The hypothesis behind this study is that PLS patients' PMNs produce more proinflammatory cytokines to compensate for their reduced capacity to neutralize leukotoxin and to eliminate Aggregatibacter actinomycetemcomitans. Production of more interleukin (IL)-8 would result in the attraction of more PMNs. Thus, the aim of this study was to evaluate the cytokine profile in PLS patients' blood cultures.
|Study Design:||Observational Model: Case Control
Time Perspective: Cross-Sectional
|Official Title:||Observational Study on Cytokine Production by Leukocytes of Papillon-Lefèvre Syndrome Patients and Healthy Probands in Whole Blood Cultures|
|Study Start Date:||July 2006|
|Study Completion Date:||December 2009|
|Primary Completion Date:||January 2007 (Final data collection date for primary outcome measure)|
Eight PLS patients (one female) from 6 families. For all patients A.actinomycetemcomitans had been detected from subgingival plaque. All adult patients have reported premature tooth loss of deciduous teeth caused by severe periodontitis. 1 patient was treated successfully and has retained 26 permanent teeth up to now. 2 patients were edentulous or had left only very few remaining permanent teeth at the time point of examination.
Healthy donors had abstained from taking drugs for two weeks prior to the study. Due to wide spread use of oral contraceptives only male probands were chosen.
|Department of Periodontology, Center for Dental, Oral, and Maxillofacial Medicine (Carolinum), JWG-University|
|Frankfurt/Main, Germany, 60596|
|Study Chair:||Peter Eickholz, Prof. Dr.||JWG-University|