Leukocyte functions in 2 cases of Papillon-Lefèvre syndrome.
OBJECTIVE
To investigate the rôle of leukocytes in the pathogenesis of Papillon-Lefevre syndrome (PLS).


METHODS
Peripheral blood polymorphonuclear neutrophils (PMNs), monocytes (MNs) and gingival crevicular fluid (GCF) were obtained from 2 cases of PLS with typical features. The chemotaxis of PMNs and MNs were evaluated using a modified Boyden chamber. The adherence of PMNs was determined by adherence of PMNs to petri dishes. Interleukin-8 (IL-8) in GCF was detected by sandwich ELISA. Elastase activity in GCF was measured with a low molecular weight substrate (S-2484) specific for granulocyte elastase.


RESULTS
PMNs from both patients showed depressed chemotactic response to FMLP and IL-8. Total amounts of IL-8 in GCF from the 2 patients were much higher than those of the normal controls. Elastase activity was not significantly different from that of the controls. The adherence of PMN and the chemotaxis of MN in the 2 patients were normal.


CONCLUSIONS
The depressed chemotactic response of PMN leads to decreased recruitment of PMN and/or release of lysozyme from PMN in the diseased gingival tissue, increasing the susceptibility of PLS patients to periodontal infection.
Liu
R
R
Department of Periodontology, School of Stomatology, Beijing Medical University, China. liurk@pku.edu.cn
Cao
C
C
Meng
H
H
Tang
Z
Z
D000328:Adult; D016022:Case-Control Studies; D002448:Cell Adhesion; D002634:Chemotaxis, Leukocyte; D002648:Child; D004198:Disease Susceptibility; D005260:Female; D005883:Gingival Crevicular Fluid / Q000201:enzymology / Q000276:immunology; D006801:Humans; D016209:Interleukin-8 / Q000032:analysis; D019272:Leukocyte Elastase / Q000032:analysis; D008213:Lymphocyte Activation; D008297:Male; D009000:Monocytes / Q000502:physiology*; D009240:N-Formylmethionine Leucyl-Phenylalanine / Q000494:pharmacology; D020556:Neutrophil Infiltration; D009504:Neutrophils / Q000187:drug effects / Q000502:physiology*; D010214:Papillon-Lefevre Disease / Q000150:complications / Q000276:immunology*; D010518:Periodontitis / Q000209:etiology / Q000276:immunology*; D018709:Statistics, Nonparametric
D016209:Interleukin-8; D009240:N-Formylmethionine Leucyl-Phenylalanine; D019272:Leukocyte Elastase