[Functional anomalies of polymorphonuclears in Papillon-Lefèver disease (author's transl)].
Polymorphonuclear functions were studied in 3 patients of the same brotherhood with Papillon-Lefèvre disease (hyperkeratosis palmaris and plantaris and acute periodontosis resulting in loss of teeth) who developed severe and recurrent infections. Chemotaxis, oxygen consumption and production of H2 O2 were investigated by polarography, O2 production by quantitative reduction of nitroblue tetrazolium and iodination by the Pinus and Klebanoff technique. functional anomalies of polymorphonuclears involving chemotaxis, induced O2 consumption and H2 O2 production were detected in all three patients. This study confirms the presence of polymorphonuclear functional anomalies in patients with Papillon-Lefèvre disease. Analysis of the family tree of the patients, which went back to 1761, showed that this was probably not a chance association.
D000328:Adult; D006801:Humans; D007239:Infections / Q000209:etiology; D007645:Keratoderma, Palmoplantar / Q000097:blood*; D008297:Male; D009504:Neutrophils / Q000502:physiology*; D010214:Papillon-Lefevre Disease / Q000097:blood* / Q000150:complications / Q000235:genetics; D010375:Pedigree
Anomalies fonctionnelles des polynucléaires dans la maladie de Papillon-Lefèvre. Trois observations.