Characterization of neutrophil function in Papillon-Lefèvre syndrome
نویسندگان
چکیده
منابع مشابه
Characterization of neutrophil function in Papillon-Lefèvre syndrome.
Papillon-Lefévre syndrome is a rare, inherited, autosomal-recessive disease, characterized by palmoplantar keratosis and severe prepubertal periodontitis, leading to premature loss of all teeth. Papillon-Lefévre syndrome is caused by a mutation in the cathepsin C gene, resulting in complete loss of activity and subsequent failure to activate immune response proteins. Periodontitis in Papillon-L...
متن کاملPapillon-Lefèvre syndrome.
Papillon- Lefèvre syndrome (PLS) is a rare autosomal recessive disorder of keratinization characterized by palmoplantar hyperkeratosis, periodontopathy and precocious loss of dentition. The exact pathomechanism of these clinical events mainly remains speculative. This paper describes two cases of PLS with classic clinical features and briefly review the relevant literature.
متن کاملPreventive periodontal regimen in Papillon-Lefèvre syndrome.
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-Lefèvre syndrome (PLS). METHODS Thirty-five consecutive PLS patients (median age=7 years; range=3-19 years) were treated and foll...
متن کاملPapillon-Lefèvre Syndrome: a case report
Introduction: Papillon-Lefèvre syndrome(PLS) characterized by palmoplantar hyperkeratosis is a rare autosomal recessive genetic disorder with rapidly progressive periodontitis and premature loss of both deciduous and permanent teeth. In this study, we report the clinical and radiographic features of Papillon-Lefèvre syndrome in an 11- year-old girl and we also discuss the history ...
متن کاملpapillon-lefèvre syndrome
introduction: papillon-lefèvre syndrome(pls) characterized by palmoplantar hyperkeratosis is a rare autosomal recessive genetic disorder with rapidly progressive periodontitis and premature loss of both deciduous and permanent teeth. in this study, we report the clinical and radiographic features of papillon-lefèvre syndrome in an 11- year-old girl and we also discuss the history and various th...
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ژورنال
عنوان ژورنال: Journal of Leukocyte Biology
سال: 2016
ISSN: 0741-5400
DOI: 10.1189/jlb.5a1015-489r