title: Long-term change of disease behavior in Papillon-Lefèvre syndrome: seven years follow-up.
abstract: Papillon-Lefèvre syndrome (PLS) is an autosomal recessive disease, characterized by severe periodontitis and palmoplantar hyperkeratosis. Mutations in the cathepsin C (CTSC) gene are the causative genetic factor. PLS starts at very early age, however, the age associated change of PLS has never been characterized. In this report, four PLS patients with CTSC mutations were followed up for seven years, periodontal condition and serum immunoglobulins (Igs) were recorded. Results showed that periodontal inflammation of PLS peaked at teenage years, but declined with time. At the same time the serum IgE change was consistent with the change, suggesting the possibility of using IgE as a monitoring index for PLS inflammation level, or to develop new target for therapy.
authors.affiliation: Department of Periodontology and Oral Medicine, School of Stomatology, State Key Laboratory of Military Stomatology, The Fourth Military Medical University, China.
authors.forename: Xinwen
authors.initials: X
authors.lastname: Wang
authors.affiliation: Department of Periodontology, PLA 309 Hospital, Beijing, China.
authors.forename: Yang
authors.initials: Y
authors.lastname: Liu
authors.affiliation: Department of Oral Pathology, School of Stomatology, State Key Laboratory of Military Stomatology, The Fourth Military Medical University, China.
authors.forename: Yuan
authors.initials: Y
authors.lastname: Liu
authors.affiliation: Department of Periodontology and Oral Medicine, School of Stomatology, State Key Laboratory of Military Stomatology, The Fourth Military Medical University, China.
authors.forename: Guangying
authors.initials: G
authors.lastname: Dong
authors.affiliation: Los Angeles School of Dentistry, University of California, USA.
authors.forename: E Barrie
authors.initials: EB
authors.lastname: Kenney
authors.affiliation: Department of Periodontology and Oral Medicine, School of Stomatology, State Key Laboratory of Military Stomatology, The Fourth Military Medical University, China.
authors.forename: Qing
authors.initials: Q
authors.lastname: Liu
authors.affiliation: Department of Periodontology and Oral Medicine, School of Stomatology, State Key Laboratory of Military Stomatology, The Fourth Military Medical University, China.
authors.forename: Zhiwei
authors.initials: Z
authors.lastname: Ma
authors.affiliation: Department of Periodontology and Oral Medicine, School of Stomatology, State Key Laboratory of Military Stomatology, The Fourth Military Medical University, China. Electronic address: wqtzym@fmmu.edu.cn.
authors.forename: Qingtao
authors.initials: Q
authors.lastname: Wang
mesh_terms: D000293:Adolescent; D020789:Cathepsin C / Q000235:genetics* / Q000378:metabolism; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood; D007073:Immunoglobulin E / Q000097:blood; D007074:Immunoglobulin G / Q000097:blood; D008297:Male; D009154:Mutation; D010214:Papillon-Lefevre Disease / Q000175:diagnosis / Q000235:genetics*; D016388:Tooth Loss / Q000235:genetics; D014094:Tooth, Deciduous / Q000378:metabolism; D055815:Young Adult
chemical_list: D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007073:Immunoglobulin E; C533918:CTSC protein, human; D020789:Cathepsin C
keywords: Cathepsin C; Disease behavior; IgE; PLS; Papillon-Lefèvre syndrome