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-  2018 

掌跖角化-牙周破坏综合征的研究进展
Research Progress of Papillon-Lefèvre Syndrome.

DOI: 10.13701/j.cnki.kqyxyj.2018.09.002

Keywords: 掌跖角化—牙周破坏综合征,基因突变,病因,发病机制,牙周治疗,
Papillon-Lefèvre syndrome
, Mutation ,Etiology, Pathogenesis, Periodontal treatment

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Abstract:

摘要 掌跖角化-牙周破坏综合征 ( Papillon-Lefèvre syndrome, PLS,OMIM 245000)是一种罕见的常染色体隐性遗传性疾病, 以掌跖角化症及快速进展的牙周组织破坏为特征。目前研究认为此疾病主要与组织蛋白酶C(cathepsin C,CTSC)基因的突变有关,亦有研究表明免疫因素、微生物因素及其他遗传因素参与,确切的病因尚未明确。国内外相关文献多为病案报道,对其病因及机制的研究较少。本文就PLS的临床表现、治疗措施、病因及发病机制的研究进展作一综述

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