The prosthodontic management of a young edentulous patient with the papillon lefevre syndrome-a rare case report.
The prosthodontic management of a young edentulous patient with the papillon lefevre syndrome-a rare case report.
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一名患有巴比龙·勒费弗综合征的年轻无牙颌患者的修复治疗——罕见病例报告。
DOI：
10.7860/jcdr/2012/4884.2607
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发表时间：
2012
期刊：
影响因子：
--
通讯作者：
G. Kiran
中科院分区：
文献类型：
--
作者：
B. Sreeramulu;S. Haragopal;K. Shalini;M. Sudha;G. Kiran
乳头状Lefevre综合征是一种常染色体隐性遗传病，以各种皮肤病和牙周炎为特征，是一种全身性疾病。其发病机制被认为是组织蛋白酶C基因突变的次要原因。各种研究表明，免疫相关细胞，如多形核白细胞和巨噬细胞及其前体细胞受到影响。本文报告了一例患有这种综合征的无牙合男性，考虑到他年龄小，社会经济地位低，接受了改良的全口义齿修复(中空、上颌、全口义齿)。
The Papillon Lefevre syndrome is an autosomal recessive disorder which is characterized by various dermatological manifestations and periodontitis as a manifestation of a systemic disease. Its aetiopathogenesis is thought to be secondary to the mutation of the Cathepsin C gene. Various studies have shown that the immune related cells like the polymorphonuclear leucocytes and the macrophages and their precursors were affected.This report elicits about an edentulous male with this syndrome, who was treated with a modified complete denture prosthesis (hollow, maxillary, complete denture), considering his young age and low socioeconomic status.