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一名患有帕皮永-勒费弗尔综合征的年轻无牙患者的口腔修复治疗——一例罕见病例报告

The prosthodontic management of a young edentulous patient with the papillon lefevre syndrome-a rare case report.

作者信息

Sreeramulu B, Haragopal S, Shalini K, Sudha Madhuri D, Kiran G

机构信息

Assistant Professor, Department of Prosthodontics, GDCH , Hyderabad. A.P., India .

出版信息

J Clin Diagn Res. 2012 Dec;6(10):1808-11. doi: 10.7860/JCDR/2012/4884.2607. Epub 2012 Sep 18.

Abstract

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.

摘要

帕皮永-勒费夫尔综合征是一种常染色体隐性疾病,其特征为多种皮肤表现以及作为全身性疾病表现的牙周炎。其发病机制被认为是组织蛋白酶C基因突变的继发结果。多项研究表明,免疫相关细胞如多形核白细胞、巨噬细胞及其前体细胞受到了影响。本报告引出了一位患有该综合征的无牙男性患者,鉴于其年轻且社会经济地位较低,对其采用改良全口义齿修复体(中空、上颌、全口义齿)进行治疗。

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